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

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.

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• Authors: Jian Li, Wei-hua Yin, Kengo Takeuchi, Hong Guan, Yu-hua Huang, John K C Chan
• Journal: Diagnostic pathology (Diagn Pathol) Vol. 8 Pg. 147 (Sep 13 2013) ISSN: 1746-1596 [Electronic] England
• PMID: 24034896 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• ALK-RANBP2 fusion protein, human
• Biomarkers, Tumor
• Molecular Chaperones
• Nuclear Pore Complex Proteins
• Oncogene Proteins, Fusion
• ran-binding protein 2
• ALK protein, human
• Anaplastic Lymphoma Kinase
• Receptor Protein-Tyrosine Kinases

Topics

• Adult
• Anaplastic Lymphoma Kinase
• Biomarkers, Tumor (analysis)
• Biopsy
• Cell Shape
• Fatal Outcome
• Female
• Gene Fusion
• Gene Rearrangement
• Genetic Predisposition to Disease
• Humans
• In Situ Hybridization, Fluorescence
• Inflammation (enzymology, genetics, pathology, surgery)
• Intestinal Neoplasms (enzymology, genetics, pathology, surgery)
• Male
• Molecular Chaperones (genetics)
• Neoplasm Recurrence, Local
• Neoplasms, Muscle Tissue (enzymology, genetics, pathology, surgery)
• Nuclear Pore Complex Proteins (genetics)
• Oncogene Proteins, Fusion (genetics)
• Phenotype
• Receptor Protein-Tyrosine Kinases (genetics)
• Reverse Transcriptase Polymerase Chain Reaction
• Time Factors
• Tomography, X-Ray Computed
• Treatment Outcome
• Tumor Burden
• Young Adult