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At least 1,387 records · Page 77Linked to original sources
PubMed1994

Neurofibroma of the supraglottic larynx in childhood.

The case of a male child with a benign neurogenic laryngeal tumour caused by von Recklinghausen's disease is presented. At the age of eight years, tracheostomy was necessary. The tumour, deforming the right half of the larynx and extending into the right half of the neck, was removed three years later. Following this procedure, decannulation was possible, and the patient has been free of laryngological complaints for six years. Attention is drawn to the fact that autosomal dominantly inherited neurofibromatosis is a generalized benign tumour with a special form. If it involves any vital structure or forms a functional obstacle, meticulous surgical removal is necessary, and this provides good prospects over a long period.

Child↗
PubMed2004

[A huge malignant degenerating neurofibroma of the lower leg coexistent with type 1 Recklinghausen disease].

HISTORY: A 65-year-old man had noted a tumor of the right lower leg that had progressively grown over the last twenty years. He had a dyspnoea on insignificant movement and was relatively immobile because of the extent of the tumor. The tumor had been diagnosed as a Klippel-Trenaunay syndrome. INVESTIGATIONS: Laboratory investigations suggested a hypochromic and microcytic anaemia and an inflammatory constellation. The tumor had no av-fistulas on ultrasonography and angiography, but there were many pathological arteries and tumor nodes. The tumor nodes had been seen also on MRI-biopsy suggested a neurogenic sarcoma. DIAGNOSIS AND TREATMENT: Amputation of the leg was necessary. The histological diagnosis was neurofibrosarcoma with extensive necrotic areas and Recklinghausen disease. Investigation did not reveal any metastasis. Postoperatively the anaemia regressed and the patient learned to walk with a prosthesis. CONCLUSIONS: This history shows the problem of separating clearly the two hereditary diseases. Often there is a different clinical picture in everyday life. One must consider the definitely higher risk of malignant tumors of patients with neurofibromatosis. Ultrasonography is a non-invasive method that can provide morphological information on soft parts and pathology of the vessels. It facilitates the classification of such.

Aged↗
PubMed1992

Perineurioma: an uncommon soft tissue neoplasm distinct from localized hypertrophic neuropathy and neurofibroma.

Perineurial cells, which normally surround the nerve fascicles within a nerve, can be distinguished from Schwann cells by their immunoreactivity for epithelial membrane antigen (EMA) and lack of reactivity for S-100 protein. We report two cases of perineurioma, a tumor composed exclusively of perineurial cells and distinct from other nerve sheath tumors. The first case involved a deep, soft-tissue mass of the neck, and the second involved a tumor located in the infraclavicular subcutaneous tissue. Both tumors were well circumscribed. Histologically, they were hypocellular and composed of spindle cells possessing elongated nuclei and bipolar, wavy, slender, strikingly elongated cytoplasmic processes, disposed in a background of collagen in the form of short bundles and whorls. In the first case, there were frequent calcospherites and remnants of a small nerve at the periphery. The spindle cells stained for EMA but not S-100 protein, chromogranin, neuron-specific enolase or Leu-7. Ultrastructurally, they possessed long cytoplasmic processes with incomplete basal lamina and occasional pinocytotic vesicles. Axons were not identified. Review of the literature shows that genuine perineuriomas are rare, and most cases reported as such are merely examples of localized hypertrophic neuropathy, a mononeuropathy characterized by fusiform swelling of a nerve, usually in the extremities. The involved segment in localized hypertrophic neuropathy contains distended fascicles composed of whorls of perineurial cells and fibrous tissue entrapping residual axons, probably representing a hyperplastic reaction to nerve damage. The term perineurioma should be reserved for the neoplasm composed only of perineurial cells and presenting as a soft tissue tumor.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1984

Osseous destruction by neurofibroma diagnosed in infancy as "desmoplastic fibroma".

A 2-month-old male infant presented with a lytic lesion of the distal radius which was diagnosed as desmoplastic fibroma. Tumor recurred following en bloc resection of the distal radius and forearm, necessitating an elbow disarticulation. Subsequently, the child developed multiple café-au-lait spots, and histologic sections have been reinterpreted as neurofibromatosis. This case illustrates the potential difficulty in diagnosing neurofibromatosis when few or no classic stigmata of the disease are present.

Adult↗