[Malignant peripheral neuroectodermal tumors. Histological and immunohistological conditions in 41 cases]

In view of the personal observation that malignant peripheral neuroectodermal tumours (MPNT) can present different histological growth patterns, 41 cases of MPNT were histologically and immunohistochemically studied. The median age of the 41 patients was 15 years (range: 9 months - 23 years). There were 27 males and 14 females. Most tumours (23/41) were located in the thoracopulmonary region. In 31/41 cases there was bone as well as soft tissue involvement. The following histopathological patterns were found: Ewing's sarcoma-like (n = 7), atypical Ewing's sarcoma-like (n = 4), neuroblastoma-like (n = 8), rhabdomyosarcoma-like (n = 8), and hemangiopericytoma-like (n = 1). In 2 cases combined patterns were noted, one tumour being characterized by neuroblastoma-like and Burkitt's lymphoma-like features. Most cases of MPNT differed from the cytological features of typical Ewing's sarcoma in that they contained hyperchromatic nuclei with distinct nucleoli. Some reticulin fibrils were found in between the cells of some cases. Immunohistochemically, 19/23 cases reacted positively to vimentin, 29/32 to neuron specific enolase (NSE), 16/28 to protein S-100, and 1/9 to glial fibrillary acidic protein. 12/24 cases reacted positively to NSE and protein S-100. Neurofilaments and desmin were not found in the formalin fixed material of the present study. The results show that most cases of MPNT can be distinguished from typical Ewing's sarcoma by cytological and histological findings. Differential diagnosis from atypical Ewing's sarcoma, neuroblastoma, and rhabdomyosarcoma is possible by immunohistochemistry.