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Mast cell sarcoma

MedGen UID:
11322
•Concept ID:
C0036221
•
Neoplastic Process
Synonyms: Malignant Mastocytoma; Malignant Mastocytomas; Mast Cell Sarcoma; Mast-Cell Sarcoma; Mast-Cell Sarcomas; Mastocytoma, Malignant; Mastocytomas, Malignant; Sarcoma, Mast Cell; Sarcoma, Mast-Cell; Sarcomas, Mast-Cell
SNOMED CT: Malignant mast cell tumor (clinical) (118615008); Malignant mast cell neoplasm (397010005); Malignant mast cell tumor (118615008); Mast cell sarcoma (13583002); Malignant mast cell tumor (13583002); Malignant mastocytoma (13583002); Mast cell sarcoma (118615008)
 
Monarch Initiative: MONDO:0019024
Orphanet: ORPHA66661

Definition

Mast cell sarcoma is a rare, neoplastic disease characterized by locally destructive sarcoma-like growth of a solitary mass, composed of atypical mast cells, and without systemic involvement. It can affect any organ and the symptoms depend on the location. Cells are medium to large, pleomorphic or epithelioid, with oval, bilobed or multilobulated nuclei, sometimes prominent multinucleated giant cells. The disease closely resembles other neoplasms and may share associated markers, however the tumor is positive for mast cell tryptase. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMast cell sarcoma
Follow this link to review classifications for Mast cell sarcoma in Orphanet.

Professional guidelines

PubMed

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
Update on Mastocytosis (Part 2): Categories, Prognosis, and Treatment.
Azaña JM, Torrelo A, Matito A
Actas Dermosifiliogr 2016 Jan-Feb;107(1):15-22. Epub 2015 Oct 30 doi: 10.1016/j.ad.2015.09.009. PMID: 26525106
Mast cell sarcoma: clinical management.
Weiler CR, Butterfield J
Immunol Allergy Clin North Am 2014 May;34(2):423-32. Epub 2014 Mar 13 doi: 10.1016/j.iac.2014.01.004. PMID: 24745684

Recent clinical studies

Etiology

NCCN Guidelines® Insights: Systemic Mastocytosis, Version 3.2024.
Gotlib J, Gerds AT, Abdelmessieh P, Ali H, Castells M, Dunbar A, Fein Revell R, George TI, Green S, Gundabolu K, Hexner E, Jain T, Jamieson C, Kaesberg PR, Kuykendall AT, Madanat Y, Manchanda N, Masarova L, May J, McMahon B, Mohan SR, Nadiminti KV, Oh S, Palmer J, Patel A, Patel AA, Podoltsev N, Rein L, Salit R, Talpaz M, Wadleigh M, Wall S, Bergman MA, Hochstetler C
J Natl Compr Canc Netw 2024 Jun;22(2 D) doi: 10.6004/jnccn.2024.0030. PMID: 38862005
Mast cell sarcoma: clinical management.
Weiler CR, Butterfield J
Immunol Allergy Clin North Am 2014 May;34(2):423-32. Epub 2014 Mar 13 doi: 10.1016/j.iac.2014.01.004. PMID: 24745684
Pathology of extramedullary mastocytosis.
Doyle LA, Hornick JL
Immunol Allergy Clin North Am 2014 May;34(2):323-39. doi: 10.1016/j.iac.2014.01.010. PMID: 24745677
Systemic mastocytosis--a systematic review.
Andersen CL, Kristensen TK, Severinsen MT, Møller MB, Vestergaard H, Bergmann OJ, Hasselbalch HC, Bjerrum OW
Dan Med J 2012 Mar;59(3):A4397. PMID: 22381091
Mastocytosis.
Fine J
Int J Dermatol 1980 Apr;19(3):117-23. doi: 10.1111/j.1365-4362.1980.tb00272.x. PMID: 6156134

Diagnosis

Advanced systemic mastocytosis-Revised classification, new drugs and how we treat.
Pardanani A, Reichard K, Tefferi A
Br J Haematol 2024 Feb;204(2):402-414. Epub 2023 Dec 6 doi: 10.1111/bjh.19245. PMID: 38054381
The international consensus classification of mastocytosis and related entities.
Leguit RJ, Wang SA, George TI, Tzankov A, Orazi A
Virchows Arch 2023 Jan;482(1):99-112. Epub 2022 Oct 10 doi: 10.1007/s00428-022-03423-3. PMID: 36214901
Mast cell sarcoma: new cases and literature review.
Monnier J, Georgin-Lavialle S, Canioni D, Lhermitte L, Soussan M, Arock M, Bruneau J, Dubreuil P, Bodemer C, Chandesris MO, Lortholary O, Hermine O, Damaj G
Oncotarget 2016 Oct 4;7(40):66299-66309. doi: 10.18632/oncotarget.11812. PMID: 27602777Free PMC Article
Pathology of extramedullary mastocytosis.
Doyle LA, Hornick JL
Immunol Allergy Clin North Am 2014 May;34(2):323-39. doi: 10.1016/j.iac.2014.01.010. PMID: 24745677
Systemic mastocytosis.
Ozdemir D, Dagdelen S, Erbas T
Am J Med Sci 2011 Nov;342(5):409-15. doi: 10.1097/MAJ.0b013e3182121131. PMID: 21629042

