Abstract
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 slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/3314123381007714
Highlights
Inflammatory myofibroblastic tumors (IMTs) are mesenchymal neoplasms of intermediate biologic potential that are derived from myofibroblastic cells and accompanied by rich inflammatory infiltrates [1]
IMTs show expression of anaplastic lymphoma kinase (ALK) protein triggered by ALK gene rearrangement, which has been found in 36–60% of IMTs [2]
The aggressive behavior of IMTs with RANBP2-ALK (IMT-RA) might be associated with the following aspects: (1) The location and size of the tumor: Coffin et al [13] analyzed the clinicopathologic features of IMTs with an invasive course, and found that IMTs that arise in the abdominopelvic site are more likely to recur
Summary
Inflammatory myofibroblastic tumors (IMTs) are mesenchymal neoplasms of intermediate biologic potential that are derived from myofibroblastic cells and accompanied by rich inflammatory infiltrates [1]. Positive staining for CD30 (Dako; Clone Ber-H2; 1:40 dilution) (Figure 3B), desmin (Dako; Clone D33; 1:100 dilution) (Figure 3C), and SMA (Dako; Clone 1A4; 1:100 dilution) was observed in some tumor cells in the 2 cases In both cases, the tumor cells were nonreactive to AE1/AE3 (Dako; Clone AE1 + AE3; 1:200 dilution), cytokeratin 8/18 (Santa Cruz Biotechnology; Clone NCL5D3; 1:100 dilution), CD117 (Dako; polyclonal; 1:100 dilution), calponin Fluorescence in situ hybridization (FISH) was performed on 4 μm-thick tissue sections with bacterial artificial chromosome probes for RANBP2 (RP11-348G16) and ALK (RP11-984I21 and RP11-62B19) In both cases, 1 fused signal was observed in the tumor cell nucleus, indicating the presence of RANBP2 and ALK gene rearrangement (Figure 3D) by unbalanced genetic rearrangement mechanism [5]. Direct sequencing of the chimeric cDNA product confirmed that the RANBP2-ALK fusion point was composed of exon 18 of RANBP2 to exon 20 of ALK (Figure 4C)
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