Robbins

Compilation of AP Board Remembrances ( page references are for Robbins 5th edition unless otherwise specified) [Note: this is as received from Sara; I modified it and added to it a bit in the document called “Board summary-AP.Modified”; take your pick, they’re roughly the same-Mike] *BASIC PATH – ROBBINS 1. Choristoma – (Robbin’s Chp 7, p.243) an ectopic (heterotopic) rest of normal tissue, eg: a rest of adrenal cells under the kidney capsule or pancreatic rest in small intestinal mucosa 2. Fat in an EtOH fatty liver is composed of a. Triglycerides (Robbins page 25) 3. Irreversible cell injury includes (chart Robbins page 7) a. Cell membrane defects b. Nucleus pyknosis, karyolysis, or karyorrhexis c. Rupture of lysosomes and autolysis d. Lysis of ER e. Myelin figures (submembrane) f. Large mitochondrial densities 4. Increased permeability in inflammation occurs where? (This question may be more complicated than it seems. If they asked about a specific stage or type of inflammation, it could be one choice, but in general if all stages and forms of injury are considered, the answer is all three – meaning the microcirculation. Panvascular is wrong. READ Robbins page 54-56) a. Venules **The most common mechanism of vascular leakage classically affects only venules 20-60um in diameter and leaves the arterioles and capillaries unaffected. b. Arterioles c. Capillaries 5. Complement C5a chemotactic effect activated by (Robbins page 66) a. Ag-Ab complex b. Microbial surfaces c. Endotoxins d. Aggregated IgA e. Complex polysaccharides f. Neutrophil lysosomal enzymes g. Plasmin h. Cobra venom 6. Cell mediated immunity decreased in a. From Robbins, could be AIDS, Severe combined immunodeficiency, Digeorge’s syndrome (thymic hypoplasia), and Common Variable Immunodeficiency b. ?prob not myeloma 7. CD4 and MHCII interaction

a. CD4 Tcells recognize antigen when it is presented associated with MHCII molecule on antigen presenting cell (CD8 recognizes Ag with MHC I) 8. Acetyl cholinesterase is positive in (k type) a. ?mast cell, polys 9. p53 - Single most common target for genetic alteration in human tumors (Robbins chp 8) 10. Eosinophils have which type of Leukocyte-endothelial adhsion molecule (table Robbins p58) a. Receptor VLA-4 (a beta-1integrin) on eos binds to endothelial cell molecule VCAM-1 (an immunoglobulin) b. All leukocytes including eos use the LFA-1 and Mac-1 receptors to bind to ICAM-1 11. Structure of amyloid a. Protein made up of 95% fibrillary B-pleated sheet and 5% P substance glycoprotein (Robb p 231-32) 12. Which is not true about amyloid a. ?Not branching fibrils (person thought this was answer) 13. GVHD – which cell is responsible a. Donor T lymphocytes (p194, Robb) 14. Neutrophils can do what? a. Could be a lot of things, but probably answer is somewhere in pp59-65 in Robbins 15. Learn the allergic response (don’t know question) a. Type I hypersensitivity, Robb p179 table 16. Which reaction is type II hypersensitivity reaction a. Antibody mediated reactions (pp182-184) b. Examples i. Transfusion reactions ii. Erythroblastosis fetalis iii. Autoimmune hemolytic anemia, agranulocytosis, or thrombocytopenia iv. Myasthenia gravis 17. Which is not produced by macrophages a. Bradykinin (source is plasma substrate, p73, table) b. Note: the following are produced by macrophages :Eicosinoids (another name for arachidonic acid metabolites – prostagandins and leukotrienes) ,pdgf, and fgf . (p77, fig 3-28) ADRENAL 18. Non-chromaffin reactive pheochromocytoma a. ? 19. Atrophic adrenal sparing glomerulosa and medulla. What is cause? a. Secondary adrenocortical insufficiency – anything that has reduced ACTH i. Exogenous steroid tx (reduces ACTH by feed back) ii. Hypothalamic disfunction iii. Pituitary disfunction

iv. These diseases relatively spare glomerulosa, meaning aldosterone synthesis is relatively normal v. Review of adrenal structure 1. Cortex a. Glomerulosa – thin outer most layer. Produces mineralocorticoids, mainly aldosterone b. Fasciculata – thick middle layer (75% of cortex) c. Reticularis – thin inner most layer next to medulla The Fasciculata an reticularis act as a functional unit to produce glucocorticoids (cortisol) and sex steroid (testosterone) 2. Medulla – neuroendocrine tissue which is source of catecholemines a. Epinephrine, neuroepinephrine, and dopamine BLADDER BONE/JOINTS 20. Which does not cause secondary malignant changes? Choices: bone infarct, fibrous dysplasia, osteoma. a. Note: I think they mean which does not undergo malignant transformation i. Answer 1. Bone infarcts can. This is rare and occurs with medullary infarcts. (p1229) 2. Fibrous dysplasia can. This is rare and is usually with polyostotic involvement (p1243) 3. Osteoma does not undergo malignant transformation (p1233) 4. Other things associated with increased incidence of bone neoplasia: (p1232) a. Chronic osteomyelitis b. Pagets c. Radiation d. Metal prostheses 21. Osteoblastic and clastic activity are seen in what disease besides Paget’s? Choices: Osteoporosis, osteomalacia, osteitis fibrosa a. An INCREASE in both osteoblastic and osteoclastic activity is found in Paget’s and Osteitis fibrosa cystica (hyperparathyroidism related – p1226). The others don’t have increased activity. They all have at least minimal activity of both, but the question probably means increased activity. 22. Low complement in synovial fluid in what ds? Choices: Gonorrhea, rheumatoid, others

a. answer – I think gonorrhea. Gonococcal arthritis is assoc with genetic deficiency of C5,C6, and C7 (p1254, 2nd column) 23. Enlarged osteoid seams in renal dialysis pt (enlarged osteoid seams correspond to the osteomalacia component of renal osteodystrophy) due to? Choices: Low Ca, hypophosphate, aluminum a. Answer: Low Ca and aluminum of the choices in part A because (This is painful) (p1227) i. Renal failure causes hyperphosphatemia ii. Hyperphosphate causes hypocalcemia, which causes secondary hyper PTH iii. HyperPTH causes bone resorption to try to maintain serum calcium iv. Renal failure also makes the kidney unable to activate Vit D to (1,25 (OH)2 -D3). Without the active form, less Ca2+ is absorbed in the intestine, which contributes to the hypocalcemia v. Aluminum deposition at the site of mineralization, iron deposition, and metabolic acidosis of renal failure also play a role in renal osteodystrophy BREAST 24. What gives 4-5x increased risk for invasive breast CA a. Atypical duct hyperplasia b. (not papilloma or FCC) 25. Why do you grade DCIS? Choices: no clinical signif., dictates management, dictates node dissection a. Recurrence/invasion occurs in 40% of people with high grade DCIS and lumpectomy alone and <10% of those with low or intermediate grade (non-palpable) – ?dictates management 26. Medullary breast CA a. Peritumor lymphoid infiltrate b. High nuclear grade c. Minimal DCIS 27. Soft lesions of the breast include all except? Choices: Lobular CA vs medullary CA vs lipoma a. Of these, lobular CA is not soft. Is usually accompanied by a fibrous stroma. 28. Metaplastic carcinoma of breast looks most like? Choices: myofibroblastoma, fibromatosis, others a. Probable myofibroblastoma (more common in male breast, spindle cell lesion) CARDIOVASCULAR 29. Monkeberg’s medial calcific sclerosis Robb p484 a. Ring-like calcification of media of small to medium-sized muscular arteries in people over 50

b. No inflammation and do not narrow lumen c. May coexist with atherosclerosis d. Most common affected arteries are i. Femoral ii. Tibial iii. Radial iv. Ulnar v. Genital tract arteries 30. Findings in syphilitic aortitis (Robb p501) a. Aneurysmal dilatation confined to thoracic aorta b. Starts with inflammation and occlusion of adventitial vasa vasorum c. Ischemia, inflammation, scarring, and revascularization of media due to occlusion of vasa vasorum d. Dissection unusual e. Tree barking – wrinkling of intima f. Involvement of aortic root with atherosclerosis (not a site for usual atherosclerosis) g. Involvment of aortic valve with dilatation and aortic insufficiency h. Cor bovinum secondary to aortic insufficiency i. ?Orig stated valve adhesion to wall, ?endocardial pocket 31. Chronic rejection in heart transplant bx associated with a. ? (don’t know choices) 32. Which arteritis is not associated with giant cells a. Giant cell arteritis, Takayuso’s arteritis, Wegener’s vasculitis, and Buerger’s ds (Thromboangiitis obliterans) all have granulomatous inflammation. Don’t know what the choices were (pp492-498) 33. Cardiac amyloid, is it primary or secondary amyloidosis (p233) a. Depends on type. Primary amyloidosis is when it is associated with plasma cell dyscrasia (AL type). Secondary is secondary to inflammatory conditions (AA type). Primary amyloidosis involves the heart more often than the secondary type. b. Senile cardiac amyloidosis in old people is not classified in Robbins as either primary or secondary. It is either transthyretin or atrial natriuretic peptide. 34. Causes of angiosarcoma (don’t know choices) a. Radiation b. Arsenic c. Thorotrast d. Polyvinyl chlorid (angiosarcs assoc with b-d are primarily in liver or spleen) 35. Death in pt with floppy mitral valve due to a. Sudden death is due to arrythmias (p546-47) b. Can get stroke from leaflet thromboemboli 36. Which valve ds is unlikely to be post infectious? Choices: AS,AI,MS,MI a. If post-infectious means rheumatic, then the most common in decreasing order are

i. Mitral stenosis ii. Aortic stenosis iii. Tricuspid stenosis iv. Rare pulmonic involvement b. Don’t know if you get secondary insufficiencies with these stenoses too. 37. Cystic medial necrosis occurs in (p502) a. Frequently occurs in Marfans b. However, occurs in other non-specific aortic dissecting aneurysms and is found at autopsy occasionally in older or hypertensive patients

