High-Grade Myxoid Spindle Cell Sarcoma of the Pleura

紡錘細胞肉瘤為罕見的軟組織腫瘤，由原始間質細胞產生，並有廣泛而多樣的組織細胞分形。這些肉瘤有著多元的表現與分岐的預後。在紡錘細胞肉瘤中，有一群腫瘤其紡錘細胞包埋在含有豐富黏液性的基質體中，為黏液性紡錘細胞肉瘤，大多發生在周邊四肢，而罕見原發於肋膜。要區分黏液性紡錘細胞肉瘤不同之亞型有相當之困難度。我們在此提出一個以大量肋膜積水表現的原發性肋膜高惡性度黏液性紡錘細胞肉瘤的個案。胸腔鏡下觀察發現在肋膜腔內有大量凝膠狀、肉瘤狀的無定形腫瘤。病理檢查確立為高惡性度黏液性紡錘細胞肉瘤。手術移除只能提供暫時性的緩解，放射線治療並無法遏止腫瘤在手術後的迅速惡化。綜合臨床表現與病理檢查的結果，此肉瘤與目前文獻已報告的亞型均有差異之處。為一獨特的表現。黏液性紡錘細胞肉瘤並不易診斷或分類，我們藉這個特殊的病例將可能鑑別診斷之亞型間的臨床病程、組織型態、免疫染色、預後與治療作一綜論與分析。

關鍵字

黏液性紡錘細胞肉瘤；肋膜腫瘤

並列摘要

Spindle cell sarcomas arise from primitive mesenchymal cells and have a broad spectrum of histological differentiation. They are rare but diversified in presentation, as well as outcomes. A small group of these rare malignant tumors presents spindle cells embedded in an abundant myxoid stromal matrix. The myxoid spindle cell sarcomas mostly involve the extremities, and their occurrence in the pleura is rare. The distinguishing of the various sarcomas within the group of myxoid spindle cell sarcomas is complicated. We report a case of high-grade myxoid spindle cell sarcoma arising from the pleura presenting with massive pleural effusion. Thoracoscopic inspection of the pleural cavity showed a fleshy and gelatinous formless mass. Surgical removal provided only temporary relief. The tumor re-grew rapidly and did not respond to salvage radiotherapy. The histological patterns and the immunohistochemical studies suggested a high-grade myxoid spindle cell sarcoma. However, the features of the tumor presented here were distinct from those of the published differential diagnoses of myxoid spindle cell sarcoma. The literature was reviewed and the differential diagnoses of the presented case explored.

並列關鍵字

myxosarcoma ； pleural neoplasms

國際替代計量

High-Grade Myxoid Spindle Cell Sarcoma of the Pleura

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