Neurofibroma, one of the peripheral nerve tumor, is a complex benign tumor arising from skin, deep soft tissue, nerve tissue and bone. The incidence of peripheral nerve tumor is relatively low and it may occur either solitarily or diffusely. Plexiform neurofibroma, a type of neurofibroma, develops characteristically in the patients with von Recklinghausen's disease and its exact incidence is unknown. The case reports of solitary plexiform neurofibroma not associated with von Recklinghausen's disease were extremely rare. We reported a case of solitary plexiform neurofibroma on the median nerve.
Humans ; Incidence ; Median Nerve* ; Nerve Tissue ; Neurofibroma ; Neurofibroma, Plexiform* ; Neurofibromatosis 1 ; Peripheral Nervous System Neoplasms ; Skin

Neurofibromas are benign, slow-growing nerve sheath tumors of the peripheral nervous system, arising from Schwann cells, and classically associated with neurofibromatosis type 1 (Nf1, von Recklinghausen's disease). They occur rarely in the gastrointestinal tract as isolated neoplasms, outside the classical clinical feature of neurofibromatosis. We herein present an isolated colonic neurofibroma without any systemic signs of neurofibromatosis. A 59-year-old female came to our hospital for constipation. On physical examination, general appearance showed no definite skin lesions. A subepithelial tumor measuring 0.8 cm was detected at the distal descending colon on colonoscopy. The lesion was removed completely by endoscopic resection. Microscopic examination showed proliferation of spindle cells in the mucosa and infiltration of inflammatory cells. Immunohistochemical staining was positive for S-100 protein. The above morphological and immunohistochemical characteristics were consistent with a diagnosis of a solitary neurofibroma of the sigmoid colon.
Colon ; Colon, Descending ; Colon, Sigmoid* ; Colonoscopy ; Constipation ; Diagnosis ; Female ; Gastrointestinal Tract ; Humans ; Middle Aged ; Mucous Membrane ; Nerve Sheath Neoplasms ; Neurofibroma* ; Neurofibromatoses ; Neurofibromatosis 1 ; Peripheral Nervous System ; Physical Examination ; S100 Proteins ; Schwann Cells ; Skin

We histopathologically analysed 221 consecutive cases of lid(97 cases ) and orbital(124 cases) tumors from 1992 through 1996 to investigate types and incidence of tumors. In cases of eyelid tumor, cystic tumors accounted for 32 cases (33.0%), basal cell carcinomas 15 cases, nevus 11 cases, sebaceous gland carcinomas 10 cases, cavernous hemangiomas 9 cases, squamous cell carcinomas 8 cases, papilloma 6 cases, pseudotumors 3 cases and lipoma 3 cases. The most common tumors were basal cell carcinoma(24.6%) in adults and cystic tumors(64.4%) in children. In cases of orbital tumor, cystic tumors were the most frequent type of lesions, responsible for 22 of the 124 cases, followed by secondary tumor 16 cases, lacrymal lesions 15 cases, pseudotumors 15 cases, lymphomas 15 cases, hemangioma 11 cases, lipoma 10 cases, peripheral nerve tumors 6 cases, optic nerve gliomas and meningiomas 5 cases, fibromas 5 cases and rhabdomyosarcomas 4 cases. The most common tumors were lacrimal tumors(17.3%) in adults and cystic tumors(50.0%) in children.
Adult ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Child ; Classification* ; Eyelids* ; Fibroma ; Hemangioma ; Hemangioma, Cavernous ; Humans ; Incidence* ; Lipoma ; Lymphoma ; Meningioma ; Nevus ; Optic Nerve Glioma ; Orbit* ; Papilloma ; Peripheral Nervous System Neoplasms ; Rhabdomyosarcoma ; Sebaceous Glands

Malignant peripheral nerve sheath tumor (MPNST) is rare, accounting for 5-10% of all soft tissue sarcomas. MPNST is characteristically aggressive and has a poor prognosis. Fifty percent of patients with MPNST have neurofibromatosis type 1 (NF1). NF-associated MPNST occurs more often at younger ages than sporadic MPNST, but the survival difference is controversial. Superficial MPNST from a recurrent neurofibroma is extremely rare and only a limited number of cases have been reported in the literature. Herein, we report an unusual case of superficial MPNST from a recurrent neurofibroma in a patient without NF1.
Abdominal Wall ; Humans ; Nerve Sheath Neoplasms ; Neurilemmoma ; Neurofibroma ; Neurofibromatoses ; Neurofibromatosis 1 ; Neurofibrosarcoma ; Peripheral Nerves ; Prognosis ; Sarcoma

