- 期刊
頭皮類上皮肉瘤(epithelioid sarcoma)局部侵犯至腦組織－病例報告及文獻回顧
Epithelioid Sarcoma at Scalp with Local Invasion to Brain- A Case Report
摘要
文獻上第一次詳細描述類上皮肉瘤，是在1970年由Franz M. Enzinger提出，從此以後類上皮肉瘤已被接受為一獨特的軟組織肉瘤，而且在學術期刊上廣為討論。它是一種罕見的肌腱滑液膜肉瘤，通常易發於年輕男性，好發位置在前臂、手部掌側、小腿、及足底，約有三分之一的病例其病灶沿著同一解剖區域的肌腱(tendon)、腱膜(aponeurosis)、筋膜(fascia)以及神經束膜(perineural)多處發生，且易轉移至局部淋巴結。頭頸部少見，原發於頭皮的病例更罕見。局部侵犯至腦部，則沒有文獻上的報告。而其結節狀生長、外觀看似無害的良性肉芽狀組織，客易誤導臨床醫師的診斷。但其典型病程是毫不留情的(relentless)、是容易多處復發及轉移的。生物學上，它具有高度的惡性(high grade or biologic malignancy)。 類上皮肉瘤在組織發生學上其來源仍未確定。組織病理學診斷、細胞種類方面須靠免疫組織化學染色(immunohistochemical reaction)檢查。例如cytokeratin、vimentin、EMA，為陽性反應。 本文報告一61歲男性病例，罹患頭皮類上皮肉瘤局部侵犯至腦組織，其診斷經過，病程之變化及文獻回顧。
並列摘要
Epithelioid sarcoma was first clearly described and classified as a distinctive neoplasm by Franz M. Enzinger in 1970. It was then widely reported and discussed in literature. These are rare sarcomas and usually arise in tendon sheath of the distal extremities in adolescents and young adults between 10 and 25 years of age, especially in male. They have a high incidence of local recurrence, regional lymph node metastasis and also a high grade of biologic malignancy. Clinical diagnosis is misleading due to its deceptive and initially benign appearance. Actually, after its silent and slow growth, its course is relentless and indolent. Pathological diagnosis is difficult due to similar picture in sarcoma group under light microscope without special stain. Under immunohistochemical stain the cells show coexpression for cytokeratin, vimetin and EMA (epithelial membrane antigen), which aids in the diagnosis and differentiation from other sarcoma or benign lesions. Sarcoma over head and neck is rather rare. Soft tissue sarcoma is even less. Epithelioid sarcoma of scalp has been reported by Enzinger et al for only 2 cases (2/202), but in concerns with local brain invasion there is no literatural report. We present a case of huge epithelioid sarcoma of scalp with local invasion through calvarium into brain. The diagnosis, clinical picture, patient's hospital course and literature are reviewed.
延伸閱讀
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