| Abstract|| |
Sertoli-Leydig Cell Tumors (SLCT) are very rare neoplasms of the ovary (0.2%) and they belong to the group of sex cord-stromal tumors. Of these, 20% of the cases show heterologous elements. We report a case of a 22-year-old woman who presented with complaints of lower abdominal pain and secondary amenorrhea for 10 months. Physical examination revealed right lower abdominal tenderness and fullness. Imaging showed a right ovarian mass. She underwent right salpingo-oophorectomy with bilateral pelvic lymphadenectomy and omentectomy. Microscopic examination revealed a neoplasm with varied histomorphological patterns. The predominant pattern was an atypical proliferative mucinous tumor with foci of microinvasion. The other component was that of moderately differentiated Sertoli-Leydig Cell Tumor. Focal areas resembling carcinoid were also noted. Immunohistochemistry was performed and the Sertoli-Leydig Cells were positive for CD56, calretinin, inhibin, vimentin, and ER. The glandular component was positive for CK20, EMA, CEA, and CDX2. Synaptophysin and chromogranin were positive within nests resembling carcinoid. With the given histomorphological features and immunohistochemistry findings, a diagnosis of moderately differentiated Sertoli-Leydig Cell Tumor of the ovary with associated mucinous carcinoma and carcinoid was rendered. The presence of heterologous elements in SLCTs has been reported to be associated with poor prognosis.
Keywords: Carcinoid tumor, leydig cells, mucinous adenocarcinoma, sertoli cells, sex cord-gonadal stromal tumors
|How to cite this article:|
Ravichandaran A, Lakshmanan A, Kurian A, Prasad S. Moderately differentiated Sertoli-Leydig cell tumor of ovary with associated mucinous carcinoma and carcinoid—A case report and review of literature. Indian J Pathol Microbiol 2021;64:528-31
|How to cite this URL:|
Ravichandaran A, Lakshmanan A, Kurian A, Prasad S. Moderately differentiated Sertoli-Leydig cell tumor of ovary with associated mucinous carcinoma and carcinoid—A case report and review of literature. Indian J Pathol Microbiol [serial online] 2021 [cited 2022 Dec 6];64:528-31. Available from: https://www.ijpmonline.org/text.asp?2021/64/3/528/322410
| Background|| |
Sertoli-Leydig Cell Tumors (SLCT) are one of the rarest ovarian tumors accounting for 0.5% of the ovarian neoplasms. SLCTs are classified into well-differentiated which are essentially benign, whereas poorly differentiated tumors have a malignant behavior. A proportion of moderately differentiated tumors behave in a malignant fashion. The presence of heterologous elements and the retiform pattern is associated with a worse prognosis.,,
We report a case of ovarian moderately differentiated SLCT with associated atypical proliferative mucinous tumor (APMT) with multiple foci of microinvasive adenocarcinoma, intestinal type, and focal carcinoid.
| Case Report|| |
The patient is a 22-year-old unmarried woman with complaints of pain abdomen. She had a history of amenorrhea for nearly 10 months. She is also a known case of hypothyroidism on regular treatment. Physical examination showed right lower abdominal tenderness and fullness. Further workup with ultrasonography and CT abdomen demonstrated right ovarian mass. She underwent right salpingo-oophorectomy with bilateral pelvic lymphadenectomy and omentectomy. The right ovary measured 10 × 9 × 6 cm with smooth intact capsule. Cut surface was predominantly solid, gray-white, and nodular with many scattered cystic spaces ranging from 0.1 to 1.5 cm. Some cysts were filled with firm gelatinous material. The microscopic examination of the ovarian mass revealed a neoplasm with varied histomorphological patterns. The predominant pattern was composed of tubular glands of varying sizes some of which were cystically dilated. The lining epithelium was mucinous with scattered goblet cells. The epithelial cells showed moderate nuclear atypia. There were multiple tiny foci of invasion in the form of small nests and singly scattered atypical cells invading the stroma. The maximum invasive focus measured 0.3 cm. There were ill-defined lobules, nests, and trabeculae of Sertoli cells and aggregates of Leydig cells in a fibro cellular stroma. Focal areas also showed small nests of a monomorphic population of cells resembling carcinoid. Immunohistochemistry was performed and the Sertoli and Leydig cells were positive for CD56, calretinin, inhibin, vimentin, and ER. The glandular component was positive for CK20, EMA, CEA, and CDX2. PAX8 was negative in both components. Synaptophysin and chromogranin were positive within the small cell nests resembling carcinoid. Hence a diagnosis of Sertoli-Leydig Cell Tumor of the ovary with associated mucinous carcinoma and carcinoid was rendered. The case was discussed in a multi-disciplinary team meeting. As the patient is young with stage I disease, it was decided not to give any adjuvant treatment. She is disease-free as on one-year follow-up.
