Sclerosing peritonitis with unilateral ovarian luteinized thecoma in a post-menopausal woman: A case report.

A unique case of unilateral ovarian luteinized thecoma with sclerosing peritonitis is reported because the association between these two conditions is extremely rare. Etiology is not clearly known. A 50-year-old post-menopausal woman presented with pain abdomen and huge swelling. Pelvic contrast-enhanced computed tomography (CECT) revealed a heterogenous enhancing mass arising possibly from ovary and adherent to uterus, bowl loops, and omentum. A spindle cell neoplasm without definite features of malignancy was reported in computed tomography (CT)-guided fine-needle aspiration cytology (FNAC). Ascitic fluid was negative for malignancy. Her serum carcinoembryonic antigen (CEA) (1.1ng/ml) and CA125 (27.6μ/ml) level was within normal limits. Total abdominal hysterectomy with bilateral supracolic omentectomy, resection of part of colon, and supracolic omentectomy was done. Histopathological diagnosis was luteinized thecoma with sclerosing peritonitis. Tumor cells were immunoreactive to smooth muscle antigen (SMA) and CD 99 and non- reactive to ER, PR, Calretinine, and Cytokeratin. Patient developed post-operative sepsis and enterocutaneous fistula, which was managed conservatively and was kept in follow-up. According to current concept, in spite of its large size, wide-spread involvement, mitotic activity, and complications due to peritoneal involvement, behavior of this rare clinical entity is benign.

INTRODUCTION

Luteinizing thecoma with sclerosing peritonitis is a rare ovarian tumor, the etiology and pathogenesis is not clear till date. The entity was proposed in 1994, and an association with anti-epileptic drugs was suggested.[1] Approximately 30 cases has been reported among which a few Indian reports has been documented so far. Luteinized thecoma of the ovary is microscopically characterized by a group of spindle to polygonal cells with clear, vacuolated, lipid-filled cytoplasm which is linked to tumor oestrogen activity.[2] This neoplasm may be associated with multiple fibrotic nodular thickening of the peritoneum called sclerosing peritonitis. Several treatment options like Antiestrogens plus luteinizing-hormone-releasing hormone (LHRH) agonists,[2] high dose steroids, conservative approaches[3] have been tried. So far, the disease entity is labelled as benign albeit with life-threatening complications due to sclerosing peritonitis. We report a case with unilateral ovarian mass with extensive omental deposits, clinically interpreted as sarcoma which was came out to be luteinized thecoma with sclerosing peritonitis in histopathological studies.

CASE REPORT

A 50-year-old post-menopausal woman, known diabetic and hypertensive, presented to us with huge abdominal swelling and pain. History of single episode of generalized tonic clonic seizure was found and prophylactic phenytoin was started. Contrast enhanced computed tomographic (CECT) scan revealed heterogeneously contrast enhancing mass of 17 × 12 × 5 cm arising possibly from ovary. Guided fine-needle aspiration cytology (FNAC) from the mass revealed spindle cell neoplasm without definite features of malignancy [Figure 1]. Her serum levels of carcinoembryonic antigen (CEA) and CA-125 were 1.1ng/ml and 27.6 u/ml, respectively. Hematological and biochemical parameters were within normal limits except high fasting plasma glucose and elevated lactate dehydrogenase level. Ascitic fluid cytology was negative for malignant cells. Total abdominal hysterectomy along with bilateral salpingo-oophorectomy and resection of adherent bowel loop along with mesocolon and supracolic omentectomy was done.

Figure 1

FNAC smear shows spindle cell neoplasm (MGG×40)

On gross examination, huge solitary mass measuring 20 × 19 × 12 cm with adherent colonic loop, mesocolon, uterus, and cervix was noted. Opposite ovary was identified which was normal except one unilocular thin walled cyst, 1-cm diameter. External surface of the mass was nodular and cut surface of mass showed solid cerebriform yellowish areas with mucohemorrhagic and cystic zones [Figure 2]. Supracolic omentectomy specimen showed numerous patchy whitish thickened areas without any definite nodule.

