上呼吸道炎性肌纤维母细胞瘤携带新的TRAF3-ALK融合转录本
Inflammatory Myofibroblastic Tumor of the Upper Airways Harboring a New TRAF3-ALK Fusion Transcript.
作者信息
Di Ruscio Valentina, Mastronuzzi Angela, Russo Ida, Neri Marianna, Stracuzzi Alessandra, Giovannoni Isabella, Tropiano Maria Luisa, De Ioris Maria Antonietta, Milano Giuseppe Maria
机构信息
Department of Hematology/Oncology, Cell and Gene Therapy, Bambino Gesù Children's Hospital, IRCCS, Piazza Sant'Onofrio 4, 00165 Rome, Italy.
Pediatric Unit Annunziata Hospital, Via Migliori 1, 87100 Cosenza, Italy.
出版信息
Children (Basel). 2021 Jun 15;8(6):505. doi: 10.3390/children8060505.
Inflammatory myofibroblastic tumor (IMT) is a rare disease that mainly involves the lung and the abdomen with an intermediate clinical course but a recurrence rate between 15-30%. Radical surgery represents the gold standard of treatment, while chemotherapy and radiotherapy are considered for unresectable lesions. The identification of translocations in IMT opened the option for the use of target therapies. Indeed, the ALK inhibitors have changed the treatment approach for aggressive lesions, improving the prognosis. Intraluminal upper-way IMT is extremely rare and represents a medical challenge. We reported an endotracheal IMT case presenting a previously unknown fusion transcript.
炎性肌纤维母细胞瘤（IMT）是一种罕见疾病，主要累及肺和腹部，临床病程呈中度，但复发率在15%至30%之间。根治性手术是治疗的金标准，而对于不可切除的病变则考虑化疗和放疗。IMT中易位的鉴定为使用靶向治疗开辟了选择。事实上，ALK抑制剂改变了侵袭性病变的治疗方法，改善了预后。腔内上段IMT极为罕见，是一项医学挑战。我们报告了1例气管内IMT病例，该病例呈现一种此前未知的融合转录本。
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