Myeloid Neoplasm with PDGFRA Rearrangement Manifesting as a Retromolar Pad Mass

Amy S Duffield, Jonathan Webster, B. Douglas Smith, Julius S. Necciai, Austin McCuiston, Alisha D. Ware

Research output: Contribution to journalArticlepeer-review

Abstract

Myeloid neoplasms with PDGFRA rearrangement are rare, and most commonly present with features of chronic eosinophilic leukemia; however, they rarely manifest as acute myeloid or lymphoblastic leukemia. Patients typically present with symptoms of hypereosinophilia including cardiovascular and pulmonary symptoms. An increase in mast cells is also a common feature of this disease, and there may be elevated serum tryptase with significant clinical overlap with systemic mastocytosis. Here, we present an unusual case of a myeloid neoplasm with PDGFRA rearrangement manifesting as a retromolar pad mass in a patient with a prior diagnosis of systemic mastocytosis. This case highlights the possibility of soft tissue involvement by myeloid neoplasms with PDGFRA rearrangement in the oral cavity. The identification of this entity is of significant clinical importance because many patients can be effectively treated with tyrosine kinase inhibitors.

Original languageEnglish (US)
JournalHead and Neck Pathology
DOIs
StateAccepted/In press - 2021

Keywords

  • Eosinophilia
  • Gingiva
  • Mastocytosis
  • Oral pathology
  • PDGFRA-associated myeloproliferative neoplasm
  • Tyrosine kinase inhibitor

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Otorhinolaryngology
  • Oncology

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