Malignant peripheral nerve tumors are very rare and derived from the neurofibromatosis in about 13%. There are two classes of malignant tumors in the peripheral nerve, one those of mesoblastic origin, is far most common and made up of malignant neurofibroma and much common fibrosarcoma, and metastasis in 20% of fibrosarcoma, and the other is neuroepithelial origin. These are characterized by increased cellularity, atypical form of cells, mitoses and invasiveness. The choice treatment is radical removal of the tumor, and not affected the prognosis by radiation or chemotherapy. We have been recently experienced of one case of malignant schwannoma on the cervical roots in a case of von Recklinhausen's disease.
Drug Therapy ; Fibrosarcoma ; Mitosis ; Neoplasm Metastasis ; Neurilemmoma* ; Neurofibroma ; Neurofibromatoses ; Peripheral Nerves ; Peripheral Nervous System Neoplasms ; Prognosis

OBJECTIVETo study the clinicopathologic features of lipomatosis of nerve (NLS).

METHODSThe clinical, radiologic and pathologic features were analyzed in 15 cases of NLS.

RESULTSThere were a total of 10 males and 5 females. The age of patients ranged from 4 to 42 years (mean age = 22.4 years). Eleven cases were located in the upper limbs and 4 cases in the lower limbs. The median nerve was the most common involved nerve. The patients typically presented before 30 years of age (often at birth or in early childhood) with a soft and slowly enlarging mass in the limb, with or without accompanying motor and sensory deficits. Some cases also had macrodactyly and carpal tunnel syndrome. MRI showed the presence of fatty tissue between nerve fascicles, resembling coaxial cable in axial plane and assuming a spaghetti-like appearance in coronal plane. On gross examination, the affected nerve was markedly increased in length and diameter. It consisted of a diffusely enlarged greyish-yellow lobulated fusiform beaded mass within the epineural sheath. Histologically, the epineurium was infiltrated by fibrofatty tissue which separated, surrounded and compressed the usually normal-appearing nerve fascicles, resulting in perineural septation of nerve fascicles and microfascicle formation. The infiltration sometimes resulted in concentric arrangement of perineural cells and pseudo-onion bulb-like hypertrophic changes. The perineurial cells might proliferate, with thickening of collagen fibers, degeneration and atrophic changes of nerve bundles. Immunohistochemical study showed that the nerve fibers expressed S-100 protein, neurofilament and CD56 (weak). The endothelial cells and dendritic fibers were highlighted by CD34. The intravascular smooth muscle cells were positive for muscle-specific actin.

CONCLUSIONSNLS is a rare benign soft tissue tumor of peripheral nerve. The MRI findings are characteristic. A definitive diagnosis can be made with histologic examination of tissue biopsy.

Adolescent ; Adult ; Antigens, CD34 ; metabolism ; CD56 Antigen ; metabolism ; Carpal Tunnel Syndrome ; complications ; pathology ; Child ; Child, Preschool ; Diagnosis, Differential ; Extremities ; innervation ; Female ; Hand Deformities, Congenital ; complications ; pathology ; Humans ; Lipoma ; pathology ; Lipomatosis ; complications ; diagnosis ; metabolism ; pathology ; surgery ; Magnetic Resonance Imaging ; Male ; Median Nerve ; metabolism ; pathology ; Nerve Sheath Neoplasms ; pathology ; Neurofibroma ; pathology ; Neurofilament Proteins ; metabolism ; Neuroma ; pathology ; Peripheral Nervous System Diseases ; complications ; diagnosis ; metabolism ; pathology ; surgery ; Peripheral Nervous System Neoplasms ; pathology ; Retrospective Studies ; S100 Proteins ; metabolism ; Vimentin ; metabolism ; Young Adult

