Autopsy and Case Reports
https://autopsyandcasereports.org/article/doi/10.4322/acr.2021.288
Autopsy and Case Reports
Clinical Case Report

Epithelioid inflammatory myofibroblastic sarcoma: the youngest case reported

Sajida Batool; Arvind Ahuja; Devender Singh Chauhan; Minakshi Bhardwaj; Atul Kumar Meena

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Abstract

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of the inflammatory myofibroblastic tumor. It has an aggressive clinical course and a high rate of recurrence. EIMS primarily affects children and young adults. Hereby, we report this entity in a 4-month-old infant who presented with an abdominal mass. Imaging studies revealed a large hypodense mesentery-based lesion involving the right half and mid-region of the abdomen. The mass with an attached segment of the small bowel was excised in toto. Grossly, a large encapsulated tumor was identified arising from the mesentery of the small bowel. The histological examination showed a tumor consisting of epithelioid to spindle cells loosely arranged in a myxoid background with numerous blood vessels and lymphoplasmacytic inflammatory infiltrate. On immunohistochemistry, the tumor cells showed positivity for ALK1 (nuclear), desmin, SMA, CD68, and focal positivity for CD30. A final diagnosis of EIMS of the small intestine was rendered. To the best of our knowledge, this case is the youngest reported case in literature.

Keywords

Sarcoma, Epithelioid Cells, Intestine, Small, Mesentery, Anaplastic Lymphoma Kinase

References

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Submitted date:
01/10/2021

Accepted date:
04/11/2021

Publication date:
05/25/2021

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