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Epithelioid Inflammatory Myofibroblastic Sarcoma Presenting as Gastrointestinal Bleed: Case Report and Literature Review

Myofibroblastic tumor is a mesenchymal neoplasm composed of myofibroblastic spindle cells with inflammatory infiltrate and considered to be of low-malignant potential tumor. Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a variant of myofibroblastic tumor with malignant characteristics;...

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Autores principales: Giannaki, Alexandra, Doganis, Dimitrios, Giamarelou, Panagiota, Konidari, Anastasia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins, Inc. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10191593/
https://www.ncbi.nlm.nih.gov/pubmed/37206935
http://dx.doi.org/10.1097/PG9.0000000000000019
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author Giannaki, Alexandra
Doganis, Dimitrios
Giamarelou, Panagiota
Konidari, Anastasia
author_facet Giannaki, Alexandra
Doganis, Dimitrios
Giamarelou, Panagiota
Konidari, Anastasia
author_sort Giannaki, Alexandra
collection PubMed
description Myofibroblastic tumor is a mesenchymal neoplasm composed of myofibroblastic spindle cells with inflammatory infiltrate and considered to be of low-malignant potential tumor. Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a variant of myofibroblastic tumor with malignant characteristics; it mainly consists of round-to-epithelioid cells with positive nuclear membrane/perinuclear immunostaining for anaplastic lymphoma kinase (ALK) receptor tyrosine kinase. A gastric EIMS case in a 7-year-old boy is discussed. Our patient presented with severe anemia and melena. Magnetic resonance imaging of the abdomen and pelvis revealed a solid tumor (2.7 × 1.9 × 2.6 cm) at the posterior stomach wall. Upper gastrointestinal endoscopy revealed an irregular, protruding, highly vascular, approximately 2 cm mass close to the gastrooesophageal junction. Endoscopic biopsies were taken for histology; tumor cells were epithelioid with eccentric nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. Immunohistochemistry showed positive staining for desmin, smooth muscle actin, epithelial membrane antigen, cluster of differentiation CD30, and strongly positive staining for ALK. Fluorescence in situ hybridization analysis confirmed the presence of ALK rearrangements. A full-thickness surgical excision of the tumor with clear margins was performed. No adjunct treatment was administered and our patient has remained in full remission at 12 months following the surgery. To the best of our knowledge, this is the first pediatric case of gastric EIMS. Raised awareness and prompt recognition of special histological and immunochemical characteristics of EIMS can lead to accurate diagnosis and targeted therapy.
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spelling pubmed-101915932023-05-18 Epithelioid Inflammatory Myofibroblastic Sarcoma Presenting as Gastrointestinal Bleed: Case Report and Literature Review Giannaki, Alexandra Doganis, Dimitrios Giamarelou, Panagiota Konidari, Anastasia JPGN Rep Review Article Myofibroblastic tumor is a mesenchymal neoplasm composed of myofibroblastic spindle cells with inflammatory infiltrate and considered to be of low-malignant potential tumor. Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a variant of myofibroblastic tumor with malignant characteristics; it mainly consists of round-to-epithelioid cells with positive nuclear membrane/perinuclear immunostaining for anaplastic lymphoma kinase (ALK) receptor tyrosine kinase. A gastric EIMS case in a 7-year-old boy is discussed. Our patient presented with severe anemia and melena. Magnetic resonance imaging of the abdomen and pelvis revealed a solid tumor (2.7 × 1.9 × 2.6 cm) at the posterior stomach wall. Upper gastrointestinal endoscopy revealed an irregular, protruding, highly vascular, approximately 2 cm mass close to the gastrooesophageal junction. Endoscopic biopsies were taken for histology; tumor cells were epithelioid with eccentric nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. Immunohistochemistry showed positive staining for desmin, smooth muscle actin, epithelial membrane antigen, cluster of differentiation CD30, and strongly positive staining for ALK. Fluorescence in situ hybridization analysis confirmed the presence of ALK rearrangements. A full-thickness surgical excision of the tumor with clear margins was performed. No adjunct treatment was administered and our patient has remained in full remission at 12 months following the surgery. To the best of our knowledge, this is the first pediatric case of gastric EIMS. Raised awareness and prompt recognition of special histological and immunochemical characteristics of EIMS can lead to accurate diagnosis and targeted therapy. Lippincott Williams & Wilkins, Inc. 2020-12-17 /pmc/articles/PMC10191593/ /pubmed/37206935 http://dx.doi.org/10.1097/PG9.0000000000000019 Text en Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY) (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Article
Giannaki, Alexandra
Doganis, Dimitrios
Giamarelou, Panagiota
Konidari, Anastasia
Epithelioid Inflammatory Myofibroblastic Sarcoma Presenting as Gastrointestinal Bleed: Case Report and Literature Review
title Epithelioid Inflammatory Myofibroblastic Sarcoma Presenting as Gastrointestinal Bleed: Case Report and Literature Review
title_full Epithelioid Inflammatory Myofibroblastic Sarcoma Presenting as Gastrointestinal Bleed: Case Report and Literature Review
title_fullStr Epithelioid Inflammatory Myofibroblastic Sarcoma Presenting as Gastrointestinal Bleed: Case Report and Literature Review
title_full_unstemmed Epithelioid Inflammatory Myofibroblastic Sarcoma Presenting as Gastrointestinal Bleed: Case Report and Literature Review
title_short Epithelioid Inflammatory Myofibroblastic Sarcoma Presenting as Gastrointestinal Bleed: Case Report and Literature Review
title_sort epithelioid inflammatory myofibroblastic sarcoma presenting as gastrointestinal bleed: case report and literature review
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10191593/
https://www.ncbi.nlm.nih.gov/pubmed/37206935
http://dx.doi.org/10.1097/PG9.0000000000000019
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