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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

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
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

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
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
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
Mastocytosis.
Fine J
Int J Dermatol 1980 Apr;19(3):117-23. doi: 10.1111/j.1365-4362.1980.tb00272.x. PMID: 6156134

Diagnosis

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
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
Diagnostic and therapeutic challenges in mast cell sarcoma.
Mannelli F, Gesullo F, Mannarelli C, Vanderwert F, Lazzi S, Mungai F, Berti V, Santi R, Guglielmelli P, Vannucchi AM
Am J Hematol 2023 Mar;98(3):529-532. Epub 2022 Dec 20 doi: 10.1002/ajh.26804. PMID: 36464921
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
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

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
Mast cell sarcoma: a rare and aggressive entity--report of two cases and review of the literature.
Georgin-Lavialle S, Aguilar C, Guieze R, Lhermitte L, Bruneau J, Fraitag S, Canioni D, Chandesris MO, Suarez F, Grandpeix-Guyodo C, Damaj G, Barete S, Aouba A, Fite C, Robert C, Gaulard P, Lortholary O, Tournilhac O, Dubreuil P, Hermine O
J Clin Oncol 2013 Feb 20;31(6):e90-7. Epub 2012 Nov 5 doi: 10.1200/JCO.2012.41.9549. PMID: 23129735
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
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
Therapy of neoplasia in domestic animals.
Owen LN
Adv Vet Sci Comp Med 1976;20:223-52. PMID: 188328

Prognosis

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: a rare and aggressive entity--report of two cases and review of the literature.
Georgin-Lavialle S, Aguilar C, Guieze R, Lhermitte L, Bruneau J, Fraitag S, Canioni D, Chandesris MO, Suarez F, Grandpeix-Guyodo C, Damaj G, Barete S, Aouba A, Fite C, Robert C, Gaulard P, Lortholary O, Tournilhac O, Dubreuil P, Hermine O
J Clin Oncol 2013 Feb 20;31(6):e90-7. Epub 2012 Nov 5 doi: 10.1200/JCO.2012.41.9549. PMID: 23129735
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
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
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
Creating CTL targets with epitope-linked beta 2-microglobulin constructs.
Uger RA, Barber BH
J Immunol 1998 Feb 15;160(4):1598-605. PMID: 9469415
Stimulation of CD28 triggers an association between CD28 and phosphatidylinositol 3-kinase in Jurkat T cells.
Truitt KE, Hicks CM, Imboden JB
J Exp Med 1994 Mar 1;179(3):1071-6. doi: 10.1084/jem.179.3.1071. PMID: 7509360Free PMC Article
Cytotoxic T lymphocyte recognition of secreted HLA class I molecules.
Kahn-Perles B, Barra C, Jehan J, Fourel D, Barad M, Maryanski JL, Lemonnier FA
J Immunol 1989 May 1;142(9):3021-5. PMID: 2468709
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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