Carcinosarcoma (True Malignant Mixed Tumor) of the Parotid Gland- Case Report

腮腺Carcinosarcoma (True Malignant Mixed Tumor)的病例報告──病例報告及文獻回顧

謝宇軒(Yu-Hsuan Hsieh) ； 潘俊豪(Chun-Hao Pan) ； 蔡嘉軒(Chia-Hsuan Tsai) ； 余德毅(De-Yi Yu) ； 陳信宏(Hsin-Hung Chen) ； 陳建宗(Chien-Tzung Chen)

《臺灣整形外科醫學會雜誌》 25卷2期 (2016/06) Pp. 144-152

摘要

背景：Carcinosarcoma 也稱作混合型惡性腫瘤(Mixed malignant tumor)，是一種很罕見的腫瘤，通常侵犯腮腺，在所有唾線腫瘤中佔有比例小於0.04%-0.2%。病理檢驗下，Carcinosarcoma同時具有原發性上皮腺癌及肉瘤癌成分，也稱作“真正的混合型惡性腫瘤”(True mixed malignant tumor)。在文獻回顧中，治療主要以外科切除合併術後放射線治療。由於病例非常稀少，預後及存活率的預測並沒有建立。目的及目標：我們分享一個患有右側腮腺腫瘤的病例，在腫瘤切除後病理報告顯示腫瘤同時具有原發性上皮腺癌及肉瘤癌成分，探討其治療方式及追蹤其預後。材料及方法：這一篇病例報告指出一具有肺腺癌病史之84歲亞洲男性病人，患有持續擴大之右側腮腺腫瘤，接受右側腮腺全切除後，腫瘤組織送往病理切片及免疫化學 (Iimmunohistochemical study (IHC))分析。結果：病人在腫瘤切除後，有右側顏面神經麻痺的症狀，在接受復健及電刺激治療後逐漸恢復原有之顏面神經功能。病理報告顯示該腫瘤同時具有原發性上皮腺癌及肉瘤癌成分。TTF-1及Napsin A染色也用來排除病人腮腺腫瘤為肺部腺癌轉移之病兆。病人術後接受放射線治療，劑量5800cGy/29 fractions。在追蹤18個月並無癌症復發之跡象。結論：Carcinosarcoma是一種非常罕見的疾病，這一篇病例報告呈現了病人在臨床上接受治療的過程、預後及文獻的回顧。

並列摘要

Background: Carcinosarcoma, also referred to as malignant mixed tumor, mostly affect parotid gland. The incidence of carcinosarcoma of the salivary gland is vary rare, constitutes less than 0.04%-0.2% of all salivary gland tumor. Histopathologic examination of the specimen revealed a biphasic pattern composed of both carcinomatous and sarcomatous elements. Treatment with surgery followed by radiotherapy is the most usual approach for tumor with aggressive behavior. Prognosis and survival statistics following treatment of parotid malignant tumors have been very difficult to interpret because these are rare tumors with a diverse histology. Aim and Objectives: We present a case with right pre-auricular tumor. A histopathologic study showed the biphasic pattern composed of both carcinomatous and sarcomatous elements. Materials and Methods: An Asian patient, 84-year-old male sustained a progressively growing right preauricular mass for more than one year. The 3 cm mass is firm and tender. No lymph node enlargement was palpable. The patient received right total parotidectomy after frozen section showed adenocarcinoma. Specimens were sent to pathology study. Results: Postoperatively, the patient had weakness of facial expression over right frontal, buccal and mandibular branch territory but improved after receiving electrical stimulation therapy. Final histopathologic study indicated carcinosarcoma (pT2) with negative margin. The tumor encompassed both patterns of adenocarcinoma and sarcoma. The final diagnosis was ＂True malignant mixed tumor＂ of salivary gland tumor. Immunohistochemical study with Thyroid transcription factor-1 (TTF-1) and Napsin A were used to rule out the metastasis of malignancy from the lung adenocarcinoma. The recovery of the patient is fair and the patient was sent to Radiation Therapy Oncology department for further treatment. After radiotherapy with 5800cGy/29 fractions, regular clinical follow up of the patient was arranged and no recurrence of tumor was noted 18 months postoperatively. Conclusion: Carcinosarcoma is very rare disease. This paper showed the whole clinical course and effective treatment of the carcinosarcoma.

並列關鍵字

carcinosarcoma ； adenocarcinoma ； sarcoma ； parotid gland tumor ； carcinomatous, sarcomatous ； radiotheraphy ； TTF-1 (Thyroid transcription factor-1) ； Napsin A