Low‐grade myofibroblastic sarcoma: A population‐based study. (5th July 2016)

• Record Type: Journal Article
• Authors: Chan, Jason Y. K.; Gooi, Zhen; Wong, Eddy W. Y.; Ng, Siu K.; Tong, Michael C. F.; Vlantis, Alexander C.
• Is Part Of: Laryngoscope. Volume 127:Number 1(2017:Jan.)
• Page Start: 116
• Page End: 121
• Publication Date: 2016-07-05
• Subjects: Low‐grade myofibroblastic sarcoma -- myofibroblastic sarcoma; Otolaryngology -- Periodicals; 617.51005
• DOI: 10.1002/lary.26146
• Languages: English
• ISSNs: 0023-852X

Abstract

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