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In adults, mediastinal neurogenic tumours constitute the third group of mediastinal tumours, after thymomas and lymphomas. If the group of neurogenic tumour is frequent, each type of tumour is relatively unusual in everyday's clinic. Among them, nerve sheath tumours are the more frequent, followed by tumour of the autonomic system. Askin tumour remains uncommon. Treatment of this tumour requires complete preoperative work-up, including standard radiography, CT-scan, MRI, and sometimes nuclear imaging. In most cases, the treatment is based on surgical resection, and may be associated with radiotherapy or chemotherapy in case of malignant tumour or incomplete resection. Better understanding of these tumours, including their molecular abnormalities, may lead to new changes in their classifications, and to their management., (Copyright (c) 2009 Elsevier Masson SAS. All rights reserved.)

Most vasoactive intestinal peptide (VIP)-producing tumours are from epithelial origin. Tumours derived from the sympathetic nervous system can produce VIP as well. We report here the case of a Verner-Morrison syndrome in a 40-year-old woman revealing a metastatic ganglioneuroblastoma. The diarrhea resolved after the resection of primary tumour and liver metastases. Neuroblastic tumours occur extremely rarely in adults. Thus, the management of these tumours is poorly defined in adults.

Peripheral Neuroblastic Tumors are classified according to the recommendations of the INPC into four categories and their subtypes: 1/Neuroblastoma stroma poor, undifferentiated, poorly differentiated, and differentiating, 2/ganglioneuroblastoma stroma composite, nodular, 3/ganglioneuroblastoma stroma composite, intermixed and 4/ganglioneuromas stroma dominant, maturing and mature. The classification is based on age and morphologic features of PNT, including the differentiation grade of the neuroblasts and the mitosis-karyorrhexis index. Histopathological classification has prognostic impact in predicting overall and event-free survival allowing the categorisation of PNT into two groups: favorable subset and unfavorable subset.