Epithelioid Inflammatory Myofibroblastic Sarcoma of the Ovary with RANB2-ALK Fusion: Report of a Case

Hong Fang, Carrie L. Langstraat, Daniel W Visscher, Andrew L. Folpe, J. Kenneth Schoolmeester

Research output: Contribution to journalArticle

5 Scopus citations

Abstract

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a recently described, clinically aggressive variant of inflammatory myofibroblastic tumor with a predilection for intraabdominal sites, tendency to arise in male patients and resistance to conventional chemotherapy and radiotherapy. We present a case of a 15-year-old girl with EIMS of the ovary with a RANBP2-ALK fusion. During 2 years of follow up, she experienced multiple recurrences. Following classification of the tumor as EIMS, the patient began treatment with an ALK inhibitor (crizotinib), which led to disease improvement within weeks of administration. To the best of our knowledge, this represents the first reported case of EIMS arising in the female genital tract.

Original languageEnglish (US)
Pages (from-to)468-472
Number of pages5
JournalInternational Journal of Gynecological Pathology
Volume37
Issue number5
DOIs
StatePublished - Sep 1 2018

Keywords

  • ALK
  • Crizotinib
  • Epithelioid inflammatory myofibroblastic sarcoma
  • Inflammatory myofibroblastic tumor
  • Ovary
  • RANBP2

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Obstetrics and Gynecology

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