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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 4  |  Page : 554-558
Metastatic rhabdomyosarcomatous elements, mimicking a primary sarcoma, in the omentum, from a poorly differentiated ovarian Sertoli-Leydig cell tumor in a young girl: An unusual presentation with a literature review


1 Department of Pathology, Tata Memorial Hospital, Mumbai, India
2 Department of Pathology, Prince Aly Khan Hospital, Mumbai, India

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Date of Web Publication1-Oct-2009
 

   Abstract 

Sertoli-Leydig cell tumors (SLCTs) of the ovary with mesenchymal heterologous elements are uncommon. Only few such cases have been documented, showing presence of only mesenchymal heterologous elements at the metastatic site. We report an unusual case in a young girl who presented with an omental mass that was consistent with histopathological features of a high-grade sarcoma, with prominent rhabdomyoblastic differentiation of the embryonal type. The sections from her ovarian mass for which she was operated a year back displayed features of a poorly differentiated SLCT with heterologous elements, including focal rhabdomyoblastic differentiation. This is one of the rare cases, to the best of our knowledge, where only rhabomyosarcomatous elements were identified at the metastatic site, mimicking a primary abdominal rhabdomyosarcoma, in a case of an ovarian SLCT. Further, this case reinforces the presence of rhabdomyosarcomatous elements in an ovarian SLCT to be associated with an aggressive disease course.

Keywords: Heterologous elements, omental metastasis in Sertoli-Leydig cell tumors, ovarian Sertoli-Leydig cell tumors, rhabdomyosarcomatous differentiation

How to cite this article:
Rekhi B, Karpate A, Deodhar KK, Chinoy R F. Metastatic rhabdomyosarcomatous elements, mimicking a primary sarcoma, in the omentum, from a poorly differentiated ovarian Sertoli-Leydig cell tumor in a young girl: An unusual presentation with a literature review. Indian J Pathol Microbiol 2009;52:554-8

How to cite this URL:
Rekhi B, Karpate A, Deodhar KK, Chinoy R F. Metastatic rhabdomyosarcomatous elements, mimicking a primary sarcoma, in the omentum, from a poorly differentiated ovarian Sertoli-Leydig cell tumor in a young girl: An unusual presentation with a literature review. Indian J Pathol Microbiol [serial online] 2009 [cited 2014 Nov 22];52:554-8. Available from: http://www.ijpmonline.org/text.asp?2009/52/4/554/56165



   Introduction Top


Sertoli-Leydig cell tumors (SLCTs) are uncommon, accounting for less than 0.5% of the ovarian tumors, predominantly noted in young females. Histologically, these tumors exhibit a wide range of morphologic appearances, including presence of somatic elements that are designated as heterologous elements, noted in 20% of these tumors. [1],[2] The most common heterologous component identified is the mucinous epithelium of the gastrointestinal (GI) tract. [3] Five percent of these tumors also contain mesenchymal elements. [2] Herein, we report a case of a young girl, diagnosed with a poorly differentiated SLCT of the ovary with heterologous elements, who, subsequently, developed omental deposits that showed presence of only rhabdomyosarcomatous elements.


   Case Report Top


A 17-year-old girl presented in the intensive care unit of our hospital with vague abdominal pain and distension. Her past history revealed an ovarian tumor for which she was operated 1 year back. On clinical examination, she had pallor and ascites without pedal edema, hormonal manifestations or lymphadenopathy. Her chest examination was normal. Her abdominal ultrasonography revealed absence of right ovary and ascites that was submitted for cytological evaluation. Subsequently, she underwent an exploratory laparotomy, wherein an omental mass was submitted for a frozen section evaluation. Thereafter, she underwent a total hysterectomy with left-sided salpingo-oophorectomy.

Her earlier medical records unravelled a reviewed diagnosis of a poorly differentiated SLCT of the right-sided salpingo-oophorectomy that she had undergone elsewhere. Adjuvant chemotherapy (CT) was planned; however, she was lost to follow-up.

The microsections from the ovarian mass as well as from the omental mass were subjected to conventional hematoxylin and eosin (H and E) staining and special stains like phosphotungstic acid-hematoxylin and immunohistochemical (IHC) staining. IHC was performed by immunoperoxidase using the avidin-biotin method and 3'-3'D-aminobenzidine tetrachloracetic acid (DAB) as the chromogen. The various antibody markers were vimentin (1:50, monoclonal, Dako, Produkionsveg, Glostrup, Denmark), cytokeratin (CK) (1:100, monoclonal), epithelial membrane antigen (EMA) (1:100, monoclonal), desmin (1:80, monoclonal), myoglobin (1:600, polyclonal), Myo D-1 (1:40 monoclonal), smooth muscle actin (SMA) (1:200, monoclonal), S-100 (1:300, polyclonal), inhibin (1:50, monoclonal), β-feto protein (AFP), CD30 and β-human chorionic gonadotrophin (β-HCG).

