PMID: 9662863Jul 15, 1998Paper

Primitive neuroectodermal tumors: difficult tumors versus modern oncology

Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
V Martínez IbáñezJ Boix-Ochoa

Abstract

Primitive peripheral neuroectodermal tumours (PNET) are rare masses and form part of the group of round small cell tumours which include a wide range of highly aggressive neoplasias such as Ewing's sarcoma, neuroblastoma, lymphoma and rhabdomyosarcoma. PNET present the same cell line as the tumours presented by F. Askin in 1979, both located in the thoracic-pulmonary region. Of the last 26 thoracic neuroblastomas and 11 mediastinal-thoracic sarcomas treated at our centre, we observed 5 PNET in children with a mean age of 12 years (range: 9-14 years). These patients presented a thoracic mass infiltrating sternum, clavicle, supraspinal muscle or, in two cases, a left lateral or paravertebral intrathoracic mass. The time elapsed between clinical observation and diagnosis was 6 weeks. Diagnosis was established by chest X-Ray, CT, bone scintigraphy, immunocytochemistry and cytology. Aggressive local treatment associated with stage IV SIOP chemotherapy for rhabdomyosarcoma was applied in all cases to prevent metastasis. Of the five PNET treated, one 16-year-old patient died (4 y 5 m post-diagnosis) from bone marrow infiltration which had evolved badly from the beginning. The remaining patients are disease-free. One patient who did no...Continue Reading

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