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Actas Dermo-sifiliograficas 2016Mastocytosis is a term used to describe a heterogeneous group of disorders characterized by clonal proliferation of mast cells in different organs. The organ most often... (Review)
Review
Mastocytosis is a term used to describe a heterogeneous group of disorders characterized by clonal proliferation of mast cells in different organs. The organ most often affected is the skin. The World Health Organization classifies cutaneous mastocytosis into mastocytoma, maculopapular cutaneous mastocytosis, and diffuse mastocytosis. The systemic variants in this classification are as follows: indolent systemic mastocytosis (SM), aggressive SM, SM with an associated clonal hematological non-mast cell lineage disease, mast cell leukemia, mast cell sarcoma, and extracutaneous mastocytoma. The two latest systemic variants are rare. Although the course of disease is unpredictable in children, lesions generally resolve by early adulthood. In adults, however, the disease tends to persist. The goal of treatment should be to control clinical manifestations caused by the release of mast cell mediators and, in more aggressive forms of the disease, to reduce mast cell burden.
Topics: Humans; Leukemia, Mast-Cell; Mast Cells; Mast-Cell Sarcoma; Mastocytosis; Mastocytosis, Cutaneous; Mastocytosis, Systemic; Prognosis
PubMed: 26525106
DOI: 10.1016/j.ad.2015.09.009 -
The Veterinary Clinics of North... Sep 2019Tumor microenvironment represents a key obstacle for the effectiveness of anticancer drugs. Electrochemotherapy involves the systemic or local delivery of lipophobic... (Review)
Review
Tumor microenvironment represents a key obstacle for the effectiveness of anticancer drugs. Electrochemotherapy involves the systemic or local delivery of lipophobic drugs such as bleomycin and cisplatin, with the application of permeabilizing electric pulses having appropriate amplitude and waveforms. This greatly enhances the uptake of these drugs by an estimated factor of 700-fold for bleomycin and 4 to 8 times for cisplatin. Because of its efficacy and limited morbidity, this therapeutic option is becoming more and more available in veterinary oncology either as an adjuvant to surgery or as first line of treatment with palliative or curative purposes.
Topics: Animals; Antineoplastic Agents; Cat Diseases; Cats; Dog Diseases; Dogs; Electrochemotherapy; Medical Oncology; Neoplasms; Veterinary Medicine
PubMed: 31176458
DOI: 10.1016/j.cvsm.2019.04.006 -
Blood Mar 2023
Topics: Humans; Leukemia, Mast-Cell; Mast-Cell Sarcoma; Sarcoma; Lymphohistiocytosis, Hemophagocytic; Mast Cells
PubMed: 36929432
DOI: 10.1182/blood.2022018256 -
Journal of the American Veterinary... Nov 2023To determine the prevalence of splenic malignancy in cats undergoing splenectomy and to investigate possible factors associated with post-operative outcome.
OBJECTIVE
To determine the prevalence of splenic malignancy in cats undergoing splenectomy and to investigate possible factors associated with post-operative outcome.
ANIMALS
62 client-owned cats that underwent splenectomy.
METHODS
Medical records of 4 UK-based referral hospitals were searched and data reviewed retrospectively over 17 years. Factors associated with outcomes post-splenectomy were analyzed.
RESULTS
50 out of 62 cats (81%) were diagnosed with splenic neoplasia. Mast cell tumor ([MCT], 42%), hemangiosarcoma ([HSA], 40%), lymphoma and histiocytic sarcoma (6% each) were the most common tumor types. Fifteen cats (24%) presented with spontaneous hemoabdomen and were all diagnosed with splenic neoplasia. The diagnostic accuracy of cytology to detect splenic malignant lesions was 73% (100% for MCTs and 54% for mesenchymal tumors). Median survival time for cats with nonneoplastic splenic lesions was 715 days (IQR, 18 to 1,368) and 136 days for cats with splenic neoplasia (IQR, 35 to 348); median survival time was longer for cats with splenic MCT when compared to cats with HSA (348 vs 94 days; P < .001). Presence of metastatic disease and anemia (PCV < 24%) at diagnosis were associated with a poorer survival when considering all cats. Presence of anemia, a splenic mass on imaging or spontaneous hemoabdomen were associated with a diagnosis of HSA (P < .001).
