具有 RANBP2 和 ALK 基因重排的炎性肌纤维母细胞瘤：两例报告及文献复习。

Inflammatory myofibroblastic tumor with RANBP2 and ALK gene rearrangement: a report of two cases and literature review.

机构信息

Department of Pathology, the Second Shenzhen People's Hospital, Sungang West Road, Shenzhen 518035, China.

出版信息

Diagn Pathol. 2013 Sep 13;8:147. doi: 10.1186/1746-1596-8-147.

UNLABELLED

Inflammatory myofibroblastic tumors (IMTs) are categorized as intermediate biologic neoplasms, whereas IMTs with genetic features of ran-binding protein 2 (RANBP2) and anaplastic lymphoma kinase (ALK) rearrangement (IMT-RAs) are possibly related to a more aggressive clinical course. However, fewer than 10 cases of IMT-RA have been reported to date. Herein, we present 2 new cases of IMT-RA in which both tumors recurred quickly after primary surgery; one patient died 3 months later from the disease, and the other patient has been living with the disease for 12 months. IMT-RAs are characterized by noncohesive epithelioid and rounded tumoral cell morphology, commonly derived from pelvic and peritoneal cavities, and frequently show larger tumor sizes. The relation between the clinicopathologic features and poor prognosis of IMT-RA is discussed.

VIRTUAL SLIDES

The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/3314123381007714.

未注明

炎性肌纤维母细胞瘤（IMTs）被归类为中等生物学肿瘤，而具有 RAN 结合蛋白 2（RANBP2）和间变性淋巴瘤激酶（ALK）重排遗传特征的 IMT（IMT-RAs）可能与更具侵袭性的临床病程有关。然而，迄今为止，报告的 IMT-RA 病例少于 10 例。在此，我们报告了 2 例新的 IMT-RA 病例，这两个肿瘤在初次手术后均迅速复发；1 例患者在 3 个月后死于该病，另 1 例患者在疾病中生存了 12 个月。IMT-RAs 的特征是无黏附性上皮样和圆形肿瘤细胞形态，通常源自骨盆和腹腔，并经常显示更大的肿瘤大小。讨论了 IMT-RA 的临床病理特征与不良预后之间的关系。