Extraskeletal myxoid chondrosarcoma of the heart and review of current literature

H. L. Geyer, N. Karlin

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Extraskeletal myxoid chondrosarcoma (ems) is a rare oncologic phenomenon characterized by chondroid and neurogenic differentiation in extraskeletal locations. These tumours represent fewer than 2.5% of all soft-tissue sarcomas and are most commonly found in the lower extremities, limb girdles, distal extremities, and trunk. Their presence in cardiac tissue is exceedingly unusual; just a single case of ems metastatic to the heart has been reported, and no cases of primary cardiac ems are known. Here, we report the case of a 26-year-old man who presented to his physician with a chest wall mass. Further evaluation led to the discovery of a large intracardiac mass with multiple end-organ growths. Complete work-up of this patient included cardiac biopsy, echocardiography, magnetic resonance imaging, positron-emission tomography, computed tomography, and fluorescence in situ hybridization studies for the translocation involving the EWSR1 gene locus (22q12). Results of the foregoing studies confirmed the diagnosis of ems, but the origin of this patient's tumours remains elusive and the contention between a primary cardiac source and cardiac metastasis has yet to be resolved. This article describes the histopathology, immunohistochemistry, and chromosomal aberrations common to ems, together with the common presenting features, natural history, and prognosis.

Original languageEnglish (US)
Pages (from-to)58-62
Number of pages5
JournalCurrent Oncology
Volume17
Issue number5
StatePublished - 2010

Fingerprint

Lower Extremity
Thoracic Wall
Natural History
Fluorescence In Situ Hybridization
Chromosome Aberrations
Sarcoma
Echocardiography
Neoplasms
Extremities
Immunohistochemistry
Magnetic Resonance Imaging
Extraskeletal Myxoid Chondrosarcoma
Neoplasm Metastasis
Physicians
Biopsy
Growth
Genes
Positron Emission Tomography Computed Tomography

Keywords

  • Cardiac
  • Extraskeletal
  • Metastatic
  • Myxoid chondrosarcoma
  • Primary

ASJC Scopus subject areas

  • Oncology

Cite this

Extraskeletal myxoid chondrosarcoma of the heart and review of current literature. / Geyer, H. L.; Karlin, N.

In: Current Oncology, Vol. 17, No. 5, 2010, p. 58-62.

Research output: Contribution to journalArticle

Geyer, H. L. ; Karlin, N. / Extraskeletal myxoid chondrosarcoma of the heart and review of current literature. In: Current Oncology. 2010 ; Vol. 17, No. 5. pp. 58-62.
@article{a3880d3974be42e4bc5bebae6bf7dbac,
title = "Extraskeletal myxoid chondrosarcoma of the heart and review of current literature",
abstract = "Extraskeletal myxoid chondrosarcoma (ems) is a rare oncologic phenomenon characterized by chondroid and neurogenic differentiation in extraskeletal locations. These tumours represent fewer than 2.5{\%} of all soft-tissue sarcomas and are most commonly found in the lower extremities, limb girdles, distal extremities, and trunk. Their presence in cardiac tissue is exceedingly unusual; just a single case of ems metastatic to the heart has been reported, and no cases of primary cardiac ems are known. Here, we report the case of a 26-year-old man who presented to his physician with a chest wall mass. Further evaluation led to the discovery of a large intracardiac mass with multiple end-organ growths. Complete work-up of this patient included cardiac biopsy, echocardiography, magnetic resonance imaging, positron-emission tomography, computed tomography, and fluorescence in situ hybridization studies for the translocation involving the EWSR1 gene locus (22q12). Results of the foregoing studies confirmed the diagnosis of ems, but the origin of this patient's tumours remains elusive and the contention between a primary cardiac source and cardiac metastasis has yet to be resolved. This article describes the histopathology, immunohistochemistry, and chromosomal aberrations common to ems, together with the common presenting features, natural history, and prognosis.",
keywords = "Cardiac, Extraskeletal, Metastatic, Myxoid chondrosarcoma, Primary",
author = "Geyer, {H. L.} and N. Karlin",
year = "2010",
language = "English (US)",
volume = "17",
pages = "58--62",
journal = "Current Oncology",
issn = "1198-0052",
publisher = "Multimed Inc.",
number = "5",

}

TY - JOUR

T1 - Extraskeletal myxoid chondrosarcoma of the heart and review of current literature

AU - Geyer, H. L.

AU - Karlin, N.

PY - 2010

Y1 - 2010

N2 - Extraskeletal myxoid chondrosarcoma (ems) is a rare oncologic phenomenon characterized by chondroid and neurogenic differentiation in extraskeletal locations. These tumours represent fewer than 2.5% of all soft-tissue sarcomas and are most commonly found in the lower extremities, limb girdles, distal extremities, and trunk. Their presence in cardiac tissue is exceedingly unusual; just a single case of ems metastatic to the heart has been reported, and no cases of primary cardiac ems are known. Here, we report the case of a 26-year-old man who presented to his physician with a chest wall mass. Further evaluation led to the discovery of a large intracardiac mass with multiple end-organ growths. Complete work-up of this patient included cardiac biopsy, echocardiography, magnetic resonance imaging, positron-emission tomography, computed tomography, and fluorescence in situ hybridization studies for the translocation involving the EWSR1 gene locus (22q12). Results of the foregoing studies confirmed the diagnosis of ems, but the origin of this patient's tumours remains elusive and the contention between a primary cardiac source and cardiac metastasis has yet to be resolved. This article describes the histopathology, immunohistochemistry, and chromosomal aberrations common to ems, together with the common presenting features, natural history, and prognosis.

AB - Extraskeletal myxoid chondrosarcoma (ems) is a rare oncologic phenomenon characterized by chondroid and neurogenic differentiation in extraskeletal locations. These tumours represent fewer than 2.5% of all soft-tissue sarcomas and are most commonly found in the lower extremities, limb girdles, distal extremities, and trunk. Their presence in cardiac tissue is exceedingly unusual; just a single case of ems metastatic to the heart has been reported, and no cases of primary cardiac ems are known. Here, we report the case of a 26-year-old man who presented to his physician with a chest wall mass. Further evaluation led to the discovery of a large intracardiac mass with multiple end-organ growths. Complete work-up of this patient included cardiac biopsy, echocardiography, magnetic resonance imaging, positron-emission tomography, computed tomography, and fluorescence in situ hybridization studies for the translocation involving the EWSR1 gene locus (22q12). Results of the foregoing studies confirmed the diagnosis of ems, but the origin of this patient's tumours remains elusive and the contention between a primary cardiac source and cardiac metastasis has yet to be resolved. This article describes the histopathology, immunohistochemistry, and chromosomal aberrations common to ems, together with the common presenting features, natural history, and prognosis.

KW - Cardiac

KW - Extraskeletal

KW - Metastatic

KW - Myxoid chondrosarcoma

KW - Primary

UR - http://www.scopus.com/inward/record.url?scp=78149449680&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=78149449680&partnerID=8YFLogxK

M3 - Article

VL - 17

SP - 58

EP - 62

JO - Current Oncology

JF - Current Oncology

SN - 1198-0052

IS - 5

ER -