The Extremity Leiomyosarcoma of Vascular Origin-A Case Report

平滑肌肉瘤約佔所有肉瘤5-10%。過去有文獻指出，血管性平滑肌肉瘤多見於大型血管。其較少發生於肢體端，更遑論發生源自於肢體之小血管。試討論本院一名病患發生在上肢血管性平滑肌肉瘤之治療及追蹤預後。一名64歲男性因右前臂背側無痛性腫塊約半年而前來就診。影像學檢查顯示一長約11公分並有血栓形成之血管性病兆，疑似血管瘤。經檢查後，逕行手術切除。術中冷凍切片，確定為一血管性平滑肌肉瘤。術中完整切除及確定雙側切端為健康組織，輔助以術後放射線治療。經術後長達48個月之追蹤及治療，恢復情況良好，無局部復發或遠處轉移之情形。平滑肌肉瘤好發於後腹腔中。回顧過去文獻，曾有血管性平滑肌肉瘤之案例，主要為大血管產生，如肺血管。而上肢血管性平滑肌肉瘤，不若後腹腔般有較多之病例數，其相關文獻之報導或治療極少。分析追蹤本院肢體端血管性平滑肌肉瘤，由其手術前臨床及影像評估上，懷疑為血管瘤。經外科切除，術中冷凍切片診斷，及輔助以術後放射線治療，已達成無局部復發或遠端轉移之良好預後。平滑肌肉瘤雖好發於後腹腔而少見於小血管。藉由此一手前臂血管性平滑肌肉瘤之治療及追蹤預後，相信經審慎術前診斷評估，外科廣泛性切除，加上輔助性放射線治療，可達成無局部復發或遠端轉移之最佳結果。

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並列摘要

Leiomyosarcoma (LMS) is a rare tumor that mutates from smooth muscle. It has occupied around 5-10 % of adult soft tissue sarcoma. Sometimes, we can find LMSs occurring from vascular origins clinically and they are usually found in large size vessels, such as the pulmonary trunk. It could seldom happen in peripheral small vessels. We present one case of LMS over the upper limb with a vascular origin and further discuss the best strategy in treating such a disease. A 64-year-old male, who has suffered from a growing palpable mass along the dorsal vein in R't dorsal forearm for 6 months, visited our clinic. In clinic division, completed image surveys revealed an intravascular lesion that favored hemangioma. Surgical excision was performed for him after serial examinations. Intraoperatively, the frozen sections of the lesion margins were checked for times and the reports showed malignancy. Afterwards, we made repeated checks both proximal and distal ends of the cut vessel and found they were free of tumor. Post-operatively, because the tumor within the vascular wall might contribute to early hematogenous spreading, we prescribed adjuvant radiotherapy for him. The patient has been followed post-operatively for 48 months, no local recurrence or distant metastasis is found, and his general condition is remaining robust. In the previous literatures, some authors reported the diseases occurred from vascular origins, mostly from large vessels of the pulmonary trunk. Besides, LMSs were diseases dominant in retroperitoneum and rare in peripheral vessels. However, now we present one case of the upper limb LMS with a vascular origin, which clinically might mimic a hemangioma or ganglion cyst. We successfully treat it with adequate resection combined with post-operative radiotherapy. Therefore, we believe that detailed pre-operative surveys, adequate resection of tumor, en block removal of adjacent tissue, careful checks of intraoperative frozen sections, and post-operative adjuvant radiotherapy are all key points to excellent diseasefree results and greatly reduce the possibilities of local recurrence.

並列關鍵字

Leiomyosarcoma LMS ； vascular origin ； radiotherapy

國際替代計量

The Extremity Leiomyosarcoma of Vascular Origin-A Case Report

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