Plexiform neurofibroma of the retropharyngeal space: a case report.
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Transmission electron microscopy was performed on seven neurofibromata to study the relationship between the frequently occurring mast cells and the other cell types present in the tumour. Intimate association was observed between mast cells and perineurial fibroblasts, but not between mast cells and Schwann cells. At the contact interface between mast cells and perineurial fibroblasts, numerous vesicle-like structures were observed with corresponding endocytotic vesicles in the fibroblast plasma membrane. The authors regard this close morphological relationship as in vivo evidence for a possible role of mast cells in fibroblast metabolism, a conclusion which has already been drawn from in vitro studies, but until now inadequately supported by observations in vivo.
A malignant schwannoma of the median nerve occurred in a 27-year-old woman with multiple neurofibromatosis (von Recklinghausen's disease). A growth had been present at the left wrist since birth and was partially excised at the age of 10 years. The patient refused an amputation and radical local excision did not control this highly malignant tumor.
BACKGROUND: Gastrointestinal involvement in von Recklinghausen's disease (RD) is rare during childhood; its symptoms are late and its prognosis is poor, related to local recurrence and risk of malignant transformation. CASE REPORT: A 13 year-old boy was admitted for hematemesis revealing gastric ulcer. A second episode of hematemesis led to identify a sessile gastric tumor in this patient having numerous skin café-au-lait spots. Recurrent bleeding required laparotomy that showed diffuse infiltration into the anterior gastric wall: histological examination of the excised piece showed characteristic features of neurofibromatosis. The patient was not compliant to the endosonographic survey so that a symptomatic relapse led to total gastrectomy: histological examination did not show malignant transformation. CONCLUSION: Endoscopy is a major tool for identifying gastrointestinal localization of RD but endosonography is necessary to precise the extent of the tumor.
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