Epithelioid inflammatory myofibroblastic sarcoma: the youngest case reported
Autops. Case Rep
;
11: e2021288, 2021. tab, graf
Article
in English
| LILACS
| ID: biblio-1249005
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.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Sarcoma
/
Intestinal Neoplasms
Type of study:
Prognostic study
Limits:
Female
/
Humans
/
Infant
Language:
English
Journal:
Autops. Case Rep
Journal subject:
Anatomia
/
Patologia Cl¡nica
/
Patologia Legal
Year:
2021
Type:
Article
Affiliation country:
India
Institution/Affiliation country:
Atal Bihari Vajpayee Institute of Medical Sciences/IN
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