Adult rhabdomyosarcoma

Outcome following multimodality treatment

Darren J. Little, Matthew Ballo, Gunar K. Zagars, Peter W T Pisters, Shreyaskumar R. Patel, Adel K. El-Naggar, Adam S. Garden, Robert S. Benjamin

Research output: Contribution to journalArticle

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Abstract

BACKGROUND. Childhood rhabdomyosarcoma (RMS) has a relatively good prognosis. Outcome for adults with this disease is poorly documented due to its rarity. METHODS. The clinicopathologic features, treatment methods, and disease outcome were reviewed retrospectively for 82 adults with locoregional RMS treated between 1960 and 1998. Patients with distant metastasis at diagnosis were excluded. Actuarial univariate and multivariate statistical methods were used to evaluate outcome. RESULTS. Patient ages ranged from 17 to 84 years (median, 27 years). Histologic subtypes were embryonal (34%), pleomorphic (43%), and alveolar (23%). Anatomic sites of origin were head and neck (52%), trunk (26%), and extremity (7%). Tumor size was 5 cm or smaller in 51% of patients. Regional lymph node metastasis was present in 33% of patients at presentation. Treatment consisted of radiation alone in 11%, radiation and surgery in 18%, radiation and chemotherapy in 34%, and all three modalities in 37%. With a median follow-up of 10.5 years, the 10-year actuarial disease-free and overall survival rates were 41% and 40%, respectively. The 10-year actuarial local, lymph node, and metastatic control rates were 75%, 82%, and 53%, respectively. The major determinant of metastatic control and survival was primary tumor size (≤ 5 vs. > 5 cm). Local control was satisfactory (10-year rate of 87%) for sites other than parameningeal (50% at 10 years). Patients whose disease responded to chemotherapy had a significantly better metastasis free period (72% at 10 years) than those whose disease failed to respond (19% at 10 years). CONCLUSIONS. Adult RMS is a highly malignant tumor with a significant incidence of metastatic recurrence. Continuing investigation of new and potentially more effective chemotherapy is crucial. Local control is satisfactory for sites other than parameningeal where new radiation technologies such as intensity-modulated therapy may be necessary to safely deliver adequate doses.

Original languageEnglish (US)
Pages (from-to)377-388
Number of pages12
JournalCancer
Volume95
Issue number2
DOIs
StatePublished - Jul 15 2002
Externally publishedYes

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Rhabdomyosarcoma
Radiation
Neoplasm Metastasis
Drug Therapy
Lymph Nodes
Therapeutics
Neoplasms
Disease-Free Survival
Neck
Survival Rate
Extremities
Head
Technology
Recurrence
Survival
Incidence

All Science Journal Classification (ASJC) codes

  • Cancer Research
  • Oncology

Cite this

Little, D. J., Ballo, M., Zagars, G. K., Pisters, P. W. T., Patel, S. R., El-Naggar, A. K., ... Benjamin, R. S. (2002). Adult rhabdomyosarcoma: Outcome following multimodality treatment. Cancer, 95(2), 377-388. https://doi.org/10.1002/cncr.10669

Adult rhabdomyosarcoma : Outcome following multimodality treatment. / Little, Darren J.; Ballo, Matthew; Zagars, Gunar K.; Pisters, Peter W T; Patel, Shreyaskumar R.; El-Naggar, Adel K.; Garden, Adam S.; Benjamin, Robert S.

In: Cancer, Vol. 95, No. 2, 15.07.2002, p. 377-388.

