Diffuse Glioma of the Brain in von Recklinghausen's Disease

Abstract
A case of von Recklinghausen''s disease in a 12-year-old girl with diffuse cerebral gliomatosis is reported. The neoplasm is similar to an astrocytoma but can not be definitely so interpreted because it also contains numerous transitional elements which resemble cells of Schwann, oligoden-droblasts and astroblasts. The neoplastic tissue is well preserved, the configuration of the brain not distorted, and edema and necrosis common in astrocytoma are absent. The findings suggest that the neoplastic cells have pluripotentiality and that the growth is related to central Schwannoma, oligodendroblastoma and astrocytoma. In a review of the literature the authors found only 15 cases of von Recklinghausen''s disease with pathology in the central nervous system. These changes can be classified into 2 main groups: extensive diffuse gliosis of the brain and discrete tumors. The 1st group comprises 7 previously reported cases and in addition the 1 presented here; 8 cases reported in the literature belong to the 2d group and are not pertinent to the present discussion.

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