Year : 2009 | Volume
: 52 | Issue : 1 | Page : 97--99
Bilateral sertoli-leydig cell tumor of the ovary: A rare case report
Kiran Alam1, Veena Maheshwari1, Seema Rashid2, Shruti Bhargava1,
1 Departments of Pathology, Obstetrics, Jawahar Lal Nehru Medical College, Aligarh Muslim University, Aligarh, India
2 Department of Gynecology, Jawahar Lal Nehru Medical College, Aligarh Muslim University, Aligarh, India
2/82, Arya Nagar, Avantika Phase 2, Ramghat Road, Aligarh 202 001, UP
Sertoli leydig cell tumors also known as arrhenoblastoma, are a rare member of the sex cord-stromal tumor group of ovarian and testicular cancers, comprising less than 1% of all ovarian tumors, which occur in young adults and are almost always unilateral. We hereby report a case of a 17-year-old female presenting with a short history of irregular menses and an abdominal lump, which was histologically proven to be a bilateral sertoli leydig cell tumor of the ovary, an exceptionally rare entity in itself.
|How to cite this article:|
Alam K, Maheshwari V, Rashid S, Bhargava S. Bilateral sertoli-leydig cell tumor of the ovary: A rare case report.Indian J Pathol Microbiol 2009;52:97-99
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Alam K, Maheshwari V, Rashid S, Bhargava S. Bilateral sertoli-leydig cell tumor of the ovary: A rare case report. Indian J Pathol Microbiol [serial online] 2009 [cited 2021 Oct 18 ];52:97-99
Available from: https://www.ijpmonline.org/text.asp?2009/52/1/97/44981
Sertoli leydig cell tumors are rare sex cord-stromal neoplasms that account for less than 1% of ovarian tumors, occurring most commonly in young adults. , A majority of the patients present with clinical features of virilization due to excessive secretion of testosterone by the tumor and more rarely estrogenization. However, 50% of the patients may have no endocrine symptomatology and may present with abdominal pain and lump only. , Almost all the cases are unilateral, bilaterality being an exceptionally rare feature of these rare ovarian tumors. ,,, We report here an unusual case of a bilateral sertoli leydig cell ovarian tumor in a 17-year-old female presenting with irregular menses and abdominal lump.
A 17-year-old unmarried female presented in the gynecology department with a 4 month history of irregular menstrual cycle and abdominal distension. Her age at menarche was 12 years old. On abdomen examination, a lump was felt in the right side of the abdomen seemingly arising from the pelvis. There were no clinical features suggesting virilization, namely hirsuitism, hoarseness of voice, acne, clitoromegaly, breast atrophy, temporal hair recession, or increase in musculature. An ultrasonography revealed the presence of bilateral solid ovarian masses with a small cyst. A laparotomy revealed ovarian tumors on both sides. A bilateral salpingo-oopherectomy was performed. Also, an intraoperative peritoneal wash was sent for cytological examination.
The peritoneal washings showed sheets of atypical looking cells that were suggestive of malignancy and a probable diagnosis of metastatic adenocarcinoma was given [Figure 1].
Grossly, the right ovary measured 25x23x8 cm with a smooth external surface and on cut section showed yellowish areas with a small cystic space. The left ovary measured 10x8x3 cm with a similar gross appearance [Figure 2].
Microscopically, the ovaries were replaced by a nodular tumor composed of dual population of cells [Figure 3a]. These consisted of cords of immature round to oval sertoli cells with darkly staining nuclei and islands of pale leydig cells with abundant vacuolated cytoplasm and round nuclei containing prominent nucleoli [Figure 3b]. Mitotic figures were less than 5 per 10 HPF. A focal retiform pattern was also seen [Figure 3c]. Few cystic spaces were seen [Figure 3d]. Periodic Acid Schiff staining was performed, which was positive in cords of sertoli cells [Figure 4]. The overall morphology was suggestive of sertoli leydig cell tumor of intermediate differentiation (Meyer's Type II), confined to the ovary, hence, Stage 1c according to the staging classification of the International Federation of Gynecology and Obstetrics.
Sertoli leydig cell tumors are rare sex cord stromal neoplasms accounting for less than 1% of ovarian tumors  and the majority present at an average age of 25 years.  The tumor is almost always unilateral unlike in our patient in which it was bilateral, which is extremely rare. A total of 40% of the cases present with androgen excess and more rarely estrogenization and 50% have a lump in the abdomen. Our patient was a 17-year-old female with oligomenorrhea and an abdominal lump.
The prognosis of sertoli leydig cell tumors is usually good and correlates with the stage and degree of differentiation. , Our case was Meyer's Type II with intermediate differentiation which accounts for 54% of sertoli leydig cell tumors and was Stage Ic at presentation in keeping with reported series in which more than 90% of sertoli leydig cell tumors are Stage 1 at presentation.  In one series, 11% of intermediate differentiated and 59% of poorly differentiated tumors behaved in a clinically malignant fashion.  Presentation with Stage II or higher disease is also associated with a poor outcome. However, tumors without any apparent poor prognostic factors may behave in an aggressive fashion.  In a series by Zaloudek and Norris, 4 out of 20 poorly differentiated tumors, 1 out of 44 tumors of intermediate differentiation and none of the 7 well-differentiated tumors were malignant. A total of 25% of the Stage I tumors of intermediate differentiation with a retiform pattern were malignant as opposed to 10% of those with no retiform component. 
Recurrence of malignant sertoli leydig cell tumors is relatively early and the recurrent tumor is usually confined to the pelvis and abdomen but distant metastasis to the lung, scalp and supraclavicular lymph nodes have been reported. 
A bilateral salpigo-oopherectomy was done in our patient. However, adjuvant therapy may be advisable for Stage I tumors, which are poorly differentiated, have mesenchymal elements, or are ruptured tumors of intermediate differentiation.  Because in this patient, the tumor is Stage 1 and differentiation appears to be intermediate, the prognosis is expected to be good. However, the patient is being closely monitored.
Cytological diagnosis, especially intraoperative peritoneal washings of sex cord-stromal ovarian tumors, which constitute less than 1% of ovarian malignancies, should always be made with caution. A histopathological examination is mandatory in such cases for a definite diagnosis, more so in a young patient presenting with a lump in the abdomen with irregular menses and the absence of virilizing symptoms.
Dr. Mehar Aziz MD; DNB (Pathology), Senior Professor, Department of Pathology, JNMC, AMU, Aligarh, India.
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