Mast cell tumor overview
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Overview
A mast cell tumor is a type of tumor consisting of mast cells that is found in many species of animals. It refers to an accumulation or nodule of mast cells that resembles a tumor. Mastocytosis is a rare and heterogeneous disease characterized by the presence of excessive numbers of mast cells in various organs, mainly the skin and the bone marrow that manifests with an unusually broad spectrum of clinical and morphological appearances.
Classification
Based on the affected organ(s), mast cell tumor may be classified into either cutaneous mastocytosis or systemic mastocytosis. Mast cell tumor may be classified into seven subtypes based on WHO classification system.[1]
Pathophysiology
Mast cell tumor arises from the mast cell, which is a type of white blood cell involved in the inflammatory process. The progression to mast cell tumor usually involves the uncontrolled stimulation of the receptor for stem cell factor following mutation of C-kit cell surface receptor.[2] On microscopic histopathological analysis, mast cells in the superficial and mid dermis that are lymphocyte like with dense granular cytoplasm which tend to be more abundant around blood vessels is characteristic finding of mast cell tumor.[3]
Causes
There are no established causes for mast cell tumor.
Differentiating Mast Cell Tumor from other Diseases
Mast cell tumor must be differentiated from other diseases that cause flushing,such as: phaeochromocytoma and carcinoid syndrome.[4] Mast cell tumor must be differentiated from other diseases that cause elevated serum tryptase levels and cytopenia, such as: myelodysplastic syndrome, primary myelofibrosis, essential thrombocythemia, and chronic eosinophilic leukemia.
Epidemiology and Demographics
Mast cell tumor is a rare disease and considered to be an "orphan disease" affecting 200,000 or fewer people in the United States. There are no definite data regarding the prevalence of mast cell tumor among the US general population.[5] Cutaneous mastocytosis is more common in children and the disease manifest itself in the first year of life in over 80% of cases.[6] There is no sex predilection and race predilection to the mast cell tumor.
Risk Factors
Common risk factors in the development of mast cell tumor are age and mutation of C-kit receptor.
Natural History, Complications and Prognosis
Common complications of mast cell tumor include episodes of anaphylaxis, osteoporosis, and progression to malignant mastocytosis or mast cell leukemia. The prognosis of mast cell tumor varies with the subtype, location, and extent of the tumor. The cutaneous mastocytosis is associated with the most favorable prognosis and mast cell leukemia is associated with poor prognosis.[4]
Staging
There is no established system for the staging of mast cell tumor.
References
- ↑ Patnaik MM, Rindos M, Kouides PA, Tefferi A, Pardanani A (2007). "Systemic mastocytosis: a concise clinical and laboratory review". Arch Pathol Lab Med. 131 (5): 784–91. doi:10.1043/1543-2165(2007)131[784:SMACCA]2.0.CO;2. PMID 17488167.
- ↑ Adolf, Stefanie; Millonig, Gunda; Seitz, Helmut Karl; Reiter, Andreas; Schirmacher, Peter; Longerich, Thomas; Mueller, Sebastian (2012). "Systemic Mastocytosis: A Rare Case of Increased Liver Stiffness". Case Reports in Hepatology. 2012: 1–6. doi:10.1155/2012/728172. ISSN 2090-6587.
- ↑ Mastocytosis. Libre Pathology. http://librepathology.org/wiki/Mastocytosis accessed on March 1st, 2016
- ↑ 4.0 4.1 Mastocytosis and mast cell disorders.Patient info.http://patient.info/doctor/mastocytosis-and-mast-cell-disorders#ref-20 accessed on March 7th, 2016
- ↑ Koenig, Martial; Morel, Jérôme; Reynaud, Jacqueline; Varvat, Cécile; Cathébras, Pascal (2008). "An unusual cause of spontaneous bleeding in the intensive care unit – mastocytosis: a case report". Cases Journal. 1 (1): 100. doi:10.1186/1757-1626-1-100. ISSN 1757-1626.
- ↑ Ferrante, Giuliana; Scavone, Valeria; Muscia, Maria; Adrignola, Emilia; Corsello, Giovanni; Passalacqua, Giovanni; La Grutta, Stefania (2015). "The care pathway for children with urticaria, angioedema, mastocytosis". World Allergy Organization Journal. 8 (1): 5. doi:10.1186/s40413-014-0052-x. ISSN 1939-4551.