International Journal of Gynecological Pathology:
Pathology of the Upper Genital Tract: Case Reports
Stacher, Elvira M.D.; Pristauz, Gunda M.D.; Scholz, Heinz S. M.D.; Moinfar, Farid M.D.
Institute of Pathology (E.S., F.M.)
Department of Gynecology and Obstetrics, (G.P., H.S.S.) Medical University of Graz, Graz, Austria
Conflict of Interest: The authors declare no conflict of interest.
Address correspondence and reprint requests to Elvira Stacher, MD, Department of Pathology, Medical University of Graz, Auenbruggerplatz 25, A-8036 Graz, Austria. e-mail: firstname.lastname@example.org
Bilateral Sertoli-Leydig cell tumors (SLCTs) of the ovary, especially in association with a cystadenoma, are exceedingly rare. Some SLCTs, usually of poor differentiation, show heterologous elements. We present a case of a 61-year-old woman with bilateral well-differentiated SLCTs in which the Sertoli-Leydig cell component showed leiomyogenic (heterologous) differentiation. Furthermore, on the left side it also was associated with a serous cystadenoma.
Sertoli-Leydig cell tumors (SLCTs) of the ovary belong to the category of sex cord-stromal neoplasms and account for less than 0.5% of all ovarian tumors (1–3). Bilateral SLCTs of the ovary are exceedingly rare. The average patient age of unilateral SLCTs is 25 years; 75% of the patients are 30 years of age or younger and only 10% are over 50 years of age. SLCTs are separated into well, intermediately, and poorly differentiated tumors depending on cytologic atypicality, mitotic activity, presence or absence of tubular or solid structures, and presence or absence of a sarcomatoid growth pattern. Heterologous elements including chondroid, leiomyogenic, rhabdomyogenic, gastrointestinal type, or carcinoid differentiation occur in approximately 20% of SLCTs, most of which are otherwise of intermediate or poor differentiation (3,4). We present here a rare and quite unusual case of a well-differentiated bilateral SLCT associated with a unilateral serous cystadenoma in combination with a heterologous, leiomyogenic differentiation.
A 61-year-old white woman who had had primary amenorrhoea due to aplasia of the uterus presented at the Department of Gynecology and Obstetrics, Medical University of Graz, with an increase of her abdominal girth and intermittent urinary outflow obstruction. No signs of virilization were noted. Routine vaginal ultrasound showed a multiloculated cystic ovarian tumor on the left side and a solid-cystic ovarian tumor on the right side. Bilateral oophorectomy was performed and the left ovary was sent for frozen section analysis. Macroscopically, the left ovary measured 27×16×7 cm, had a weight of 1965 g, and showed multiple cysts with numerous grayish-to-yellowish solid nodules measuring up to 1 cm. The frozen section showed a serous cystadenoma/adenofibroma. The right ovary in which no frozen section was obtained measured 4×2.5×2 cm and showed on cut surface a 2.5×1.5×0.5 cm brown to gray solid cystic tumor. The tissue was processed and stained (hematoxylin and eosin) according to standard protocols. The examination of the formalin-fixed, paraffin-embedded tissue of the left ovary displayed a serous cystadenoma. In addition, a well-differentiated SLCT was identified showing irregular infiltration of the cystic wall of the serous adenoma (Figs. 1A–E). The tumor cells lacked cytologic atypia and mitotic activity. To confirm the sex cord-stromal component, immunohistochemical staining with an antibody against α-inhibin (Serotec, dilution 1:20) was performed. Immunohistochemistry showed intense and diffuse positivity for α-inhibin (Fig. 1F). Within the well-differentiated sex cord-stromal tumor, there were mesenchymal areas showing fascicular arrangement of spindle cells with eosinophilic, fibrillary cytoplasm (Fig. 1G). These mesenchymal areas did not show any cytologic atypia, increased mitotic activity, or tumor necrosis. These heterologous areas were immunoreactive for smooth muscle actin (SMA1A4, Sigma, dilution 1:5000) (Fig. 1H).
The histologic examination of the right ovary showed a well-differentiated SLCT without any heterologous differentiation. In contrast to the left ovary, the right ovary was not associated with a serous cystic tumor. The cystic component represented a benign inclusion of surface (serous) epithelium.
The patient had an unremarkable postoperative recovery; regular follow-up examinations have been insuspect until now.
This case constitutes a unique example of SLCTs of the ovary. The very unusual and unique features of an SLCT in this case are: (a) bilaterality of the tumor, (b) unilateral association with a serous cystadenoma, and (c) heterologous, leiomyogenic differentiation in association with a well-differentiated SLCT.
To our knowledge, this is the first reported case of a bilateral well-differentiated SLCT of the ovary in combination with a unilateral serous cystadenoma and unilateral leiomyogenic differentiation. It is of note that heterologous elements occur in approximately 20% of SLCTs, most of which are otherwise of intermediate differentiation, but some of which are poorly differentiated or retiform (4). The occurrence of heterologous elements in an otherwise well-differentiated SLCT, as observed in our case, is an exceedingly rare phenomenon.
It is well known that the prognosis in patients with SLCT is closely related to its differentiation and stage. In 1 large study, none of the well-differentiated tumors, 11% of those with intermediate differentiation, 59% of the poorly differentiated tumors, and 19% of those with heterologous elements were clinically malignant (2).
Usually, well-differentiated SLCTs of the ovary show closely packed neoplastic tubular structures and commonly have a lobulated and well-described tumor border. However, they may also show a truly infiltrating pattern (5). In this regard, an infiltrating growth pattern alone should not be considered as a sign (or diagnostic criterion) of malignancy. Moreover, an infiltrating growth pattern alone should not be used as a criterion for grading of a SLCT.
Although the SLCT in this case showed areas of heterologous, leiomyogenic differentiation, we expect a clinically benign course of this tumor because of its well differentiation. The unusual histomorphologic features of this case and its bilateral occurrence, however, warrant a closer and longer follow-up of the patient.
This case shows that heterologous elements may also be encountered in well-differentiated SLCTs. Principally, the prognosis of a well-differentiated SLCT is good with a 5-year survival of almost 100% (2).
The coexistence of a serous cystadenoma and a well-differentiated SLCT in 1 ovary in this case may raise the possibility of whether these 2 tumors were related or even perhaps, whether the cystic serous tumor might represent an epithelial differentiation of an otherwise sex cord-stromal tumor of the involved ovary. Although this issue remains highly speculative and can hardly be proven or disproven by the current methods, we think that these 2 tumors in 1 ovary are most likely not related to each other and probably represent independent neoplastic ovarian lesions. To the best of our knowledge, this is the first described case of an SLCT of the ovary occurring in a serous cystadenoma. Leiomyogenic differentiation and bilaterality are further unique features of this case.
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5. Crum CP, Lee KR. Diagnostic gynecologic and obstetric pathology. Elsevier Saunders 2006:963
Sertoli-Leydig cell tumor; Ovary; Sex cord-stromal tumor; Heterologous elements; Bilaterality; Serous cystadenoma