血管外皮細胞瘤是一種相當罕見的血管性肉瘤,它來自微血管外圍的外皮細胞。此腫瘤正確的診斷是依據其病理特性作鑑別診斷;放射學方面並無專一特性,但血管攝影仍可輔助診斷。廣泛性手術切除是最重要且為大多數學者所選擇的治療方式,輔助性的治療包括:(1)術前血管內栓塞,可減少腫瘤本身血管分佈狀態,特別是切除一些較困難的後腹腔及骨盆腔腫瘤,能避免術中大量出血。(2)術後放射治療及化學治療。三軍總醫院自1985至1995年間共有14例病例,平均追蹤期間為39個月。其中有7例因遠處轉移而死亡,占50%。有2例在手術切除後局部復發,但仍然存活,占14.3%。有5例在手術切除後存活,無再復發,占35.7%。在我們病例中可發現預後與腫瘤的組織等級有關,然而血管外皮細胞瘤因惡性潛力高,故應當做是一種侵犯性的腫瘤積極治療。末期復發和遠處轉移是很常見,故長期定期追蹤治療將是很重要的。
Hemangiopericytoma is an uncommon vascular neoplasm originating from spindle, plump or mixed cells around the capillaries which Zimmermann had called pericytes. The plain X-ray film is nonspecific, but CT scan and angiography may aid in the diagnosis. A definitive diagnosis depends on histologic and immunohistochemical features. Wide excision is the treatment of choice. The adjuvant treatments include (1) pre-operative embolization of hemangiopericytomas (2) post-operative radiation therapy and (3) chemotherapy. This report reviews our experience of 14 cases of hemangiopericytomas treated at TSGH from 1985 to 1995. Surgery wise: 10 patients received wide excision and 4 received incisional biopsy. Adjuvant therapy wise: 3 received post-operative radiotherapy, 1 received extra- corporeal irradiation during operation, 1 received palliative radiotherapy, and 2 received both post-operative radiotherapy and chemotherapy. The average follow-up period was 39 months. 7 of the 14 patients (50%) had distant metastases and then died. 2 patients (14.3%) exhibited local recurrences after excision, and remained alive. 5 (35.7%) were alive without recurrence. Based on our experiences, the prognosis seems to correlate with the histologic grade of the tumor. Late recurrences and distant metastases are common. All hemangiopericytomas should be treated as aggressive tumors and long-term follow-up is necessary.