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Immunology and Allergy Clinics of North... May 2014Mast cell sarcoma is a disorder that results in abnormal mast cells as identified by morphology, special stains, and in some publications, c-kit mutation analysis. It... (Review)
Review
Mast cell sarcoma is a disorder that results in abnormal mast cells as identified by morphology, special stains, and in some publications, c-kit mutation analysis. It affects animal species such as canines more commonly than humans. In humans it is a very rare condition, with variable clinical presentation. There is no standard therapy for the disorder. It can affect any age group. It is occasionally associated with systemic mastocytosis and/or urticaria pigmentosa. The prognosis of mast cell sarcoma in published literature is very poor in humans.
Topics: Adult; Aged; Animals; Bone and Bones; Child; Colon; Disease Progression; Dogs; Female; Humans; Larynx; Male; Mast Cells; Mast-Cell Sarcoma; Prognosis; Protein Kinase Inhibitors
PubMed: 24745684
DOI: 10.1016/j.iac.2014.01.004 -
Oncotarget Oct 2016Mast cell sarcoma (MCS) is a rare form of mastocytosis characterized by the presence of solid tumor(s) comprising malignant mast cells that harbor destructive... (Review)
Review
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.
Topics: Disease Progression; Humans; Mast-Cell Sarcoma; Prognosis
PubMed: 27602777
DOI: 10.18632/oncotarget.11812 -
American Journal of Hematology Oct 2022
Topics: Humans; Mast-Cell Sarcoma
PubMed: 35266568
DOI: 10.1002/ajh.26525 -
Journal of Comparative Pathology Nov 2014Lymph node metastasis in dogs with mast cell tumour has been reported as a negative prognostic indicator; however, no standardized histological criteria exist to define...
Lymph node metastasis in dogs with mast cell tumour has been reported as a negative prognostic indicator; however, no standardized histological criteria exist to define metastatic disease. The primary aim of this study was to determine whether different histological patterns of node-associated mast cells correlate with clinical outcome in dogs with mast cell tumour. A secondary goal was to propose a criteria-defined classification system for histological evaluation of lymph node metastasis. The Colorado State University Diagnostic Medicine Center database was searched for cases of canine mast cell tumours with reported lymph node metastasis or evidence of node-associated mast cells. Additional cases were obtained from a clinical trial involving sentinel lymph node mapping and node extirpation in dogs with mast cell neoplasia. Forty-one cases were identified for inclusion in the study. Demographic data, treatment and clinical outcome were collected for each case. Lymph nodes were classified according to a novel classification system (HN0-HN3) based on the number of, distribution of, and architectural disruption by, nodal mast cells. The findings of this study indicate that characterization of nodal mast cells as proposed by this novel classification system correlates with, and is prognostic for, clinical outcome in dogs with mast cell tumours.
Topics: Animals; Dog Diseases; Dogs; Female; Lymphatic Metastasis; Male; Mast-Cell Sarcoma; Sentinel Lymph Node Biopsy
PubMed: 25172053
DOI: 10.1016/j.jcpa.2014.07.004 -
British Journal of Haematology May 2020
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Humans; Leukemia, Mast-Cell; Mast-Cell Sarcoma; Middle Aged; Young Adult
PubMed: 32242922
DOI: 10.1111/bjh.16581 -
Veterinary Pathology Nov 2016A 2-tiered histologic grading scheme for canine cutaneous mast cell tumors (MCTs) is based on morphologic characteristics of neoplastic cells, including karyomegaly,...
