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

Jian Li1, Wei-hua Yin1, Kengo Takeuchi23, Hong Guan4*, Yu-hua Huang4 and John KC Chan5

Author Affiliations

1 Department of Pathology, Peking University Shenzhen Hospital, No. 1120, Lianhua North Road, Shenzhen 518000, China

2 Pathology Project for Molecular Targets of the Cancer Institute, Tokyo 135-8550, Japan

3 Division of Pathology of the Cancer Institute Hospital, Japanese Foundation for Cancer Research, 3-8-31 Ariake, Koto, Tokyo 135-8550, Japan

4 Department of Pathology, the Second Shenzhen People’s Hospital, Sungang West Road, Shenzhen 518035, China

5 Department of Pathology, Queen Elizabeth Hospital, Hong Kong, SAR, China

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Diagnostic Pathology 2013, 8:147  doi:10.1186/1746-1596-8-147

Published: 13 September 2013


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.

The virtual slide(s) for this article can be found here: webcite

Inflammatory myofibroblastic tumor; RANBP2-ALK; Fluorescence in situ hybridization