鼻腔及鼻竇橫紋肌肉瘤

Rhabdomyosarcoma of the Nose and Paranasal Sinuses

蘇穩發(Ween-Fa Su) ； 何青吟(Ching-Yin Ho) ； 林清榮(Ching-Zong Lin)

《中華民國耳鼻喉科醫學會雜誌》 33卷3期 (1998/06) Pp. 244-249

https://doi.org/10.6286/1998.33.3.244

摘要

背景：橫紋肌肉瘤是小孩子最常見的軟組織肉瘤，可發生在身體任何部位，大人最常發生於四肢及軀幹，小孩則發生於頭頸部及泌尿生殖器。而頭頸部以眼球最常見，其次是鼻咽、中耳、鼻腔及鼻竇。發生在鼻腔及鼻竇的橫紋肌肉瘤，因其罕見，病理診斷不易，在診斷確立時，常已有局部及遠處轉移，因此預後相當不好。本篇報告即針對鼻腔及鼻竇的橫紋肌肉瘤，就其臨床病程做一回溯性探討。
方法：本院耳鼻喉部收集從1984年至1996年發生於鼻腔及鼻竇的橫紋肌肉瘤共6例，就病患年紀、性別、腫瘤位置、臨床症狀、病理、分期、法療方法及追蹤，加以分析討論。
結果：本報告共收集6個病例，男1例，女5例，平均齡22歲。除第6例剛接受完手術切除，現正接受放射和化學治療，以及第3例失去追蹤外，其餘均已死亡。從確定診斷到死亡的時間，從6個月到2年。病患到本部門診求治時，腫瘤均已侵犯相當廣泛。而其起始症狀多為鼻塞、流鼻涕、鼻分泌帶血絲，並無特異性。也因此使得病患及醫師失去警覺性，而延誤治療時機。
結論：鼻腔及鼻竇橫紋肌肉瘤極為罕見且預後不好。因其起症狀無特異性，且病理診斷不易，加上病程發展相當快速，因此在診斷確立時，多已有局部侵犯及遠處轉移。這也是為什麼此疾病容易復發、轉移，治療效果不彰的原因。看門診時提高對此病的警覺性，才能對此疾病早期發現、正確診斷，且儘速給予結合手術、放射及多種藥物化學治療的多型式治療（multiomdality therapy），以增進此病存活率。

關鍵字

橫紋肌肉瘤 ； 頭頸部 ； 鼻腔及鼻竇

並列摘要

Background: Rhabdomyosarcoma is the most common soft tissue sarcoma of children. This tumor can arise anywhere in the body but rarely in the nose and paransal sinuses. It most commonly occurs in the childhood and adolescent years. The prognosis is poor but it had been imprved since 1972 when multimodality therappy was introduced by the Intergroup Rhabdomyosarcoma Study (IRS). The purpose of this study is to collect and analyze clinical data of patients with this disease.
Methods: We collected 6 cases of rhabdomyosarcoma of the nose and paranasal sinuses diagnosed in our department from 1984 to 1996. Age, sex, tumor location, clinical presentation, pathology, tumor stage, treatment regimen and follow-up were analyzed.
Rusults: Study subjects included one man and five women with mean age 22 years old. All died except two patients: one lost follow-up and anther one was still alive under radiotherapy and chemotherapy. The duration from diagnosis to death varied from 6 to 24 months. Chief complaints included nasal obstruction, rhinorrhea and bolld-tinged nasal discharge. The initial symptoms were not specific and were easily mistaken for those of inflammatory condition by primary care givers.
Conclusions: Nasosinal rhabdomyosarcoma is a relatively highly malignant tumor. The poor prognosis may be related to nonspecific initial symptoms, delayed diagnosis, easy local ifiltration, early dissemination and surgical difficulty. Improved results can be obtained with apropriate aggressive multmodality therapy – a combination of surgery, radiotherapy and continuous repeated courses of chemotherapy. It is also important to pay more attention to this rare disease at our clinics.

並列關鍵字

rhabdomyosarcoma ； head and neck ； nose and paranasal sinuses