CYTOLOGY 38. Use of cytology in colon a. ?Good for evaluation of stricture 39. PAP cytology, nucleolus useful in distinguishing a. Reg/repair of endocervix vs squamous dysplasia 40. PAP with atypical degenerated squames in elderly a. ?trial of estrogen 41. What makes a PAP unsatisfactory? a. ? 42. Cytology of cirrhosis shows all except (don’t know choices) a. ?nucleoli 43. What determines adequate sputum a. ?carbon containing macrophages EM 44. EM intranuclear and cytoplasmic inclusions? Choices: PML, EBV, herpes a. Thought all were intranuclear only, can find only mention of intranuclear inclusions ENDOCRINE 45. Which pituitary adenoma is associated with calcification a. Prolactinoma (Robbins says 10-15% calcify) 46. What is the most common functioning endocrine tumor? a. ?Islet cell tumor of pancreas 47. Islet cell tumors of pancreas (Robbins pp922-24) a. May rarely produce more than one hormone when active b. Most common type is insulinoma (beta cell) 48. Do beta cell tumors occur outside the pancreas? a. Yes, but these occur in ectopic pancreatic tissue (p923, pink part) b. Also, 5% are malignant and metastasize to other organs. 49. Neuroendocrine microtumors of the fundus

a. Assoc with ZE syndrome (hypergastrinemia secondary to duodenal or pancreatic gastrinoma which then causes fundic enterochromaffin cell hyperplasia, microcarcinoids, or carcinoids of the stomach) 50. “pseudo ZE syndrome” – primary G cell hyperplasia of antrum which causes increased gastrin without gastrinoma and symptoms like ZE. Can have nodules of hyperplastic G cells in stomach 51. Dx of parathyroid adenoma – requires at least one other normal parathyroid 52. MEN syndromes a. MEN I (Wermer’s syndrome) – i. Chromosome 11q13 ii. Parathyroid ds – PTH hyperplasia or adenoma iii. Pacreatic islet cell tumors of any kind 1. Insulinomas present with hypoglycemia 2. Gastrinomas present with ZE syndrome iv. Pituitary adenomas – Prolactinoma most common v. May also have carcinoids, thyroid adenomas, adrenal cortical adenomas, lipomas b. MEN II (2a), (Sipple’s sydrome) i. RET protooncogene mutation of receptor tyrosine kinase which binds glial neurotroophic factor and normally transmits growth signals. The mutation causes an upregulation or gain in function, which is an unusual mech of tumorogenesis – normally get loss of tumor suppressor gene function instead of gain of growth signal fx. ii. Parathyroid ds as above iii. Pheochromocytoma iv. Thyroid medullary CA – commonly multifocal and may be associated with C cell hyperplasia also. May be aggressive and secrete calcitonin c. MEN III (2b) i. Different RET protooncogene mutation – single amino acid mutation of tyrosine kinase catalytic core ii. Pheochromocytoma iii. Thyroid medullary CA iv. Mucocutaneous neuromas and ganglioneuromas v. Marfanoid habitus vi. May have parathyroid hyperplasia (less common than in MEN I and II) 53. Parathyroid - which cells make hormone? a. Chief cells are major source of PTH b. Oxyphil cells contain lots of mitochondria 54. Cushing’s with thymic carcinoid (don’t know question) a. 1/3 of thymic carcinoids are associated with cushing’s (Ackerman surgical pathology, p 458.) Some of these are associated with MEN I or Iia syndrome. Many of them simply produce ACTH on their own. ENT

55. Salivary acinic cell CA differs from renal cell CA (?fat, glycogen) a. RCC clear cells contain both fat and glycogen b. Acinic cell CA i. Basophilic granular cells contain zymogen granules (PAS-D +) and occasional mucin. ii. The clear or vacuolated cells contain glycogen but not fat or mucin 56. Most common site of cannalicular adenoma of salivary gland a. Upper lip 57. Oral hairy leukoplakia caused by a. EBV b. May be associated with HPV 58. Laryngeal papilloma a. HPV 6,11 most common b. (original copy said “laryngeal polyp”, but these are AKA singers nodules, are not virus associated, but are trauma associated) 59. Warthin’s tumor association a. Males 50-70 yrs b. 10% multifocal, 10% bilateral c. Benign (James says no malignant change but there are sporadic reports of malignant change) 60. Endolymphatic sac tumor of the ear (don’t know question or choices) Ackerman surgical pathology p2515 a. Called “aggressive papillary middle ear tumor (APMET) or “low-grade adenocarcinoma of probable enolymphatic sac origin” – thought to be the same entitiy b. Papillary structures with vascular stroma lined by uniform cuboidal cells c. May have cystic dilatation resembling thyroid follicular tumor or cytoplasmic clearing resembling renal cell CA d. Associated with Von Hipple Lindau e. Locally invasive, bony invasion 61. Most common tumor of the submandibular gland (don’t know choices) a. Adenoid cystic is the most common malignant tumor. Margie’s book doesn’t even say the most common tumor overall b. Robbins p752 says adenoid cystic is “the most common neoplasm in the other salivary glands” excluding the parotid 62. Necrotizing sialometaplasia (don’t know question) a. Benign, necrotizing ulcerative inflammatory condition b. Most common in minor salivary glands of palate, also other salivary glands c. Commonly associated with hx of trauma and vascular compromise of area, (eg – compression from endotracheal intubation) d. Lobular necrosis and sialadenitis with squamous met of ducts e. May have pseudoepitheliomatous hyperplasia of overlying epithelium f. May be confused with squamous cell CA or mucoepi CA

g. Heals spontaneously, but occasionally arises in association with a malignant tumor 63. Prognosis of adenoid cystic CA (don’t know question) a. Related to histologic grade (I-III – grade III is >30% solid pattern and is poor prognosis) b. Also dependent on Stage c. Dependent on location, from best to worst prognosis: i. Palate (best) ii. Parotid iii. Submandibular gland (worst) d. High rate of local recurrence 64. HIV with bilateral cystic parotid lesions a. Called “AIDS related parotid cysts”, or “cystic benign lymphoepithelial lesion of AIDS” (Margie’s head and neck pathology, p334) b. Squamous lined cysts with hyperplastic lymphoid tissue surrounding c. Has similar histologic appearance to regular BLEL, but usually less cystic 65. What is in cytoplasm of Warthin’s tumor a. Oncocytic cells in the epithelial lining – so answer is mitochondria if it’s a choice. FORENSICS 66. How can cocaine cause MI? (k type)? Choices:+/-pre-existing CAD, arrythmia a. (p395) can cause vasoconstriction with or without pre-existing CAD b. can cause arrythmias c. Can cause direct catecholamine injury to myocytes 67. What information can be obtained from the vitreous humor of an embalmed pt? Choices: multiple electrolytes, k type a. Hyperglycemia b. Increased urea nitrogen c. Drugs: - barbiturates, meprobamate, propoxyphene, pentazocine, ampetamines, digoxin d. Not alcohol. GENETIC DISORDERS 68. Chronic granulomatous disease (Robbins page 63) a. Inherited defect in genes encoding components of NADPH oxidase, which in the neutorphil converts NADPH to NADP and superoxide. Superoxide then forms peroxide, which is converted to HOCL by myeloperoxidase and kills bacteria (This process is call OXIDATIVE BURST) b. Causes recurrent bacterial infection c. Can be x-linked or autosomal recessive 69. Neurofibromatosis associated with a. NF-1 (von Recklinghausen) (p149) i. Half are autosomal dominant inheritance, half are new mutations ii. Chromosome 17q11.2 mutation

iii.

iv. v. vi. vii.

b. NF-2 i. ii. iii. iv. v. vi.