A case of macrodactyly associated with neurofiborma of the median nerve, a congenital anomaly of the hand, affecting only one(left middle) finger is reported with a review of the literature. Macrodactyly which is also termed local gigantism, megalodactylism, megalodactylia, or macrodactylism in other literature, is a rare congenital malformation characterized by overgrowth of one or more fingers of hand. Macrodactyly associated with neurofibroma of the median nerve is especially rare. For this reason the following case is presented together with a review of the literature.
Adolescent ; Angiography ; Female ; Fingers/abnormalities* ; Fingers/radiography ; Fingers/surgery ; Human ; Korea ; Median Nerve*/surgery ; Neurofibroma/complications* ; Neurofibroma/pathology ; Neurofibroma/surgery ; Peripheral Nervous System Neoplasms/complications* ; Peripheral Nervous System Neoplasms/pathology ; Peripheral Nervous System Neoplasms/surgery ; Radial Nerve/surgery

PURPOSE: Malignant peripheral nerve sheath tumors most often arise from the anatomically discernible peripheral nerve or neurofibroma. METHODS: A 55-year-old man had a rapidly growing pedunculated large mass on the sacrolumbar junction for 2 years. He has congenital neurofibromatosis type I. He had multiple caf-au-lait spots and multiple neurofibromas on the entire body. The mass developed from a subcutaneous nodule on the sacrolumbar junction and grew rapidly. The preoperative punch biopsy revealed a malignant peripheral nerve tumor. The mass was completely excised with 1 cm free margin above the deep fascial plane. RESULTS: There was no evidence of recurrence of tumor for 19 months of follow-up examination. CONCLUSION: Malignant peripheral nerve sheath tumor is very rare and has unique feature. We report a successful case of malignant peripheral nerve sheath tumor with the review of the literatures.
Biopsy ; Follow-Up Studies ; Humans ; Middle Aged ; Neurilemmoma ; Neurofibroma ; Neurofibromatoses* ; Neurofibromatosis 1 ; Peripheral Nerves* ; Peripheral Nervous System Neoplasms ; Recurrence

Neurofibromatosis is an autosomal dominant hereditary disorder with an overall incidence of one in 3,000~4,000, and type 1 (Von Recklinghausen's neurofibromatosis) characterized by the presence of multiple cutaneous neurofibromas, axillary and groin freckling, and cafe- au-lait spot. The neurofibromatosis type 1 gene is a tumor suppressor gene. Patients with the neurofibromatosis type 1 are at increased risk of developing nervous system neoplasm, including plexiform neurofibromas, optic gliomas, ependymomas, meningiomas, astrocytomas, and pheochromocytomas. Neurofibromas may undergo secondary malignant degeneration and sarcomatous changes. Patients with neurofibromatosis type 1 show a high incidence of Wilm's tumor, rhabdomyosarcoma, nonlymphocytic leukemia, and pheochromocytoma but the gastrointestinal involvement appears to be relatively rare and usually consists of neurofibroma, ganglioneuroma, and leiomyoma. We have identified a case of early gastric adenocarcinoma and intraabdominal schwannoma in a 65-year-old man afflicted with neurofibromatosis type 1.
Adenocarcinoma* ; Aged ; Astrocytoma ; Ependymoma ; Ganglioneuroma ; Genes, Tumor Suppressor ; Groin ; Humans ; Incidence ; Leiomyoma ; Leukemia ; Meningioma ; Nervous System Neoplasms ; Neurilemmoma* ; Neurofibroma ; Neurofibroma, Plexiform ; Neurofibromatoses* ; Neurofibromatosis 1* ; Optic Nerve Glioma ; Pheochromocytoma ; Rhabdomyosarcoma ; Wilms Tumor

The authors report on a case of primary malignant melanoma of the 7th cervical spinal nerve root in a 45-year-old woman. Neuro-radiological features of this extra-dural mass were suggestive of a nerve sheath tumor. The lesion underwent total gross resection through the anterolateral approach. The patient's postoperative course was uneventful. Histopathological investigation confirmed malignant melanoma. There was no evidence of tumor recurrence or other melanotic lesions on regular follow-up examinations until the postoperative eighth month. When treating a common, benign-looking lesion of the cervical spinal nerve root, surgeons should be aware of the potential to encounter such a malignant tumor.
Cervical Vertebrae ; Female ; Human ; Melanoma/*pathology/surgery ; Middle Aged ; Nerve Sheath Tumors/*pathology/surgery ; Peripheral Nervous System Neoplasms/*pathology/surgery ; Spinal Nerve Roots/*pathology