An Unusual Ovarian Tumor

An unusual ovarian tumor: Case presentation and review of literature Dr. Paban Kumar Sharma, MD, Obgyn Dr. Sujan Vaidya, MD, Pathology MIDAT Hospital, Lagankhel, Lalitpur Contact: [email protected]

Abstract: Ovarian tumor is one of the common problem. Lipoid Ovarian tumor associated with unexplained anemia is one of the rare condition. We present such a case who had unexplained anemia with right sided solid ovarian tumor. After removal of tumor, anemia was corrected without treatment. Ovarian steroid cell tumours account for approximately 0.1% of all ovarian neoplasms. This is a rare tumor with malignant potential and association with multiple different endocrine or paraneoplastic changes. This case is reported for its rarity and its unusual presentation of not having any clinical signs and symptoms typical of a steroid hormone secreting tumour but having explained anemia. Key words: Steroid cell tumor, Lipoid tumor.

Introduction: Case: A 27 yrs unmarried lady, student, from Kathmandu was admitted in MIDAT clinc for laparotomy with a diagnosis of mass in lower abdomen. She gives on and off history of pain in lower back for last 10 yrs for which she was treated with pain medicine and became better. One year back, she developed generalized weakness, bruising of skin and diagnosed to have anemia. She received 2 units of blood at that time. Hemoglobin at that time was 6 gm % and PCV 18%. Her leukocyte with differential and platelet counts all were within normal limits. Peripheral smears showed hypochromic picture with anisocytosis. She had normal liver function test and normal reticulocyte count. Serum urea, creatinine, blood sugar, electrolytes and liver function tests, all were within normal limits but her hemoglobin level never came to the normal level even after iron therapy. She had normal menstrual cycles

and no other significant medical problem except anemia. A sonography was done after eight months (2065.3.22) which showed 10.4 x 7 x 7.3 cm fairly well defined solid mass seen in the pelvic cavity just superior to the fundus of the uterus. Ultrasound examination was repeated after one week by another radiologist, which showed a large oval mass of 9.8x6.4 cm well defined solid lesion apparently continuous with a pedicle suggestive of pedunculated fibroid. CT scan showed a solid mass of 9x7.6x7.5 cm, which was well defined and having heterogeneous density. Post contrast study was showing moderate enhancement of the mass. The mass was located anterior to the uterus and moderately compressing the urinary bladder. The mass did not appear to be ovarian origin but there was no comment regarding ovaries. There was an enlarged lymph node in aortic bifurcation area but no other lymph node were enlarged. Uterus was found to be normal size and there was no free fluid in the peritoneal cavity. The differential diagnosis given in the report was lymphoma, GIST and ovarian mass. Ca 125, beta hCG and alfa feto protein all were normal limit. laparotomy was done on 2065.4.14, at that time her hemoglobin level was 9 gm%, but all pre-operative investigations were within normal limit. During laparotomy there was around 100 ml of clear fluid in the pelvis. Uterus, other ovary, tubes and other viscera all were normal looking. No lymph node was palpable. There was a solid tumor in left ovary with intact, no adhesion and on deposit on the surface. Size of the tumor was 9 x 8 cm. Right adenectomy was done along with omental biopsy. On cut section, there was a white capsule with inside yellow solid lobulated areas and a septae separating the different lobules. Microscopically, Omentum was normal looking and cytology was negative for malignant cells. An impression of Steroid (Lipoid) cell tumor, not otherwise specified was made. Post operative period was uneventful and discharged on 3rd post operative day. She had two episodes of menorrhagia in subsequent months, which was managed with progesterone and iron. She is completely alright after 4 months of surgery and her hemoglobin level is now, more than 10 gm%.

Picture 1: intra-operative picture of tumor

Picture 2: Other ovary and uterus

Picture 3: Cut section of the tumor

Picture 4: cut section of the tumor

Picture:5 Microscopic view

DISCUSSION:

Ovarian steroid cell tumours account for approximately 0.1% of all ovarian neoplasms.1These are rare tumours for which there is interest, not for their malignant potential, but for their association with multiple different endocrine or paraneoplastic changes. The histogenesis of steroid cell tumor of ovary is still controversial. They are thought to arise from ovarian stroma, hilus or adrenal cortical rests and

secrete steroid hormones.1Until recently, these tumors were thought to contain lipid and hence called “lipid cell tumor” or “lipoid cell tumor”. Because 25 % of these tumors contain little or no lipid, Hayes and Scully in 1979 initiated the term “steroid cell tumor” to describe this rare group of tumours1. This term justifies the morphological appearance of these tumors as well as provides insights into their clinical manifestations. They are subclassified according to their cells of origin. Subclassification: 1. Stromal luteoma 2. Leydig cell tumour: a. Hilus cell type b. Leydig cell tumour, non hilar type 3. Steroid cell tumour, not otherwise specified (NOS) Steroid cell tumours that cannot be diagnosed specifically as stromal luteomas or Leydig cell tumour subtypes are classified as steroid cell tumours, not otherwise specified (NOS) 1. They are the most common of the steroid cell tumours and account for 60% of all steroid cell tumours and occur at a younger age (average - 43 years) than other types of steroid cell tumours and in contrast to the latter, they occasionally occur before puberty14.

