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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

Mast cell neoplasm: a challenging pathological diagnosis.
Natal RA, Carneiro HC, de Carvalho Barra FF, Pagnano KBB, Soares FA, Vassallo J
Hum Pathol 2022 Aug;126:55-62. Epub 2022 May 10 doi: 10.1016/j.humpath.2022.05.007. PMID: 35550832
New Insights into Clonal Mast Cell Disorders Including Mastocytosis.
Volertas S, Schuler CF 4th, Akin C
Immunol Allergy Clin North Am 2018 Aug;38(3):341-350. Epub 2018 Jun 9 doi: 10.1016/j.iac.2018.04.014. PMID: 30007455
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

Diagnosis

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 neoplasm: a challenging pathological diagnosis.
Natal RA, Carneiro HC, de Carvalho Barra FF, Pagnano KBB, Soares FA, Vassallo J
Hum Pathol 2022 Aug;126:55-62. Epub 2022 May 10 doi: 10.1016/j.humpath.2022.05.007. PMID: 35550832
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
Treatment of systemic mast cell disorders.
Worobec AS
Hematol Oncol Clin North Am 2000 Jun;14(3):659-87, vii. doi: 10.1016/s0889-8588(05)70301-4. PMID: 10909045

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
Pediatric mastocytosis.
Hartmann K, Metcalfe DD
Hematol Oncol Clin North Am 2000 Jun;14(3):625-40. doi: 10.1016/s0889-8588(05)70299-9. PMID: 10909043

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
Dog mast cell alpha-chymase activates progelatinase B by cleaving the Phe88-Gln89 and Phe91-Glu92 bonds of the catalytic domain.
Fang KC, Raymond WW, Blount JL, Caughey GH
J Biol Chem 1997 Oct 10;272(41):25628-35. doi: 10.1074/jbc.272.41.25628. PMID: 9325284
Requirements for CD28-dependent T cell-mediated cytotoxicity.
Azuma M, Cayabyab M, Phillips JH, Lanier LL
J Immunol 1993 Mar 15;150(6):2091-101. PMID: 8383716
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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