Epithelioid inflammatory myofibroblastic sarcoma – A rare case report
Inflammatory myofibroblastic tumors are mesenchymal neoplasms made up of spindle cells with inflammatory infiltrates and are intermediate-grade tumors. Inflammatory Myofibroblastic Tumor (IMT) has a variant known as Epithelioid Inflammatory Myofibroblastic Sarcoma (EIMS) with aggressive behavior. Mo...
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| Format: | Article |
| Language: | English |
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Elsevier
2024-03-01
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| Series: | Human Pathology Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2772736X23000361 |
| _version_ | 1797272439918428160 |
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| author | Dona Maria George Archana Lakshmanan S Annapurneswari Karuppasamy Usharani |
| author_facet | Dona Maria George Archana Lakshmanan S Annapurneswari Karuppasamy Usharani |
| author_sort | Dona Maria George |
| collection | DOAJ |
| description | Inflammatory myofibroblastic tumors are mesenchymal neoplasms made up of spindle cells with inflammatory infiltrates and are intermediate-grade tumors. Inflammatory Myofibroblastic Tumor (IMT) has a variant known as Epithelioid Inflammatory Myofibroblastic Sarcoma (EIMS) with aggressive behavior. Morphologically they have round to epitheliod cells that are positive for the Anaplastic Lymphoma Kinase (ALK) receptor tyrosine kinase. The abdomen and pelvis are the most common sites of involvement. Children and young adults are frequently affected. We report a rare case of EIMS arising on the serosal aspect of the ileocaecal junction in a 33-year-old male patient. Morphologically there were sheets and loose fascicles of epithelioid cells admixed with inflammatory cells in a variably myxoid background. Tumor cells were positive for Vimentin, Desmin, CD30, and Smooth muscle actin and showed cytoplasmic and nuclear membrane ALK positivity. Molecular studies in our case showed rearrangement of ALK by Vysis LSI ALK dual color break apart FISH probe. To our knowledge, this is the fifty-ninth reported case to date. Our case report highlights the clinical, histopathological, and immunohistochemical features of EIMS. Given their rarity and novelty, increased understanding and correct identification of the histological and immunohistochemical features of EIMS can result in an accurate diagnosis and focused treatment. |
| first_indexed | 2024-03-07T14:28:18Z |
| format | Article |
| id | doaj.art-b67796485de94b02a32407da103d9e9a |
| institution | Directory Open Access Journal |
| issn | 2772-736X |
| language | English |
| last_indexed | 2024-03-07T14:28:18Z |
| publishDate | 2024-03-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Human Pathology Reports |
| spelling | doaj.art-b67796485de94b02a32407da103d9e9a2024-03-06T05:29:15ZengElsevierHuman Pathology Reports2772-736X2024-03-0135300726Epithelioid inflammatory myofibroblastic sarcoma – A rare case reportDona Maria George0Archana Lakshmanan1S Annapurneswari2Karuppasamy Usharani3Corresponding author at: Department of Histopathology, Apollo Hospitals, Greams Road, Chennai, Tamil Nadu, India.; Department of Histopathology Apollo Main Hospital, Chennai, IndiaDepartment of Histopathology Apollo Main Hospital, Chennai, IndiaDepartment of Histopathology Apollo Main Hospital, Chennai, IndiaDepartment of Histopathology Apollo Main Hospital, Chennai, IndiaInflammatory myofibroblastic tumors are mesenchymal neoplasms made up of spindle cells with inflammatory infiltrates and are intermediate-grade tumors. Inflammatory Myofibroblastic Tumor (IMT) has a variant known as Epithelioid Inflammatory Myofibroblastic Sarcoma (EIMS) with aggressive behavior. Morphologically they have round to epitheliod cells that are positive for the Anaplastic Lymphoma Kinase (ALK) receptor tyrosine kinase. The abdomen and pelvis are the most common sites of involvement. Children and young adults are frequently affected. We report a rare case of EIMS arising on the serosal aspect of the ileocaecal junction in a 33-year-old male patient. Morphologically there were sheets and loose fascicles of epithelioid cells admixed with inflammatory cells in a variably myxoid background. Tumor cells were positive for Vimentin, Desmin, CD30, and Smooth muscle actin and showed cytoplasmic and nuclear membrane ALK positivity. Molecular studies in our case showed rearrangement of ALK by Vysis LSI ALK dual color break apart FISH probe. To our knowledge, this is the fifty-ninth reported case to date. Our case report highlights the clinical, histopathological, and immunohistochemical features of EIMS. Given their rarity and novelty, increased understanding and correct identification of the histological and immunohistochemical features of EIMS can result in an accurate diagnosis and focused treatment.http://www.sciencedirect.com/science/article/pii/S2772736X23000361Epithelioid inflammatory myofibroblastic sarcomaEIMSAnaplastic lymphoma kinaseALK-positive tumorGastrointestinal epithelioid sarcoma |
| spellingShingle | Dona Maria George Archana Lakshmanan S Annapurneswari Karuppasamy Usharani Epithelioid inflammatory myofibroblastic sarcoma – A rare case report Human Pathology Reports Epithelioid inflammatory myofibroblastic sarcoma EIMS Anaplastic lymphoma kinase ALK-positive tumor Gastrointestinal epithelioid sarcoma |
| title | Epithelioid inflammatory myofibroblastic sarcoma – A rare case report |
| title_full | Epithelioid inflammatory myofibroblastic sarcoma – A rare case report |
| title_fullStr | Epithelioid inflammatory myofibroblastic sarcoma – A rare case report |
| title_full_unstemmed | Epithelioid inflammatory myofibroblastic sarcoma – A rare case report |
| title_short | Epithelioid inflammatory myofibroblastic sarcoma – A rare case report |
| title_sort | epithelioid inflammatory myofibroblastic sarcoma a rare case report |
| topic | Epithelioid inflammatory myofibroblastic sarcoma EIMS Anaplastic lymphoma kinase ALK-positive tumor Gastrointestinal epithelioid sarcoma |
| url | http://www.sciencedirect.com/science/article/pii/S2772736X23000361 |
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