CLINICAL RELEVANCE
Benign splenic lesions were uncommon in this cohort of cats. Spontaneous hemoabdomen should prompt the clinician to suspect neoplasia in cats with splenic disease. Anemia and evidence of metastasis at diagnosis were poor prognostic factors regardless of the final diagnosis.
Topics: Humans; Cats; Animals; Dogs; Splenectomy; Retrospective Studies; Prevalence; Splenic Neoplasms; Anemia; Hemoperitoneum; Dog Diseases; Hemangiosarcoma; Cat Diseases
PubMed: 37582488
DOI: 10.2460/javma.23.05.0258 -
Annals of Oncology : Official Journal... Sep 2014Mast cell leukemia (MCL), the leukemic manifestation of systemic mastocytosis (SM), is characterized by leukemic expansion of immature mast cells (MCs) in the bone... (Review)
Review
Mast cell leukemia (MCL), the leukemic manifestation of systemic mastocytosis (SM), is characterized by leukemic expansion of immature mast cells (MCs) in the bone marrow (BM) and other internal organs; and a poor prognosis. In a subset of patients, circulating MCs are detectable. A major differential diagnosis to MCL is myelomastocytic leukemia (MML). Although criteria for both MCL and MML have been published, several questions remain concerning terminologies and subvariants. To discuss open issues, the EU/US-consensus group and the European Competence Network on Mastocytosis (ECNM) launched a series of meetings and workshops in 2011-2013. Resulting discussions and outcomes are provided in this article. The group recommends that MML be recognized as a distinct condition defined by mastocytic differentiation in advanced myeloid neoplasms without evidence of SM. The group also proposes that MCL be divided into acute MCL and chronic MCL, based on the presence or absence of C-Findings. In addition, a primary (de novo) form of MCL should be separated from secondary MCL that typically develops in the presence of a known antecedent MC neoplasm, usually aggressive SM (ASM) or MC sarcoma. For MCL, an imminent prephase is also proposed. This prephase represents ASM with rapid progression and 5%-19% MCs in BM smears, which is generally accepted to be of prognostic significance. We recommend that this condition be termed ASM in transformation to MCL (ASM-t). The refined classification of MCL fits within and extends the current WHO classification; and should improve prognostication and patient selection in practice as well as in clinical trials.
Topics: Bone Marrow Examination; Diagnosis, Differential; Disease Progression; Humans; Leukemia, Mast-Cell; Leukemia, Myelomonocytic, Acute; Leukemia, Myelomonocytic, Chronic; Mast Cells; Mastocytosis
PubMed: 24675021
DOI: 10.1093/annonc/mdu047 -
American Journal of Clinical Pathology May 2023Sessions 8 and 9 of the 2021 Society for Hematopathology and the European Association for Haematopathology Workshop aimed to collect examples of transdifferentiation,...
OBJECTIVES
Sessions 8 and 9 of the 2021 Society for Hematopathology and the European Association for Haematopathology Workshop aimed to collect examples of transdifferentiation, lineage infidelity, progression, and transformation in precursor and mature T/natural killer (NK)-cell neoplasms.
METHODS
Twenty-eight cases were submitted and analyzed, with whole-exome sequencing and genome-wide RNA expression analysis performed in a subset of the cases.
RESULTS
In session 8, 7 T-lymphoblastic lymphoma/leukemia cases were received that showed transdifferentiation to clonally related mature myeloid hematopoietic neoplasms, including 6 histiocytic/dendritic cell lineage neoplasms and a mast cell sarcoma. Session 9 included 21 mature T-cell neoplasms that were grouped into 3 themes. The first one addressed phenotypic infidelity in mature T-cell lymphomas (TCLs) and included 8 TCLs expressing aberrant antigens, mimicking classic Hodgkin and non-Hodgkin B-cell lymphomas. The second theme addressed disease progression in TCL and included 5 cutaneous T-cell lymphoproliferative disorders and 2 T-cell large granular lymphocyte proliferations with subsequent progression to systemic TCL. The third theme included 6 patients with TCL with T-follicular helper phenotype, mainly angioimmunoblastic T-cell lymphoma, with concurrent/subsequent clonal hematopoiesis or myeloid neoplasms and/or subsequent/concomitant diffuse large B-cell lymphoma.