1. codes for neurofibromin protein which down regulates p21 ras oncoprotein, a tumor suppressor gene Multiple neural tumors anywhere on body – neurofibromas including plexiform type 1. These neurofibromas can undergo malignant transformation, especially the ones attached to large central nerve trunks Pigmented lesions including Café au lait spots Lisch nodules – pigmented iris hamartomas Can have unilateral acoustic neuromas (bilateral are in type 2) Associated lesions 1. Skeletal a. Erosive defects b. Scoliosis c. Intraosseous and subperiosteal cysts d. Psseudarthrosis of the tibia 2. Other tumors a. Meningiomas b. Optic gliomas c. Pheochromocytoma Less frequent than type 1 Bilateral acoustic neuromas May or may not have skin neurofibromas Café au lait spots are present No lisch nodules Chromosome 22 mutation of a tumor suppressor gene

70. Peutz Jeghers syndrome associated with which tumor a. Sex-cord tumor with annular tubules b. See GI section for review of Peutz Jeghers 71. Testicular feminization (aka complete androgen insensitivity syndrome) Robb pp162. (eg Jamie Lee Curtis) a. Normal male genotype (46XY) b. Female phenotype or ambiguous external genitalia due to mutation in the gene for the androgen receptor located on Xq11-Xq12 c. Inherited as x-linked recessive d. Is the most common form of male pseudohermaphroditism 72. Klinefelter’s syndrome p159-160) a. Most are 47, XXY i. Rarer genotypes include mosaic 46XY/47XXY, 47XXY/48XXXY, etc b. Male phenotype c. Atrophic testis d. Long eunichoid body habitus

e. Infertility f. Slightly lower IQ, but not usually retarded g. Elevated FSH 73. Sickle cell trait assoc with which renal tumor a. Renal medullary CA – a variant of renal cell CA which is centered in the medulla and shows a reticular, yolk sac-like, or adenoid cystic appearance on histology which can be focally poorly diff and associated with desmoplastic stroma with neutophillic infiltrates and surrounding lymphocytes. (Ackerman p1150). The book says it’s assoc with SS disease. b. Review of Sickle Cell ds i. Autosomal recessive ii. Point mutation – substitutes Valine for glutamic acid at 6th position of beta-globin chain iii. 8% of black Americans are carriers (heterozygous) iv. Heterozygotes have about 40% HbS, remaining is Hb A v. When present along with HbC, is less serious because the Hb C prevents polymerization of the HbS, which causes the sickling 74. Why not do Polycystic Kid ds 1 mutation analysis on RT-PCR a. ? 75. Father is mosaic for an autosomal dominant ds with one son already affected. What is the highest % that the second offspring will be affected? a. ? 50% 76. Adrenoleukodystrophy (p1281, table 28-3) a. Long chain fatty acids collect because of defect in fatty acyl-CoA ligase b. X-linked c. Neurologic symptoms and adrenal insufficiency 77. Von Hipple Lindau (don’t know question) p268 and p1163, tables a. Chromosome 3p25-26 b. Autosomal dominant c. Renal cell carcinomas d. Pheochromocytoma e. Retinal and cerebellar hemangioblastoma f. Renal, hepatic, pancreatic, and epididymal cysts g. Angiomatosis 78. Adrenal hypoplasia with anencephaly a. ? 79. Monosomy 21 in XY pt. What is disease a. ?Down’s, Marfan’s , female, others b. Could be the Robertsonian translocation in a parent of Down’s syndrome, depending how the question is worded. If it is a karyotype with monosomy at 21 and an extra piece on chromosome 22 or 14, this is the Robertsonian translocation in a parent which leads to Down’s if the offspring gets the extra piece and non-viable if the offspring actually has monosomy 21. (p155, Robb). 4% of Down’s syndrome arise from

parents with this Robertsonian translocation. The other 96% are from non-disjunction during meiosis, which is maternal age related. 80. Which is not usual in NF1 a. Bilateral acoustic Schwannoma/neuroma (found in type 2) b. Can get unilateral in type 1 81. Description of Marfan’s pt, what is protein involved a. Fibrillin (p131, table) 82. Which ds not caused by point mutation – learn same table, p131 Robb. a. Thalassemia was one of choices, is not point mutation, b. Sickle cell is point mutation GI 83. Mucinous cystic tumor of the pancreas (Ackerman) a. Most located in body/tail, b. Usually multilocular but may be unilocular, c. Have cellular or ovarian type stroma d. ?communicates with pancreatic duct (don’t know yet, know that intraductal papillary mucinous tumor does communicate with the main pancreatic duct) 84. Most common pediatric polyp a. Retention polyp – sometimes simply called juvenile polyp i. Located in recto-sigmoid ii. Autoamputation is common iii. Surface ulceration and granulation tissue iv. Underlying cystically dilated glands with mucus v. Inflamed, edematous stroma vi. May have hyperplastic mucosal changes. Only rare cases have shown dysplasia vii. 1/3 of cases occur in adults 85. Pattern of UC involvement (maybe this question showed a picture) a. Rectum and a continuous segment of colon involved. No skip lesions as seen in Crohn’s, however may have cecal patch. May have small bowel involvement in the form of backwash ileitis, but otherwise small bowel not involved. b. Muscularis is spared, no mural thickening c. Many pseudopolyps d. Toxic megacolon is complication (can rarely occur in Crohn’s too) 86. Review of Crohn’s a. Skip lesions b. Fistulas, creeping fat c. Transmural involvement with non-caseating granulomas d. Aphtous ulcers which coalesce to form linear ulcers with cobblestoning of intervening normal e. Strictures may lead to “string sign” on barium study 87. Celiac ds injury a. Diffuse small intestinal enteritis, more severe proximally

b. Loss of villi c. Intraepithelial lymphocytosis, increase inflammation of lamina propria d. Caused by immune response to the gliadin protein in gluten – wheat, rye, oats, etc (pts have antigliadin antibodies) 88. Polyposis syndromes – review a. Peutz-jeghers (p811) i. Autosomal dominant ii. Multiple hamartomatous polyps, no malignant potential in these polyps. distribution 1. stomach 25% 2. colon 30% 3. small bowel 100% iii. Melanotic pigmentation of lips, face,oral genital iv. Increased risk of extra intestestinal malignancies in pancreas, breast, lung, ovary, and uterus v. Assoc with sex cord tumor with annular tubules of ovary and adenoma malignum of cervix b. Familial adenomatous polyposis FAP (p814) i. Autosomal dominant ii. Gene 5q21 iii. Multiple TA’s of colon iv. At least 100 tubular adenomas to make dx, usually >500 v. 100% progress to cancer within 10-15 years of dx c. Gardner’s syndrome i. Variant of FAP, same gene 5q21 ii. Autosomal dominant with variable penetrance iii. Multiple TA’s, multiple osteomas of bone, epidermal cysts, fibromatosis, dental abnormalities, duodenal and thyroid CA d. Turcot’s syndrome i. Variant of FAP ii. Multiple TA’s, CNS tumors (gliomas) 89. Which is not GI tract hamartomatous polyposis? a. Answer – Familial polyposis (is adenomatous) 90. Most common gastric polyp a. Hyperplastic (p779, Robb) 91. Small intestine obstruction due to enlarged lymphoid nodules ( choices Giardia vs Salmonella/Shigella) a. Lymphoid inflammation/peyers patch hyperplasia is seen in infection with Salmonella or Yersinia enterocolitica (p793, table, and 794, Robb) 92. Necrotizing enterocolitis associated with (don’t know question) a. Perforation, bacterial overgrowth, enteral feeding of premature infants 93. Which has worst prognosis in anus/rectum? Choices: Melanoma, squamous cell, mucinous adenoCA a. Melanoma 94. Prognosis of colon CA most related to? Choices:LN mets, inflammation, depth, necrosis

a. Single most important prognostic indicator is extent of tumor, AKA stage of tumor (p817, Robb) – I think this means lymph node mets would be the answer, although depth of invasion is also included in extent. If k-type, both would be good choices. 95. First genetic defect in colon CA (p814 Robb) a. First mutation is in APC (adenomatous polyposis coli tumor suppressor gene) b. Sequence in the multi-hit theory of colon cancer is: i. APC deletion ii. DNA loss of methylation iii. Oncogene activation - ras (most common), myc, myb, trk, hst iv. 18q allelic loss (called “DCC” for deleted in colon cancer) v. loss at 17p (site of p53suppressor gene) vi. microsatellite DNA stability on chromosome 2 GYN 96. What determines if a teratoma is immature /how is teratoma graded? (this is for ovary) a. Grades 1-3 based on the histologic proportion of the tumor which contains immature neuroepithelial elements i. Grade I – Abundat mature tissues intermixed with loose mesenchymal tissue with occasional mitoses, immature cartilage, tooth anlage ii. Grade II – Fewer mature tissues; rare foci of neuroepithelium with common mitoses, not exceeding 3 low power(x40) fields on any one slide iii. Grade III – Few or no mature tissues; numerous neuroepithelial elements merging with a cellular stroma occupying 4 or more low power fields 97. IH pattern of multinucleated stellate cell of vaginal polyp a. If the dx is fibroepithelial polyp, here is a review of fibroepithelial polyp i. Adult women (esp pregnant) or neonates ii. Not neoplasm, but hormone induced hyperplasia iii. Central fibrovascular core covered by normal squamous epithelium iv. May have edematous or cellular stroma v. Stromal cells may be highly atypical and stellate shape and stain with 1. vimentin, desmin , and steroid receptors 2. not usually actin vi. May be mistaken for botryoid rhabdomyosarc in babies because of desmin positivity and atypia, but do not have a cambium layer, epithelial invasion, or cross striations 98. Small cell ovarian CA associated with hypercalcemia 99. Cytogenetics in partial vs complete mole (p1082-1083) a. Complete i. >90% are 46,XX

