Soft-tissue sarcomas of the diaphragm: A report from the intergroup rhabdomyosarcoma study group from 1972 to 1997

the Intergroup Rhabdomyosarcoma Study Group (IRSG), Children’s Cancer Group, the Pediatric Oncology Group, the Intergroup Rhabdomyosarcoma Statistical Office

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Purpose To describe clinical details and outcome of children and adolescents with primary sarcomas of the diaphragm treated on Intergroup Rhabdomyosarcoma Studies (IRS) I through IV. Patients and Methods We reviewed the records of 15 patients with sarcoma of the diaphragm who were entered on IRS Group protocols between 1972 and 1997. Patient ages at diagnosis ranged from 0.5 to 20 years (median, 13 yrs), and 10 were girls. Patients had chest pain, dyspnea, and/or coughing, decreased breath sounds, and occasionally hepatomegaly. Results Localized, gross residual disease after initial surgery was present in 10 patients, and five had metastases at diagnosis (pleura, 3; pericardium, 1; lungs and bones, 1). Tumor subtypes were alveolar rhabdomyosarcoma (RMS) in five cases, embryonal RMS in three, undifferentiated sarcoma in three, extraosseous Ewing sarcoma in three, and unclassified sarcoma in one. Treatment consisted of radiation therapy to the primary tumor and metastases when feasible, and combination chemotherapy with vincristine, actinomycin D, and cyclophosphamide with or without doxorubicin, ifosfamide, cisplatin, and etoposide. Ten patients achieved complete remission (67%), four obtained a partial remission, and one was improved. Five patients (33%) are continuously failure-free and alive at a median of 8.8 years from diagnosis (range, 1.1-15 yrs). However, the other 10 patients experienced relapse at 0.3 to 2 years from start of therapy (median, 1 yr). Sites of relapse were local in five, distant in three, and combined in two. Death after relapse occurred at 0.39 to 2.6 years (median, 1.6 yrs) from diagnosis. Conclusions Sarcomas of the diaphragm are generally deemed unresectable at diagnosis and/or are metastatic. Most of them are not embryonal rhabdomyosarcomas. Treatment with more effective primary chemotherapy to shrink the tumor, followed-up by surgical resection and radiation therapy, should improve the prognosis for patients with sarcomas arising in the diaphragm, especially for the majority who have localized tumors.

Original languageEnglish (US)
Pages (from-to)510-514
Number of pages5
JournalAmerican Journal of Pediatric Hematology/Oncology
Volume22
Issue number6
StatePublished - Jan 1 2000

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Rhabdomyosarcoma
Diaphragm
Sarcoma
Embryonal Rhabdomyosarcoma
Recurrence
Neoplasms
Radiotherapy
Alveolar Rhabdomyosarcoma
Neoplasm Metastasis
Ifosfamide
Ewing's Sarcoma
Hepatomegaly
Pleura
Pericardium
Dactinomycin
Vincristine
Etoposide
Combination Drug Therapy
Chest Pain
Dyspnea

Keywords

  • Childhood
  • Diaphragm
  • Soft-tissue sarcoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

the Intergroup Rhabdomyosarcoma Study Group (IRSG), Children’s Cancer Group, the Pediatric Oncology Group, & the Intergroup Rhabdomyosarcoma Statistical Office (2000). Soft-tissue sarcomas of the diaphragm: A report from the intergroup rhabdomyosarcoma study group from 1972 to 1997. American Journal of Pediatric Hematology/Oncology, 22(6), 510-514.

Soft-tissue sarcomas of the diaphragm : A report from the intergroup rhabdomyosarcoma study group from 1972 to 1997. / the Intergroup Rhabdomyosarcoma Study Group (IRSG); Children’s Cancer Group; the Pediatric Oncology Group; the Intergroup Rhabdomyosarcoma Statistical Office.

In: American Journal of Pediatric Hematology/Oncology, Vol. 22, No. 6, 01.01.2000, p. 510-514.

Research output: Contribution to journalArticle

the Intergroup Rhabdomyosarcoma Study Group (IRSG), Children’s Cancer Group, the Pediatric Oncology Group & the Intergroup Rhabdomyosarcoma Statistical Office 2000, 'Soft-tissue sarcomas of the diaphragm: A report from the intergroup rhabdomyosarcoma study group from 1972 to 1997', American Journal of Pediatric Hematology/Oncology, vol. 22, no. 6, pp. 510-514.
the Intergroup Rhabdomyosarcoma Study Group (IRSG), Children’s Cancer Group, the Pediatric Oncology Group, the Intergroup Rhabdomyosarcoma Statistical Office. Soft-tissue sarcomas of the diaphragm: A report from the intergroup rhabdomyosarcoma study group from 1972 to 1997. American Journal of Pediatric Hematology/Oncology. 2000 Jan 1;22(6):510-514.
the Intergroup Rhabdomyosarcoma Study Group (IRSG) ; Children’s Cancer Group ; the Pediatric Oncology Group ; the Intergroup Rhabdomyosarcoma Statistical Office. / Soft-tissue sarcomas of the diaphragm : A report from the intergroup rhabdomyosarcoma study group from 1972 to 1997. In: American Journal of Pediatric Hematology/Oncology. 2000 ; Vol. 22, No. 6. pp. 510-514.
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TY - JOUR

T1 - Soft-tissue sarcomas of the diaphragm

T2 - A report from the intergroup rhabdomyosarcoma study group from 1972 to 1997

AU - the Intergroup Rhabdomyosarcoma Study Group (IRSG)

AU - Children’s Cancer Group

AU - the Pediatric Oncology Group

AU - the Intergroup Rhabdomyosarcoma Statistical Office

AU - Beverly Raney, R.