CONCLUSIONS
This cohort of cases allowed us to illustrate, discuss, and review current concepts of transdifferentiation, aberrant antigen expression, and progression in various T/NK-cell neoplasms.
PubMed: 37167533
DOI: 10.1093/ajcp/aqad045 -
The Veterinary Clinics of North... May 2015Surgical oncology is experiencing rapid transition in veterinary medicine. Mast cell tumors and soft tissue sarcomas are two of the most common neoplasms in small animal... (Review)
Review
Surgical oncology is experiencing rapid transition in veterinary medicine. Mast cell tumors and soft tissue sarcomas are two of the most common neoplasms in small animal patients. Clinicians should be familiar with the need for staging and the procedures involved in treating patients with these tumors. Clinicians should be comfortable with available adjuvant therapies and when to use them in certain patients.
Topics: Animals; Cats; Dogs; Mastocytosis; Sarcoma; Surgery, Veterinary
PubMed: 25758851
DOI: 10.1016/j.cvsm.2015.01.003 -
Postgraduate Medicine Nov 2017Mastocytosis, a heterogeneous group of disorders, is characterized by an abnormal increase in the number of mast cells that is limited to the skin (cutaneous... (Meta-Analysis)
Meta-Analysis Review
INTRODUCTION
Mastocytosis, a heterogeneous group of disorders, is characterized by an abnormal increase in the number of mast cells that is limited to the skin (cutaneous mastocytosis), involving extracutaneous tissues (systemic mastocytosis), or presenting as solid tumours (mastocytoma and mast cell sarcoma). Recent studies estimate that 1 in 10,000 people are diagnosed with mastocytosis. Although prompt diagnosis and appropriate management are crucial, little is known about the natural history and currently there are no established management guidelines. We have conducted a systematic review to assess the natural history and management of different mastocytosis subtypes.
METHODS
A systematic review and meta-analysis were conducted using the PubMed and Ovid database of studies published in English and French over the last fifteen years, from January 2001 to December 2016. Keywords 'Cutaneous mastocytosis', 'Systemic mastocytosis', 'pathophysiology', 'clinical course', 'prognosis', 'drug therapy', and 'therapy' were searched. Rate of complete resolution was subjected to pooled analysis for different mastocytosis subtypes. Meta-analysis was conducted using Stata version 12.0.
RESULTS
We reviewed 634 papers, of which 5 were included in the analysis of resolution, and 138 were included in the assessment of management. Pooled estimate for rate of complete resolution varied depending on the mastocytosis subtype. In cutaneous mastocytosis, the complete resolution rate for mastocytoma was 10% per year (95% CI: 4.8%, 15.1%) while the rate for urticaria pigmentosa was 1.9% per year (95% CI: -0.5%, 4.3%). Diffuse cutaneous mastocytosis and systemic mastocytosis subtypes did not show evidence of complete resolution in the studies reviewed. Treatment of cutaneous and systemic mastocytosis is purely symptomatic with topical corticosteroids, antihistamines, omalizumab and imatinib being common choices.
CONCLUSION
Rate of resolution of mastocytosis is only shown in urticaria pigmentosa and mastocytoma. Better management guidelines are required to improve the health of these patients.
Topics: Adrenal Cortex Hormones; Biological Products; Combined Modality Therapy; Histamine Antagonists; Humans; Mastocytosis; Mastocytosis, Cutaneous; Mastocytosis, Systemic; Phototherapy
PubMed: 28770635
DOI: 10.1080/00325481.2017.1364124 -
Journal of the American Veterinary... Sep 2023To determine the incidence of histologic grade shift (alteration of grade relative to the original tumor) in recurrent canine soft tissue sarcoma (STS) and mast cell...
OBJECTIVE
To determine the incidence of histologic grade shift (alteration of grade relative to the original tumor) in recurrent canine soft tissue sarcoma (STS) and mast cell tumor (MCT), and to determine the level of agreement between blinded pathologist review and original histology interpretation of STS and MCT grades.
ANIMALS
15 dogs with recurrent cutaneous/subcutaneous STS and 5 dogs with recurrent cutaneous MCT. All included dogs underwent excision of both the primary and recurrent tumors and had tumor samples available for review.
