|Year : 2014 | Volume
| Issue : 4 | Page : 198-201
Sclerosing peritonitis with unilateral ovarian luteinized thecoma in a post-menopausal woman: A case report
Auradha De (Pati)1, Supti Mukhopadhyay2, Ayandip Nandi3, Indranil Das2
1 School of Tropical Medicine, Kolkata, West Bengal, India
2 Department of Pathology, Saroj Gupta Cancer Centre and Research Institute, Thakurpukur, Kolkata, India
3 Medical College, Kolkata, West Bengal, India
|Date of Web Publication||21-Nov-2014|
Auradha De (Pati)
Department of Pathology, School of Tropical Medicine, 108, C R Avenue, Kolkata - 700 073, West Bengal
Source of Support: None, Conflict of Interest: None
| Abstract|| |
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.
Keywords: Immunohistochemistry, luteinized thecoma, rare ovarian neoplasm, sclerosing peritonitis
|How to cite this article:|
De (Pati) A, Mukhopadhyay S, Nandi A, Das I. Sclerosing peritonitis with unilateral ovarian luteinized thecoma in a post-menopausal woman: A case report. J Mid-life Health 2014;5:198-201
|How to cite this URL:|
De (Pati) A, Mukhopadhyay S, Nandi A, Das I. Sclerosing peritonitis with unilateral ovarian luteinized thecoma in a post-menopausal woman: A case report. J Mid-life Health [serial online] 2014 [cited 2022 Dec 6];5:198-201. Available from: https://www.jmidlifehealth.org/text.asp?2014/5/4/198/145173
| 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.  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.  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,  high dose steroids, conservative approaches  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.
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.
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)|
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|Figure 4: Photomicrograph showing omental nodule with spindle cell proliferation and hyalinization|
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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.
| 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.  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.  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. 
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.  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.  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. ,, Leuprolide and tamoxifene has been tried as targeted therapy with success.  Current approach is preservation of fertility either with oocyte extraction and preservation  or unilateral oophorectomy along with high dose steroid and hormone suppression.  Post-operative complications due to short bowel syndrome and sepsis are frequent and are managed conservatively. ,,
| 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.
| References|| |
|1.||Levavi H, Sabah G, Heifetz M, Feinmesser M. Sclerosing peritonitis associated with bilateral luteinized thecoma, linked to anticonvulsant therapy. Eur J Gynaecol Oncol 2009;30:695-700. |
|2.||Bianco R, de Rosa G, Staibano S, Somma P, Bianco AR. Ovarian luteinized thecoma with sclerosing peritonitis in an adult woman treated with leuprolide and toremifene in complete remission at 5 years. Gynecol Oncol 2005;96:846-9. |
|3.||Schonman R, Klein Z, Edelstein E, Czernobilsky B, Fishman A. Luteinized thecoma associated with sclerosing peritonitis--conservative surgical approach followed by corticosteroid and GnRH agonist treatment--a case report. Gynecol Oncol 2008;111:540-3. |
|4.||Clement PB, Young RH, Hanna W, Scully RE. Sclerosing peritonitis associated with luteinized thecomas of the ovary. A clinicopathological analysis of six cases. Am J Surg Pathol 1994;18:1-13. |
|5.||Fletcher CD. Diagnostic histopathology of tumours. Churchill livingstone: Elsevier publications; Vol. 1., Ch 13. Tumours of female genital tract. part A: Ovary, fallopian tubes and broad and round ligaments. p. 593. |
|6.||Mellembakken JR, Engh V, Tanbo T, Czernobilsky B, Edelstein E, Lunde O, et al. Mitotically active cellular luteinized thecoma of the ovary and luteinized thecomatosis associated with sclerosing peritonitis: Case studies, comparison, and review of the literature. Pathol Res Pract 2010;206:744-8. |
|7.||Staats PN, McCluggage WG, Clement PB, Young RH. Luteinized thecomas (thecomatosis) of the type typically associated with sclerosing peritonitis: A clinical, histopathologic, and immunohistochemical analysis of 27 cases. Am J Surg Pathol 2008;32:1273-90. |
|8.||Iwasa Y, Minamiguchi S, Konishi I, Onodera H, Zhou J, Yamabe H. sclerosing peritonitis associated with luteinized thecoma of the ovary. Pathol Int 1996;46:510-4. |
|9.||Noyes N, Perretta RC, Fino ME, Matulewicz T, Barakat R. Use of hormone suppression then oocyte freezing to preserve reproductive capability in an adolescent girl with ovarian luteinized thecoma associated with sclerosing peritonitis. Fertil Steril 2009;92:393.e11-4. |
|10.||Spiegel GW, Swiger FK. Luteinized thecoma with sclerosing peritonitis presenting as an acute abdomen. Gynecol Oncol 1996;61:275-81. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]