Sertoli Leydig Cell Carcinoma

History

9 year old female: Patient presented with vomiting and abdominal pain. On physical examination, the referring physician felt a midline, hard mass.

Findings

Ultrasound

The patient had a large midline mass which extended slightly towards the right. The mass was well defined and measured 13.5 x 9.4cm. It had heterogeneous echotexture with the more anterior portions appearing solid and the more posterior portions being cystic. Vascular flow in the mass was minimal. Imaging of the abdomen showed a normal liver, spleen, and gallbladder. The kidneys were normal size and there was no evidence of hydronephrosis or hydroureter. The pancreas was not visualized. The mass displaced structures leftward and anteriorly and deformed the anterior abdominal wall.

CT

A large, heterogeneous complex mixed solid and cystic mass was identified lying predominately within the right abdomen. Origin from the retroperitoneum or peritoneal cavity could not be determined given the large size of the mass. Contrast enhancement was seen in the margins of the mass as well in some internal solid components. Several medium size vessels were seen to course through the mass. The dimensions were 9.4 x 12.9 x 16cm. There was mass-effect on the IVC, right gonadal vein, adjacent bowel, pancreas, and right ureter resulting in mild right pelvicaliectasis. Multiple small periaortic and retroperitoneal lymph nodes were noted but these were not enlarged by CT size criteria.

Diagnosis

Diagnosis was made after the mass was removed and sent for pathologic inspection. Pathology reported viable tumor sections corresponding to the solid lobulated surfaces comprised of immature and mature sertoli cells lining a network of irregularly branched (retiform) tissue. In the view of the histological features of this rare pediatric ovarian tumor, the slides were sent out for a second consultation. This second evaluation concurred with the diagnosis of sertoli-leydig cell tumor. Additional pathologic specimens of omentum and regional lymph nodes were negative for tumor involvement.

Discussion

A Sertoli-Leydig cell tumor is a rare tumor, also known as an androblastoma or an arrhenoblastoma. It makes up less than 1% of all ovarian neoplasms. It generally occurs in women under 30 years of age; with a mean age of 25. Almost all are unilateral. Malignancy occurs in 10–20% of these tumors. The malignant tumors tend to recur soon after initial diagnosis, with relatively few recurrences after five years.

These tumors are comprised of Sertoli cells, which are normally found in testes, and Leydig cells which secrete testosterone. Therefore, patients can develop signs of virilization. Virilization is the development of male sexual charateristics in a female. These characteristics include, deepening of the voice, growth of facial hair, increase in body hair, swelling of the clitoris, and male pattern baldness. Virilization occurs in approximately 30% of patients with Sertoli-Leydig cell tumors. Loss of feminization is seen clinically before frank virilization occurs. Abnormalities of menstrual cycle or amenhorhea may be presenting symptom. Serum testosterone levels are frequently elevated. These tumors can also be associated with estrogen production.

Sonographically, Sertoli-Leydig cell tumors usually appear as solid, hyoechoic masses. They may mimic the appearance of a granulosa cell tumor: ranging from small solid masses to tumors with variable degrees of hemorrhage or fibrotic changes to multilocular cystic lesions.

Specific Discussion

This patient had an exploratory laparotomy and right salpingo-oophorectomy after the CT scan. She had another CT post-op that showed no residual tumor. She then began chemotherapy and continues to have CT follow-ups. At the 3 month, 6 month and 1 year follow up exams the patient has had no recurrence or metastatic disease.

References

1. Definition of Virilization. 2007 [cited; Available from: 8/18/2008: http://www.nlm.nih.gov/medlineplus/ency/article/002339.htm.
2. Outwater EK, Wagner BJ, Mannion C, et al., Sex cord-stromal and steroid cell tumors of the ovary. Radiographics. 18(6):1523-46, 1998
3. Sachdeva P, Arora R, Dubey C, et al., Sertoli-Leydig cellt umor: a rare ovarian neoplasm. Case report and review of literature. Gynecol Endocrinol. 24(4):230-234, 2008
4. Salem S and Wilson SR, Gynecologic Ultrasound, in Diagnostic Ultrasound, Rumack CM, Wilson SR, and Charboneau JW, Editors. 2005, Elsevier Mosby: St. Louis. p. 570-571.
5. Young RH and Scully RE, Ovarian Sertoli-Leydig cell tumors. A clinicopathological analysis of 207 cases. Am J Surg Pathol. 9(8):543-69, 1985