ii. rare 46, XY b. Partial i. Most are triploid (69,XXY) ii. Rare tetraploid (92,XXXY) 100.Karyotype XO from abortus, which is least likely a. Mole is not likely, but don’t know other choices. 101.Polycystic ovary associated with a. Endometrial hyperplasia (p1057, 1064, 1065) and also increased risk of endometrial CA (Essentials of OBGYN)– This is because of unapposed estrogen stimulation due to persistant anovulation. These patients also have increased androgens which are converted to estrone also) b. Polycystic ovary with oligomenorrhea = Stein Leventhal syndrome i. Hirsuitism, obesity, masculinization, infertility 102.Difference between borderline and malignant tumors a. Destructive infiltrative growth is not seen in borderline 103.Metastatic colon CA to ovary most commonly mimics a. ?endometrioid adenoCA 104.Fibroepithelial polyp of vagina, cells stain for a. See above – Desmin, vimentin, steroid receptors. INFECTIOUS DS 105.Histoplasmosis found geographically a. Ohio River valley b. Mississippi River valley c. Caribbean 106.Changes which occur with transition from HIV infection to AIDS – don’t know choices (Robb pp227-228) a. Increased viral replication (increased viral titer in blood) b. CD4 count less than 200 cells/ul c. Opportunistic infections/AIDS defining illnesses (table 6-10,p228) i. Cryptosporidium or isosporidium enteritis ii. PCP iii. Toxoplasma pneumonia or CNS infection iv. Candidiasis – esophageal, tracheal, pulmonary v. Cryptococcal CNS infec vi. Disseminated coccidiodomycosis vii. Disseminated histoplasmosis viii. Tuberculosis – pulmonary or extrapulmonary ix. MAI – disseminated or extrapulmonary x. Nocardia - pneumonia, meningitis or disseminated xi. Disseminated salmonella xii. CMV – pulmonary, intestinal, retinits, CNS xiii. Localized or disseminated HSV xiv. Varicella Zoster xv. Progressive mulitfocal leukoencephalopathy (JC virus

xvi. Neoplasms – KS, lymphoma, primary CNS lymphoma, invasive cervical cancer 107.Which is not due to Chlamydia species (don’t know choices). a. The following are due to Chlamydia (Lev and Jaw, p150) i. C. psittaci –psittscosis pneumonia from birds ii. C. trachomatis 1. urethritis 2. genital tract infections (associated with Reiter’s syndrome) 3. conjunctivitis (neonatal) 4. lymphogranuloma venereum 5. Trachoma (endemic chronic conjunctivitis) iii. C. pneumoniae – atypical pneumonia 108.Mucosporidiosis associated with a. ?gelfoam 109.EBV associated diseases include (p287) a. Infectious mono b. Burkitt lymphoma (African form) c. Other B cell lymphoma (especially in immunosuppressed/HIV) d. Nasopharyngeal CA e. Oral hairy leukoplakia f. Association with Hodgkin’s 110.Which is not a protozoa – don’t know exact choices here. a. ?Rhinosporidium vs cryptococcus. Crytptococcus is a yeast. Cryptosporidium is a protozoa. b. ?Babesia, microsporidium, isospora, rhinosporidium – Babesia, microsporidium, and isospora are protozoa. 111.Ornithosis (don’t know question) a. Chlamydia psittici pneumonia caused by aspirated particles from bird excrement. 112.Not a feature of aspergillus – don’t know choices a. These are features of aspergillus (Robb p355) i. 3 toxins 1. Aflatoxin – carcinogen which may lead to African liver CA 2. Restrictocin – degrades host mRNA 3. Mitogillin - degrades host mRNA. Induces IgE involved in allergy and aspergillus ii. Causes allergies through types III and IV hypersensitivity reactions iii. Allergic bronchopulmonary aspergillosis – common in asthmatics. Hypersensitivity due to bronchial colonization with Aspergillus and may lead to COPD iv. Aspergilloma – colonization within pre-existing cavity. No invasion. v. Invasive aspergillosis 1. Usually immunocompromised 2. Starts in lung. May involve heart valves, brain, kidneys

3. Target lesions – gross foci of necrotizing pneumonia with hemorrhagic border 4. Blood vessel invasion vi. Morphology 1. Septate hyphae 5-10um thick 2. Acute angle (40 degree) branching 3. May see fruiting body KIDNEY 113.Learn kidney chp pictures and tables Robbins chp 20 114.Angiomyolipoma (don’t know question, here is review) a. Tumor of fat, blood vessles, and smooth muscle b. May grossly resemble RCC c. Commonly multiple or bilateral (15-30%) d. Blood vessles lack elastic lamina e. Mature fat f. Smooth muscle may show hypercellularity and atypical forms i. Type of bizarre, epithelioid cell in some cases which may be multinucleated and is thought to be of smooth muscle origin but has other properties 1. cytoplasm may contain glycogen 2. Stains with actin, but also HMB-45 3. Cytoplasmic crystalloids 4. premelanosome-like organelles 5. May resemble oncocytes or RCC cells g. 1/3 of pts with renal angiomyolipoma have tuberous sclerosis – i. tuberous sclerosis – 1. CNS hamartomas (cortical tubers, subependymal hamartomas), 2. Seizures and mental retardation 3. retinal glial hamartomas, 4. pulmonary and cardiac myomas 5. cysts of liver, kidney, pancreas 6. Cutaneous lesions – angiofibromas, shagreen patches (leathery thickenings), ash-leaf patches (hypopigmented), subungual fibromas 7. 2 different loci on chromo 9 and 16 (16 codes for tuberin protein similar to GTPase acitivating protein) 8. Autosomal dominant with variable penetrance h. Angiomyolipoma also assoc with lymphangioleiomyomatosis i. Also occur in retroperitoneal space unconnected to kidney, liver, and other sites 115.Cystic renal dysplasia, is it bilateral or inherited? (pp934-35) a. Not inherited (sporadic disorder) b. Can be unilateral or bilateral

c. Congenital defect with persistence of islands of undifferentiated mesenchymal elements in the kidney with cystification and abnormal lobar organization d. Assoc with other urinary tract deformities (atresia, etc) e. Other cystic renal ds, review (p934-37, Robb) i. Autosomal dominant (adult) polycystic kidney ds 1. 90% on chromosome 16, remaining unknown 2. Almost 100% penetrance by age 70-80 3. Always bilateral 4. Initially cysts involve only portion of nephron, so function usually retained until 5th or 6th decade of life a. Cysts eventually involve whole nephron 5. Also cysts in liver, spleen, pancreas, lungs 6. Berry aneurysms in 10-30%, is cause of death in 10% of pts ii. Autosomal recessive (childhood) polycystic kidney ds 1. Usually have severe symptoms in neonatal period, can present later in childhood 2. Always bilateral 3. Smaller cysts than AD polycystic kidney with elongated channels present at right angles to cortical surface a. Cysts involve cortex and medulla 4. May develop congenital hepatic fibrosis if they survive infancy iii. Medullary cystic ds 1. Medullary sponge kidney – a. Cysts of medulla collecting ducts b. Adults c. Unknown pathogenesis d. Usually normal function e. Secondary complications include calcification, infection, hematuria, and calculi 2. Nephronophthisis-Uremic medullary cystic disease complex (AKA hereditary tubulointerstitial nephritis) a. Usually childhood onset. Responsible for >20% of childhood renal failure b. Cysts in medulla c. Cortical tubular atrophy and interstitial fibrosis which is the cause of renal insufficiency d. 4 types i. Sporadic nonfamilial (20%) ii. Familial juvenile nephroonophthisis (50%) – recessive inheritance. iii. Renal-retinal dysplasia (15%) – assoc with retinitis pigmentosa. Recessive inheritance iv. Adult onset medullary cystic ds (15%) – dominant inheritance

