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Mastocytoma

MedGen UID:
6237
•Concept ID:
C0024897
•
Neoplastic Process
Synonym: mastocytoma
SNOMED CT: Mastocytoma (89796001); Mastocytoma (404171008)
 
Monarch Initiative: MONDO:0003079

Definition

A localized tumor composed of sheets of mast cells without atypia. It includes the cutaneous mastocytoma which involves the dermis and subcutaneous tissue, and the extracutaneous mastocytoma. Most cases of extracutaneous mastocytoma have been reported in the lung. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMastocytoma

Professional guidelines

PubMed

Genetic Changes in Mastocytes and Their Significance in Mast Cell Tumor Prognosis and Treatment.
Zmorzynski S, Kimicka-Szajwaj A, Szajwaj A, Czerwik-Marcinkowska J, Wojcierowski J
Genes (Basel) 2024 Jan 22;15(1) doi: 10.3390/genes15010137. PMID: 38275618Free PMC Article
Childhood Solitary Cutaneous Mastocytoma: Clinical Manifestations, Diagnosis, Evaluation, and Management.
Leung AKC, Lam JM, Leong KF
Curr Pediatr Rev 2019;15(1):42-46. doi: 10.2174/1573396315666181120163952. PMID: 30465511Free PMC Article
Natural history and treatment of cutaneous and systemic mastocytosis.
Le M, Miedzybrodzki B, Olynych T, Chapdelaine H, Ben-Shoshan M
Postgrad Med 2017 Nov;129(8):896-901. Epub 2017 Aug 21 doi: 10.1080/00325481.2017.1364124. PMID: 28770635

Recent clinical studies

Etiology

Cutaneous Mastocytosis in Adults and Children: New Classification and Prognostic Factors.
Matito A, Azaña JM, Torrelo A, Alvarez-Twose I
Immunol Allergy Clin North Am 2018 Aug;38(3):351-363. Epub 2018 May 17 doi: 10.1016/j.iac.2018.04.001. PMID: 30007456
Natural history and treatment of cutaneous and systemic mastocytosis.
Le M, Miedzybrodzki B, Olynych T, Chapdelaine H, Ben-Shoshan M
Postgrad Med 2017 Nov;129(8):896-901. Epub 2017 Aug 21 doi: 10.1080/00325481.2017.1364124. PMID: 28770635
Canine neoplasia--introductory paper.
Gamlem H, Nordstoga K, Glattre E
APMIS Suppl 2008;(125):5-18. doi: 10.1111/j.1600-0463.2008.125m2.x. PMID: 19385278
Pediatric mastocytosis.
Kettelhut BV, Metcalfe DD
J Invest Dermatol 1991 Mar;96(3 Suppl):15S-18S; discussion 18S, 60S-65S. doi: 10.1111/1523-1747.ep12468942. PMID: 16799603
Leukotrienes.
Hammarström S
Annu Rev Biochem 1983;52:355-77. doi: 10.1146/annurev.bi.52.070183.002035. PMID: 6311078

Diagnosis

Cutaneous mastocytosis: A dermatological perspective.
Di Raimondo C, Del Duca E, Silvaggio D, Di Prete M, Lombardo P, Mazzeo M, Spallone G, Campione E, Botti E, Bianchi L
Australas J Dermatol 2021 Feb;62(1):e1-e7. Epub 2020 Oct 11 doi: 10.1111/ajd.13443. PMID: 33040350
Childhood Solitary Cutaneous Mastocytoma: Clinical Manifestations, Diagnosis, Evaluation, and Management.
Leung AKC, Lam JM, Leong KF
Curr Pediatr Rev 2019;15(1):42-46. doi: 10.2174/1573396315666181120163952. PMID: 30465511Free PMC Article
Cutaneous manifestations in patients with mastocytosis: Consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology.
Hartmann K, Escribano L, Grattan C, Brockow K, Carter MC, Alvarez-Twose I, Matito A, Broesby-Olsen S, Siebenhaar F, Lange M, Niedoszytko M, Castells M, Oude Elberink JNG, Bonadonna P, Zanotti R, Hornick JL, Torrelo A, Grabbe J, Rabenhorst A, Nedoszytko B, Butterfield JH, Gotlib J, Reiter A, Radia D, Hermine O, Sotlar K, George TI, Kristensen TK, Kluin-Nelemans HC, Yavuz S, Hägglund H, Sperr WR, Schwartz LB, Triggiani M, Maurer M, Nilsson G, Horny HP, Arock M, Orfao A, Metcalfe DD, Akin C, Valent P
J Allergy Clin Immunol 2016 Jan;137(1):35-45. Epub 2015 Oct 21 doi: 10.1016/j.jaci.2015.08.034. PMID: 26476479
Rare syndromes.
Jabbour SA, Davidovici BB, Wolf R
Clin Dermatol 2006 Jul-Aug;24(4):299-316. doi: 10.1016/j.clindermatol.2006.04.005. PMID: 16828412
Pediatric mastocytosis.
Kettelhut BV, Metcalfe DD
J Invest Dermatol 1991 Mar;96(3 Suppl):15S-18S; discussion 18S, 60S-65S. doi: 10.1111/1523-1747.ep12468942. PMID: 16799603