A 2-tiered histologic grading scheme for canine cutaneous mast cell tumors (MCTs) is based on morphologic characteristics of neoplastic cells, including karyomegaly, multinucleation, nuclear pleomorphism, and mitotic figures. Aspirates from MCTs may provide the same information more quickly, inexpensively, and less invasively. This study used these criteria to develop a cytologic grading scheme for canine MCTs to predict outcome. Three anatomic pathologists graded histologic samples from 152 canine MCTs. Three clinical pathologists evaluated aspirates from these masses using similar criteria. A cytologic grading scheme was created based on correlation with histologic grade and evaluated with a kappa statistic. Survival was evaluated with Kaplan-Meier survival curves. Cox proportional hazards regression was used to estimate hazard ratios for tumor grades and individual grading components. Simple logistic regression tested for relationships between risk factors and mortality. The cytologic grading scheme that best correlated with histology (kappa = 0.725 ± 0.085) classified a tumor as high grade if it was poorly granulated or had at least 2 of 4 findings: mitotic figures, binucleated or multinucleated cells, nuclear pleomorphism, or >50% anisokaryosis. The cytologic grading scheme had 88% sensitivity and 94% specificity relative to histologic grading. Dogs with histologic and cytologic high grade MCTs were 39 times and 25 times more likely to die within the 2-year follow-up period, respectively, than dogs with low grade MCTs. High tumor grade was associated with increased probability of additional tumors or tumor regrowth. This study concluded that cytologic grade is a useful predictor for treatment planning and prognostication.
Topics: Animals; Dog Diseases; Dogs; Female; Male; Mast-Cell Sarcoma; Neoplasm Grading; Prognosis; Skin; Skin Neoplasms
PubMed: 27034386
DOI: 10.1177/0300985816638721 -
Veterinary Pathology Sep 1984Eighty-three cutaneous mast cell tumors in the dog were classified morphologically into three grades. The neoplasms were excised surgically, and the dogs were followed... (Comparative Study)
Comparative Study
Eighty-three cutaneous mast cell tumors in the dog were classified morphologically into three grades. The neoplasms were excised surgically, and the dogs were followed for 1500 days. Comparison of morphologic grade of tumor with survival time revealed significant differences among the three groups (p less than 0.00010). Comparison of sex and age (above and below ten years) with morphologic grade of tumor revealed no significant differences.
Topics: Animals; Dog Diseases; Dogs; Female; Follow-Up Studies; Male; Mast-Cell Sarcoma; Skin Neoplasms
PubMed: 6435301
DOI: 10.1177/030098588402100503 -
Journal of Clinical Oncology : Official... Feb 2013
Review
Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Fatal Outcome; Genotype; Humans; Immunohistochemistry; Interleukin-2 Receptor alpha Subunit; Ki-1 Antigen; Male; Mast Cells; Mast-Cell Sarcoma; Proto-Oncogene Proteins c-kit
PubMed: 23129735
DOI: 10.1200/JCO.2012.41.9549 -
American Journal of Hematology Mar 2023
Topics: Humans; Mast-Cell Sarcoma; Mast Cells
PubMed: 36464921
DOI: 10.1002/ajh.26804 -
Modern Pathology : An Official Journal... Apr 2013Mast cell sarcoma is a rare, aggressive neoplasm composed of cytologically malignant mast cells presenting as a solitary mass. Previous descriptions of mast cell sarcoma...
Mast cell sarcoma is a rare, aggressive neoplasm composed of cytologically malignant mast cells presenting as a solitary mass. Previous descriptions of mast cell sarcoma have been limited to single case reports, and the pathologic features of this entity are not well known. Here, we report three new cases of mast cell sarcoma and review previously reported cases. Mast cell sarcoma has a characteristic morphology of medium-sized to large epithelioid cells, including bizarre multinucleated cells, and does not closely resemble either normal mast cells or the spindle cells of systemic mastocytosis. One of our three cases arose in a patient with a remote history of infantile cutaneous mastocytosis, an association also noted in one previous case report. None of our three cases were correctly diagnosed as mast cell neoplasms on initial pathological evaluation, suggesting that this entity may be under-recognized. Molecular testing of mast cell sarcoma has not thus far detected the imatinib-resistant KIT D816V mutation, suggesting that recognition of these cases may facilitate specific targeted therapy.
Topics: Aged; Child; Female; Humans; Male; Mast-Cell Sarcoma; Young Adult
PubMed: 23196796
DOI: 10.1038/modpathol.2012.199