Introduction Sex cord tumor with annular tubules (SCTAT) represents less than 1% of ovarian neoplasms. It includes two forms; a form associated with Peuz-Jeghers syndrome (PJS) which is usually benign, and the other one, sporadic, with malignant behavior. We aim to study the clinical, radiological, and pathological particularities of SCTAT.

Methods We report four cases of SCTAT treated in Salah Azaiz Institute of Oncology, Tunis, Tunisia between 2000 and 2013.

Results The average age was 30 years, ranging from 4 to 76 years. The age of our patients ranged from 10 to 32 years. One of our patients had a concomitant diagnosis of PJS. The tumor was unilateral in all cases and was revealed by precocious puberty in one case. Gross findings showed a solid tumor, with a yellow cut surface. The size ranged from 0,5 to 28 cm. The morphological features were characteristic but intermediate between granulosa and Sertoli cells. Immunohistochemistry showed that tumor cells expressed inhibin and calretinin. The treatment was surgical, often conservative.

The diagnosis of malignancy wasn’t focused on histological features, but on tumor extension, clinical course, and presence of metastases.

Conclusion SCTAT is a rare tumor, usually benign. The diagnosis is based on histological examination. Malignant potential is noted in sporadic forms. Surgery remains the corner stone of the treatment which is most often conservative, based on oophorectomy.