AU - Anderson, James R.

AU - Andrassy, Richard J.

AU - Crist, William M.

AU - Donaldson, Sarah S.

AU - Maurer, Harold M.

AU - Arndt, Carola A.S.

AU - Baker, K. Scott

AU - Barr, Frederic G.

AU - Bleyer, W. Archie

AU - Breitfeld, Philip

AU - Breneman, John C.

AU - Bridge, Julia

AU - Brown, Kenneth

AU - Grier, Holcombe E.

AU - Hawkins, Douglas

AU - Houghton, Peter J.

AU - Link, Michael

AU - Lobe, Thom L.

AU - Meyer, William H.

AU - Michalski, Jeff

AU - Murphy, Sharon

AU - Paidas, Charles N.

AU - Pappo, Alberto S.

AU - Parham, David M.

AU - Qualman, Stephen J.

AU - Robison, Leslie

AU - Sandler, Eric

AU - Smith, Lynn

AU - Sorensen, Poul H.B.

AU - Teot, Lisa

AU - Triche, Timothy

AU - Vietti, Teresa J.

AU - Walterhouse, David

AU - Wharam, Moody

AU - Wiener, Eugene S.

AU - Wolden, Suzanne

AU - Womer, Richard

PY - 2000/1/1

Y1 - 2000/1/1

N2 - Purpose To describe clinical details and outcome of children and adolescents with primary sarcomas of the diaphragm treated on Intergroup Rhabdomyosarcoma Studies (IRS) I through IV. Patients and Methods We reviewed the records of 15 patients with sarcoma of the diaphragm who were entered on IRS Group protocols between 1972 and 1997. Patient ages at diagnosis ranged from 0.5 to 20 years (median, 13 yrs), and 10 were girls. Patients had chest pain, dyspnea, and/or coughing, decreased breath sounds, and occasionally hepatomegaly. Results Localized, gross residual disease after initial surgery was present in 10 patients, and five had metastases at diagnosis (pleura, 3; pericardium, 1; lungs and bones, 1). Tumor subtypes were alveolar rhabdomyosarcoma (RMS) in five cases, embryonal RMS in three, undifferentiated sarcoma in three, extraosseous Ewing sarcoma in three, and unclassified sarcoma in one. Treatment consisted of radiation therapy to the primary tumor and metastases when feasible, and combination chemotherapy with vincristine, actinomycin D, and cyclophosphamide with or without doxorubicin, ifosfamide, cisplatin, and etoposide. Ten patients achieved complete remission (67%), four obtained a partial remission, and one was improved. Five patients (33%) are continuously failure-free and alive at a median of 8.8 years from diagnosis (range, 1.1-15 yrs). However, the other 10 patients experienced relapse at 0.3 to 2 years from start of therapy (median, 1 yr). Sites of relapse were local in five, distant in three, and combined in two. Death after relapse occurred at 0.39 to 2.6 years (median, 1.6 yrs) from diagnosis. Conclusions Sarcomas of the diaphragm are generally deemed unresectable at diagnosis and/or are metastatic. Most of them are not embryonal rhabdomyosarcomas. Treatment with more effective primary chemotherapy to shrink the tumor, followed-up by surgical resection and radiation therapy, should improve the prognosis for patients with sarcomas arising in the diaphragm, especially for the majority who have localized tumors.

AB - Purpose To describe clinical details and outcome of children and adolescents with primary sarcomas of the diaphragm treated on Intergroup Rhabdomyosarcoma Studies (IRS) I through IV. Patients and Methods We reviewed the records of 15 patients with sarcoma of the diaphragm who were entered on IRS Group protocols between 1972 and 1997. Patient ages at diagnosis ranged from 0.5 to 20 years (median, 13 yrs), and 10 were girls. Patients had chest pain, dyspnea, and/or coughing, decreased breath sounds, and occasionally hepatomegaly. Results Localized, gross residual disease after initial surgery was present in 10 patients, and five had metastases at diagnosis (pleura, 3; pericardium, 1; lungs and bones, 1). Tumor subtypes were alveolar rhabdomyosarcoma (RMS) in five cases, embryonal RMS in three, undifferentiated sarcoma in three, extraosseous Ewing sarcoma in three, and unclassified sarcoma in one. Treatment consisted of radiation therapy to the primary tumor and metastases when feasible, and combination chemotherapy with vincristine, actinomycin D, and cyclophosphamide with or without doxorubicin, ifosfamide, cisplatin, and etoposide. Ten patients achieved complete remission (67%), four obtained a partial remission, and one was improved. Five patients (33%) are continuously failure-free and alive at a median of 8.8 years from diagnosis (range, 1.1-15 yrs). However, the other 10 patients experienced relapse at 0.3 to 2 years from start of therapy (median, 1 yr). Sites of relapse were local in five, distant in three, and combined in two. Death after relapse occurred at 0.39 to 2.6 years (median, 1.6 yrs) from diagnosis. Conclusions Sarcomas of the diaphragm are generally deemed unresectable at diagnosis and/or are metastatic. Most of them are not embryonal rhabdomyosarcomas. Treatment with more effective primary chemotherapy to shrink the tumor, followed-up by surgical resection and radiation therapy, should improve the prognosis for patients with sarcomas arising in the diaphragm, especially for the majority who have localized tumors.

KW - Childhood

KW - Diaphragm

KW - Soft-tissue sarcoma

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