Post-irradiation Sarcoma in Patients with Nasopharyngeal Carcinoma-Experience at National Taiwan University Hospital

鼻咽癌患者經放射線治療後續發之惡性肉瘤－台大醫院的臨床經驗

陳建璋(Chien-Chang Chen) ； 戴浩志(Hao-Chih Tai) ； 簡雄飛(Hsiung-Fei Chien) ； 鄭乃禎(Nai-Chen Cheng) ； 洪學義(Shyue-Yih Horng) ； 湯月碧(Yueh-Bih Tang)

《臺灣整形外科醫學會雜誌》 21卷3期 (2012/09) Pp. 188-200

https://doi.org/10.7096/TJTSPS.201209.0188

摘要

背景：放射線治療後續發性肉瘤（PIS）是臨床上少見的惡性腫瘤，它具有較高的惡性度與侵犯性，而且早期診斷與及時治療均不容易，目前的文獻病人數都不多或僅僅是個案報告，尤其是在鼻咽癌盛行的東亞地區，完整的文獻報告更是少見。目的及目標：因此我們統計台大醫院於1978年至2008年的病例，分析鼻咽癌患者經放射線治療後續發性肉瘤的發生率、特徵表現、病理組織分類、危險因子、與臨床預後。材料及方法：透過癌症登記資料庫以病歷回溯方式，我們蒐集台大醫院於1978年至2008年間，共35位放射線治療後續發性肉瘤患者的資料。其中23位（65.7％）患者因之前患有鼻咽癌而接受頭頸部放射線治療，包含16位男性及7位女性，診斷出放射線治療後續發性肉瘤的平均年齡為51.5歲。我們去分析可能的危險因子與存活曲線。結果：在這個研究中，鼻咽癌是最常造成放射線治療後續發性肉瘤（PIS）的前置疾病。在鼻咽癌患者經頭頸部放射線治療後約有0.43％的人會罹患續發的惡性肉瘤，這發生率和稍早的幾個研究類似。患者接受放射線的年齡從17歲至53歲（平均37.4歲），接受放射線治療後到診斷出惡性肉瘤平均經過14.1年（2.9-25年）。年紀、性別、放射線劑量、鼻咽癌期別與是否有不良生活習慣（抽菸、喝酒、嚼檳榔）均不是導致放射線治療後續發性肉瘤顯著的危險因子。這樣的肉瘤大多以無症狀的腫塊來表現（65.2％）。病理組織學上最常見的是骨肉瘤。病患一年、兩年及五年的存活率分別為60％、25％及20％。23位患者中有19位接受過切除手術，平均每人接受過2.1次。我們的統計指出，如果續發性肉瘤可以依照安全範圍來切除，病患的預後是較好的，而由於重建及顯微皮瓣技術的發展，我們可以較積極地切除腫瘤以達到較佳的預後。結論：在高鼻咽癌發生率的地區，當在診斷鼻咽癌病人復發性腫塊時須特別小心，因為這極有可能就是放射線治療後續發性肉瘤。及早診斷與範圍廣泛性切除方可改善病人預後。

並列摘要

Background: Post-irradiation sarcoma (PIS) is a rare disease. However, these tumors are relatively aggressive and often elude early detection and timely treatment. Literature is limited to small series and case reports. Besides, there is a lack of thorough reports regarding post-irradiation sarcoma in East Asia, the high nasopharyngeal cancer (NPC) risk area. Aim and Objectives: This study investigates the clinical pictures, pathological features, treatments, and prognoses of patients with PIS stemming from NPC at National Taiwan University Hospital.Materials and Methods: A retrospective review of charts from the Cancer Registry Department of National Taiwan University Hospital (NTUH) was performed from 1978-2008. A total of 35 patients with post-irradiation sarcomas were noted. 23 PIS victims who had previous NPC within the irradiated field were included and evaluated (16 males and 7 females; mean age at diagnosis: 51.5 years). Possible risk factors were analyzed and survival curve was generated with the Kaplan-Meier method.Results: NPC was the most common index lesion before radiotherapy in our series. The incidence rate of PIS for patients with NPC was 0.43%, similar to other series. The age at the time of radiotherapy ranged from 17 to 53 (mean: 37.4). The latency period from radiation to PIS ranged from 2.9 to 25 years (mean: 14.1). As for risk factors in our series, they did not include gender, age, radiation dosage, NPC stages, or habits. Mass lesions over the head and neck region were the most common symptom of PIS (65.2%). Of these, osteosarcoma was the most prevalent histological type. Their overall 1-, 2-, 5-year survival rates were 60%, 25%, and 20% at respectively. 19 patients had undergone surgical intervention. On average, each patient had 2.1 operations for PIS. Evidence indicates that surgical safety margins assure PIS victims a better prognosis. With the development of reconstruction techniques and free flaps, more aggressive excision and higher survival rates have been obtained.Conclusions: In areas with a high NPC prevalence, it is important to keep in mind PIS during the differential diagnosis of nasopharyngeal cancer patients with a probably local recurrence. Early diagnosis and wide excision with safety margins are keys to a better prognosis. (J Taiwan Soc of Plast Surg 2012;21:188~200)

並列關鍵字

post-irradiation sarcoma (PIS) ； radiation carcinogenesis ； radiation side effects ； head and neck ； nasopharyngeal carcinoma (NPC)