Posttransplant epithelioid inflammatory myofibroblastic sarcoma: A case report
Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare entity and a novel variant of inflammatory myofibroblastic tumor (IMT), usually seen in children and nonsmoking young adults. Their occurrence in a posttransplant setting is still rare. These tumors are characterized by prominent epit...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2019-01-01
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| Series: | Indian Journal of Pathology and Microbiology |
| Subjects: | |
| Online Access: | http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2019;volume=62;issue=2;spage=303;epage=305;aulast=Garg |
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| author | Ritu Garg Sumaid Kaul Deepshikha Arora Vikas Kashyap |
| author_facet | Ritu Garg Sumaid Kaul Deepshikha Arora Vikas Kashyap |
| author_sort | Ritu Garg |
| collection | DOAJ |
| description | Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare entity and a novel variant of inflammatory myofibroblastic tumor (IMT), usually seen in children and nonsmoking young adults. Their occurrence in a posttransplant setting is still rare. These tumors are characterized by prominent epithelioid morphology, large histiocytoid “Reed Sternberg”-like cell, unique pattern of ALK immuno-reactivity, and aggressive clinical behavior. Their etiology and metastatic potential is controversial. In a post-transplant setting, many factors such as trauma, infections with EBV, HIV, Hepatitis C, mycobacteria, fungus, and chemotherapy-induced immunosuppression have been implicated in their etiology. We present the case of a 2-year-old female child who developed multiple omental and mesenteric tumor nodules, 8 months post liver transplant for progressive familial intrahepatic cholestasis (PFIC). Following a histopathological diagnosis of “mesenchymal neoplasm of possible malignant nature” on a trucut biopsy and frozen section, tumor debulking was performed. A final histological diagnosis of EMIS was made on the completely resected tumor. The patient remains in remission nearly 7 months after presentation, without any follow-up systemic chemotherapy. IMT after a solid organ transplant is rare, only 5 cases have been reported in the literature until now. Similar phenomenon has also been noted with hematopoietic stem cell transplant. However, to our knowledge, this case of EMIS in a post liver transplant patient is first of its kind. |
| first_indexed | 2024-12-12T14:11:29Z |
| format | Article |
| id | doaj.art-03bc5cc9dd6543d6935d61edb878adb8 |
| institution | Directory Open Access Journal |
| issn | 0377-4929 |
| language | English |
| last_indexed | 2024-12-12T14:11:29Z |
| publishDate | 2019-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Pathology and Microbiology |
| spelling | doaj.art-03bc5cc9dd6543d6935d61edb878adb82022-12-22T00:22:02ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49292019-01-0162230330510.4103/IJPM.IJPM_284_17Posttransplant epithelioid inflammatory myofibroblastic sarcoma: A case reportRitu GargSumaid KaulDeepshikha AroraVikas KashyapEpithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare entity and a novel variant of inflammatory myofibroblastic tumor (IMT), usually seen in children and nonsmoking young adults. Their occurrence in a posttransplant setting is still rare. These tumors are characterized by prominent epithelioid morphology, large histiocytoid “Reed Sternberg”-like cell, unique pattern of ALK immuno-reactivity, and aggressive clinical behavior. Their etiology and metastatic potential is controversial. In a post-transplant setting, many factors such as trauma, infections with EBV, HIV, Hepatitis C, mycobacteria, fungus, and chemotherapy-induced immunosuppression have been implicated in their etiology. We present the case of a 2-year-old female child who developed multiple omental and mesenteric tumor nodules, 8 months post liver transplant for progressive familial intrahepatic cholestasis (PFIC). Following a histopathological diagnosis of “mesenchymal neoplasm of possible malignant nature” on a trucut biopsy and frozen section, tumor debulking was performed. A final histological diagnosis of EMIS was made on the completely resected tumor. The patient remains in remission nearly 7 months after presentation, without any follow-up systemic chemotherapy. IMT after a solid organ transplant is rare, only 5 cases have been reported in the literature until now. Similar phenomenon has also been noted with hematopoietic stem cell transplant. However, to our knowledge, this case of EMIS in a post liver transplant patient is first of its kind.http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2019;volume=62;issue=2;spage=303;epage=305;aulast=GargEIMS in post solid organ transplantepithelioid inflammatory myofibroblastic sarcoma in liver transplanted childinflammatory myofibroblastic tumor (IMT) in liverprogressive familial intrahepatic cholestasis (PFIC) |
| spellingShingle | Ritu Garg Sumaid Kaul Deepshikha Arora Vikas Kashyap Posttransplant epithelioid inflammatory myofibroblastic sarcoma: A case report Indian Journal of Pathology and Microbiology EIMS in post solid organ transplant epithelioid inflammatory myofibroblastic sarcoma in liver transplanted child inflammatory myofibroblastic tumor (IMT) in liver progressive familial intrahepatic cholestasis (PFIC) |
| title | Posttransplant epithelioid inflammatory myofibroblastic sarcoma: A case report |
| title_full | Posttransplant epithelioid inflammatory myofibroblastic sarcoma: A case report |
| title_fullStr | Posttransplant epithelioid inflammatory myofibroblastic sarcoma: A case report |
| title_full_unstemmed | Posttransplant epithelioid inflammatory myofibroblastic sarcoma: A case report |
| title_short | Posttransplant epithelioid inflammatory myofibroblastic sarcoma: A case report |
| title_sort | posttransplant epithelioid inflammatory myofibroblastic sarcoma a case report |
| topic | EIMS in post solid organ transplant epithelioid inflammatory myofibroblastic sarcoma in liver transplanted child inflammatory myofibroblastic tumor (IMT) in liver progressive familial intrahepatic cholestasis (PFIC) |
| url | http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2019;volume=62;issue=2;spage=303;epage=305;aulast=Garg |
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