title: In vivo model for mastocytosis: A comparative review.
abstract: Human mastocytosis are heterogeneous group of neoplastic diseases characterized by a different degree of uncontrolled mast cell (MC) proliferation and activation. Interestingly, human mastocytosis share several biological and clinical features with canine mast cell disorders, so called canine mast cell tumors (CMCTs). These CMCTs are the most common spontaneous cutaneous tumors found in dogs representing a valid model to study neoplastic mast cell disorders. It has been discovered that the pathological activation of c-Kit receptor (c-KitR), expressed by MCs, has been involved in the pathogenesis of neoplastic MC disorders. In this review we have focused on human mastocytosis in terms of: (i) epidemiology and classification; (ii) pathogenesis at molecular levels; (iii) clinical presentation. In addition, we have summarized animal models useful to study neoplastic MC disorders including CMCTs and murine transgenic models. Finally, we have revised therapeutic approaches mostly common in human and canine MCTs and novel tyrosine kinase inhibitors approved for CMCTs and recently translated in human clinical trials.
authors.affiliation: Diagnostic and Interventional Radiology Unit with Integrated Section of Translational Medical Oncology, National Cancer Research Centre Istituto Tumori "Giovanni Paolo II", Bari, Italy. Electronic address: giroran@tiscalinet.it.
authors.forename: Girolamo
authors.initials: G
authors.lastname: Ranieri
authors.affiliation: Diagnostic and Interventional Radiology Unit with Integrated Section of Translational Medical Oncology, National Cancer Research Centre Istituto Tumori "Giovanni Paolo II", Bari, Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Marech
authors.affiliation: Department of Emergency and Organ Transplantation (D.E.T.O.), Veterinary Medical School, Università "Aldo Moro", Bari, Italy.
authors.forename: Marianna
authors.initials: M
authors.lastname: Pantaleo
authors.affiliation: Department of Emergency and Organ Transplantation (D.E.T.O.), Veterinary Medical School, Università "Aldo Moro", Bari, Italy.
authors.forename: Mariagrazia
authors.initials: M
authors.lastname: Piccinno
authors.affiliation: Department of Emergency and Organ Transplantation (D.E.T.O.), Veterinary Medical School, Università "Aldo Moro", Bari, Italy.
authors.forename: Maria
authors.initials: M
authors.lastname: Roncetti
authors.affiliation: Department of Emergency and Organ Transplantation (D.E.T.O.), Veterinary Medical School, Università "Aldo Moro", Bari, Italy.
authors.forename: Maddalena
authors.initials: M
authors.lastname: Mutinati
authors.affiliation: Department of Emergency and Organ Transplantation (D.E.T.O.), Veterinary Medical School, Università "Aldo Moro", Bari, Italy.
authors.forename: Annalisa
authors.initials: A
authors.lastname: Rizzo
authors.affiliation: Diagnostic and Interventional Radiology Unit with Integrated Section of Translational Medical Oncology, National Cancer Research Centre Istituto Tumori "Giovanni Paolo II", Bari, Italy.
authors.forename: Cosmo Damiano
authors.initials: CD
authors.lastname: Gadaleta
authors.affiliation: Department of Pathology, Veterinary Medical School, Università "Aldo Moro", Bari, Italy.
authors.forename: Marcello
authors.initials: M
authors.lastname: Introna
authors.affiliation: Department of Prevention and Animal Health, ASL BAT, Barletta, Italy.
authors.forename: Rosa
authors.initials: R
authors.lastname: Patruno
authors.affiliation: Department of Emergency and Organ Transplantation (D.E.T.O.), Veterinary Medical School, Università "Aldo Moro", Bari, Italy.
authors.forename: Raffaele Luigi
authors.initials: RL
authors.lastname: Sciorsci
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D006801:Humans; D008415:Mastocytosis / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology / Q000628:therapy*
keywords: Animal mast cell tumors; Human mastocytosis; Tyrosine kinase inhibitors (TKIs); c-Kit receptor