The malignant potential of this tumour is uncertain. In contrast to benign nature of both the stromal luteomas and Leydig cell tumours, 25 to 43% of steroid cell tumours, NOS are malignant1,5. In one study, patients with clinically malignant tumours were on average 16 years older than those with “probably benign” tumours1. However, a rare case of malignancy has also been reported in a 8 year old girl3. The histological criteria for malignancy are grade 2 or 3 nuclear atypia, tumour diameter (> 7 cm), mitotic rate (> 2/hpf), necrosis and haemorrhage. Capsular penentration and vascular invasion also point to malignancy, although metastasis is the only definite sign of malignancy1. Steroid cell tumours, NOS are associated with androgenic changes in 50% of the cases, oestrogenic in 6 to 23% cases while few cases have shown progestational changes1,2. About 10 to 15% of the patients are asymptomatic, with tumours detected incidentally during routine pelvic examination or in a surgical intervention, as in our case6. Virilization is the most common clinical manifestation of this tumour.1 Other unusual clinical presentations include, Cushing’s syndrome (6 to 10%

cases), oestrogenic manifestations and rare cases of isosexual pseudoprecocious puberty.1,2,7 A rare example of virilizing steroid cell tumours, NOS associated with hypothyroidism and secondary hyperlipidemia, has been reported.8 A renin secreting tumour associated with secondary polycythaemia has also been reported.9 Other rare examples have been associated with erythrocytosis, hypercalcaemia, ascitis and aldosterone secretion.1,10 In our case, there were no such clinical manifestations of hormone secretion of any kind by the tumour. It was diagnosed only after surgical intervention and subsequent histopathological examination of the ovarian mass. Therefore, no hormonal assays were done. The gross and microscopic features were suggestive of a steroid cell tumour, NOS which was limited to the ovary. Except for its large size (> 7 cm) no other gross or microscopic features of malignancy were noted. The patient is on regular follow up and is doing well. The differential diagnoses of steroid cell tumour, NOS include, leutinised granulosa cell tumour, thecoma, clear cell carcinoma, metastatic renal cell carcinoma and lipid rich sertoli cell tumour. All these tumours were excluded before reaching our diagnosis. This case is reported for its rarity and its unusual presentation of not having any clinical signs and symptoms typical of a steroid hormone secreting tumour.

REFERENCES: 1. Hayes MC, Scully RE. Ovarian steroid cell tumors (not otherwise specified). A clinicopathological analysis of 63 cases. Am J Surg Pathol 1987;11:835-845.. 2. Adeyemi SD, Grange AO, Giwa-Osagie OF, Elesha SO. Adrenal rest tumor of the ovary associated with isosexual precocious pseudopuberty and cushingoid features. Eur J Pediatr 1986;145:236. 3. Naik VS, Jashnani KD, Kandalkar BM, Oak SN. Steroid (lipid) cell tumour of the ovary in an eight year old girl. Bhj.org/journal/2005_july/html/case_steroid. 4. Campbell PE, Danks DM. Pseudoprecocity in an infant due to a luteoma of the ovary. Arch Dis Child 1963;38:518-23. 5. Taylor HB, Norris HJ. Lipid cell tumours of the ovary. Cancer 1967;20:1953-62.

6. Hartmann LC, Young RH, Podratz KC. Ovarian sex cord-stroma tumors. In: Hoskins WJ, Perez CA, Young RC, eds. Principles and Practice of Gynecologic Oncology. 3rd edn. Lippincott Williams and Wilkins, 2000:1059 7. Donovan BJ, Otis CN, Powell JL, Cathcart HK. Cushing’s syndrome secondary to malignant lipoid cell tumour of the ovary. Gynecol Oncol 1993;50:249-53. 8. Tsai HJ, Chen SC, Wei HY, Chen GD. Hypothyroidism and Hyperlipidemia with a Virilizing Ovarian steroid cell tumor, Not Otherwise Specified. 9. Stephen MR, Lindop GBM. A renin secreting ovarian steroid cell tumour associated with secondary polycythaemia. J Clin Pathol 1998;51:75-82. 10. Kulkarni JN, Mistry RC, Kamat MR, Chinoy R, Lotlikar RG. Case report of autonomous aldosterone-secreting ovarian tumour. Gynaecologic Pathology 1990;37:284-9.