Treatment-refractory ALK-positive inflammatory myofibroblastic tumour of the oral cavity

BMJ Case Rep. 2018 Apr 11:2018:bcr2017221553. doi: 10.1136/bcr-2017-221553.

Abstract

We present a challenging case of a previously healthy 23-year-old man who developed an inflammatory myofibroblastic tumour of the hard palate, harbouring a rearrangement of the anaplastic lymphoma kinase (ALK) locus. Despite surgical intervention, radiotherapy and ALK-inhibition therapy, the tumour recurred locally and metastasised to regional lymph nodes, and the patient passed away roughly 9 months after diagnosis from local progression. The rapid progression of this patient's disease and its resistance to treatment demonstrate the potentially aggressive clinical course of inflammatory myofibroblastic tumours. ALK-inhibition therapy was unsuccessful in this ALK-positive tumour, highlighting the need for further investigation of markers predictive of disease progression and treatment response.

Keywords: cancer intervention; genetics; head and neck cancer; oral and maxillofacial surgery; radiotherapy.

Publication types

  • Case Reports

MeSH terms

  • Anaplastic Lymphoma Kinase
  • Crizotinib
  • Fatal Outcome
  • Granuloma, Plasma Cell / diagnostic imaging
  • Granuloma, Plasma Cell / pathology*
  • Granuloma, Plasma Cell / therapy
  • Humans
  • Lymph Nodes / pathology
  • Male
  • Mouth Diseases / diagnostic imaging
  • Mouth Diseases / enzymology
  • Mouth Diseases / pathology*
  • Mouth Diseases / therapy
  • Palate, Hard
  • Protein Kinase Inhibitors / therapeutic use
  • Pyrazoles / therapeutic use
  • Pyridines / therapeutic use
  • Receptor Protein-Tyrosine Kinases / antagonists & inhibitors*
  • Recurrence
  • Tomography, X-Ray Computed
  • Young Adult

Substances

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