Gastrointestinal Manifestations in Systemic Mastocytosis: The Need of a Multidisciplinary Approach
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 origina...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-07-01
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| Series: | Cancers |
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| Online Access: | https://www.mdpi.com/2072-6694/13/13/3316 |
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| author | Magda Zanelli Marco Pizzi Francesca Sanguedolce Maurizio Zizzo Andrea Palicelli Alessandra Soriano Alessandra Bisagni Giovanni Martino Cecilia Caprera Marina Moretti Francesco Masia Loredana De Marco Elisabetta Froio Moira Foroni Giuditta Bernardelli Maria Isabel Alvarez de Celis Alessandro Giunta Francesco Merli Stefano Ascani |
| author_facet | Magda Zanelli Marco Pizzi Francesca Sanguedolce Maurizio Zizzo Andrea Palicelli Alessandra Soriano Alessandra Bisagni Giovanni Martino Cecilia Caprera Marina Moretti Francesco Masia Loredana De Marco Elisabetta Froio Moira Foroni Giuditta Bernardelli Maria Isabel Alvarez de Celis Alessandro Giunta Francesco Merli Stefano Ascani |
| author_sort | Magda Zanelli |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-10T09:52:26Z |
| format | Article |
| id | doaj.art-04e0c406550e44bcabfb033a3bd8a195 |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-10T09:52:26Z |
| publishDate | 2021-07-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cancers |
| spelling | doaj.art-04e0c406550e44bcabfb033a3bd8a1952023-11-22T02:36:03ZengMDPI AGCancers2072-66942021-07-011313331610.3390/cancers13133316Gastrointestinal Manifestations in Systemic Mastocytosis: The Need of a Multidisciplinary ApproachMagda Zanelli0Marco Pizzi1Francesca Sanguedolce2Maurizio Zizzo3Andrea Palicelli4Alessandra Soriano5Alessandra Bisagni6Giovanni Martino7Cecilia Caprera8Marina Moretti9Francesco Masia10Loredana De Marco11Elisabetta Froio12Moira Foroni13Giuditta Bernardelli14Maria Isabel Alvarez de Celis15Alessandro Giunta16Francesco Merli17Stefano Ascani18Pathology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, ItalyGeneral Pathology and Cytopathology Unit, Department of Medicine-DMED, University of Padova, 35121 Padova, ItalyPathology Unit, Policlinico Riuniti, University of Foggia, 71122 Foggia, ItalySurgical Oncology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, ItalyPathology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, ItalyDepartment of Pathology, Case Western Reserve University, Cleveland, OH 44106, USAPathology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, ItalyPathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, 05100 Terni, ItalyPathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, 05100 Terni, ItalyOncoHematology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, 05100 Terni, ItalyOncoHematology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, 05100 Terni, ItalyPathology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, ItalyPathology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, ItalyPathology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, ItalyPathology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, ItalyHematology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, ItalySurgical Oncology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, ItalyHematology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, ItalyPathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, 05100 Terni, ItalyMastocytosis 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.https://www.mdpi.com/2072-6694/13/13/3316mast cellmastocytosismast cell activationbone marrowgut |
| spellingShingle | Magda Zanelli Marco Pizzi Francesca Sanguedolce Maurizio Zizzo Andrea Palicelli Alessandra Soriano Alessandra Bisagni Giovanni Martino Cecilia Caprera Marina Moretti Francesco Masia Loredana De Marco Elisabetta Froio Moira Foroni Giuditta Bernardelli Maria Isabel Alvarez de Celis Alessandro Giunta Francesco Merli Stefano Ascani Gastrointestinal Manifestations in Systemic Mastocytosis: The Need of a Multidisciplinary Approach Cancers mast cell mastocytosis mast cell activation bone marrow gut |
| title | Gastrointestinal Manifestations in Systemic Mastocytosis: The Need of a Multidisciplinary Approach |
| title_full | Gastrointestinal Manifestations in Systemic Mastocytosis: The Need of a Multidisciplinary Approach |
| title_fullStr | Gastrointestinal Manifestations in Systemic Mastocytosis: The Need of a Multidisciplinary Approach |
| title_full_unstemmed | Gastrointestinal Manifestations in Systemic Mastocytosis: The Need of a Multidisciplinary Approach |
| title_short | Gastrointestinal Manifestations in Systemic Mastocytosis: The Need of a Multidisciplinary Approach |
| title_sort | gastrointestinal manifestations in systemic mastocytosis the need of a multidisciplinary approach |
| topic | mast cell mastocytosis mast cell activation bone marrow gut |
| url | https://www.mdpi.com/2072-6694/13/13/3316 |
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