Mast cell tumor history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

Overview

Symptoms of mast cell tumor depends mainly on the release of clinically relevant mediators histamine, leukotrienes, tryptase, and heparin. Symptoms include flushing, pruritic cutaneous lesions, hypotension, anemia, malabsorption, peptic ulcers, and bone pain.

History and Symptoms

  • Symptoms of mast cell tumor depends on mast cells burden in different organs and release of clinically relevant mediators such as histamine, leukotrienes, tryptase, and heparin.[1]
  • There is considerable heterogeneity in the presentation of mastocytosis, and in the rate of disease progression. Clinical features include:[2][3][4][5]
Type Symptoms

Skin symptoms

May be present in both systemic and cutaneous mastocytosis. Most common in urticaria pigmentosa:

  • Pruritic cutaneous lesions
  • Flushing

Mast cell reslease symptoms

May be present in both cutaneous mastocytosis and systemic mastocytosis. Symptoms may occur in episodes:

Non cutaneous organ infiltration:

Only present in systemic mastocytosis

Urticaria Pigmentosa[6]

  • Urticaria pigmentosa is the most common clinical variant in which fixed, reddish brown lesions occurring as maculo-papules, plaques, nodules, or blisters are found.
  • 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[7]

  • 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

References

  1. 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.
  2. Sokol H, Georgin-Lavialle S, Canioni D, Barete S, Damaj G, Soucie E, Bruneau J, Chandesris MO, Suarez F, Launay JM, Aouba A, Grandpeix-Guyodo C, Lanternier F, Grosbois B, de Gennes C, Cathébras P, Fain O, Hoyeau-Idrissi N, Dubreuil P, Lortholary O, Beaugerie L, Ranque B, Hermine O (October 2013). "Gastrointestinal manifestations in mastocytosis: a study of 83 patients". J. Allergy Clin. Immunol. 132 (4): 866–73.e1–3. doi:10.1016/j.jaci.2013.05.026. PMID 23890756.
  3. Moura DS, Georgin-Lavialle S, Gaillard R, Hermine O (May 2014). "Neuropsychological features of adult mastocytosis". Immunol Allergy Clin North Am. 34 (2): 407–22. doi:10.1016/j.iac.2014.02.001. PMID 24745683.
  4. Lidor C, Frisch B, Gazit D, Gepstein R, Hallel T, Mekori YA (August 1990). "Osteoporosis as the sole presentation of bone marrow mastocytosis". J. Bone Miner. Res. 5 (8): 871–6. doi:10.1002/jbmr.5650050811. PMID 2239371.
  5. Sokol H, Georgin-Lavialle S, Canioni D, Barete S, Damaj G, Soucie E, Bruneau J, Chandesris MO, Suarez F, Launay JM, Aouba A, Grandpeix-Guyodo C, Lanternier F, Grosbois B, de Gennes C, Cathébras P, Fain O, Hoyeau-Idrissi N, Dubreuil P, Lortholary O, Beaugerie L, Ranque B, Hermine O (October 2013). "Gastrointestinal manifestations in mastocytosis: a study of 83 patients". J. Allergy Clin. Immunol. 132 (4): 866–73.e1–3. doi:10.1016/j.jaci.2013.05.026. PMID 23890756.
  6. CAPLAN RM (February 1963). "The natural course of urticaria pigmentosa. Analysis and follow-up of 112 cases". Arch Dermatol. 87: 146–57. PMID 14018418.
  7. Lange M, Niedoszytko M, Nedoszytko B, Łata J, Trzeciak M, Biernat W (December 2012). "Diffuse cutaneous mastocytosis: analysis of 10 cases and a brief review of the literature". J Eur Acad Dermatol Venereol. 26 (12): 1565–71. doi:10.1111/j.1468-3083.2011.04350.x. PMID 22092511.

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