Therapy

Advanced systemic mastocytosis-Revised classification, new drugs and how we treat.
Pardanani A, Reichard K, Tefferi A
Br J Haematol 2024 Feb;204(2):402-414. Epub 2023 Dec 6 doi: 10.1111/bjh.19245. PMID: 38054381
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
Mast cell sarcoma: clinical management.
Weiler CR, Butterfield J
Immunol Allergy Clin North Am 2014 May;34(2):423-32. Epub 2014 Mar 13 doi: 10.1016/j.iac.2014.01.004. PMID: 24745684
Systemic mastocytosis--a systematic review.
Andersen CL, Kristensen TK, Severinsen MT, Møller MB, Vestergaard H, Bergmann OJ, Hasselbalch HC, Bjerrum OW
Dan Med J 2012 Mar;59(3):A4397. PMID: 22381091
Aggressive systemic mastocytosis.
Inaoui R, Petit B, Jaccard A, Bertin P, Trèves R
Joint Bone Spine 2003 Feb;70(1):64-6. doi: 10.1016/s1297-319x(02)00015-5. PMID: 12639621

Prognosis

Mast cell sarcoma: new cases and literature review.
Monnier J, Georgin-Lavialle S, Canioni D, Lhermitte L, Soussan M, Arock M, Bruneau J, Dubreuil P, Bodemer C, Chandesris MO, Lortholary O, Hermine O, Damaj G
Oncotarget 2016 Oct 4;7(40):66299-66309. doi: 10.18632/oncotarget.11812. PMID: 27602777Free PMC Article
Mast cell sarcoma: clinical management.
Weiler CR, Butterfield J
Immunol Allergy Clin North Am 2014 May;34(2):423-32. Epub 2014 Mar 13 doi: 10.1016/j.iac.2014.01.004. PMID: 24745684
Pathology of extramedullary mastocytosis.
Doyle LA, Hornick JL
Immunol Allergy Clin North Am 2014 May;34(2):323-39. doi: 10.1016/j.iac.2014.01.010. PMID: 24745677
Systemic mastocytosis.
Ozdemir D, Dagdelen S, Erbas T
Am J Med Sci 2011 Nov;342(5):409-15. doi: 10.1097/MAJ.0b013e3182121131. PMID: 21629042
Mastocytosis.
Fine J
Int J Dermatol 1980 Apr;19(3):117-23. doi: 10.1111/j.1365-4362.1980.tb00272.x. PMID: 6156134

Clinical prediction guides

Comprehensive mastocytosis data analysis from a single center.
Tiryaki TO, Özkan SG, Erdem S, Aday AD, Hindilerden İY, Gelincik A, Baykal C, Yegen G, Doğan İÖ, Büyükbabani N, Nalçacı M, Yavuz AS
BMC Cancer 2023 Jan 24;23(1):82. doi: 10.1186/s12885-022-10498-3. PMID: 36694141Free PMC Article
FDG-PET/CT findings in systemic mastocytosis: a French multicentre study.
Djelbani-Ahmed S, Chandesris MO, Mekinian A, Canioni D, Brouzes C, Hanssens K, Pop G, Durieu I, Durupt S, Grosbois B, Besnard S, Tournilhac O, Beyne-Rauzy O, Agapé P, Delmer A, Ranta D, Jeandel PY, Georgin-Lavialle S, Frenzel L, Damaj G, Eder V, Lortholary O, Hermine O, Fain O, Soussan M
Eur J Nucl Med Mol Imaging 2015 Dec;42(13):2013-20. Epub 2015 Jul 5 doi: 10.1007/s00259-015-3117-3. PMID: 26140850
Intratumoural interleukin-2 therapy can induce regression of non-resectable mastocytoma in dogs.
Ziekman PG, Otter WD, Tan JF, Teske E, Kirpensteijn J, Koten JW, Jacobs JJ
Anticancer Res 2013 Jan;33(1):161-5. PMID: 23267141
Lipopolysaccharide and splenic tumoricidal macrophage activation.
Verstovsek S, Zaleskis G, Maccubbin DL, Mihich E, Ehrke MJ
J Leukoc Biol 1994 Dec;56(6):714-22. doi: 10.1002/jlb.56.6.714. PMID: 7996047
Mastocytosis.
Fine J
Int J Dermatol 1980 Apr;19(3):117-23. doi: 10.1111/j.1365-4362.1980.tb00272.x. PMID: 6156134

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
Systemic mastocytosis--a systematic review.
Andersen CL, Kristensen TK, Severinsen MT, Møller MB, Vestergaard H, Bergmann OJ, Hasselbalch HC, Bjerrum OW
Dan Med J 2012 Mar;59(3):A4397. PMID: 22381091

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