Gastrointestinal Manifestations in Systemic Mastocytosis: The Need of a Multidisciplinary Approach

Abstract

Mastocytosis represents a heterogeneous group of neoplastic mast cell disorders. The basic classification into a skin-limited disease and a systemic form with multi-organ involvement remains valid. Systemic mastocytosis is a disease often hard to diagnose, characterized by different symptoms originating from either the release of mast cell mediators or organ damage due to mast cell infiltration. Gastrointestinal symptoms represent one of the major causes of morbidity, being present in 60–80% of patients. A high index of suspicion by clinicians and pathologists is required to reach the diagnosis. Gastrointestinal mastocytosis can be a challenging diagnosis, as symptoms simulate other more common gastrointestinal diseases. The endoscopic appearance is generally unremarkable or nonspecific and gastrointestinal mast cell infiltration can be focal and subtle, requiring an adequate sampling with multiple biopsies by the endoscopists. Special stains, such as CD117, tryptase, and CD25, should be performed in order not to miss the gastrointestinal mast cell infiltrate. A proper patient’s workup requires a multidisciplinary approach including gastroenterologists, endoscopists, hematologists, oncologists, and pathologists. The aim of this review is to analyze the clinicopathological features of gastrointestinal involvement in systemic mastocytosis, focusing on the relevance of a multidisciplinary approach.

Clinical challenge for gastroenterologists–Gastrointestinal manifestations of systemic mastocytosis: A comprehensive review

“…Even though no remarkable endoscopic findings could be found[ 4 ], there are several endoscopic pictures described in SM, and endoscopic findings could be secondary both to MC mediator release and to MC infiltration in the GI wall or liver.…”

“…Systemic mastocytosis (SM) is, on the contrary, a condition of MCs proliferation associated with extra-cutaneous involvement, and it is more frequent in adult patients. Neoplastic MCs form focal and/or diffuse infiltrates in various internal organs, including the bone marrow (BM), spleen, liver, and gastrointestinal (GI) tract[ 2 - 4 ]. Regardless of the type of SM, the BM is involved in virtually all patients.…”

What Do We Have to Know about PD-L1 Expression in Prostate Cancer? A Systematic Literature Review (Part 6): Correlation of PD-L1 Expression with the Status of Mismatch Repair System, BRCA, PTEN, and Other Genes

“…Systematic literature reviews and metanalyses (SLRs) conducted according to the PRISMA guidelines (including an evidence-based minimum set of items for reporting) ( , accessed on 8 May 2021) are increasingly important in health care, keeping medical doctors up to date, and also representing the background for developing clinical guidelines/trials, as well as the justification for financial supports of research projects. Usually conducted by multidisciplinary teams, SLRs performed according to these guidelines could be applicable in various topics/contexts, improving the research quality not only of pure meta-analyses but also of SLRs applied to case report/series [ 215 , 216 , 217 , 218 , 219 , 220 , 221 , 222 , 223 , 224 , 225 , 226 , 227 , 228 , 229 , 230 , 231 , 232 , 233 , 234 , 235 , 236 , 237 , 238 , 239 , 240 , 241 , 242 , 243 , 244 , 245 , 246 , 247 , 248 , 249 , 250 , 251 , 252 , 253 , 254 , 255 , 256 , …”

What Do We Have to Know about PD-L1 Expression in Prostate Cancer? A Systematic Literature Review. Part 3: PD-L1, Intracellular Signaling Pathways and Tumor Microenvironment

“…They highlight various subtopics, including: PD-L1 immunohistochemical expression in PC cases, with discussion of pre-analytical and interpretation variables; correlations of PD-L1 expression with clinic-pathological features in PC patients; genetic and epigenetic regulation of PD-L1; data of pre-clinical studies (cell lines, mouse models) about the effects of experimental treatments on PD-L1 expression in PC cells; investigated correlations of PD-L1 expression with the status of mismatch repair system, BRCA, PTEN and other main genes in PC; PD-L1 expression in liquid biopsy samples; information of clinical trials, etc. We address the Readers to the other papers for further details on these subtopics [259][260][261][262][263][264][265][266][267][268][269][270][271][272][273][274][275][276].…”