Malignant small cell tumor of the thoracopulmonary region in childhood.A distinctive clinicopathologic entity of uncertain histogenesis

TLDR

The study reports a distinct malignant small cell tumor of the thoracopulmonary region in 20 children and adolescents, averaging 14.5 years old. The tumor shows a 75 % female predominance, arises in chest wall or peripheral lung soft tissues, recurs locally but rarely metastasizes, has a median survival of 8 months, and electron microscopy hints at neuroepithelial origin though its histogenesis remains unclear.

Abstract

This report describes a unique clinicopathologic entity characterized as a malignant small cell tumor of the thoracopulmonary region in 20 children and adolescents (average age 14.5 years). There was a female predilection (75%) for this tumor which appeared to originate in the soft tissues of the chest wall or the peripheral lung. The neoplasm tended to recur locally and did not seem to disseminate as widely as some of the other small cell tumors of childhood (rhabdomyosarcoma, Ewing's sarcoma, neuroblastoma and malignant lymphoma). However, the median survival was only 8 months. Electron microscopy of 3 cases suggested a neuroepithelial derivation, but, at the present, the histogenesis remains a subject for further investigation.