Alveolar Soft Part Sarcoma: A Case Report, Literature Review, and Differentiation from Vascular Malformation

腺泡狀軟組織肉瘤－案例報告及文獻回顧

郭宇正(Yu-Chen Kuo) ； 李建智(Jian-Jr Lee) ； 劉致和(Charles Chih-Ho Liu) ； 洪瑞隆(Ruey-Long Hong) ； 陳明庭(Ming-Ting Chen)

《臺灣整形外科醫學會雜誌》 21卷4期 (2012/12) Pp. 351-358

https://doi.org/10.7096/TJTSPS.201212.0351

摘要

背景：線泡狀軟組織肉瘤是非常罕見的惡性腫瘤，通常疾病的進程是緩慢的，但在早期卻有很高的肺部轉移發生率；多影響青少年及年輕成年人，最常發生的部位為下肢的肌肉群中；典型的特徵是充滿血管的，常被觀察到有明顯的血管存在腫瘤內；因此，我們必須謹記在心，試著將線泡狀軟組織肉瘤與血管畸形作區分，然後選擇正確的治療方式，以期能延長病人的生命。目的及目標：提出一個罕見的惡性肉瘤案例，讓手術者增加對它的熟悉度，並跟良性的血管病灶做出區別。材料及方法：我們報告一位十五歲年輕女性，從小學時期左大腿開始有個摸得到的腫塊；經歷一系列的影像學檢查，診斷為左大腿惡性腫瘤合併肺轉移，她先接受左大腿腫瘤廣泛性切除，一公分的安全邊際，然後接受口服的化學療法。結果：一年後追蹤左大腿核磁共振顯示無局部復發；胸部X光片持續追蹤三年，並無明顯的肺部結節；電腦斷層則顯示腫瘤仍處於穩定狀態。結論：因為線泡狀軟組織肉瘤的進程緩慢，常被延誤，甚至轉移；此外，它的高血管性特徵常會被誤認為血管畸形，必須小心做鑑別診斷；假如懷疑是線泡狀軟組織肉瘤，病人必須接受完整的手術切除，若有任何遠端轉移，則建議接受進一步的化療。

並列摘要

Background: Alveolar soft part sarcoma (ASPS) is a rare malignant tumor, usually presenting as an indolent slow-growing malignancy with a high incidence of pulmonary metastases early in the disease course. It affects adolescents and young adults, and most frequently occurs in the muscle of the lower extremity. The vascularity of ASPS is typically high, with prominent vessels see within the tumor. It is therefore crucial to differentiate ASPS from a benign vascular lesion. Aim and Objectives: We present this rare case of ASPS and review current literature. Surgeons need to be made aware of the disease to avoid treating it as a benign vascular lesion. Materials and Methods: We present a 15-year-old girl with a growing mass over left thigh since primary school age (preadolescence). Following a series of imaging studies including magnetic resonance imaging and angiography, soft tissue malignancy or vascular lesion was suspected. Abdominal echo, brain magnetic resonance imaging, bone scan and chest computed tomography were also performed. She received surgical excision and specimen was sent for pathological examinations. Patient continues to receive regular out-patients follow-up to date. Results: Alveolar soft part sarcoma was diagnosed pathologically. Multiple small pulmonary metastases were noted on chest computed tomography. After resection of primary tumor, she has received oral thalidomide and endoxan. Follow-up magnetic resonance imaging of left thigh demonstrated no tumor recurrence one year later. Follow-up chest x-ray three years later showed no apparent nodule lesions though CT scan still demonstrated bilateral lung metastases, some remained stationary and some slightly enlarged. Conclusion: Although a rare disease, accurate diagnosis of ASPS needs to be made pathologically. Surgeons need to have a high index of suspicion based on clinical and imaging studies. Adequate surgical resection with adjuvant therapy is essential for disease management in cases with pulmonary metastasis.

並列關鍵字

alveolar soft part sarcoma ； vascular malformation