ABSTRACT
Neurilemmomatosis is a clinical entity clearly distinguished from neurofibromatosis of von Recklinghausen's disease, and characteristically consists of multiple, cutaneuos neurilemmomas, central nervous system tumors, and neurologic disorders. A 52-year-old man, with multiple spinal cord neurilemmomas and unilateral acoutic neurilemmoma, presented with multiple subcutaneous nodules on the flexor parts of the extremities. Histologically, it was diagnosed as neurilemmoma. We report a case of neurilemmomatosis associated with CNS tumors.
Subject(s)
Humans , Middle Aged , Central Nervous System Neoplasms , Extremities , Nervous System Diseases , Neurilemmoma , Neurofibromatoses , Neurofibromatosis 1 , Spinal CordABSTRACT
Plexiform neurilemmoma is a relatively rare, benign peripheral nerve sheath tumor, whieh usually arises in either the dermis or subcutaneous tissue. These tumors may occur singly or as multiple lesions (plexiform neurilemmomatosis), We report an unusual case of plexiform neurilemmomatosis associated with cafe au lait spots reminiscent of neurofibromatosis clinically and another case of plexiform neurilemmoma on the finger. A Biopsy revealed the characteristic palisaded arrangement of spindle cells within well circumscribed elongated nodules, The skin lesions were completely excised without recurrence thereafter.
Subject(s)
Biopsy , Cafe-au-Lait Spots , Dermis , Fingers , Neurilemmoma , Neurofibromatoses , Peripheral Nerves , Recurrence , Skin , Subcutaneous TissueABSTRACT
Neurilemmomatosis is a clinical entity consisting of multiple cutaneous neurilemmomas, central nervous system tumors, and neurologic disorders. Since Shishiba et al first described the disorder in 1984, several cases have been reported. We report a Korean case of neurilemmomatosis showing multiple neurilemmomas of the skin and spinal cord, with associated motor and sonsory nerve disturbance. Histologic, immunohistopathologic and electron microscopic studies confirmed the diagnosis.