Sex cord stromal tumors of the ovary in children: a clinicopathological report from the Italian TREP project

Background. Ovarian sex-cord stromal tumors (SCST) are rare in childhood and include a variety of neoplasms with different clinical features and biologic behavior. Aim of the analysis was to report the clinical findings and treatment results of a series of patients with SCST of the ovary, registered in a multi-institutional Italian network on rare tumors in children and adolescent between 2000 and 2009. Methods. Data on 23 patients, 5-176 months old, from 13 Centers were reviewed. All patients were grouped on the basis of the results of the first surgical approach, according to the Children Oncology Group staging system. A cisplatin based chemotherapy was recommended in patients with a localized disease, who had undergone an incomplete excision/initial biopsy, and in case of metastatic spread. Results. A frequent symptom was abdominal pain; 9/23 cases had signs of hormonal secretion and two patients were hospitalized for acute pain following ovarian torsion. Twelve patients had a Juvenile-Granulosa Cell tumor, six a Sertoli-Leydig Cell tumor, three a Fibrothecoma, and two a Sclerosing-Stromal tumor. Twenty-one patients maintained the complete remission(follow-up: 9-91 months), 2 with a ST II Sertoli-Leydig Cell tumor relapsed and one of them died. Immonohistochemical studies could be done in 10 cases. Conclusions. Completeness of resection and histology were important prognostic factors; in our series the Sertoli-Leydig Cell tumor was the most aggressive variety. Hormonal signs (precocious puberty, telarca, menarche) were common in younger patients and led to an early diagnosis. Cisplatin based chemotherapy seemed to be effective for locally advanced tumors. Pediatr Blood Cancer 2011;56:1062-1067. (C) 2010 Wiley-Liss, Inc