ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis
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...
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| Language: | English |
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BMC
2017-09-01
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| Series: | Diagnostic Pathology |
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| Online Access: | http://link.springer.com/article/10.1186/s13000-017-0645-0 |
| _version_ | 1818193274836353024 |
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| author | Jun Fan Bo Huang Xiuping Yang Ming Yang Jun He Xiu Nie |
| author_facet | Jun Fan Bo Huang Xiuping Yang Ming Yang Jun He Xiu Nie |
| author_sort | Jun Fan |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-12T00:43:48Z |
| format | Article |
| id | doaj.art-c31021a7fd244c0490bf3caff8bc3936 |
| institution | Directory Open Access Journal |
| issn | 1746-1596 |
| language | English |
| last_indexed | 2024-12-12T00:43:48Z |
| publishDate | 2017-09-01 |
| publisher | BMC |
| record_format | Article |
| series | Diagnostic Pathology |
| spelling | doaj.art-c31021a7fd244c0490bf3caff8bc39362022-12-22T00:44:11ZengBMCDiagnostic Pathology1746-15962017-09-011211610.1186/s13000-017-0645-0ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposisJun Fan0Bo Huang1Xiuping Yang2Ming Yang3Jun He4Xiu Nie5Department of pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and TechnologyDepartment of pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and TechnologyDepartment of pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and TechnologyDepartment of pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and TechnologyDepartment of pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and TechnologyDepartment of pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and TechnologyAbstract 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.http://link.springer.com/article/10.1186/s13000-017-0645-0Inflammatory myofibroblastic tumorsFamilial adenomatous polyposisGastric fundic gland polyposisAnaplastic lymphoma kinase |
| spellingShingle | Jun Fan Bo Huang Xiuping Yang Ming Yang Jun He Xiu Nie ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis Diagnostic Pathology Inflammatory myofibroblastic tumors Familial adenomatous polyposis Gastric fundic gland polyposis Anaplastic lymphoma kinase |
| title | ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis |
| title_full | ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis |
| title_fullStr | ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis |
| title_full_unstemmed | ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis |
| title_short | ALK-positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis |
| title_sort | alk positive gastric inflammatory myofibroblastic tumor in an adult with familial adenomatous polyposis and diffuse fundic polyposis |
| topic | Inflammatory myofibroblastic tumors Familial adenomatous polyposis Gastric fundic gland polyposis Anaplastic lymphoma kinase |
| url | http://link.springer.com/article/10.1186/s13000-017-0645-0 |
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