Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.2.1.36 (hyaluronidase)
 4,606 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A series of 46 malignant schwannomas occurring in soft parts of patients having von Recklinghausen's neurofibromatosis was analyzed. The diagnosis of malignant schwannoma was based upon the occurrence of malignant spindled cells closely resembling Schwann cells in the neoplasm and the close association or origin of the malignant schwannoma in a neurofibroma (27 tumors), or a large peripheral nerve (31 tumors). Additional histologic features useful in making the diagnosis of malignant schwannoma included the arrangement of the spindled tumor cells in a whorled pattern about thin-walled, gaping blood vessels, perivascular cellular proliferation and the presence of prominent myxoid stroma containing abundant hyaluronidase-sensitive acid mucopolysaccharides. Nuclear palisading was present in only one case. Eight tumors containing both neoplastic Schwann cells and rhabdomyoblasts and five containing both neoplastic Schwann cells and rhabdomyoblasts (malignant "Triton" tumors) and five containing foci of malignant cartilage cells were included in the series. The neoplasms occurred principally in adults (median age, 34 years) and were most common in the lower extremity (18 cases) and retroperitoneum (11 cases). A mass with or without pain was the most common presenting symptom (28 cases). The median size of excised tumors was 11 cm. The malignant schwannomas were highly malignant neoplasms, causing the death of 39 patients within five years and two patients within 6--10 years after diagnosis. Only four patients were alive and free of tumor 5--15 years after diagnosis.
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PMID:Malignant Schwannoma associated with von Recklinghausen's neurofibromatosis. 15 12

Hyaluronidase, an enzyme which depolymerizes the mucopolysaccharide hyaluronic acid, appears to be tolerated by the human central nervous system and in the anterior chamber of the rabbit eye. Two patients with hydrocephalus and meningomyelocele had their condition curtailed by intraventricular injections of hyaluronidase, and in a third patient its use permitted delay of shunting. It was apparently effective in preventing a reaccumulation of cystic fluid in an intramedullary neurofibroma, and in reversing adverse effects of adhesive arachnoiditis of the spinal cord. Hylauronidase seems worthy of further investigation in disorders of the central nervous system.
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PMID:Use of hyaluronidase in the central nervous system. 45 58

Four cases of uncommon soft tissue tumors were investigated histopathologically. All of them consisted of fibrous and myxoid components, and mature bone showed shell-like characteristics. Histological features revealed these tumors were well circumscribed by a thick collagenous fibrous capsule and composed of uniform-sized fusiform cells with eosinophilic cytoplasm and a round or oval nucleus in the myxoid matrix. An incomplete shell of mature bone with lamellar structure was also observed at the periphery. Immunohistochemical and ultrastructural studies were performed. The major component of the proliferating cells in the tumors had positive staining for vimentin, S-100 protein, neuron-specific enolase and synaptophysin. The myxoid matrix was stained by alcian blue and was digested completely by pretreatment with hyaluronidase. Electron microscopy showed the cytoplasm contained dense-core granules measuring 100-200 nm and abundant filaments of an intermediate size. It is suggested that these uncommon tumors might be diagnosed as the 'ossifying fibromyxoid tumor of soft parts' previously described by Enzinger et al., which were derived from peripheral nerve sheath tumors such as neurofibroma and myxoid neurofibroma.
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PMID:Ossifying fibromyxoid tumor of soft parts: clinicopathologic, immunohistochemical and ultrastructural study of four cases. 1050 43