A Rare Ovarian Tumor -- Sertoli-Leydig Cell Tumor With Heterologous Element

Rimpy Tandon, MD; Poonam Goel, MD; Pradip Kumar Saha, MD; Navneet Takkar, MD; RPS Punia, MD

Disclosures
In This Article

Discussion

We here present an unusual case of SLCT with heterologous elements. Heterologous elements are observed in approximately 20% of SLCT. These can be further separated into 2 basic types: endodermal elements and mesenchymal elements. The endodermal type is represented by gastric or intestinal type mucin-secreting epithelium. The mesenchymal elements are represented by immature cartilage and or skeletal muscle. The SLCT with heterologous mesenchymal elements are usually poorly differentiated in contrast to neoplasms with endodermal elements, which typically are of intermediate differentiation.[3] In our patient the heterologous element was in the form of mucinous epithelium of the gastrointestinal type. Though Meyer et al. described the presence of mucinous epithelium in a SLCT in 1930, the first illustration is in the case reported by McLester in 1936, who described a SLCT containing a cyst lined by nonmucinous cells and mucinous cells, including glandular cells.[5,6] The majority of these patients are seen during the second and third decades of life, with the average age at diagnosis 25 years. Around 50% of cases come to clinical attention because of progressive defeminization, as was seen in this patient.[1]

The most important prognostic factors in these tumors are their stage and degree of differentiation. In a review of 207 cases by Young and Scully in 1985,[1] all well-differentiated tumors were benign, whereas 11% of tumors with intermediate differentiation, 59% of tumors with poor differentiation, and 19% of those with heterologous elements were malignant. In another study of 64 patients who had intermediate or poorly differentiated SLCT, a survival rate of 92% was noted at both 5 and 10 years.[7]

Most of these tumors are unilateral and diagnosed in stage I, so conservative surgery in a young patient is an appropriate treatment. There have also been case reports of successful laparoscopic management of these tumors.[8] Adjuvant chemotherapy is considered for patients who have poor prognostic factors.

The malignancy rate in tumors with heterologous elements is 15% to 20%.[1] Adjuvant chemotherapy in stage I is given to those patients who have poorly differentiated SLCT or SLCT with heterologous elements or a metastatic tumor of any histologic type.[9]

The patient in this report received adjuvant chemotherapy for SLCT in stage I. The 2 factors responsible for this decision were incomplete staging of the tumor and the presence of heterologous elements on histopathologic study of the tumor. The following chemotherapy regimens were considered:

  1. Cisplatin, doxorubicin, cyclophosphamide (PAC);[10]

  2. Vincristine, actinomycin- D, cyclophosphamide (VAC);[11] and

  3. Bleomycin, etoposide, and cisplatin (BEP).[12]

The BEP regimen is a comparatively safe chemotherapeutic regimen because it does not affect the fertility status of the patient.[12]

In conclusion, SLCT is a rare ovarian sex-cord tumor that usually occurs unilaterally. SLCT should always be considered in a young female patient who has symptoms of virilization and an ovarian mass on examination or investigation.

Management issues mostly revolve around the histopathology of the tumor. Poorly differentiated tumors require aggressive management because the chances of them being malignant are high. Intermediately differentiated tumors need an individualized approach. The patient should be involved in all decision making.

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