Mast cell tumor history and symptoms: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Mast cell tumor}} | {{Mast cell tumor}} | ||
{{CMG}} | {{CMG}};{{AE}} {{PSK}} | ||
==Overview== | ==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== | ==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]].<ref name="KoenigMorel2008">{{cite journal|last1=Koenig|first1=Martial|last2=Morel|first2=Jérôme|last3=Reynaud|first3=Jacqueline|last4=Varvat|first4=Cécile|last5=Cathébras|first5=Pascal|title=An unusual cause of spontaneous bleeding in the intensive care unit – mastocytosis: a case report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=100|issn=1757-1626|doi=10.1186/1757-1626-1-100}}</ref> There is considerable heterogeneity in the presentation of mastocytosis, and in the rate of disease progression. Clinical features include:<ref name= | * 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]].<ref name="KoenigMorel2008">{{cite journal|last1=Koenig|first1=Martial|last2=Morel|first2=Jérôme|last3=Reynaud|first3=Jacqueline|last4=Varvat|first4=Cécile|last5=Cathébras|first5=Pascal|title=An unusual cause of spontaneous bleeding in the intensive care unit – mastocytosis: a case report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=100|issn=1757-1626|doi=10.1186/1757-1626-1-100}}</ref> | ||
* There is considerable heterogeneity in the presentation of mastocytosis, and in the rate of disease progression. Clinical features include:<ref name="pmid23890756">{{cite journal |vauthors=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 |title=Gastrointestinal manifestations in mastocytosis: a study of 83 patients |journal=J. Allergy Clin. Immunol. |volume=132 |issue=4 |pages=866–73.e1–3 |date=October 2013 |pmid=23890756 |doi=10.1016/j.jaci.2013.05.026 |url=}}</ref><ref name="pmid24745683">{{cite journal |vauthors=Moura DS, Georgin-Lavialle S, Gaillard R, Hermine O |title=Neuropsychological features of adult mastocytosis |journal=Immunol Allergy Clin North Am |volume=34 |issue=2 |pages=407–22 |date=May 2014 |pmid=24745683 |doi=10.1016/j.iac.2014.02.001 |url=}}</ref><ref name="pmid2239371">{{cite journal |vauthors=Lidor C, Frisch B, Gazit D, Gepstein R, Hallel T, Mekori YA |title=Osteoporosis as the sole presentation of bone marrow mastocytosis |journal=J. Bone Miner. Res. |volume=5 |issue=8 |pages=871–6 |date=August 1990 |pmid=2239371 |doi=10.1002/jbmr.5650050811 |url=}}</ref><ref name="pmid238907562">{{cite journal |vauthors=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 |title=Gastrointestinal manifestations in mastocytosis: a study of 83 patients |journal=J. Allergy Clin. Immunol. |volume=132 |issue=4 |pages=866–73.e1–3 |date=October 2013 |pmid=23890756 |doi=10.1016/j.jaci.2013.05.026 |url=}}</ref> | |||
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|valign=top| | | valign="top" | | ||
|+ | |+ | ||
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Type'''}} | ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Type'''}} | ||
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; align=left" | | | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; align=left" | | ||
Skin symptoms | Skin symptoms | ||
| style="padding: 5px 5px; background: #F5F5F5;" align=left | | | style="padding: 5px 5px; background: #F5F5F5;" align="left" | | ||
Most common in urticaria pigmentosa | May be present in both systemic and cutaneous mastocytosis. Most common in urticaria pigmentosa: | ||
*Pruritic cutaneous lesions | *Pruritic cutaneous lesions | ||
*Flushing | |||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; align=left" | | | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; align=left" | | ||
Mast cell reslease symptoms | Mast cell reslease symptoms | ||
| style="padding: 5px 5px; background: #F5F5F5;" align=left | | | style="padding: 5px 5px; background: #F5F5F5;" align="left" | | ||
May be present in both cutaneous mastocytosis and systemic mastocytosis. Symptoms may occur in episodes: | |||
*[[Hypotension]] | *[[Hypotension]] | ||
*[[Flushing]] | *[[Flushing]] | ||
*Itching | *Itching | ||
*[[Syncope]] | *[[Syncope]] | ||
*[[Fatigue]] | |||
*Abdominal discomfort | *Abdominal discomfort | ||
*[[Vomiting]] | *[[Vomiting]] | ||
*[[Diarrhea]] | *[[Diarrhea]] | ||
*[[Headache]] | |||
*Bloating | |||
*Acid Reflex | |||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; align=left" | | | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; align=left" | | ||
Non cutaneous organ infiltration | Non cutaneous organ infiltration: | ||
| style="padding: 5px 5px; background: #F5F5F5;" align=left | | | style="padding: 5px 5px; background: #F5F5F5;" align="left" | | ||
Only present in systemic mastocytosis | |||
*[[Anemia]] | *[[Anemia]] | ||
*[[Malabsorption]] | *[[Malabsorption]] | ||
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*Pathological fracture | *Pathological fracture | ||
*[[Weight loss]] | *[[Weight loss]] | ||
*Anxiety, Depression | |||
*Mood changes, aggressive behaviour | |||
*Fatigue | |||
|} | |} | ||
'''Urticaria Pigmentosa<ref name="pmid14018418">{{cite journal |vauthors=CAPLAN RM |title=The natural course of urticaria pigmentosa. Analysis and follow-up of 112 cases |journal=Arch Dermatol |volume=87 |issue= |pages=146–57 |date=February 1963 |pmid=14018418 |doi= |url=}}</ref>''' | |||
*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 | |||
*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. | *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. | *The trunk and thigh are more commonly involved with sparing of face, palms and soles. | ||
'''Diffuse Cutaneous Mastocytosis''' | '''Diffuse Cutaneous Mastocytosis'''<ref name="pmid22092511">{{cite journal |vauthors=Lange M, Niedoszytko M, Nedoszytko B, Łata J, Trzeciak M, Biernat W |title=Diffuse cutaneous mastocytosis: analysis of 10 cases and a brief review of the literature |journal=J Eur Acad Dermatol Venereol |volume=26 |issue=12 |pages=1565–71 |date=December 2012 |pmid=22092511 |doi=10.1111/j.1468-3083.2011.04350.x |url=}}</ref> | ||
*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. | *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 | *The clinical course is more severe and can even be life-threatening, due to hypovolemic shock, mast cell leukemia, gastrointestinal hemorrhage, and cachexia | ||
==References== | ==References== | ||
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[[Category:Types of cancer]] | [[Category:Types of cancer]] | ||
[[Category:Pathology]] | [[Category:Pathology]] | ||
[[Category:Up-To-Date]] | |||
[[Category:Oncology]] | |||
[[Category:Medicine]] | |||
[[Category:Hematology]] | |||
[[Category:Immunology]] | |||
Latest revision as of 02:40, 14 May 2019
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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:
|
|
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
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ CAPLAN RM (February 1963). "The natural course of urticaria pigmentosa. Analysis and follow-up of 112 cases". Arch Dermatol. 87: 146–57. PMID 14018418.
- ↑ 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.