Rhabdomyosarcoma of the Heart

Dutschmann, L; Silva, Duarte; Costa, Jason

doi:10.1177/000331977402500303

Cited by 7 publications

(2 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…'~~-~ Since tumors arising in different primary sites have been demonstrated to have different prognosis, we have chosen to analyze patients by site of origin as well as by group. The purpose of this report is to describe 17 patients with intrathoracic primary tumors entered in the Intergroup Rhabdomyosarcoma Study (IRS)-I" or the pilot study for IRS-I1 (2).…”

mentioning

confidence: 99%

Intrathoracic soft tissue sarcomas in children

Rb²,

Newton³

et al. 1982

Cancer

View full text Add to dashboard Cite

Seventeen of 646 (2.6%) children registered on the Intergroup Rhabdomyosarcoma Study (IRS)‐I and pilot study for IRS‐II as of December 1977, had apparent intrathoracic primary soft tissue sarcomas. The primary tumor appeared to arise in the mediastinum in ten patients, the pleura in four, and the lung in three. The age range was 1–20 years (mean, 9.9 years), and the male/female ratio was 1.8. Tumor histologic type was embryonal in seven, alveolar in five, undifferentiated in four, and extraosseous Ewing's (EOE) in one. Fourteen patients (82%) had unresectable disease at presentation, while three had grossly resected disease. These children were treated according to the IRS‐I15 or a subsequent pilot study protocol. Nine of 15 patients (60%) completing induction therapy achieved a complete remission (CR) and three a partial remission (PR) (CR + PR rate 80%). Two of three patients with grossly resected disease (Groups I or II) are long‐term survivors (4+ years), while only one of ten (10%) who had unresectable gross residual disease after surgery (Groups III or IV) is known to be surviving free of disease more than two years. We conclude that the “group” dependent survival experience of patients with intrathoracic primaries appears similar to that observed in the IRS‐I Study as a whole, but that this particular primary site is associated with advanced disease at presentation and its attendant high mortality with the treatment used. Additionally, intrathoracic primary tumors occurred in older children and were more likely to be of undifferentiated histologic types than tumors from most other primary sites.

show abstract

mentioning

confidence: 99%

Intrathoracic soft tissue sarcomas in children

Rb²,

Newton³

et al. 1982

Cancer

View full text Add to dashboard Cite

show abstract

“…The patient described here is unique because he had symptoms by the age of two weeks, and it must thus be presumed that the tumour was present before birth . From previously published reports, we have identified 62 cases of rhabdomyosarcoma of the heart [1,[4][5][6][8][9][10] 13,[16][17][18][20][21][22][23][25][26][27][28][29][31][32][33]36,[38][39][40]43, 441 . Localization Cardiac sarcomas [16] seem in general to have a predilection for the right atrium, but the origin 0172-0643/82/0002-0073 $01 .00…”

mentioning

confidence: 99%

Primary rhabdomyosarcoma of the heart

Schmaltz

Apitz

1982

Pediatr Cardiol

View full text Add to dashboard Cite

A primary rhabdomyosarcoma of the heart observed in a 3-week-old infant, provides an opportunity to review this rare clinical entity; 61 reported cases are collated. The more common sites are right ventricle, left atrium, and right atrium; the tumour may protrude into the cavity and cause obstruction. Echocardiography can demonstrate the tumour, which may also been seen as a filling defect on angiography. Primary heart tumours seldom occur, especially in infancy and childhood. According to Van der Hauwaert, the most common cardiac tumour in infancy is the rhabdomyoma. No primary malignant tumour was seen in 15 European centres within a 5-year period.

show abstract

Primary Tumors of the Heart

Debois¹

2000

Selected Topics in Clinical Oncology

View full text Add to dashboard Cite

Rhabdomyosarcoma of the Heart

Cited by 7 publications

References 11 publications

Intrathoracic soft tissue sarcomas in children

Intrathoracic soft tissue sarcomas in children

Primary rhabdomyosarcoma of the heart

Primary Tumors of the Heart

Contact Info

Product

Resources

About