116.Pediatric renal tumors – prognosis (don’t know question, here is review) a. In order from best prognosis to worst (compiled from Ackerman pp11351144) i. Multicystic nephroma/multilocular cyst – benign cystic lesion with areas that have appearance of a fully differentiated Wilm’s tumor (tubules, etc) ii. Mesoblastic nephroma – AKA fetal mesenchymal hamartoma, congenital, benign but may recur 1. Spindle cell tumor with fibroblasts, myofibroblast, or smooth muscle differentiation 2. No epithelial component, but surrounds epithelial cells iii. Wilm’s tumor (nephroblastoma) – 80-90% cure rate overall with surgery +/- chemo. Prognostic indicators include 1. Stage (most important) – based on tumor extent capsular/vascular invasion, renal sinus invasion, mets, bilaterality 2. Age of pt - <2 years is good 3. Tumor size – larger is worse in stage I tumors 4. Presence of anaplasia = unfavorable histology 5. Review of Wilms (p463-464) a. Most common renal primary tumor of childhood, usually <6 years b. Mutation on chromosome i. 11p13 – (a transcription factor protein) – WT-1 ii. 11p15.5 (function unknown) - WT-2 c. Blastemal, stromal, and renal epithelial elements on histology d. May have heterologous elements (eg cartilage) e. 5% contain anaplasia f. Express insulin-like growth factor g. Syndromes associated with Wilms i. WAGR - Wilms, aniridia, genital anomalies, mental retardation – WT-1 ii. Denys-Drash – gonadal dysgenesis (male pseudohermaphroditism) and nephropathy WT - 1 iii. Beckwith-Wiedemann – organomegaly, hemihypertrophy, adrenal cytomegaly, may have hepatoblastoma, adrenocortical tumors, rhabdomyosarc, pancreatic tumors. WT-2 iv. Clear cell sarcoma (AKA bone metastasizing renal tumor) – was previously considered variant of Wilms. Rare. Small blue round cell histology, not necessarily clear cells. Near 70% mortality (May be improving with new chemo)

1. Expresses insulin-like growth factor but not WT-1 transcripts v. Rhabdoid tumor – Blue round cell tumor of usually infants (may have spindle features) 1. Cytoplasmic eosinophilic hyaline globule made of intermediate filaments which displaces nucleus giving a plasmacytoid appearance a. Positive for Vimentin and CK, not usually muscle or neural markers b. High mortality (>75%) c. Occur in other sites besides kidney i. Soft tissue , pelvis, bladder, thymus 117.Glomerulonephritis in pt with SLE. Where are immune deposits a. Depends on what type of lesion, but if question is k-type, in Lupus in general, depositis can be seen in the following areas (p204-205 – pink text) i. Mesangial ii. Intramembranous iii. Subepithelial iv. Subendothelial 118.Renal papillary necrosis occurs in (Ackerman p1123) a. Pyelonephritis b. Diabetes mellitus (Robbins says pyelonephritis assoc with DM) c. Infection associated with obstruction d. Alcoholism e. Vascular thrombosis f. Sickle cell ds g. Analgesic abuse h. Long term Rifampin therapy for TB i. Hemodialysis 119.Which will recur in renal transplant a. ?Dense deposit, IgA, post infx GN 120.Which glomerular diseases recur after transplant a. ? 121.Features of renal oncocytoma (don’t know question, here is review) a. Circumscribed b. May be multiple or bilateral c. Mahogany brown with a central fibrous scar d. Oncocytes with uniform nuclei, abundant granular cytoplasm (mitochondria), nested or tubular pattern e. No prominent papillary formations, clear cells, or necrosis f. Usually benign regardless of size g. Immuno i. Keratin positive ii. Dot-like positivity for Keratin 8 and 18 iii. Negative Vimentin (usually)

LIVER 122.Mallory hyaline bodies are seen in a. EtOH b. PBC c. Wilson’s d. Chronic cholestatic syndromes e. Wilson’s disease (question may specify in the portal tract, need to look up if one is specifically in portal tract) 123.Significance of cholestatic sclerosing cholangitis in posttransplantation a. ?Harvesting injury 124.Kayser-Fleischer rings (original said “Kleinfeisher ring”) Robb pp864 a. Green-brown depostis of copper in Descemet’s membrane of the cornea b. Seen in Wilson’s ds. , but may be seen in other forms of chronic cholestasis (don’t know if they contain copper in other forms) 125.Primary biliary cirrhosis assoc with a. Anti mitochondrial antibodies (M2 antibodies to mitochondrial pyruvate dehydrogenase) (p868, Robb) 126.Drug and toxin induced liver disease a. See chart on page 857 Robbins – there are at least 2 questions. One had to do with thorotrast and vinyl chloride associated with HCC and angiosarcoma. The other was an unspecified question about drugs and liver injury – LEARN THE CHART! 127.Description of HCV histology (k type, don’t know choices. Here is review) a. Lymphoid aggregates in portal tracts with accompanying mixed inflammatory infiltrate b. Fatty change more so than Hep B c. No ground glass hepatocytes as seen in Hep B d. Bile duct damage e. Piecemeal necrosis f. Bridging necrosis, fibrosis ,eventual cirrhosis 128.Histology of alcoholic hepatitis (p858) a. Hepatocyte ballooning necrosis – centrilobular b. Mallory bodies (cytokeratin and other filaments – eosinophillic cytoplasmic inclusion, not specific to alcohol, see above) c. Neutrophillic infiltrate in lobule d. Fibrosis – sinusoidal and perivenular e. Fatty change may or may not be present 129.Alcoholic fatty change a. Completely reversible. Fibrosis is not reversible. 130.Hemachromatosis defect a. Hemachromatosis gene located on chromosome 6, but the functional defect is unknown still (as of Robbin 5th edition) 131.Anti Actin Ab seen in ? choices:Hep A, B, C, autoimmune hep a. Anti smooth muscle AB definitely seen in autoimmune. (p853 Robb)

b. ? Hep A, B, C 132.What stain gives a cannalicular pattern in hepatoma/hepatocytes? Choices: CK20, EMA, CEA, s100 a. Answer (ackerman p 909) Alk phos, CEA (biliary glycoprotein component of it), low molecular weight cytokeratin LYMPHOID 133.Pattern of lymph node involvement by Langerhans histiocytosis and what is immuno staining of the langerhans histiocytes? a. Sinusoidal infiltrates of Langerhans cells and eos b. Review – disease with Langerhans histiocytosis include i. Eosoinophilic granuloma – localized ii. Hand-Schuller-Christian – multifocal iii. Letter-Siwe – systemic iv. 2-chlorodeoxyadenosine activity in adults v. Langerhans cells – large mononuclear cells with folded or grooved nuclei and abundant pale cytoplasm. Imuno is same as normal Langerhans cells 1. s-100 2. cd68 3. PNA (peanut agglutinin) 4. cd1a, cd4, cd11c, and cd14 in frozen tissue (cd1a is also available on paraffin) 5. adenosine triphosphate 6. alpha-D-mannosidase vi. Have birbeck granules on EM 134.Most common site of lymph nodes with benign mesothelial inclusions? Choices : Retroperitoneal or mesenteric or pleural a. Looks like mediastinal and abdominal lymph nodes are the most common according to Strauchen . b. Other benign lymph node inclusions i. Mullerian ii. Salivary iii. Thyroid iv. Mammary v. Pancreatic heterotopia vi. Renal tubular inclusions with Tamm-Horsfall protein in children with Wilms vii. Nevus cells – usually in superficial lymph nodes – axillary, inguinal , cervical 135.PTLD – know features and associations a. Heart or lung transplant is higher risk than other organs b. Therapy with cyclosporine A or OKT3 is high risk c. Assoc with EBV d. Spectrum of appearance i. Plasmacytic hyperplasia (low grade PTLD)

1. 2. 3. 4.

Tonsil/Waldeyer’s ring/ and LN involvement Polyclonal Multiple forms of EBV Preservation of architecture with interfollicular zone expansion by infiltrates of plasmacytoid lymphocytes and plasma cells ii. Polymorphic B-cell hyperplasia/Polymorphic B cell lymphoma – Intermediate grade PTLD) 1. Nodal or extranodal 2. Single form of EBV 3. Majority are monoclonal 4. Effacement of architecture by polymorphous proliferation of lymphocytes and immunoblasts with varying degrees of plasmacytoid differentiation. Lympohoma version has more atypia and necrosis iii. Immunoblastic lymphoma/ Multiple myeloma (high grade PTLD) 1. Nodal or extranodal 2. Monoclonal 3. contains 1 or more oncogene or tumor suppressor mutations a. N-RAS b. p53 c. C-MYC 4. Single form of EBV 5. Effacement of architecture by monomorphic proliferation of plasma cells and immunoblasts e. Therapy in all cases is based on withdrawal of immunosuppresion. Higher grades may also need chemo/radiation 136.Ki-1 lymphoma markers a. Ki-1 = CD30 b. Positive in Reed-Sternberg cells in Hodgkin’s c. Positive in anaplastic large cell lymphoma d. May also be positive in nasopharyngeal carcinoma, embryonal carcinoma, melanoma (faint) and rarely in other carcinomas (faint) 137.Follicular dendritic cells a. MHC II antigen presenting cells – present antigen to B-cells in follicular center b. Immuno i. CD21 (complement C3d receptor) ii. CD35 (complement C3b receptor) c. Other dendritic cells of the lymph nodes include i. Interdigitating reticulum cells 1. In paracortex 2. Present antigen to T-cells 3. S-100 positive (neg for CD21 and 35) ii. Langerhans cells

