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ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis.
Diagn Pathol 2017; 12(1):68DP

Abstract

BACKGROUND

Inflammatory myofibroblastic tumor (IMT) of the stomach is extremely rare in adults and exhibits a variable biological behavior that ranges from frequently benign lesions to more aggressive variants. Here we report a case of gastric IMT with lymph node metastasis in an adult who had undergone total colectomy for familial adenomatous polyposis (FAP).

CASE PRESENTATION

A 37-year-old man presented gradual-onset epigastric discomfort; he had undergone total colectomy for FAP 6 years before. The upper endoscopy revealed diffuse polyposis in the body of stomach and a submucosal protruding tumor of approximately 4.5 × 3.5 cm in the gastric angular incisure, appearing like gastrointestinal stromal tumor. Histology after surgery verified the diagnosis of fundic gland polyposis (FGPs) and gastric IMT with lymph node metastasis. Both the primary IMT tissue and its metastatic lesion but not the FGP or FAP tissue were positive for anaplastic lymphoma kinase (ALK) on immunohistochemical staining. Fluorescent in situ hybridization confirmed the existence of ALK rearrangement in IMT tissues. However, the patient exhibited no abnormalities in microsatellite instability or mismatch repair-system components, including MSH6, MSH2, MLH1 and PMS2, in IMT, FGP or FAP tissue.

CONCLUSIONS

This case allowed for exploring the relationship among IMT, FGP and FAP and indicates that gastric IMT should be considered in the diagnosis of a gastric mass in patients with FAP. ALK may be a useful biomarker in the diagnosis of IMT and its metastatic lesions.

Authors+Show Affiliations

Department of pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China.Department of pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China.Department of pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China.Department of pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China.Department of pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China.Department of pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China. niexiuyishi@126.com.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

28923119

Citation

Fan, Jun, et al. "ALK-positive Gastric Inflammatory Myofibroblastic Tumor in an Adult With Familial Adenomatous Polyposis and Diffuse Fundic Polyposis." Diagnostic Pathology, vol. 12, no. 1, 2017, p. 68.
Fan J, Huang B, Yang X, et al. ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis. Diagn Pathol. 2017;12(1):68.
Fan, J., Huang, B., Yang, X., Yang, M., He, J., & Nie, X. (2017). ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis. Diagnostic Pathology, 12(1), p. 68. doi:10.1186/s13000-017-0645-0.
Fan J, et al. ALK-positive Gastric Inflammatory Myofibroblastic Tumor in an Adult With Familial Adenomatous Polyposis and Diffuse Fundic Polyposis. Diagn Pathol. 2017 Sep 18;12(1):68. PubMed PMID: 28923119.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis. AU - Fan,Jun, AU - Huang,Bo, AU - Yang,Xiuping, AU - Yang,Ming, AU - He,Jun, AU - Nie,Xiu, Y1 - 2017/09/18/ PY - 2017/03/20/received PY - 2017/07/17/accepted PY - 2017/9/20/entrez PY - 2017/9/20/pubmed PY - 2018/6/12/medline KW - Anaplastic lymphoma kinase KW - Familial adenomatous polyposis KW - Gastric fundic gland polyposis KW - Inflammatory myofibroblastic tumors SP - 68 EP - 68 JF - Diagnostic pathology JO - Diagn Pathol VL - 12 IS - 1 N2 - BACKGROUND: Inflammatory myofibroblastic tumor (IMT) of the stomach is extremely rare in adults and exhibits a variable biological behavior that ranges from frequently benign lesions to more aggressive variants. Here we report a case of gastric IMT with lymph node metastasis in an adult who had undergone total colectomy for familial adenomatous polyposis (FAP). CASE PRESENTATION: A 37-year-old man presented gradual-onset epigastric discomfort; he had undergone total colectomy for FAP 6 years before. The upper endoscopy revealed diffuse polyposis in the body of stomach and a submucosal protruding tumor of approximately 4.5 × 3.5 cm in the gastric angular incisure, appearing like gastrointestinal stromal tumor. Histology after surgery verified the diagnosis of fundic gland polyposis (FGPs) and gastric IMT with lymph node metastasis. Both the primary IMT tissue and its metastatic lesion but not the FGP or FAP tissue were positive for anaplastic lymphoma kinase (ALK) on immunohistochemical staining. Fluorescent in situ hybridization confirmed the existence of ALK rearrangement in IMT tissues. However, the patient exhibited no abnormalities in microsatellite instability or mismatch repair-system components, including MSH6, MSH2, MLH1 and PMS2, in IMT, FGP or FAP tissue. CONCLUSIONS: This case allowed for exploring the relationship among IMT, FGP and FAP and indicates that gastric IMT should be considered in the diagnosis of a gastric mass in patients with FAP. ALK may be a useful biomarker in the diagnosis of IMT and its metastatic lesions. SN - 1746-1596 UR - https://www.unboundmedicine.com/medline/citation/28923119/ALK_positive_gastric_inflammatory_myofibroblastic_tumor_in_an_adult_with_familial_adenomatous_polyposis_and_diffuse_fundic_polyposis_ L2 - https://diagnosticpathology.biomedcentral.com/articles/10.1186/s13000-017-0645-0 DB - PRIME DP - Unbound Medicine ER -