[Long-term survival case of primitive neuroectodermal tumor in the chest wall of a young adult]

A very rare case of Askin's tumor is described. A 22-year-old male was admitted to our hospital because of an abnormal shadow on chest roentgenogram. His chest X-ray film showed a tumor shadow (4.0×5.0 cm) of chest wall protruding into the upper right lung field. The tumor shadow had markedly enlarged in size compared to previous examinations of chest X-ray film 1 month before. Chest computed tomography(CT) and magnetic resonance imaging( MRI) revealed an heterogenous tumor fixed on the right 4th rib, which did not infiltrate the surrounding tissues. In the examination of bone scintigram, no abnormal accumulation was seen at the site of the chest wall tumor. Because of the possibility of malignancy, an en bloc excision of the tumor was performed including the surrounding pleura. The histologic examination revealed primitive neuroectodermal tumors of the chest wall (Askin's tumor).The patient underwent both additional adjuvant chemotherapy and radiation therapy postoperatively, being alive and well without any sign of recurrence 10 years after the operation. Askin's tumor is a very rare disease with an extremely poor prognosis, however, the effective treatment has not been established yet. Complete excision at initial operation seemed to be correlated with long-term survival in this case.