1. In paracortex 2. Present antigen to T-cells 3. S-100 and CD1a positive 138.Toxoplasma lymphadenitis vs Kikuchi’s ds vs SLE histo a. Toxoplasma lymphadenitis i. Heterphile-negative mononucleosis syndrome ii. Lymphadenitis of Piringer-Kuchinka 1. Follicular lymphoid hyperplasia 2. Clusters of epitheliod histiocytes within and between follicular centers 3. Monocytoid B-cells in sinuses 4. Combination of above 1,2,3 together is fairly specific for toxo. 5. May rarely see toxo cysts in lymph nodes b. Lupus lymphadenitis i. Follicular lymphoid hyperplasia ii. Paracortical foci of necrosis iii. +/- Hematoxylin bodies – Masses of altered DNA – Feulgen positive iv. Neutrophils are absent v. Immunoblasts and plasma cells may be prominent c. Kikuchi’s disease i. Classic – young women with cervical adenopathy and viral prodrome ii. Circumscribed paracortical foci of necrosis with karyorrhectic debris, which are surrounded by zone of immunblasts and macrophages iii. Crescentic histiocytes (macs with c-shaped nuclei) are prominent iv. Scant polys and plasma cells v. Xanthoma cells may be prominent vi. 3 histologic patterns 1. Proliferative 2. necrotizing 3. xanthomatous vii. CD 8 t-cells and macrophages in necrotic zones d. Kawasaki’s ds (mucocutaneous lymph node syndrome) i. Febrile illness of childhood (less than 6 years of age) ii. Skin rash – desquamation of palms and soles, erythema of conjunctiva, lips, and oral mucosa; cervical adenopathy, systemic vasculitis assoc with coronary artery involvement and coronary artery aneurysm iii. Lymph node histo ( not common to get LN bx, dx based on clinical) 1. Fibrin thrombi in small vessles 2. Irregular foci of necrosis in paracortex and follicles

iv. Abnormal macrophage and t-cell activation of unknown cause (possible postinfectious) v. Treated with IV immunoglobulin e. Kimura’s ds i. Angiolymphoid proliferation of head and neck involving males from the Far East – unknown etiology ii. Peripheral eosinophilia and increased serum IgE iii. Histo 1. follicular lymphoid hyperplasia 2. Interfollicular eosinophils 3. Eosinophillic folliculolysis 4. proliferation of thin-walled vessles 5. May have Warthin Finkeldy giant cells, hyalinization of follicles, and Charcot-Leyden crystals 6. Preponderance of B-cells 7. IgE in follicular centers iv. Confused with angiolymphoid hyperplasia with eosinophilia, which is in western women in the head and neck and considered to be a form of hemangioma with epitheliod appearance and eosinophils 1. No peripheral IgE or eosinophilia 139.Hodgkin’s ds staging (k-type question in which all were correct. Don’t know choices, but here is a review of Hodgkin’s staging) a. Ann-Arbor staging I-IV i. Stage I – single LN region (I)or single extralymphatic organ or site (Ig) ii. Stage II – 2 or more LN regions on same side of the diagphragm (II) or localized involvement of extralymphatic organ or site (IIg) iii. LN regions on both sides of diaphragm (III) or localized involvement of an extralymphatic organ or site (IIIg) or spleen (IIIg) or both (IIIse) iv. Stage IV – Diffuse or disseminated involvement of one or more extralymphtic organs +/- LN involvement. – A=asymptomatic and B=with symptoms (fever, wt loss, etc) b. Early peak in adolescence is usually nodular sclerosis of Stage I or II c. Later peak in older age is usually mixed cellularity or lymphocyte depletion and presents with Stage III or IV 140.Hodgkin’s ds –B cell malignancy most likely confused with which type of lymphoma (don’t know choices) a. Possibilities include peripheral T-cell, anaplastic large cell, and t-cell rich B-large cell. b. Most likely to be confused is anaplastic large cell. c. Don’t forget infectious mono can be confused with Hodgkin’s i. RS-like cells in mono are CD15 negative, CD30 positive, CD45 negative. 141.PCR of cat scratch disease shows what organism

a. Bartonella henselae most common b. Rare – Afipia felis 142.HIV lymphoma (don’t know question but here is review) a. Non-Hodgkins lymphoma (can commonly C-MYC rearrangements and EBV) i. Commonly associated with HIV – aggressive B-cell neoplasia 1. Diffuse large cell, B-cell 2. Burkitt’s like ii. More rare associated with HIV 1. Primary effusion lymphoma assoc with HHV-8 (like Kaposi’s sarcoma) 2. CD 30 positive anaplastic large cell lymphoma 3. Peripheral T cell lymphoma 4. Immunoblastic T cell lymphoma b. Hodgkin’s i. Distinct clinical presentation – associated with b-symptoms and 50% bone marrow involvement ii. RS cells contain EBV iii. CD4:CD8 ratio of lympocytes is reversed compared immunocompetant Hodgkin’s iv. Usually mixed cellularity type 143.MDS, what affects prognosis? Choices: Age, 5qdeletion, % blasts a. (WHO classification of heme, p67) Age <60 is better prognosis b. Increase percentage of blasts is worse prognosis c. Genetics: i. Normal karyotype, 5q del, 20qdel, and –Y are in the Good prognosis category ii. >3 genetic abnormalities or chromosome 7 mutation = Poor iii. Any other = Intermediate 144.Characteristics of Sinus histiocytosis with massive lymphadenopathy a. AKA Rosai Dorfman b. Usually cervical adenopathy in young people, can occur anywhere c. Fever, elevated sed rate, hypergammaglobulin d. Unknown etiology e. Histology i. Dilated sinuses with large histiocytes with prominent nucleoli and finely vacuolated cytoplasm 1. Histiocytes S-100, CD68, CD11c, CD14, CD33, antichmotrypsin, lysozyme positive ii. Emperipolesis – histiocytes engulf other hematologic elements iii. Thickened capsule iv. Plasma cells in intersinusoidal tissue f. Can have extranodal involvement g. Benign, self limiting condition 145.Immuno of L&H cells in Hodgkin’s

a. L & H cells occur in nodular lymphocyte predominance Hodgkin’s disease, not in classical Hodgkin’s. b. L&H cells stain with a typical B-cell phenotype i. CD20, CD79a, CD45, EMA positive ii. Usually CD15 and CD30negative iii. May express CD30 weakly iv. Usually surface immunoglobulin negative 146. Which are chloroacetate positive cells a. ? MEDIASTINUM 147.Most common mass of posterior mediastinum in children (don’t know choices) a. ?Probably neuroblastoma (para vertebral region of posterior mediastinum is 2nd most common site of neuroblastoma after adrenal medulla) (p459 Robbins) 148.Most likely dx of anteorsuperior mediastinal mass, 20 yo? Choices: Lymphoma vs seminoma etc a. Nodular sclerosis Hodgkin’s probably most common in this age b. Thymoma occurs in ant-superior mediastinum, but usually in patients older than 40years. c. Thymoma makes up only approx 25% of ant-sup mediastinal tumors because this is a common location for Hodgkin’s and non-hodgkins lymphoma. MOLECULAR 149.RFLP – be able to interpret (p 168, Robbins, diagram) 150.Genome of a retrovirus (p221, Robb, diagram) a. Gag gene codes for core proteins b. Pol gene codes for reverse transcriptase c. Env codes for envelope glycoproteins 151.C-myc overexpression occurs in Burkitt’s lymphoma a. Normal myc gene on chromo 8 b. Get translocation b/t 8q24 and chromo 14 so that myc gene is under control of immunoglobulin gene enhancer and get overexpression 152.Identify these chromosomal abnormalities (karyotypes with arrow at affected chromo) a. Burkitt’s – t(8;14) b. Ewing’s – t(11;22) c. Follicular lymphoma – t(14;18) - BCL-2 153.Know DNA ploidy grams (hyperdiploid, hypo, etc) 154.BRCA 1 and 2 are a. ?tumor suppressor genes NEURO 155.Morton’s neuroma – don’t know question. Choices:?3/4 metatarsal, ?grossly nodular tumor of nerve, fibrous thickening of nerve

