Epithelioid inflammatory myofibroblastic sarcoma treated with ALK inhibitor: a case report and review of literature

Int J Clin Exp Pathol. 2015 Nov 1;8(11):15328-32. eCollection 2015.

Abstract

Epithelioid inflammatory myofibroblastic sarcoma is extremely rare and belongs to a variant of inflammatory myofibrobalstic tumor with aggressive clinical course. We describe a case of a 22 years old man presented with an abdominal huge tumor. Microscopically, the neoplasm cells were rounded and epithelioid in shape. Abundant interstitial edema and less myxoid stroma were also present together with an inflammatory infiltrate. Fluorescence in situ hybridization revealed that ALK gene presented mutation. After surgery the patient received chemotherapy with an anaplastic lymphoma kinase (ALK) inhibitor, crizotinib. The patient continues to be alive with disease for 16 months and has no recurrence. Although EIMS has a poor prognosis, this is the few successful case with sustained response of targeted therapy.

Keywords: ALK inhibitor; Epithelioid inflammatory myofibroblastic sarcoma; RANBP2-ALK fusion; crizotinib.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Abdominal Neoplasms / drug therapy*
  • Abdominal Neoplasms / enzymology
  • Abdominal Neoplasms / genetics
  • Abdominal Neoplasms / pathology
  • Abdominal Neoplasms / surgery
  • Anaplastic Lymphoma Kinase
  • Antineoplastic Agents / therapeutic use*
  • Chemotherapy, Adjuvant
  • Crizotinib
  • DNA Mutational Analysis
  • Epithelioid Cells / drug effects*
  • Epithelioid Cells / enzymology
  • Epithelioid Cells / pathology
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization, Fluorescence
  • Male
  • Molecular Targeted Therapy
  • Mutation
  • Myofibroblasts / drug effects*
  • Myofibroblasts / enzymology
  • Myofibroblasts / pathology
  • Protein Kinase Inhibitors / therapeutic use*
  • Pyrazoles / therapeutic use*
  • Pyridines / therapeutic use*
  • Receptor Protein-Tyrosine Kinases / antagonists & inhibitors*
  • Receptor Protein-Tyrosine Kinases / genetics
  • Receptor Protein-Tyrosine Kinases / metabolism
  • Sarcoma / drug therapy*
  • Sarcoma / enzymology
  • Sarcoma / genetics
  • Sarcoma / pathology
  • Sarcoma / surgery
  • Time Factors
  • Tomography, X-Ray Computed
  • Treatment Outcome
  • Tumor Burden
  • Young Adult

Substances

  • Antineoplastic Agents
  • Protein Kinase Inhibitors
  • Pyrazoles
  • Pyridines
  • Crizotinib
  • ALK protein, human
  • Anaplastic Lymphoma Kinase
  • Receptor Protein-Tyrosine Kinases