Therapy

Simple and effective bacterial-based intratumoral cancer immunotherapy.
Carroll CSE, Andrew ER, Malik L, Elliott KF, Brennan M, Meyer J, Hintze A, Almonte AA, Lappin C, MacPherson P, Schulte KM, Dahlstrom JE, Tamhane R, Neeman T, Herbert EW, Orange M, Yip D, Allavena R, Fahrer AM
J Immunother Cancer 2021 Sep;9(9) doi: 10.1136/jitc-2021-002688. PMID: 34531247Free PMC Article
Childhood Solitary Cutaneous Mastocytoma: Clinical Manifestations, Diagnosis, Evaluation, and Management.
Leung AKC, Lam JM, Leong KF
Curr Pediatr Rev 2019;15(1):42-46. doi: 10.2174/1573396315666181120163952. PMID: 30465511Free PMC Article
Comparative oncology: The paradigmatic example of canine and human mast cell neoplasms.
Willmann M, Hadzijusufovic E, Hermine O, Dacasto M, Marconato L, Bauer K, Peter B, Gamperl S, Eisenwort G, Jensen-Jarolim E, Müller M, Arock M, Vail DM, Valent P
Vet Comp Oncol 2019 Mar;17(1):1-10. Epub 2018 Sep 24 doi: 10.1111/vco.12440. PMID: 30136349Free PMC Article
Natural history and treatment of cutaneous and systemic mastocytosis.
Le M, Miedzybrodzki B, Olynych T, Chapdelaine H, Ben-Shoshan M
Postgrad Med 2017 Nov;129(8):896-901. Epub 2017 Aug 21 doi: 10.1080/00325481.2017.1364124. PMID: 28770635
Solitary mastocytoma treated with tranilast.
Katoh N, Hirano S, Yasuno H
J Dermatol 1996 May;23(5):335-9. doi: 10.1111/j.1346-8138.1996.tb04026.x. PMID: 8675824

Prognosis

Cutaneous mastocytosis: A dermatological perspective.
Di Raimondo C, Del Duca E, Silvaggio D, Di Prete M, Lombardo P, Mazzeo M, Spallone G, Campione E, Botti E, Bianchi L
Australas J Dermatol 2021 Feb;62(1):e1-e7. Epub 2020 Oct 11 doi: 10.1111/ajd.13443. PMID: 33040350
Mast cell disorders: From infancy to maturity.
Wilcock A, Bahri R, Bulfone-Paus S, Arkwright PD
Allergy 2019 Jan;74(1):53-63. Epub 2018 Nov 28 doi: 10.1111/all.13657. PMID: 30390314
Natural history and treatment of cutaneous and systemic mastocytosis.
Le M, Miedzybrodzki B, Olynych T, Chapdelaine H, Ben-Shoshan M
Postgrad Med 2017 Nov;129(8):896-901. Epub 2017 Aug 21 doi: 10.1080/00325481.2017.1364124. PMID: 28770635
Cutaneous manifestations in patients with mastocytosis: Consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology.
Hartmann K, Escribano L, Grattan C, Brockow K, Carter MC, Alvarez-Twose I, Matito A, Broesby-Olsen S, Siebenhaar F, Lange M, Niedoszytko M, Castells M, Oude Elberink JNG, Bonadonna P, Zanotti R, Hornick JL, Torrelo A, Grabbe J, Rabenhorst A, Nedoszytko B, Butterfield JH, Gotlib J, Reiter A, Radia D, Hermine O, Sotlar K, George TI, Kristensen TK, Kluin-Nelemans HC, Yavuz S, Hägglund H, Sperr WR, Schwartz LB, Triggiani M, Maurer M, Nilsson G, Horny HP, Arock M, Orfao A, Metcalfe DD, Akin C, Valent P
J Allergy Clin Immunol 2016 Jan;137(1):35-45. Epub 2015 Oct 21 doi: 10.1016/j.jaci.2015.08.034. PMID: 26476479
Pediatric mastocytosis.
Kettelhut BV, Metcalfe DD
J Invest Dermatol 1991 Mar;96(3 Suppl):15S-18S; discussion 18S, 60S-65S. doi: 10.1111/1523-1747.ep12468942. PMID: 16799603