Figure 2

Cut surface of ovarian mass shows solid yellowish areas

On microscopy, the tumor comprised of fascicles and sheets of plump spindle and oval cells that resembled the cells of theca interna. The cells had fusiform nuclei, fine chromatin, and clear vacuolated to pale eosinophilic cytoplasm. Variable number of fibroblastic cells, hyalinized connective tissue plaques are interspersed among the tumor cells [Figure 3]. The tumor was completely separated from myometrium, and the adjacent gut wall by its capsule. Opposite-sided ovarian stroma was normal and showed single follicular cyst. Omental sections showed proliferations of spindle cells along with few cells with clear cytoplasm in whirling fashion [Figure 4]. Histomorphological diagnosis of thecoma with sclerosing peritonitis was made and immunohistochemistry (IHC) was advised.

Figure 3

Photomicrograph showing luteinized thecoma of the ovary (H & E×40)

Figure 4

Photomicrograph showing omental nodule with spindle cell proliferation and hyalinization

On IHC, the cells were reactive for smooth muscle antigen (SMA) [Figure 5] and CD99 [Figure 6] supporting sex cord stromal origin. However, cells were nonreactive for estrogen receptor, progesterone receptor, calretinin, cytokeratin. Patient developed post-operative sepsis and enterocutaneous fistula and was managed with conservative treatment and was kept in follow-up. Presently, she is doing well.

Figure 5

Tumor cells showing immunoreactivity to SMA (×10)

Figure 6

Tumor cells show immunoreactivity to CD99 (×10)

DISCUSSION

Thecomas are relatively rare neoplasms (4% of all ovarian neoplasms) that belong to sex-cord stromal group of tumors. It is closely related to fibroma, sometimes collectively called as fibrothecoma group. Usually post-menopausal woman presents with unilateral large ovarian mass which has well-defined capsule, cut-surface having typical yellow bosselated appearance. Luteinized thecomas are a special group which usually occur in younger woman are usually bilateral and often associated with a peculiar condition called sclerosing peritonitis. The process of peritonitis consists of fibroblastic and myofibroblastic cell proliferation separated by collagen and fibrin, mesothelial cell proliferation, and occasionally mononuclear inflammatory cells. The etiology and pathogenesis is not clear. An association with anti-epileptic drug has been discussed. Some proposed fibrosing soluble cytokines secreted by tumor cells to be responsible for the peritoneal manifestations.[4] The tumor cells are shown to have estrogenic activity and are responsible for menstrual irregularities and endometrial hyperplasia, even co-existing carcinomas in 15–30% cases.[5] The clinical presentation of thecoma varies according to the size of the mass, larger ones typically present with abdominal swelling, pain, and ascites. While smaller ones are brought to attention due to hormonal disturbances, those associated with sclerosing peritonitis are typically associated with multiple subocclusive symptoms and needs recurrent laparotomy.[2]

Microscopically, the lesion comprises of fascicles of spindle cells with centrally placed nuclei and a moderate amount of pale vacuolated cytoplasm. Intervening tissue shows considerable collagen deposition and hyaline plaque formation which was particularly prominent in this case. A mitotically active variant has been discussed which are particularly common in sclerosing peritonitis but the clinical behavior does not seem to be affected by high mitotic count.[6] Immunohistochemical studies have shown the tumor cells to be reactive with calretinin, CD56, AE1/3, smooth muscle actin, and desmin, and variably with alpha-inhibin, epithelial membrane antigen, beta-catenin, CD34, and transforming growth factor-beta, with focal nuclear positivity for estrogen and progesterone receptor.[7] Our study found the cells were immunoreactive for SMA and CD 99 but nonreactive for estrogen receptor, progesterone receptor, calretinin, cytokeratin.

Differential diagnoses to be considered in this case were fibroma, sclerosing stromal tumor, stromal leydig cell tumor, and secondary deposits in peritoneum.

Currently, the concept is that the tumor is benign in behavior although complications due to sclerosing peritonitis bring down the quality of life. Few studies advocated conservative approach and followed up the patient for as far as 7 years without any morbidity.[248] Leuprolide and tamoxifene has been tried as targeted therapy with success.[2] Current approach is preservation of fertility either with oocyte extraction and preservation[9] or unilateral oophorectomy along with high dose steroid and hormone suppression.[3] Post-operative complications due to short bowel syndrome and sepsis are frequent and are managed conservatively.[1210]

CONCLUSION

As far our knowledge, only a few cases of unilateral thecoma in post-menopausal woman in India presenting with sclerosing peritonitis has been reported. We reviewed the literature and also re-evaluated the immunohistochemical analysis. Standard guideline of treatment is yet to be formulated for this rare entity.