Myofibroma (MF)
Overview
Myofibroma (MF) is a soft tissue neoplasm under Soft Tissue Sarcoma in OncoTree (parent: SOFT_TISSUE). Note: in the myeloproliferative neoplasms literature the abbreviation MF is commonly used for myelofibrosis; in OncoTree the primary myelofibrosis code is PMF, while MF = Myofibroma. The corpus reference below uses MF in the context of myelofibrosis patients within an MPN cohort.
Cohorts in the corpus
- mpn_cimr_2013 — 39 patients described as myelofibrosis (MF) within a 151-patient MPN whole-exome sequencing cohort. PMID:24325359
Recurrent alterations
- Among 39 myelofibrosis patients in the CALR discovery cohort, median somatic mutation burden was 13.0 per patient (significantly higher than 6.5 for ET and PV; one-way ANOVA P=0.008). JAK2 V617F was present in 27/39 (69%); CALR exon 9 frameshift indels were found in 18/32 (56%) primary MF and 12/14 (86%) post-ET MF cases lacking JAK2/MPL mutations. The L367fs*46 CALR deletion was enriched in MF vs ET (chi-square P=0.009). PMID:24325359
Subtypes
- Primary MF vs. post-ET MF and post-PV MF; CALR prevalence differs (56% vs 86% in post-ET MF for JAK2/MPL-negative cases). PMID:24325359
Therapeutic landscape
- CALR mutation discovery motivates CALR-mutant-specific therapeutic strategies beyond JAK inhibition. PMID:24325359
Sources
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