頸部蜂房狀軟組織肉瘤併鼻腔轉移－病例報告

蜂房狀軟組織肉瘤是一種罕見的軟組織惡性種瘤，通常好發於下肢且較常見於年輕女性。當這些物腫瘤出現在頭頸部時，最常發生的位置為眼框及舌部，回顧至1999年底為止，發生於頭頸部之病例佈道80例，出現在鼻腔更為罕見，以英文發表之文獻總共也只有4例報告。其組織發生來源至今仍然不明，病程往往相當長且初期症狀不明顯，然而這類腫瘤終究不可避免會復發和轉移，因此是個不折不扣的惡性腫瘤。手術摘除是治療這類疾病的首要原則。本科經例一35歲女性病患，於懷孕初期在後頸部出現腫瘤；產後腫瘤逐漸變大，同時發現鼻腔及多發性肺部轉移。病患接受手術摘除鼻腔及頸部之腫瘤，經病理診斷證實為蜂房狀軟組織肉瘤。術後定期追蹤，起出病人因無費不及其他相關症狀而拒絕接受進一步的治療，4個月後出現胸痛及咳血等症狀開始接受化學治療，之後不久發現腦部轉移，頸部亦出現復發腫瘤並接受放射治療，最後病人仍於術後半年死於急性呼吸道衰竭。

關鍵字

蜂房狀軟組織肉瘤；鼻腔；頸部；懷孕

並列摘要

Akveolar soft part sarcoma (ASPS)is a rare soft tissus malignancy typically found the extremities of young females. When arising in the head and neck area, theky usually appear in the orbit or tongue.Less than 80 head and neck occrrences have been reported in the English literature prior to 1999, with onli four nasal cavity occurrrences described.The deriation these tumors is still uncertain. The clinical course is protracted and initially indolent, indolent, but local recurrence and metastasis inevitably occure over time. As such, they aee best regarded as malignant neoplasms. Siurgical excision is the mainstay of ther-apy. A patient presented with ASPS of posterir area appeared during her pregnacy with concmitant nasal cavity and multiple lung mwtastases. She underwent wide excision of the nasal and neck masses and was followed up regularly afer surgery. She refused furtheter therapy because her lung disease was non-proressive. For months later, chest pain and hemopysis were noted and chemotherapy was commenced. Soon after, brain metastases and local recurrence in the neck were noted and radiotherapy was commenced for the neck lesion . Unfortunately, the patient died of acuted respiratory failure six months after surgerky. Owing to its unque hisological and clinical characteristics, comliete surgi- cal excision and long-tem follow-up are indicated.

並列關鍵字

Alveolar soft part sarcoma ； neck ； nasal cavity ； pregnancy

國際替代計量

頸部蜂房狀軟組織肉瘤併鼻腔轉移－病例報告

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