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)

Parachordoma is a rare soft-tissue tumor resembling extraskeletal myxoid chondrosarcoma and chordoma. Because fewer than 30 cases have been reported and precisely characterized, we studied the clinicopathologic features of seven new cases, comparing the immunohistochemical (six cases) and cytogenetic (one case) profiles with 15 cases of chordoma and six cases of extraskeletal myxoid chondrosarcoma. Patients with these tumors ranged in age from 7 to 62 years (mean, 35 years) and included four women and three men. The tumors presented as subfascial masses of the thigh (two cases), arm (three cases), chest wall (one cases), and buttocks (one case). In six of seven cases, there was neither recurrence nor metastasis within the follow-up, which ranged from 4 months to 7 years. The tumors were composed of vague nodules of large, rounded eosinophilic cells embedded in a matrix that varied from myxoid to densely hyaline, and the latter areas occasionally resembled primitive cartilage. Transitions between the large eosinophilic cells and smaller rounded and shorter spindled ones were often noted. Multivacuolated (physaliferouslike) cells were noted in all cases but were usually few in number. The matrix stained with Alcian blue (pH 2.5), and this staining was abolished with hyaluronidase predigestion. Immunohistochemistry for a variety of cytokeratins (CKs) (8/18, 1/10, 7, and 20), epithelial membrane antigen (EMA), S-100 protein, vimentin CD34, type IV collagen, smooth muscle actin, smooth muscle myosin heavy chain, calponin, and glial fibrillary acid protein was performed. All parachordomas strongly expressed CK 8/18, but not the other cytokeratins. Additionally, they expressed EMA (five of six). S-100 protein (six of six), and vimentin (six of six) and had a linear pattern of type IV collagen immunoreactivity around nests of cells (four of five). Calponin was noted in one case, but no cases expressed smooth muscle actin, smooth muscle myosin heavy chain, or glial fibrillary acid protein. In contrast, chordoma expressed CK 8/18 (15 of 15) and CK 1/10 (14 of 15), whereas extraskeletal myxoid chondrosarcoma consistently lacked CK. Although chordoma and extraskeletal myxoid chondrosarcoma showed considerable overlap with parachordoma, with respect to EMA and S-100 protein, they infrequently displayed type IV collagen, as was seen in parachordoma. One case of parachordoma studied cytogenetically disclosed trisomy 15, and monosomies of 1, 16, and 17 in contrast to the t(9;22) reported in extraskeletal myxoid chondrosarcoma and the monosomies of 3, 4, 10, and 13 seen in chordoma. We conclude that the immunohistochemical and cytogenetic profile distinguishes parachordoma from extraskeletal myxoid chondrosarcoma and chordoma. Lack of myoepithelial markers, furthermore, suggests parachordoma is not a deeply situated adnexal tumor. Because of these differences, parachordoma is best regarded as a distinct lesion without a clear relationship to other well-characterized tumors.
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PMID:Parachordoma is immunohistochemically and cytogenetically distinct from axial chordoma and extraskeletal myxoid chondrosarcoma. 1047 65

A 49-year-old woman, with a medical history of rheumatism, was admitted to our hospital with chief complaints of bilateral enlargement and redness of breasts. She underwent weekly breast examinations. Mammography findings were reported as category 3 for both breasts. Breast ultrasonography, magnetic resonance imaging, and chest contrast computed tomography revealed a massive tumor in the left BD region, however, there were no findings for suspected malignancy. Needle biopsy did not yield histologically malignant cells in both breasts. Mammary interstitium was edematous, and capillary-like slit structures were observed. The stroma stained with alcian blue and destained with hyaluronidase treatment. Since the stroma tested positive for vimentin, calponin, and CD34 and negative for CD31, the patient was diagnosed as (PASH). Because both breasts had similar diagnosis based on histopathologic findings, bilateral mastectomy was performed. Details about the origin of bilateral PASH are unknown but it may be related to the development of rheumatoid arthritis. Additionally, systemic autoimmune diseases like rheumatism may be the reason for repeated contraction and enlargement of PASH.
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PMID:Rare Finding of Bilateral Pseudoangiomatous Stromal Hyperplasia of the Breast: A Case Report. 3176 94