Mast cell tumor history and symptoms
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
History and Symptoms
There is considerable heterogeneity in the presentation of mastocytosis, and in the rate of disease progression. Clinical features include:[1]
- Pruritic cutaneous lesions
- Flushing
- Headache
- Dyspnea
- Wheezing
- Rhinorrhea
- Nausea
- Vomiting
- Diarrhea
- Hypotension and syncope
- Abdominal pain
- Malabsorption
- Bone pain and pathological fractures
- Weight loss
- Fever and night sweats
Urticaria Pigmentosa
- Urticaria pigmentosa is the most common clinical variant in which fixed, reddish brown lesions occurring as maculo-papules, plaques, nodules, or blisters are found.[2]
- These lesions urticate in response to physical irritation (Darier’s sign).
- Urticaria Pigmentosa (UP) lesions tend to be larger, better delineated, and more hyperpigmented in children, as compared to adults, who tend to have numerous small lesions that coalesce to form mottled areas.
- The trunk and thigh are more commonly involved with sparing of face, palms and soles.
Diffuse Cutaneous Mastocytosis
- Diffuse cutaneous mastocytosis is a rare variant of childhood mastocytosis that appears as diffuse infiltrative yellow-orange xanthogranuloma-like subcutaneous nodules, or as a widespread urticarial eruption with bullae and redness.
- The clinical course is more severe and can even be life-threatening, due to hypovolemic shock, mast cell leukemia, gastrointestinal hemorrhage, and cachexia[2]
It can follow a benign or indolent course, or it may be associated with invalidating or even life-threatening symptoms such as hypotension, syncope, flushing, urticaria, bronchospasm, peptic ulcer disease, diarrhea, malabsorption, osteoporosis, weigh loss and fatigue. Patients with aggressive systemic mastocytosis usually present with enlarged liver, spleen, and lymph nodes, with or without eosinophilia. Clinical pattern depends on mast cells burden in different organs and release of clinically relevant mediators such as histamine, leukotrienes, tryptase and heparin [3]
References
- ↑ Mastocytosis. Dr Alexandra Stanislavsky. Radiopaedia.org 2015. http://radiopaedia.org/articles/mastocytosis Accessed on February 29, 2016
- ↑ 2.0 2.1 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.
- ↑ 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.