| Discussion|| |
Sertoli-Leydig Cell Tumor constitutes 0.5% of ovarian neoplasms. It is most commonly seen in the age group of 20–25 years. It presents with signs of hirsutism or virilization, rarely estrogenic manifestation, pelvic or abdominal mass. SLCTs are categorized into 3 subtypes such as well-differentiated, moderately differentiated, and poorly differentiated. Microscopically, well-differentiated SLCTs have hollow or solid tubules which are usually small and round to oval, lined by stratified cells rarely resembling glands of endometrioid tumors. Sertoli cells show no atypia and minimal mitosis. Only a minor component of Leydig cells is present in the fibromatous stroma. Moderately differentiated SLCTs are lobulated with hypercellular areas and hypocellular edematous stroma. The cellular areas show an admixture of dark blue Sertoli cells in nests, cords, and trabecular pattern and Leydig cells with eosinophilic to pale vacuolated cytoplasm at the periphery of the lobules. Cells may show a bizarre nucleus. Poorly differentiated SLCTs resemble sarcoma or poorly differentiated carcinoma with a minor component of tubules, sex cords, and Leydig cells.
Heterologous elements are seen in more than 20% of cases, most often with SLCT of moderate differentiation. Heterologous elements could be endodermal or mesodermal in origin. The most common element is gastrointestinal type epithelium with scattered goblet cells and argentaffin cells, occasionally showing nuclear pseudo-stratification and cytologic atypia. Other elements include immature skeletal muscle, cartilage, hepatocytes, neuroendocrine, and neuroectodermal elements., Young et al. in his study on SLCTs with endodermal elements described 2 cases similar to our case with atypical proliferative mucinous epithelium with foci of microinvasion. Rarely SLCT with heterologous elements have been reported where the endodermal elements were predominant, thereby mimicking a surface epithelial neoplasm. However thorough sampling would help us in arriving at the correct diagnosis.
The important prognostic factors are grade, stage, and presence of heterologous elements. The prognosis of SLCTs is generally favorable and is significantly affected by the grade. Well-differentiated tumors have an excellent prognosis. Poorly differentiated tumor and those with retiform pattern have an adverse prognosis. Moderately differentiated SLCTs are predominantly benign and a small proportion (approximately 10%) behave in a malignant fashion. The presence of heterologous elements especially mesenchymal components is associated with poor outcomes.,, Young RH et al. in their study on ovarian SLCTs found that all the well-differentiated tumors were benign, but 11% of moderately differentiated, 59% of poorly differentiated tumors, and 19% of those with heterologous elements were malignant. Our patient is disease-free as on one year follow up which might be explained by the predominance of the atypical proliferative mucinous tumor with foci of microinvasion. Most of the published literature state that the overall survival of APMTs with microinvasion is similar to that of APMTs without invasion., Germline and somatic DICER1 mutations have been reported in SLCTs of the ovary with varying frequencies. Higher frequencies have been reported to be associated with moderate and poorly differentiated SLCTs. The SLCT with DICER1 mutations carries a favorable prognosis with prolonged overall survival.,The histomorphologic findings are summarized in [Figure 1] and the immunohistochemical findings are summarized in [Figure 2] and [Figure 3].
|Figure 1: (a) (H and E-100X) and (b) (H and E-400X): Closely packed glands of varying sizes lined by intestinal-type epithelium. (c) (H and E-400X): Foci of invasive carcinoma. (d) (H and E-100X): Sertoli Leydig component. (e) (H and E-400X): Leydig cells are closely admixed with mucinous glands. (f) (H and E-400X): Carcinoid like areas|
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|Figure 2: 400X-The mucinous component is positive for CEA and CDX2. The Sertoli-Leydig cell component is positive for calretinin, Inhibin, and CD56|
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|Figure 3: The carcinoid component is positive for Synaptophysin (a-400X) and Chromogranin (b-400X)|
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| Conclusion|| |
The presence of heterologous elements in SLCTs has been reported to be associated with poor prognosis. The small number of cases that have been reported in the literature precludes us from reaching far-fetching conclusions regarding prognostic implications. This case emphasizes the wide morphologic spectrum of SLCTs, thereby avoiding misdiagnosing these neoplasms.
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There are no conflicts of interest.
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Department of Pathology, Apollo Hospitals, Chennai, Tamil Nadu
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3]