Cardiac Undifferentiated Pleomorphic Sarcoma

MedGen UID:: 352855
•Concept ID:: C1704615
•: Neoplastic Process

Synonyms:

Cardiac Unclassified Pleomorphic Sarcoma; Cardiac Unclassified Pleomorphic Sarcoma (Formerly Cardiac "Malignant Fibrous Histiocytoma"); Cardiac Unclassified Pleomorphic Sarcoma (Formerly Cardiac "MFH"); Heart Undifferentiated Pleomorphic Sarcoma

Definition

An undifferentiated pleomorphic sarcoma usually arising from the left atrium of the heart. It is characterized by the presence of fibrohistiocytic cells, giant cells, and spindle cells arrranged iin a storiform pattern. Clinical presentation includes signs and symptoms associated with left atrial hemodynamic changes. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVCardiac Undifferentiated Pleomorphic Sarcoma

Disorder by Site
- Disorder of cardiovascular system
  - Cardiovascular neoplasm
    - Cardiovascular cancer
      - Heart cancer
        Cardiac sarcoma
        Cardiac Undifferentiated Pleomorphic Sarcoma

Professional guidelines

PubMed

Treatment strategies for pediatric patients with primary cardiac and pericardial tumors: a 30-year review.

Günther T, Schreiber C, Noebauer C, Eicken A, Lange R
Pediatr Cardiol 2008 Nov;29(6):1071-6. Epub 2008 Jul 4 doi: 10.1007/s00246-008-9256-6. PMID: 18600370

Primary and metastatic high-grade pleomorphic sarcoma/malignant fibrous histiocytoma of the gastrointestinal tract: an approach to the differential diagnosis in a series of five cases with emphasis on myofibroblastic differentiation.

Agaimy A, Gaumann A, Schroeder J, Dietmaier W, Hartmann A, Hofstaedter F, Wünsch PH, Mentzel T
Virchows Arch 2007 Nov;451(5):949-57. Epub 2007 Sep 14 doi: 10.1007/s00428-007-0495-3. PMID: 17874130

Surgical treatment of primary pulmonary sarcomas.

Bacha EA, Wright CD, Grillo HC, Wain JC, Moncure A, Keel SB, Donahue DM, Mathisen DJ
Eur J Cardiothorac Surg 1999 Apr;15(4):456-60. doi: 10.1016/s1010-7940(99)00045-7. PMID: 10371121

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Recent clinical studies

Diagnosis

A Rare Case of Undifferentiated Pleomorphic Cardiac Sarcoma with Inflammatory Pattern.

Stoica AI, Harpa MM, Banceu CM, Ghiragosian C, Opris CE, Al-Hussein H, Al-Hussein H, Flamind Oltean S, Mezei T, Mares RG, Suciu H
Medicina (Kaunas) 2022 Jul 28;58(8) doi: 10.3390/medicina58081009. PMID: 36013476 Free PMC Article

Cardiac Undifferentiated Pleomorphic Sarcoma Mimicking Left Atrial Myxoma.

Vallés-Torres J, Izquierdo-Villarroya MB, Vallejo-Gil JM, Casado-Domínguez JM, Roche Latasa AB, Auquilla-Clavijo P
J Cardiothorac Vasc Anesth 2019 Feb;33(2):493-496. Epub 2018 Feb 7 doi: 10.1053/j.jvca.2018.02.010. PMID: 29551281

Cardiac undifferentiated pleomorphic sarcoma incidentally diagnosed during mitral valve replacement.

Aguilar C, Soca R, Guillen M, Paucca E, Illatopa V
J Card Surg 2017 Feb;32(2):91-92. Epub 2017 Jan 27 doi: 10.1111/jocs.13087. PMID: 28127801

Life-threatening acute heart failure due to primary cardiac undifferentiated pleomorphic sarcoma.

Abe D, Sato A, Takeyasu N, Tokunaga C, Akishima S, Iijima T, Sakakibara Y, Aonuma K
Intern Med 2014;53(16):1775-7. Epub 2014 Aug 15 doi: 10.2169/internalmedicine.53.2124. PMID: 25130109

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