Clinical prediction guides

Genetic Changes in Mastocytes and Their Significance in Mast Cell Tumor Prognosis and Treatment.
Zmorzynski S, Kimicka-Szajwaj A, Szajwaj A, Czerwik-Marcinkowska J, Wojcierowski J
Genes (Basel) 2024 Jan 22;15(1) doi: 10.3390/genes15010137. PMID: 38275618Free PMC Article
Simple and effective bacterial-based intratumoral cancer immunotherapy.
Carroll CSE, Andrew ER, Malik L, Elliott KF, Brennan M, Meyer J, Hintze A, Almonte AA, Lappin C, MacPherson P, Schulte KM, Dahlstrom JE, Tamhane R, Neeman T, Herbert EW, Orange M, Yip D, Allavena R, Fahrer AM
J Immunother Cancer 2021 Sep;9(9) doi: 10.1136/jitc-2021-002688. PMID: 34531247Free PMC Article
Childhood Solitary Cutaneous Mastocytoma: Clinical Manifestations, Diagnosis, Evaluation, and Management.
Leung AKC, Lam JM, Leong KF
Curr Pediatr Rev 2019;15(1):42-46. doi: 10.2174/1573396315666181120163952. PMID: 30465511Free PMC Article
Cutaneous Mastocytosis in Adults and Children: New Classification and Prognostic Factors.
Matito A, Azaña JM, Torrelo A, Alvarez-Twose I
Immunol Allergy Clin North Am 2018 Aug;38(3):351-363. Epub 2018 May 17 doi: 10.1016/j.iac.2018.04.001. PMID: 30007456
Cutaneous manifestations in patients with mastocytosis: Consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology.
Hartmann K, Escribano L, Grattan C, Brockow K, Carter MC, Alvarez-Twose I, Matito A, Broesby-Olsen S, Siebenhaar F, Lange M, Niedoszytko M, Castells M, Oude Elberink JNG, Bonadonna P, Zanotti R, Hornick JL, Torrelo A, Grabbe J, Rabenhorst A, Nedoszytko B, Butterfield JH, Gotlib J, Reiter A, Radia D, Hermine O, Sotlar K, George TI, Kristensen TK, Kluin-Nelemans HC, Yavuz S, Hägglund H, Sperr WR, Schwartz LB, Triggiani M, Maurer M, Nilsson G, Horny HP, Arock M, Orfao A, Metcalfe DD, Akin C, Valent P
J Allergy Clin Immunol 2016 Jan;137(1):35-45. Epub 2015 Oct 21 doi: 10.1016/j.jaci.2015.08.034. PMID: 26476479

Recent systematic reviews

Natural history and treatment of cutaneous and systemic mastocytosis.
Le M, Miedzybrodzki B, Olynych T, Chapdelaine H, Ben-Shoshan M
Postgrad Med 2017 Nov;129(8):896-901. Epub 2017 Aug 21 doi: 10.1080/00325481.2017.1364124. PMID: 28770635
Paediatric mastocytosis: a systematic review of 1747 cases.
Méni C, Bruneau J, Georgin-Lavialle S, Le Saché de Peufeilhoux L, Damaj G, Hadj-Rabia S, Fraitag S, Dubreuil P, Hermine O, Bodemer C
Br J Dermatol 2015 Mar;172(3):642-51. Epub 2015 Feb 8 doi: 10.1111/bjd.13567. PMID: 25662299

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