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Tumor de células de la granulosa juvenil del ovario asociado a síndrome de Maffucci

2013; Elsevier BV; Volume: 12; Issue: 03 Linguagem: Inglês

2565-005X

Yanet Jaimes-García, Gabriela Hernández-Pliego, Stanislaw Sadowinski‐Pine, Miguel Ángel Palomo-Collí, Luis Juárez‐Villegas, Jorge Cortés-Sauza,

Sarcoma Diagnosis and Treatment

Maffucci syndrome (MS) is a non-hereditary condition that has been associated with mesenchymal neoplasms and sex cord-stromal tumors. Because of estradiol production, up to 80% of cases have precocious puberty. About 20% of these patients develop tumors, particularly malignant, among which are: astrocytomas, pituitary adenomas, granulosa cell tumors and pancreatic adenocarcinoma. Enchondromas and hemangiomas may present malignant transformation to chondrosarcoma and hemangiosarcomas. Ovarian granulosa cell tumors are rare, the juvenile form is diagnosed in patients less than 30 years of age, they present with abdominal masses and precocious puberty. The prognosis is favorable and surgery is the treatment of choice. A 13 year-old girl, with a history of MS. During medical follow-up precocious puberty was detected and the hormonal profile detected estradiol elevation. Abdominal tomography showed an ovarian tumor. The tumor was completely resected and histology established the diagnosis of granulosa cell tumor, which was classified as stage IA. Mafucci syndrome is a rare condition and so are granulosa cell tumors. This case illustrates the importance of performing a comprehensive clinical examination in MS patients to identify not only the malignant transformation of enchondromas and hemangiomas, but also other tumors, since early diagnosis is essential for the outcome. Documento descargado de http://www.elsevier.es el 09/03/2016. Copia para uso personal, se prohibe la transmision de este documento por cualquier medio o formato.