Laboratory findings

Her hemoglobin level was 11.1 g/dl. Other biochemical investigations were within normal limits.

Twelve cc of hemorrhagic ascitic fluid was centrifuged for cytological evaluation. Smears were negative for malignant cells. Her tumor marker levels for AFP, β-HCG and CA-125 were normal. She underwent an exploratory laparotomy wherein an excised omental mass was submitted for frozen section analysis.

Histopathological findings

Grossly, multiple soft tissue bits measuring 25 cm x 15 cm x 4 cm were received and reported as a malignant tumor. H and E-stained, paraffin-embedded tissue microsections showed a malignant spindle cell tumor with interspersed plump cells displaying distinct cross-striations, reminiscent of a rhabdomyoblastic differentiation of the embryonal type. Mitoses were >10/10 high-power fields (hpf). IHC showed diffuse positivity for vimentin and conspicuous areas of desmin and myoglobin positivity. SMA, CK, EMA and inhibin were negative. Diagnosis of a sarcoma with rhabdomyoblastic differentiation was formed [Figure 1]A-E. The uterus, left-sided ovary and the  Fallopian tube More Details were free of tumor.

Histopathological findings of the earlier excised ovarian mass

As per the referring pathologists' report, grossly, the right-sided ovarian mass weighed 1.5 kg and measured 25 cm x 20 cm x 15 cm. The external surface was bosselated. The cut surface was partly solid area and partly cystic. Attached fallopian tube was unremarkable.

On review of the submitted microsections, H and E staining revealed a tumor composed of tubular structures lined by plump, eosinophilic cells with hyperchromatic nuclei and prominent nucleoli reminiscent of  Sertoli cells More Details. Areas displaying small irregular "slit-like" areas/retiform pattern were noted. The tubules were interspersed within ovoid, dense stromal cells along with spindly sarcomatous cells that exhibited moderate pleomorphism. In addition, there were focal clusters of cells with pale, eosinophilic cytoplasm reminiscent of Leydig cells. Areas of necrosis and hemorrhage were noted. Mitoses were >10/10 hpf. In addition, mucinous glands and discrete rhabdomyosarcomatous elements were identified. IHC showed focal positivity for CK and EMA. Vimentin, desmin and Myo D-1 were distinctly positive in sarcomatous elements while SMA, inhibin, CD30, AFP and β-HCG were negative [Figure 2] A-I. A diagnosis of a poorly differentiated SLCT with heterologous elements was offered.

Henceforth, the omental deposits were equated to metastasizing rhabdomyosarcomatous elements from the poorly differentiated ovarian SLCT.


   Discussion Top


SLCTs of the ovary are uncommon, potentially malignant neoplasms. Many such patients present with virilizing symptoms or estrogenic manifestations. However, half of the patients might not have any endocrine manifestations, as was noted in our case. These tumors are hypothesized to be originating from a variety of cells, including granulosa cells, female-directed cells within the ovarian stroma and male-directed cells as seen in the ductuli efferent. [1],[2] Histologically, these tumors display varying combinations of Sertoli and Leydig cells along with variable heterologous elements. This diverse morphology has led to their five histologic types, including well differentiated, intermediate differentiated and poorly differentiated, the latter showing retiform patterns and heterologous elements. Mixed cellular patterns account for the diversity with this tumor. [1],[4],[5]

The heterologous elements are, most commonly, mucin-secreting GI elements and, uncommonly, hepatoid and yolk sac elements, the latter associated with increased AFP levels. [6] The presence of mesenchymal components is rare. [1] These include cartilage, rhabdomyosarcomatous differentiation, well-differentiated smooth muscles, bone and adipose tissues. [7] Prat et al. [4] identified mesenchymal components in 12 of their 48 cases of SLCTs. Zaloudek et al. [5] identified only two such cases. These elements can be present in the primary tumor and/or in the recurrent lesions. However, their exclusive presence in the abdominal/omentum, mimicking a primary sarcoma, has rarely been documented. [4] In the present case, the heterologous elements in the primary tumor were epithelial and sarcomatous, whereas the omental deposits exclusively revealed rhabdomyosarcomatous elements. Because of a variegated gross morphology that can be with this ovarian tumor, it is always necessary to sample it extensively, i.e. one section per centimeter tumor. Our case was referred with limited sections. Nonetheless, on retrospective analysis of the primary tumor, discrete rhabdomyosarcomatous elements were also identified that ruled out the possibility of a primary rhabdomyosarcoma.

