Malignant schwannoma arising in patients with von Recklinghausen's disease: report of two cases and the comparison of mast cells between benign and malignant portions.

We described two cases of malignant schwannoma arising in patients with von Recklinghausen's disease and examined the mast cells infiltrated into histologic sections. One of the two cases histologically revealed apparent mast cell infiltration in some areas of malignant schwannoma as well as in the benign neurofibroma. The malignant lesion demonstrated significantly increased percentages of degranulated mast cells over the benign lesion using FITC-avidin staining. In an electron microscopic study, mast cells in the malignant lesion displayed empty granules, piecemeal degranulation, and canaliculi structures suggesting activation. These findings were not observed in the benign lesion. The other patient histologically showed no mast cells in the malignant lesion, although the benign neurofibroma in the patient disclosed numerous mast cells. The first patient had neither recurrence nor distant metastasis. On the other hand, the second patient without mast cells in the histology had multiple distant metastases.