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)

Four cases are described of a distinctive morphologic variant of thymic carcinoid that was characterized by abundant stromal mucin admixed with the neuroendocrine elements resulting in a histologic picture reminiscent of metastatic mucin-secreting carcinoma. The patients were three men and a woman, aged 22 to 43 years. The tumors presented with symptoms of chest discomfort, cough, and dyspnea and were described as large anterior mediastinal masses on chest radiographs and computerized scans. Histologically, all cases showed nests and strands of tumor cells embedded in an abundant lightly eosinophilic, mucinous stroma with small cellular clusters as well as scattered single tumor cells seen floating in the mucin. The mucinous matrix was negative for periodic acid Schiff's and mucicarmine stains; alcian blue stains at pH 2.5 showed strong positivity of the mucinous material; this reaction was abolished by treatment with hyaluronidase, indicating the presence of nonepithelial stromal mucosubstances. Immunohistochemical stains showed strong positivity of the tumor cells with CAM 5.2, chromogranin, synaptophysin, and neuron-specific enolase, and negative staining with carcinoembyronic antigen and epithelial membrane antigen. Electron microscopy done in one case showed abundant dense-core cytoplasmic neurosecretory granules; there was no evidence of glandular secretory activity by the tumor cells. The tumors in two patients behaved in a highly aggressive fashion, with invasion of the chest wall, recurrence, and metastases to the lungs, pleura, and axillary, retroperitoneal, and mesenteric lymph nodes. Thymic carcinoid should be considered in the differential diagnosis of mediastinal neoplasms displaying prominent mucinous features. Application of immunostains and electron microscopy will be of value for establishing the correct diagnosis in this setting.
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PMID:Thymic carcinoid with prominent mucinous stroma. Report of a distinctive morphologic variant of thymic neuroendocrine neoplasm. 757 90

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

We describe a case of extraskeletal myxoid chondrosarcoma with neuroendocrine differentiation. The tumor occurred in subcutaneous tissue of the right popliteal region in a 50-year-old man. It measured 5 cm in diameter, was well circumscribed, lobular and gelatinous, and lacked any necrosis or hemorrhage. Histologically, the tumor structure was a typical of extraskeletal myxoid chondrosarcoma. The lesion was lobulated and contained small to medium-sized chondroblast-like cells with ovoid hyperchromatic nuclei and without prominent nucleoli. The cells created cords and nests and showed focally a perivascular rosette-like arrangement. A few of the tumor cells were spindle shaped. The myxoid matrix was stained with alcian blue and this reaction was resistant to prior treatment with hyaluronidase. PAS-positive glycogen was found in the cytoplasm of some tumor cells. Immunohistochemically, the tumor cells were diffusely positive for neuron specific enolase, monoclonal synaptophysin and vimentin. Following antibodies gave negative results: desmin, actins, S-100 protein, pancytokeratin, epithelial membrane antigen, chromogranin A, neurofilament protein, myelinic basic protein, glial fibrillary acidic protein. The patient is well four years after the wide excision of tumor and radiotherapy. Neuroendocrine differentiation in extraskeletal myxoid chondrosarcoma was described at first by Chhieng et al. in 1998 (1). Our observation confirms this interesting finding.
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PMID:[Extraskeletal myxoid chondrosarcoma with neuroendocrine differentiation]. 1103 63