Lahlou Ghizlene, Classe Marion, Wassef Michel, Just Pierre-Alexandre, Le Clerc Nicolas, Herman Philippe, Verillaud Benjamin
ENT Department, Lariboisiere Hospital, 2 Rue Ambroise Paré, 75010, Paris, France.
Pathology Department, Lariboisiere Hospital, APHP, Paris 7 University, Paris, France.
Head Neck Pathol. 2017 Jun;11(2):131-138. doi: 10.1007/s12105-016-0744-3. Epub 2016 Jul 21.
Inflammatory myofibroblastic tumors (IMTs) are rare mesenchymal tumors initially described in the lung. About half of them exhibit expression of the ALK1 protein, generally resulting from a gene rearrangement. Paranasal sinus IMTs are extremely uncommon, and gene rearrangement of ALK1 is very rare in this localization. A 47-year-old woman presented with rapidly progressive vision loss in her left eye. Clinical and imaging work-up revealed a tumor invading the left ethmoidal and sphenoidal sinuses and extending into the nasal cavity, the orbit and the skull base. Complete tumor resection was performed using an endonasal approach. Pathological examination revealed a paranasal localization of IMT, positive for ALK1 immunostaining. FISH analysis showed an ALK1 gene rearrangement. This case illustrates the local aggressive potential for IMTs. Treatment is primarily surgical, but targeted therapies (crizotinib) might be a solution for ALK1 rearranged cases with a poor prognosis.
炎性肌纤维母细胞瘤（IMTs）是一种罕见的间叶性肿瘤，最初在肺部被描述。其中约一半表现出ALK1蛋白表达，这通常是由基因重排导致的。鼻窦IMTs极为罕见，ALK1基因重排在该部位非常少见。一名47岁女性出现左眼视力迅速下降。临床和影像学检查发现一个肿瘤侵犯左侧筛窦和蝶窦，并延伸至鼻腔、眼眶和颅底。采用鼻内镜入路进行了肿瘤全切术。病理检查显示为鼻窦部位的IMT，ALK1免疫染色阳性。荧光原位杂交（FISH）分析显示存在ALK1基因重排。该病例说明了IMTs具有局部侵袭性。治疗主要是手术，但对于预后不良的ALK1重排病例，靶向治疗（克唑替尼）可能是一种解决办法。
