Mast cell tumor natural history, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
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. | 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. | ||
==Natural History== | ==Natural History== | ||
*Mast cell tumor usually occurs as a sporadic disease that is often transient and limited in children and progressive in adults. | *Mast cell tumor usually occurs as a sporadic disease that is often transient and limited in children and progressive in adults.<ref name="Patient">Mastocytosis and mast cell disorders.Patient info.http://patient.info/doctor/mastocytosis-and-mast-cell-disorders#ref-20 accessed on March 7th, 2016</ref> | ||
*Systemic mastocytosis may develop in childhood cases of urticaria pigmentosa that persist beyond puberty, and in approximately 40% of adults with urticaria pigmentosa, usually of long standing. | *Systemic mastocytosis may develop in childhood cases of urticaria pigmentosa that persist beyond puberty, and in approximately 40% of adults with urticaria pigmentosa, usually of long standing. | ||
*Patients with mast cell tumor often initially have symptom-free intervals interspersed among symptomatic periods. | *Patients with mast cell tumor often initially have symptom-free intervals interspersed among symptomatic periods. | ||
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==Complications== | ==Complications== | ||
Common complications of mast cell tumor include: | Common complications of mast cell tumor include:<ref name="Patient" /> | ||
*[[Anaphylaxis]] | *[[Anaphylaxis]] | ||
*[[Osteoporosis]] | *[[Osteoporosis]] | ||
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==Prognosis== | ==Prognosis== | ||
The prognosis varies with the location and extent of the tumor:<ref name=Patient>Mastocytosis and mast cell disorders.Patient info.http://patient.info/doctor/mastocytosis-and-mast-cell-disorders#ref-20 accessed on March 7th, 2016</ref> | The prognosis varies with the location and extent of the tumor:<ref name="Patient">Mastocytosis and mast cell disorders.Patient info.http://patient.info/doctor/mastocytosis-and-mast-cell-disorders#ref-20 accessed on March 7th, 2016</ref> | ||
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! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Subtype'''}} | ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|'''Subtype'''}} | ||
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Cutaneous mastocytosis | Cutaneous mastocytosis | ||
| style="padding: 5px 5px; background: #F5F5F5;" align=left | | | style="padding: 5px 5px; background: #F5F5F5;" align="left" | | ||
*Childhood cases: resolve spontaneously | *Childhood cases: resolve spontaneously | ||
*Adults: may progress to systemic form | *Adults: may progress to systemic form | ||
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Systemic mastocytosis | Systemic mastocytosis | ||
| style="padding: 5px 5px; background: #F5F5F5;" align=left | | | style="padding: 5px 5px; background: #F5F5F5;" align="left" | | ||
Prognosis varies with degree of hematological and organ involvement | Prognosis varies with degree of hematological and organ involvement | ||
*Indolent systemic mastocytosis: good prognosis | *Indolent systemic mastocytosis: good prognosis | ||
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Localized mastocytosis | Localized mastocytosis | ||
| style="padding: 5px 5px; background: #F5F5F5;" align=left | | | style="padding: 5px 5px; background: #F5F5F5;" align="left" | | ||
*Mastocytoma: benign tumor with good prognosis | *Mastocytoma: benign tumor with good prognosis | ||
*Mast cell sarcoma: poor prognosis | *Mast cell sarcoma: poor prognosis |
Latest revision as of 14:38, 13 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
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.
Natural History
- Mast cell tumor usually occurs as a sporadic disease that is often transient and limited in children and progressive in adults.[1]
- Systemic mastocytosis may develop in childhood cases of urticaria pigmentosa that persist beyond puberty, and in approximately 40% of adults with urticaria pigmentosa, usually of long standing.
- Patients with mast cell tumor often initially have symptom-free intervals interspersed among symptomatic periods.
- Over time, symptom-free intervals shorten, and finally symptoms become chronic with intensity which fluctuates but with an overall trend toward steadily increasing intensity.
Complications
Common complications of mast cell tumor include:[1]
- Anaphylaxis
- Osteoporosis
- Systemic mastocytosis may progress to malignant mastocytosis and/ or mast cell leukemia
Prognosis
The prognosis varies with the location and extent of the tumor:[1]
Subtype | Prognosis |
---|---|
Cutaneous mastocytosis |
|
Systemic mastocytosis |
Prognosis varies with degree of hematological and organ involvement
|
Localized mastocytosis |
|
References
- ↑ 1.0 1.1 1.2 Mastocytosis and mast cell disorders.Patient info.http://patient.info/doctor/mastocytosis-and-mast-cell-disorders#ref-20 accessed on March 7th, 2016