a. Morton’s neuroma, review i. Traumatic neuroma ii. Classic location is metatarsal area, esp. interdigital nerve b/t 3rd and 4th toe iii. More common in adult female iv. Extensive perineurial fibrosis, often concentric v. Thickened arterioles which may have thrombi 156.Ependymoma with best prognosis – don’t know choices a. Myxopapillary type i. Restricted to conus medullaris and filum terminale. >90% curable by surgery, though rare examples have spread b. Subependymoma has a better prognosis than any ependymoma including myxopapillary. Don’t know if it was choice i. Subependymoma 1. Derived from subependymal neuroglial cells around ventricles 2. Most commonly in 4th ventricle in older men, asymptomatic and found at autopsy 3. Solid, dense white tissue with calcification 4. Usually non-invasive, well defined. 5. May be secondary to irritation of ventricles 6. Fibrillar meshwork with groups of small tumor cells huddled together throughout. Microcystic changes in fibrillar background 7. May have nuclear atypia, but behave benign 157.Craniopharygioma a. Derived from Rathke’s pouch remnants b. Most commonly suprasellar, may be sellar c. Cystic, multiloculated, some solid d. AKA ameloblastoma or adamantioma because recapitulates tooth enamel organ e. Calcification and metaplastic bone formation common f. Benign, rare malignant behavior g. Crank case oil/machinery oil grossly in cystically degenerated tumors which contains cholesterol crystals h. Kids, young adults, but may be any age i. Question: Which is most likely to present as pituitary tumor? – don’t know other choices, answer was thought to be craniopharyngioma 158.Most common tumor of the pineal gland a. Germinoma (replicates either seminoma or dysgerminoma) 159.Brain with necrosis of globus pallidus, what is cause a. ? 160.Which has more aggressive course? Choices: Hemangioblastoma vs Gemistocytic astrocytoma a. Of these 2, gemistocytic astrocytoma is the more aggressive.

b. Hemangioblastoma i. Young adults, cerebellar. ii. May be extracranial – spinal, optic nerves, etc iii. Von Hippel Lindau iv. May produce EPO and cause erythrocytosis v. Histogenesis uncertain, thought to be stromal cell with neuroendocrine diff (s-100 positive) vi. Vascular tumor with bland stromal cells containing clear or vacuolated fat-containing cytoplasm vii. Benign, curable by resection c. Gemistocytic astrocytoma i. Predominance of gemistocytes and perivascular lymphoid cuffing ii. May transform into more aggressive tumor (GBM) 161.What changes can be seen in Antoni B areas a. Antoni B area of Schwannoma are the looser, less cellular myxoid edematous regions that undergo cystification b. Antoni A – cellular areas with palisading/ Verocay bodies 162.Which of these tumors has hemangiopericytoma like areas (no pics, don’t know choices) a. Solitary fibrous tumor has frequent hemangiopericytoma-like areas b. Others? 163.Chordoma immuno a. CK and EMA positive PROSTATE 164.Most common cause of caseating granulomatous inflammation in prostate in U.S. a. ?BCG infusion vs tuberculosis 165.High molecular weight cytokeratin can be used to distinguish PIN from prostate CA 166.High vs low grade PIN (don’t know question) a. Based on i. cell crowding and stratification, ii. nuclear enlargement, pleomorphism and chromatin iii. nucleolar appearance b. Low grade PIN includes grades 1-2 c. High grade PIN includes grade 3 and has cytologic appearance of prostatic carcinoma. 167.What is the basis of the Gleason grading system a. 2 most prevalent grades are added to get Gleason score (combined grade) RESPIRATORY (Most of the non-neoplastic information came from Katzenstein and Askin Surgical Path of Non-neoplastic Lung Disease) 168.Vasculitis and granulomatous inflammation (don’t know question). Choices:Wegenner’s, TB, ?other infections, ? Langerhan’s histiocytosis a. Wegener’s, TB, and other infections (blasto, histo, other fungal) definitely have both granulomatous inflammation and vasculitis. (Vasculitis is

common in mycobacterial and fungal infections, but is usually not necrotizing as in Wegener’s) – b. Langerhans histiocytosis (eosinophillic granuloma) has infiltration of the vessles with inflammatory cells which has been called vasculitis by many authors, but Katzenstein thinks that term is wrong because it is not a true vasculitis. It is just invasion of the vessel by the eosinophillic granuloma. 169.Coccidioidomyocosis does not have which type of inflammation? Choices: granulomatous, polys, eosinophilic, LIP a. Coccidio shows a necrotizing granulomatous inflammation with purulent (neutrophillic) zones and prominent eosinophils b. Does not show LIP 170.Giant cell CA of the lung is derived from squamous or adeno a. No definitive origin, but mucin positivity in some tumors suggests adeno 171.Lung abscess formation a. Usually associated with aspiration of oral contents, usually anaerobe 172.Bronchioloalveolar CA prognostic factors a. Localized lesion better prognosis than multiple/diffuse b. Non-mucinous better than mucinous 173.IVBAT – intravascular bronchoalveolar tumor a. is considered a form of epithelioid hemangioendothelioma 174.Allergic bronchocentric granulomatosis – don’t know question. a. Name of disease is Bronchocentric granulomatosis i. It is associated with asthma and allergic bronchopulmonary aspergillosis in about half of the patients ii. Combination of angiitis and granulomatous ds which centers around bronchioles. Get bronchiolar destruction by granulomatous inflammation 175.Pulmonary alveolar proteinosis associated with(don’t know choices) a. AKA lipoproteinosis or phospholipoproteinosis b. Alveoli filled with pink material, PAS positive c. Interstitial architecture not significantly altered d. May be isolated or associated with i. Occupational exposure to silica, aluminum, and other dusts ii. Immunosuppresion, iii. Hematolymphoid malignancy iv. Nocardia infection is encountered with increased frequency v. Infection with mycobacteria and various fungi have also been associated 176.Difference in diffuse alveoalar damage in adults vs kids (p677) a. Infants – mechanism is pulmonary surfactant deficiency with hyaline formation b. Adults – mechanism is damage to alveolar capillary walls, increased capillary permeability, alveolar edema and fibrin with hyaline formation 177.Causes hypoglycemia a. Solitary fibrous tumor of pleura

i. Spindle cell lesion with fibroblast-like cells, abundant focally dense collagen, and hemangiopericytoma-like areas ii. CD34, vimentin positive. (Occasional desmin) iii. Majority benign (>90% cure with surgery) 178.Lung cancer producing CRH a. ? b. Small cell CA of lung produces ACTH, ACTH-like substance, and ADH. Can get related paraneoplastic syndromes. (Don’t know about CRH) – See table 7-9. p296, Robbins 179.Etiology of UIP (don’t know question or choices) a. Associated with collagen vascular ds, esp RA and scleroderma b. Auto antibodies esp RF and ANA are frequent c. Circulating immune complexes frequent d. Progressive downhill course e. Older pts – avg age 60 f. Interstitial chronic inflammation, fibrosis and honeycomb change with intervening normal – temporal heterogeneity 180.Morphology of diffuse alveolar damage/ARDS a. Early stage (less than 1 week) i. Interstitial and alveolar edema ii. Hemorrhage and fibrin deposition iii. Hyalin membrane 1 or more days after injury iv. Sloughing of alveolar lining cells v. Sparse interstitial inflammatory infiltrate – lymphs, plasma cells, macs vi. Fibrin thrombi in capillaries b. Late stage i. Alveolar lining hyperplasia (begins 3-7days after injury) ii. Organization – fibroblastic foci in interstitium and air spaces iii. Increased interstitial inflammation iv. Hyalin membranes and edema not prominent v. Arterial changes – medial hypertrophy and intimal fibrosis vi. Bronchiolar squamous met c. The stages overlap somewhat 181.Bleomycin induced lung ds, which are not seen? Choices: organizing pneumonia, DAD, alveolar hemorrhage, fibrosis a. The Following are seen (p82, Katzenstein and Askin, table 4-2) i. DAD ii. Pleural fibrosis iii. Eosinophilic pneumonia iv. BOOP v. Pulmonary veno occlusive ds vi. Alveolar hemorrhage can be associated with the DAD (p86, Katzenstein), so answer must be a different choice

SKIN 182.Nevus sebaceous most commonly associated tumor a. ?BCC/syringocystadenoma papilliforum 183.Warty dyskeratoma a. Assoc with acantholysis, not virus 184.Most common site of cellular blue nevus a. ?Buttock 185.Most commonly associated with pseudoepitheliomatous hyperplasia a. ?Granular cell tumor 186.Hydropic degeneration of skin occurs in which K type a. ?SLE 187.What is helpful in differentiating Spitz nevus from melanoma a. ?age ?morphologic atypia, ?mitoses 188.Neurothekoma a. Cutaneous nerve sheath myxoma b. Benign c. Cellular and myxoid variant d. Cellular can be confused with melanoma e. S-100 +, HMB-45 neg f. Not assoc with NF or other syndromes 189.Microscopic features of intradermal nevus (don’t know choices) a. You know this 190.Melanoma vs intradermal nevus a. IDN has discrete, regular round borders and regular pigmentation. 191.Prognosis of melanoma related to a. ?depth vs thickness 192.Trichilemomma location a. Facial skin predominantly b. (Cowden’s ds – multiple trichilemomma, multi hamatomas of brst, thyroid, GI, multi acral keratosis, hyperkeratotic papillomas, INCREASED BRST CA RISK) 193.Melanocytes (or melanin) are seen in which of the following lesions (don’t know choices) a. ? 194.Langerhans cells in skin (don’t know question) a. Dendritic histiocytic antigen presenting cells 195.Eccrine poroma a. ?Positive EMA 196.Basaloid squamous cell, which does not fit a. ?location, LN mets, distant mets, etc 197.DFSP immuno a. CD34 198.Mucin deposition in skin a. Granuloma annulare 199.Mixed tumor of skin is AKA a. Chondroid syringoma