PROCEDURES
The medical records and histology database from a single institution were reviewed, and data were recorded and analyzed. A single board-certified veterinary pathologist performed blinded evaluation of all excisional tumor samples, including both primary and recurrent disease, and these were evaluated independently and in conjunction with initial pathologic diagnoses.
RESULTS
Based on single pathologist review, 7 of 15 (46.7%) dogs with recurrent STS had grade shift characterized by a higher or lower recurrent tumor grade in 4 of 7 and 3 of 7 cases, respectively, and 1 of 5 dogs with recurrent MCT had grade shift characterized by an increased grade of the recurrent tumor. Variability in reported grade between original histology report and pathologist review occurred for 13 of 30 (43.3%) STS excisional biopsy samples and 0 of 10 MCT excisional biopsy samples.
CLINICAL RELEVANCE
Grade shift has been reported in multiple tumor types in people and has the potential to alter prognosis and treatment recommendations. This is the first study to document this phenomenon in dogs. Additional large-scale studies are needed to determine factors associated with grade shift as well as prognostic significance of grade shift for recurrent canine STS and MCT.
Topics: Animals; Dogs; Female; Male; Dog Diseases; Incidence; Recurrence; Retrospective Studies; Soft Tissue Neoplasms; Sarcoma
PubMed: 37257826
DOI: 10.2460/javma.23.01.0050 -
Frontiers in Immunology 2023Hypermethylated in Cancer 1 (HIC1) was originally confirmed as a tumor suppressor and has been found to be hypermethylated in human cancers. Although growing evidence...
BACKGROUND
Hypermethylated in Cancer 1 (HIC1) was originally confirmed as a tumor suppressor and has been found to be hypermethylated in human cancers. Although growing evidence has supported the critical roles of HIC1 in cancer initiation and development, its roles in tumor immune microenvironment and immunotherapy are still unclear, and no comprehensive pan-cancer analysis of HIC1 has been conducted.
METHODS
HIC1 expression in pan-cancer, and differential HIC1 expression between tumor and normal samples were investigated. Immunohistochemistry (IHC) was employed to validate HIC1 expression in different cancers by our clinical cohorts, including lung cancer, sarcoma (SARC), breast cancer, and kidney renal clear cell carcinoma (KIRC). The prognostic value of HIC1 was illustrated by Kaplan-Meier curves and univariate Cox analysis, followed by the genetic alteration analysis of HIC1 in pan-cancer. Gene Set Enrichment Analysis (GSEA) was conducted to illustrate the signaling pathways and biological functions of HIC1. The correlations between HIC1 and tumor mutation burden (TMB), microsatellite instability (MSI), and the immunotherapy efficacy of PD-1/PD-L1 inhibitors were analyzed by Spearman correlation analysis. Drug sensitivity analysis of HIC1 was performed by extracting data from the CellMiner™ database.
RESULTS
HIC1 expression was abnormally expressed in most cancers, and remarkable associations between HIC1 expression and prognostic outcomes of patients in pan-cancer were detected. HIC1 was significantly correlated with T cells, macrophages, and mast cell infiltration in different cancers. Moreover, GSEA revealed that HIC1 was significantly involved in immune-related biological functions and signaling pathways. There was a close relationship of HIC1 with TMB and MSI in different cancers. Furthermore, the most exciting finding was that HIC1 expression was significantly correlated with the response to PD-1/PD-L1 inhibitors in cancer treatment. We also found that HIC1 was significantly correlated with the sensitivity of several anti-cancer drugs, such as axitinib, batracylin, and nelarabine. Finally, our clinical cohorts further validated the expression pattern of HIC1 in cancers.
CONCLUSIONS
Our investigation provided an integrative understanding of the clinicopathological significance and functional roles of HIC1 in pan-cancer. Our findings suggested that HIC1 can function as a potential biomarker for predicting the prognosis, immunotherapy efficacy, and drug sensitivity with immunological activity in cancers.
Topics: Humans; Ferroptosis; Immune Checkpoint Inhibitors; Programmed Cell Death 1 Receptor; Prognosis; Carcinoma, Renal Cell; Kidney Neoplasms; Tumor Microenvironment; Kruppel-Like Transcription Factors
PubMed: 37388742
DOI: 10.3389/fimmu.2023.1182030