TY - JOUR
T1 - Gastrointestinal manifestations in systemic mastocytosis: The need of a multidisciplinary approach
AU - Zanelli, Magda
AU - Pizzi, Marco
AU - Sanguedolce, Francesca
AU - Zizzo, Maurizio
AU - Palicelli, Andrea
AU - Soriano, Alessandra
AU - Bisagni, Alessandra
AU - Martino, Giovanni
AU - Caprera, Cecilia
AU - Moretti, Marina
AU - Masia, Francesco
AU - De Marco, Loredana
AU - Froio, Elisabetta
AU - Foroni, Moira
AU - Bernardelli, Giuditta
AU - de Celis, Maria Isabel Alvarez
AU - Giunta, Alessandro
AU - Merli, Francesco
AU - Ascani, Stefano
N1 - Publisher Copyright:
© 2021 by the authors. Licensee MDPI, Basel, Switzerland.
PY - 2021/7/1
Y1 - 2021/7/1
N2 - 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 clinicopatholog-ical features of gastrointestinal involvement in systemic mastocytosis, focusing on the relevance of a multidisciplinary approach.
AB - 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 clinicopatholog-ical features of gastrointestinal involvement in systemic mastocytosis, focusing on the relevance of a multidisciplinary approach.
KW - Bone marrow
KW - Gut
KW - Mast cell
KW - Mast cell activation
KW - Mastocytosis
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U2 - 10.3390/cancers13133316
DO - 10.3390/cancers13133316
M3 - Review article
AN - SCOPUS:85108875236
VL - 13
JO - Cancers
JF - Cancers
SN - 2072-6694
IS - 13
M1 - 3316
ER -