SOFT TISSUE 200.Rhabdomyosarcoma ID’d by immuno – what is translocation a. t(2;13) (seen in alveolar rhabdo but not consistent in other types) b. Immuno for Rhabdomyosarc i. Myoglobin, Desmin, myosin, tropomyosin alpha-actinin, titn, Z protein, Actin, Vimentin, MyoD1, ILGF II, muscle enzymes 201.Synovial sarcoma a. t(X;18) 202.Giant cells associated with dead skeletal muscle (don’t know question) Choices: Infarct, gun shot wound, polymyositis a. Probably the giant cells are myofiber bags (atrophic muscle cells) b. Would see atrophic muscle cells in: i. ? 203.Most frequent location for myxoid liposarcoma a. Lower extremities, esp thigh b. Is the most common type of liposarc 204.Spindle cell tumor most likely to metastasize? Choices:DFSP vs liposarc vs myxoid liposarc (hopefully there is another more obvious choice) a. Myxoid and well diff liposarc seldom metastasize b. DFSP also has very low rate of mets c. Pleomorphic and round cell liposarc metastasize often 205.Translocation of myxoid liposarc a. t(12;16) – creates TLS/CHOP fusion (p1263) 206.Genetic abnormality in desmoplastic small round cell tumor a. t(11;22) (p13;q24) – involves EWS in 22q24and WT1 in 11p13 SPLEEN 207.Splenic blood lakes a. ? 208.Fibrosis around splenic vessles caused by a. Lupus – causes onion-skin lesions of central penicilliary arteries (p207) 209.Histologic findings of ITP (p619) a. Sinusiodal congestion b. Hyperplastic enlargement of follicles with germinal centers – White pulp hyperplasia c. Increased macrophages in sinusoids 210.What infiltrates red pulp (don’t know choices) a. ? 211.Spleen in TTP(don’t know choices) a. Thrombi in arteries and arterioles without inflammation b. PAS + hyaline subendothelial deposits c. Hyperplasia of germinal centers, periarteriolar concentric fibrosis, hemosiderin macs, hemophagocytosis, extramedullary hematopoiesis TESTIS

212.Positivity for what is seen in embryonal CA but not most other germ cell tumors a. CD30 is positive in >80% embryonal but not in other germ cell tumors (very rare) b. Cytokeratins (pancytokeratin AE1/AE3, Cam 5.2) are positive in embryonal also. c. PLAP positive in >85% d. EMA is negative e. Good panel for embryonal: CK +, PLAP +, CD30 +, EMA 213.Which germ cell tumor is most likely to metastasize? a. Choriocarcinoma is correct if it is a choice, then the other nonseminomatous germ cell tumors, then seminoma 214.What is associated with a worse prognosis in seminoma? a. The presence of other non-seminomatous elements (embryonal, etc) b. Not isolated syncitiotrophoblasts 215.Enlarged testis in 70 year old, most likely dx a. Lymphoma THYMUS 216.Thymic carcinoid and ACTH a. See #54 above– Cushing’s and thymic carcinoid THYROID 217.Black thyroid associated with a. Minocyclin 218.Hurthle cells are not seen in – a. ? James thought that it was either Graves or colloid goiter, but you can see Hurthle cells in both of these. He said the other choices were straight forward, but obviously not. 219.Papillary thyroid CA oncogene a. RET is the protooncogene, PTC is the name of the new oncogene or abnormal form (forms a RET/PTC hybrid) 220.Definition of micropapillary thyroid CA a. Papillary microcarcinoma (Ackerman p 520) is defined as a papillary CA measuring 1cm or less in diameter 221.De Quervain subacute granulomatous thyroiditis, although the cause is uncertain, is associated with (p1127) a. Viral infection (history of previous mumps, measles, flu, adenovirus, coxsackie, echo) MISCELLANEOUS 222.Acute rejection shows (don’t know what organ the question specified) a. Infiltration of mixed population of inflammatory cells b. Cellular injury/necrosis c. Endothelialitis d. May see regenerative changes (eg bile ducts in liver) 223.HLA matched hyperacute rejection shows (don’t know what organ specified)

a. Arthus type reaction with neutrophils in arterioles, glomeruli, and peritubular capillaries, endothelial injury, platelet thrombi, eventual vascular occlusion and infarct (p192, robb) 224.Most common location for angiosarc (don’t know choices) a. Skin, then soft tissue, then breast , then liver (Robb page 510) 225.Congophilic angiopathy associated with lobar hemorrhage occurs in a. Alz ds. (Amyloid angiopathy ) ? not systemic amyoidosis (don’t know about this lobar hemorrhage, which lobes? In lung or brain somewhere? I found this thing about HCHWA-Dutch – familial ds form of Alzheimers – hereditary cerebral hemorrhage with amyloidosis, get hemorrhage and amyloid angiopathy b. Features of Alzheimer’s (pp1329-31, Robb) i. Cortical atrophy with wide sulci ii. Neurofibrillary tangles ( not specific to Alz ds, found in other ds) 1. Contain tau protein, ubiquitin, amyloid beta, MAP2 iii. Neuritic plaques (not specific to Alz ds, but increased numbers of plaques is suspicious) 1. Spherical collection of dystrophic neurites around an amyloid core iv. Plaques and tangles relatively spare motor and sensory cortex but occur in other cortex areas v. Amyloid angiopathy 1. Amyloid beta protein in vessel walls(APP is precursor protein) vi. Granulovacuolar degeneration 1. Intraneuronal cytoplasmic vacule containing argyrophillic granule 2. In hippocampal pyramidal cells vii. Hirano bodies (non-specific) 1. Elongated, eosinophillic bodies containing actic 2. In hippocampal pyramidal cells 226.Fibrinous changes occur in a. Gastric ulcer, rheumatoid arthritis, Polyarteritis nodosa b. Not medial cystic degeneration ( occurs in aortic dissection in Marfan’s p502-503 227.Adenomatoid tumor immuno a. ?CK + 228.Mesothelial hyperplasia a. ?Does not go into bowel wall or form gross nodules 229.Problem with insurance/billing a. ?Look up CPT code 230.Fibrosclerosing diseases include: a. Riedel’s thyroiditis b. Retroperitoneal fibrosis c. Peyronie’s disease

d. ?PBC or PSC e. ?Mesenteric retraction 231.Most common site for papillary mesothelioma a. ?Pleura vs peritoneum (probably pleura) 232.Benign papillary mesothelioma associated with a. ?Young females, psammoma bodies, etc 233.CD 34 is positive in which of the following tumors a. DFSP, solitary fibrous tumor b. ?others 234.S-100 is positive in which of the following a. ? 235.Retrolental fibroplasia a. Retinopathy of prematurity caused by excessive arterial oxygen tensions used to treat respiratory insufficiency in premature newborns b. Get fibrovascular proliferation in retina, causing blindness (Stevie Wonder) 236.Rhabdoid tumor (don’t know question) a. ? 237.EW-1/FT-1 associated with? (Don’t know choices) a. Think this is a typo and they meant EW-1/WT-1 (for ewings sarcoma and wilms tumor (see #206) b. Answ is desmoplastic small round cell tumor 238.Peutz jehger syndrome and sex cord tumor with annular tubules and adenocarcinoma of the cervix. (don’t know question) a. Yes, they are associated. The adenoCA of cervix is a well diff adeno called adenoma malignum 239.Which is least likely to exhibit stromal fatty infiltration a. Heart, liver, pancreas, lymph nodes b. Answer is liver (fat is intracellular, not stromal) 240.Wegener’s, what is most specific a. Positive anca vs blue granular necrosis 241.Wegener’s granulomatosis is associated with a. C-ANCA 242.Wegener’s question: if all occur at same time a. Sinus, renal, lung – I think they do (p496, Robb) 243.Which of the following does not have giant cells a. ?Giant cell reparative granuloma, brown tumor of bone, cherubism, fibrous dysplasia 244.Least likely to metastasize to CNS in male a. ? Prostate, GI, renal, liver, melanoma 245.Features of the neonatal alcohol syndrome a. ?Small head 246.Pt with angiomyolipoma and facial fibroma likely to have a. ?TSC 247.Ghost cells are seen in the following except

a. ?Pilomatrixoma, craniopharyngioma, chordoma, calcifying odontogenic fibroma 248.Cause of myospherulosis a. Gelfoam 249.Placenta in monozygotic twins a. ? 250.What is invariably present in TOF ?