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Low-grade myofibroblastic sarcoma: A population-based study
Jason Y K Chan1, Zhen Gooi2, Eddy W Y Wong1
1Department of Otorhinolaryngology-Head and Neck Surgery, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong.
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.
