Alveolar Soft Part Sarcoma: Clinical Outcome of Two Patients and Review of the Literature

肺泡狀軟組織肉瘤：兩病例臨床報告與文獻回顧

陳海雯(Helen H.W. Chen) ； 陳芬芬(Fen-Fen Chen) ； 黃文聰(Wen-Tsung Huang) ； 鄭莉莉(Li-Li Cheng) ； 梁永昌(Henry W.C. Leung)

《放射治療與腫瘤學》 7卷3期 (2000/09) Pp. 207-213

https://doi.org/10.6316/TRO/200007(3)207

摘要

肺泡狀軟組織肉瘤為一罕見的惡性軟組織肉瘤，好發於年輕成人的下肢。我們報導兩個典型的病例及回顧文獻報告，並對此疾病的臨床表現及治療預後做一討論。
病患一是33歲女性，發現左大腿有一無痛腫瘤，經手術切除證實是肺泡狀軟組織肉瘤後，病患接受了局部放射治療。五年後胸部X光發現肺部有多發性腫瘤，經切片證實為肺泡狀軟組織肉瘤轉移，三個月後腦部電腦斷層攝影也發現轉移腫瘤，病人在外院接受加馬刀放射手術，六個月後因復發及出現新轉移病灶而接受了腦部轉移腫瘤之切除；接著病人接受化學治療，六個月後的腦部電腦斷層攝影仍發現多發性腫瘤，病患遂接受全腦放射治療，但是無法接受完整的療程而於一個月後去世。病患二為26歲男性，臨床表現為下背痛，胸部X光發現肺古巴有多發性腫瘤，胸腰椎的磁振造影檢查發現有多處骨骼病變合併脊鞘囊壓迫，經手術切除後證實為肺泡狀軟組織肉瘤，病人並接受胸腰椎骨的局部放射治療。磁振造影檢查顯示出原發體瘤位於病患的左腿，接下來病患雖接受化學治療但是療效不佳，於發病後第十五個月去世。
肺泡狀軟組織肉瘤極易於疾病的早期或晚期出現遠端轉移，手術切除為主要的治療方式，放射治療可以提高疾病的局部控制率，也是在疾病出現轉移時可達到局部症狀緩解的有效姑息療法。

關鍵字

肺泡狀軟組織肉瘤,軟組織肉瘤,放射治療

並列摘要

Alveolar soft part sarcoma (ASPS) is a very rare soft tissue tumor which usually occurs in the lower extremity of young adults. We described two cases of ASPS in terms of natural history and clinical outcome.
The first case was a female patient who noticed a painless swelling in her left thigh at the age of 33. She underwent total removal of the tumor followed by post-operative radiation therapy for 50 Gy in 25 fractions. The diagnosis was ASPS. Five years after surgery, CXR showed multiple nodules which were proved to be metastasis by open lung biopsy. Brain metastasis developed three months later and was irradiated by Gamma-knife. Six months after radiosurgery for metastatic brain lesion, the patient underwent surgical excision for recurrent and newly developed metastatic brain lesions, followed by chemotherapy with Epirubicin, Ifosfamide and Mesna. Follow-up brain CT scan 6 months later revealed multiple nodules. The patient received radiation therapy to the whole brain but could not complete the course. She died of lung and brain metastases one month later. The second case was a 26-yearold man presented with low back pain. MRI of T-L spine revealed multiple lesions in the vertebral bodies with thecal sac compression at T11. ASPS was confirmed by a surgical decompression with partial removal of the extradural tumor. Routine CXR revealed multiple lung nodules. A meticulous clinical search led to find the primary tumor located at lateral aspect of the left thigh. He underwent T-L spine irradiation for 40.5 Gy in 17 fractions and systemic chemotherapy with Epirubicin and Cyclophosphamide for 3 months. However, no response was shown. The patient died of the disease 15 months after diagnosis.
ASPS tends to metastasize both early and late. Surgical excision is the mainstay of therapy. Radiation therapy may enhance local control after surgery or provide meaningful palliation for metastasis.

並列關鍵字

Alveolar soft part sarcoma,Soft tissue tumor,Radiation therapy