Kelessis N G, Apostolikas N N, Filopoulos E J, Griniatsos J K, Vassilopoulos P P
Surgical Department, Hellenic Anticancer Institute, Saint-Savvas Hospital, Athens, Greece.
Anticancer Res. 1996 Sep-Oct;16(5B):3193-5.
Peripheral neuroepitheliomas are rare malignant tumors of presumed neural crest origin, arising outside the central and sympathetic nervous system. We present herein a case of a young man with diffuse peripheral neuroepithelioma of the abdominal cavity. Although we twice attempted a debulking procedure on this patient, and treated him with alternating VIP-CAV combined chemotherapy, his disease showed a relentless course without responding to any treatment. The development of peripheral neuroepithelioma in the abdominal cavity is an extremely rare occurrence. These rare malignant tumors, of presumed neural crest origin, arise outside the central and sympathetic nervous systems (1). Other names for this tumor are: extracranial primitive neuroectodermal tumor (PNET), Askin tumor, peripheral or adult neuroblastoma (2,3,4). We present a case of a young man with diffuse development of this tumor in the abdominal cavity.
外周神经上皮瘤是一种罕见的恶性肿瘤，推测起源于神经嵴，发生于中枢神经系统和交感神经系统之外。我们在此报告一例患有弥漫性腹腔外周神经上皮瘤的年轻男性病例。尽管我们对该患者进行了两次减瘤手术，并采用VIP-CAV交替联合化疗进行治疗，但他的病情仍持续进展，对任何治疗均无反应。腹腔内发生外周神经上皮瘤极为罕见。这些罕见的恶性肿瘤推测起源于神经嵴，发生于中枢神经系统和交感神经系统之外（1）。该肿瘤的其他名称有：颅外原始神经外胚层肿瘤（PNET）、阿斯金瘤、外周或成人神经母细胞瘤（2,3,4）。我们报告一例年轻男性腹腔内弥漫性发生这种肿瘤的病例。
