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肌肉內黏液瘤:病例報告及文獻回顧

Intramuscular Myxoma: Report of a Case and Review of Literatures

摘要


肌肉內黏液瘤是一種少見軟組織腫瘤中之良性病變,臨床診斷及組織形態學必須與其它之黏液性之腫瘤,如黏液性脂肪肉瘤等做鑑別診斷。此類腫瘤外觀呈一界限明顯且介於肌肉組織中之腫塊,其好發於肢體部位,組織形態學上為具有豐富之黏液狀基質及少量之血管變化,夾襯些散在性之星形或梭狀腫瘤細胞。本文報告一19歲未婚女性,因右大腿內側中部有一突出且無壓痛之腫塊,腫塊經外科手術完全切除,病理學檢查及顯微觀察診斷證實為肌肉內黏液瘤,腫瘤之黏液性基質對組織化學染色法中之alcian blue和mucicarmine染色呈陽性反應,但對periodic acid-Schiff's(PAS)與alcian blue with hyaluronidase染色呈陰性反應;散在性之星形細胞對免疫組織化學染色法中之vimentin染色呈強烈陽性反應,而對S-100 protein,cytokeratin(CK),desmin,carcinoembryonic antigen(CEA),epithelial membrane antigen(EMA),alfa-1-antichymotrypsin(AAT),lysozyme染色為陰性反應。依以上之病理學之研究結果顯示,肌肉內黏液瘤可能源自於原始間葉組織中之纖維母細胞(fibroblasts)。故特提出討論及相關文獻之回顧。病人接受腫瘤全摘除術後已逾半年,目前情況良好且無局部復發或轉移之跡象。

並列摘要


Intramuscular myxoma(IMM) has long been considered as an uncommon benign soft tissue tumor. IMM imitates the primitive mesenchyme in both its gross and microscopic appearance. We, herein, present a 19-year-old female with intramuscular myxoma of the right thigh. The roentgenography of the right femoral angiography appeared encasement of femoral artery by a circumscribed soft tissue tumor deeply within muscle without evident bony destruction. A complete workup was performed and followed by the total removal of the tumor. Microscopically, the tumor exhibited the characteristic appearance of the solitary intramuscular myxoma, consisting of a slightly basophilic proteoglycan myxomatous matrix, hypocellular, and, only a few stellate or bipolar cells with oval nuclei and with surrounding compression muscular bundles were presented. There was markedly chondroid and osseous metaplasia within the central location of the tumor. It showed no evidence of typical lipoblast, pleomorphism, celluar atypia or mitotic figure, and no detection of plexiform capillary network such as the myxoid liposarcoma or mitotic figure. Histochemically, the mucoid tumor matrix revealed positive finding for the mucicarmine and alcian blue stains, but negative for PAS stain. Masson trichrome stains displayed an irregular condensation of collagen fibers around the microcystic space and tumor boundary adjacent to the musculature. Silver stains appeared the presence of a loose meshwork of intersparsely reticular fibers. Immunohistochemically, the scattered stellate or spindle cells demonstrated positive immunostaining with the PAP technique for the vimentin stains, but absence of immunoreacting production for the S-100 protein, desmin, cytokeratin, carcinoembryonic antigen and epithelial membrane stains. However, these scattered histiocytes, foam cells showed positive for the alfa-1-antichymotrypsin and lysozyme stains, but were absent in the myxoma cells. We report this additional cases, suggest that is compatible with the primitive mesenchymal cells of fibroblastic-myofibroblastic nature of the myxoma cells. However, the literature about this tumor was also reviewed. After simple wide excision of the tumor, the patient has been closely followed-up for 6 months with no evidence of local recurrence.

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