Low-grade myofibroblastic sarcoma: A population-based study

Laryngoscope. 2017 Jan;127(1):116-121. doi: 10.1002/lary.26146. Epub 2016 Jul 5.

Abstract

Objectives/hypothesis: Low-grade myofibroblastic sarcoma (LGMS) is a rare entity that is described as having a predilection for occurring in the head and neck region. Here we analyze its demographics, clinic-pathologic, and survival characteristics.

Study design: Retrospective database analysis.

Methods: A cohort from the Surveillance, Epidemiology, and End Results Program database of cases with LGMS between 2001 and 2012.

Results: There were 49 cases with a 5-year overall survival of 71.6% and disease- specific survival of 76.3%. The majority of cases were in patients <60 years old, female, and white ethnicity. The most common sites were the extremities in 40.8% of cases followed by the head and neck region with 26.5% of cases. Multivariate analysis showed that only older age was significantly associated with worse survival (P < .05).

Conclusions: LGMS is uncommon in the United States and occurs most commonly in the extremities followed by the head and neck region, despite an existing characterization of a predilection for the head and neck region. Treatment most commonly involves surgery, but the optimal surgical extent and/or radiotherapy needs to be further investigated.

Level of evidence: 2c Laryngoscope, 127:116-121, 2017.

Keywords: Low-grade myofibroblastic sarcoma; myofibroblastic sarcoma.

MeSH terms

  • Adult
  • Diagnosis, Differential
  • Extremities / pathology*
  • Female
  • Fibrosarcoma / epidemiology
  • Fibrosarcoma / pathology*
  • Fibrosarcoma / therapy
  • Head and Neck Neoplasms / epidemiology
  • Head and Neck Neoplasms / pathology*
  • Head and Neck Neoplasms / therapy
  • Humans
  • Male
  • Middle Aged
  • Myosarcoma / epidemiology
  • Myosarcoma / pathology*
  • Myosarcoma / therapy
  • Neoplasm Grading
  • Neoplasm Staging
  • Retrospective Studies
  • SEER Program
  • Survival Rate
  • United States / epidemiology