Retiform SLCTs are known to be associated with the heterologous elements, as seen in our case. [6] The presence of mesenchymal heterologous elements is associated with the intermediate and poorly differentiated forms. [3],[4],[5] In their nine cases, Prat et al.[4] observed heterologous elements at peritoneal and pelvic sites in eight cases, among which these were noted in the omentum in one case, as seen in our case. The duration of these recurrences ranged from 3 months to 5 years. Zaloudek et al. [5] identified only one such presentation. In our case, the omental deposits were noted within a year of the primary tumor.

The prognosis of SLCTs depends on the tumor stage. The other proposed poor prognostic factors include poor differentiation, frequent mitotic figures (>5/10 hpf) and the presence of mesenchymal heterologous elements, as noted in our case. One of the two such cases in the study of Zaloudek et al., [5] had peritoneal metastasis and was fatal. Prat el al.[4] had a single case of SLCT with immature skeletal elements in the omental deposits, who succumbed to the disease within 18 months. In our case, the primary tumor had rhabdomyosarcomatous elements. Further, the poorly differentiated morphologic type with retiform pattern was associated with an adverse clinical course in the form of occurrence of omental deposits within a year. In an earlier study, only four cases of SLCT have been documented with presence of immature skeletal elements [8],[9],[10] [Table 1].

Therapeutically, a salpingo-oophorectomy is the treatment of choice for a well-differentiated international federation of gynecology and obstetrics FIG O stage-1 SLCT. Adjuvant CT is added in poorly differentiated variants. [4],[5] Our case was planned for adjuvant CT.

In terms of its etiopathogenesis, origin of heterologous elements is reminiscent of a metaplastic dedifferentiation, which is further supported by the fact that neither SLCT elements nor gonadal stromal elements are found in teratomas. [3] Nonetheless, presence of rhabdomyoblastic dedifferentiation is an adverse histomorphological parameter.

To sum up, our case reinforces the value of identification of rhabdomyosarcomatous elements in a case of an SLCT in view of its association with an aggressive disease course.

 
   References Top

1.Tavassoli FA, Mooney E, Gersell DJ, et al. Sex cord-stromal tumours. In: Tavassoli FA, Devilee P, editors. World Health Organization (WHO) Classification of Tumors, Pathology and Genetics of Tumors of the Breast and Female Genital Organs 2003. Lyon: International Agency for Research on Cancer; 2003. p. 146-61.  Back to cited text no. 1      
2.Young RH, Scully RE. Ovarian Sertoli-Leydig cell tumors. A clinicopathological analysis of 207 cases. Am J Surg Pathol 1985;9:543-69.  Back to cited text no. 2      
3.Young RH, Prat J, Scully RE. Ovarian Sertoli-Leydig cell tumors with heterologous elements. I. Gastrointestinal epithelium and carcinoid: A clinicopathologic analysis of thirty-six cases. Cancer 1982;50:2448-56.  Back to cited text no. 3      
4.Prat J, Young RH, Scully RE. Ovarian Sertoli-Leydig cell tumors with heterologous elements. II. Cartilage and skeletal muscle: A clinicopathologic analysis of twelve cases. Cancer 1982;50:2465-75.  Back to cited text no. 4      
5.Zaloudek C, Norris HJ. Sertoli-Leydig tumors of the ovary. A clinicopathologic study of 64 intermediate and poorly differentiated neoplasms. Am J Surg Pathol 1984;8:405-18.  Back to cited text no. 5      
6.Mooney EE, Nogales FF, Bergeron C, Tavassoli FA. Hepatocytic differentiation in retiform sertoli-leydig cell tumours: Clinical, morphological and immunohistochemical findings. Histopathology 2002;41:110-7.  Back to cited text no. 6      
7.Roth LM, Anderson MC, Govan AD, Langley FA, Gowing NF, Woodcock AS. Sertoli-Leydig cell tumors. A clinicopathologic study of 34 cases. Cancer 1981;48:187-97.  Back to cited text no. 7      
8.Grove A, Vestergaard V. Ovarian Sertoli-Leydig cell tumor of intermediate grade with heterologous elements of rhabdomyosarcoma. A case report and a review of the literature. Ann Diagn Pathol 2006;10:288-93.  Back to cited text no. 8      
9.Guιrard MJ, Ferenczy A, Arguelles MA. Ovarian Sertoli-Leydig cell tumor with rhabdomyosarcoma: An ultrastructural study. Ultrastruct Pathol 1982;3:347-58.  Back to cited text no. 9      
10.Kostopoulou E, Talerman A. Ovarian Sertoli-Leydig cell tumor of intermediate differentiation with immature skeletal muscle heterologous elements. Acta Obstet Gynecol Scand 2003;82:197-8.  Back to cited text no. 10      

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Correspondence Address:
Bharat Rekhi
Department of Pathology, 8th Floor, Annex Building, Tata Memorial Hospital, Dr. E.B. Road, Parel, Mumbai-400012
India
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DOI: 10.4103/0377-4929.56165

PMID: 19805972

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