上頜竇炎性肌纖維母細胞瘤－病例報告及文獻回顧

Inflammatory Myofibroblastic Tumor of the Maxillary Sinus: A Case Report and Literature Review

陳伯岳(Po-Yueh Chen) ； 陳煥森(Huan-Sen Chen) ； 蘇昱仁(Yu-Jen Su) ； 張燕良(Yen-Liang Chang) ； 梁哲維(Cher-Wei Liang)

《台灣耳鼻喉頭頸外科雜誌》 44卷1期 (2009/02) Pp. 25-32

https://doi.org/10.6286/2009.44.1.25

摘要

炎性肌纖維母細胞瘤是一群不明病因的腫瘤，其在全身各處皆有可能發生，最早是由Brunn於1939年首度描述於肺部，肺部是最常發生的部位而肺部外最常見於腸繫膜及後腹腔，至於頭頸部則較常見於眼眶，位於上頜竇實屬罕見，且其臨床表徵、病程以及治療與身體其他部位的不盡相同。在此，我們報告1名56歲男性，以右上側齒槽腫痛爲最初表現，電腦斷層攝影顯示出一右上頜腫瘤，具局部侵犯及骨質破壞，經病理切片診斷爲炎性肌纖維母細胞瘤，於2006年8月接受右側上頜竇近全切除手術，於2006年12月因鼻腔內復發再次接受廣泛切除，因腫瘤局部復發，於2007年3月至2007年5月接受放射治療，因病灶持續，於2007年5月至2007年6月接受類固醇治療，於2007年6月因頸部復發接受頸部腫瘤切除手術，因腫瘤進展快速，於2007年9月再次接受廣泛切除，並於2007年11月至2008年1月接受術後放射治療，至2008年3月臨床及影像學檢查上都偏向出現遠端轉移，包括背部、腹部、肺部及胃，切片後證實爲轉移且有肉瘤樣的轉變(sarcomatous transformation)，隨後採取姑息性的放射線治療，但病患仍於2008年5月因敗血症及腎衰竭而死亡。此爲罕見的上頜竇炎性肌纖維母細胞瘤經多次復發且併發惡性轉變及遠端轉移並致死的病例，特此提出報告，並討論其臨床表現、診斷及治療。

關鍵字

炎性肌纖維母細胞瘤 ； 上頜竇 ； 惡性轉變 ； 遠端轉移 ； 致死

並列摘要

Inflammatory myofibroblastic tumor (IMT) is a rare lesion of uncertain etiology. IMT most commonly involves the lung, abdomen and orbit, but it has been reported to occur in nearly every site in the body. Sinonasal IMT is rare and has characteristics such as multifocal appearance, infiltrative local growth, vascular invasion, tendency for local recurrence, metastatic potential and more aggressive behavior. A 56-year-old man presented with swelling and pain in his right face and right upper gingiva area. A computed tomography of sinus showed a soft tissue mass at right maxillary sinus with erosion of walls of sinus. Exploratory antrotomy with biopsy was done and pathological results favored the diagnosis of IMT. Right subtotal maxillectomy was performed in July, 2006. Unfortunately, tumor recurred in right sinonasal area and wide excision was performed in December, 2006. Due to persisted lesion, he received radiotherapy (7020cGy) and steroid therapy. In June, 2007, right submandibular mass was noted and excision was done to confirm recurrence. Because of rapidly progressive disease, wide excision was undertaken again in September, 2007 and postoperative radiotherapy (6000cGy) followed. Unfortunately, distant metastases including back, abdomen, lung and stomach were noted in March, 2008 and histologically, sarcomatous transformation was also noted. Although palliative radiotherapy done, the patient expired due to sepsis and renal failure in May, 2008.

並列關鍵字

inflammatory myofibroblastic tumor ； maxillary sinus ； malignant transformation ； distant metastasis ； death