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2016 ; 7
(40
): 66299-66309
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Mast cell sarcoma: new cases and literature review
#MMPMID27602777
Monnier J
; Georgin-Lavialle S
; Canioni D
; Lhermitte L
; Soussan M
; Arock M
; Bruneau J
; Dubreuil P
; Bodemer C
; Chandesris MO
; Lortholary O
; Hermine O
; Damaj G
Oncotarget
2016[Oct]; 7
(40
): 66299-66309
PMID27602777
show ga
Mast cell sarcoma (MCS) is a rare form of mastocytosis characterized by the
presence of solid tumor(s) comprising malignant mast cells that harbor
destructive infiltration capability and metastatic potential. Here, we present an
extensive literature review and report on 23 cases of MCS, including 3 new cases
from the French National Reference Center for Mastocytosis. From our analysis, it
appears that MCS can occur at any age. It can manifest de novo or, to a lesser
extent, may evolve from a previously established mastocytosis. Bone tumor is a
frequent manifestation, and symptoms of mast cell activation are rare.
Histological diagnosis can be difficult because MCS is frequently composed of
highly atypical neoplastic mast cells and can thus mimic other tumors.
Unexpectedly, the canonical KIT D816V mutation is found in only 21% of MCS;
therefore, complete KIT gene sequencing is required. The prognosis of patients
with MCS is poor, with a median survival time of less than 18 months, and
progression to mast cell leukemia is not unusual. Because conventional
chemotherapies usually fail, the role of targeted therapies and bone marrow
transplantation warrants further investigation in such aggressive neoplasms.
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