Rhabdomyosarcoma of the Adult Nasopharynx: A Case Report

成人鼻咽橫紋肌肉瘤

黃月蘭(Yueh-Lan Huang) ； 曾金鳳(Chin-Feng Tseng) ； 楊麗瓊(Li-King Yang) ； 蔡振華(Chen-Hua Tsai)

《內科學誌》 16卷3期 (2005/06) Pp. 146-150

https://doi.org/10.6314/JIMT.2005.16(3).07

摘要

成人頭頸部腫瘤中，橫紋肌瘤是罕見且具高轉移率的極惡性腫瘤，有關其存活率的文獻報告也並不多。儘管其預後極差，但有報告指出藉由化學治療、放射治療及外科手術等多科合作治療方式，有某些病人仍可提高存活率。在此報告一病例。一位六十七歲男性病人於三週前發現右上側頸部快速明顯變粗，但無鼻塞、耳痛、耳鳴或頭痛現象。頭頸部的核磁共振攝影顯示腫瘤由鼻咽右後壁延伸至右側咽喉部，並同時發現由下顎延伸至上鎖骨之雙側頸部淋巴亦已腫大。鼻咽部位的切片呈現出小深藍圓形分化不良之癌細胞，此並非一般鼻咽癌細胞。且此細胞對myoD1、myogenin、desmin及CD56等特殊染色方式呈陽性反應，因此確認此爲少見之橫紋肌肉瘤。病人接受爲期五個月的積極化學治療(VAC、vincristine, adriamycin and cyclophosphamide)及放射治療，在第十八個月後追蹤（包括頭頸部核磁共振攝影、骨頭掃描、胸部X光），並無局部復發或遠處轉移的現象。

並列摘要

Rhabdomyosarcoma is a rare and highly malignant neoplasm of the adult head and neck with a significant incidence of metastases. The outcome for adults with this disease is poorly documented.
Despite its ominously poor prognosis, combined polychemotherapy, radiotherapy and surgery, whenever feasible, has markedly improved survival rates in some patients. We report the case of a 67-year-old man who presented with a three-week history of a rapidly growing mass on the right upper neck with no symptoms of nasal obstruction, pain in the ears, tinnitus, or headache. Magnetic resonance image (MRI) study of the head and neck revealed a mass in the right and posterior walls of the nasopharynx with extension to the right parapharyngeal space, sphenoid and ethmoid sinuses and enlarged bilateral cervical lymph nodes extending from the submandibular to the supraclaricular regions. Nasopharyngeal biopsy showed poorly differentiated small, blue round cells. A diagnosis of rhabdomyosarcoma was confirmed by positive staining for myo D1, myogenin, desmin and CD56. Combination chemotherapy with VAC (vincristine, adriamycin and cyclophosphamide) regimen and radiotherapy was completed within 5 months. Follow up MRI of the head and neck, bone scan and chest X-ray 18 months later revealed neither local recurrence nor distant metastasis.

並列關鍵字

Rhabdomyosarcoma ； Adult nasopharynx ； Radiotherapy ； Chemotherapy