Research output: Contribution to journalArticle

Little, DJ, Ballo, M, Zagars, GK, Pisters, PWT, Patel, SR, El-Naggar, AK, Garden, AS & Benjamin, RS 2002, 'Adult rhabdomyosarcoma: Outcome following multimodality treatment', Cancer, vol. 95, no. 2, pp. 377-388. https://doi.org/10.1002/cncr.10669
Little DJ, Ballo M, Zagars GK, Pisters PWT, Patel SR, El-Naggar AK et al. Adult rhabdomyosarcoma: Outcome following multimodality treatment. Cancer. 2002 Jul 15;95(2):377-388. https://doi.org/10.1002/cncr.10669
Little, Darren J. ; Ballo, Matthew ; Zagars, Gunar K. ; Pisters, Peter W T ; Patel, Shreyaskumar R. ; El-Naggar, Adel K. ; Garden, Adam S. ; Benjamin, Robert S. / Adult rhabdomyosarcoma : Outcome following multimodality treatment. In: Cancer. 2002 ; Vol. 95, No. 2. pp. 377-388.
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abstract = "BACKGROUND. Childhood rhabdomyosarcoma (RMS) has a relatively good prognosis. Outcome for adults with this disease is poorly documented due to its rarity. METHODS. The clinicopathologic features, treatment methods, and disease outcome were reviewed retrospectively for 82 adults with locoregional RMS treated between 1960 and 1998. Patients with distant metastasis at diagnosis were excluded. Actuarial univariate and multivariate statistical methods were used to evaluate outcome. RESULTS. Patient ages ranged from 17 to 84 years (median, 27 years). Histologic subtypes were embryonal (34{\%}), pleomorphic (43{\%}), and alveolar (23{\%}). Anatomic sites of origin were head and neck (52{\%}), trunk (26{\%}), and extremity (7{\%}). Tumor size was 5 cm or smaller in 51{\%} of patients. Regional lymph node metastasis was present in 33{\%} of patients at presentation. Treatment consisted of radiation alone in 11{\%}, radiation and surgery in 18{\%}, radiation and chemotherapy in 34{\%}, and all three modalities in 37{\%}. With a median follow-up of 10.5 years, the 10-year actuarial disease-free and overall survival rates were 41{\%} and 40{\%}, respectively. The 10-year actuarial local, lymph node, and metastatic control rates were 75{\%}, 82{\%}, and 53{\%}, respectively. The major determinant of metastatic control and survival was primary tumor size (≤ 5 vs. > 5 cm). Local control was satisfactory (10-year rate of 87{\%}) for sites other than parameningeal (50{\%} at 10 years). Patients whose disease responded to chemotherapy had a significantly better metastasis free period (72{\%} at 10 years) than those whose disease failed to respond (19{\%} at 10 years). CONCLUSIONS. Adult RMS is a highly malignant tumor with a significant incidence of metastatic recurrence. Continuing investigation of new and potentially more effective chemotherapy is crucial. Local control is satisfactory for sites other than parameningeal where new radiation technologies such as intensity-modulated therapy may be necessary to safely deliver adequate doses.",
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T2 - Outcome following multimodality treatment

AU - Little, Darren J.

AU - Ballo, Matthew

AU - Zagars, Gunar K.

AU - Pisters, Peter W T

AU - Patel, Shreyaskumar R.

AU - El-Naggar, Adel K.

AU - Garden, Adam S.

AU - Benjamin, Robert S.

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N2 - BACKGROUND. Childhood rhabdomyosarcoma (RMS) has a relatively good prognosis. Outcome for adults with this disease is poorly documented due to its rarity. METHODS. The clinicopathologic features, treatment methods, and disease outcome were reviewed retrospectively for 82 adults with locoregional RMS treated between 1960 and 1998. Patients with distant metastasis at diagnosis were excluded. Actuarial univariate and multivariate statistical methods were used to evaluate outcome. RESULTS. Patient ages ranged from 17 to 84 years (median, 27 years). Histologic subtypes were embryonal (34%), pleomorphic (43%), and alveolar (23%). Anatomic sites of origin were head and neck (52%), trunk (26%), and extremity (7%). Tumor size was 5 cm or smaller in 51% of patients. Regional lymph node metastasis was present in 33% of patients at presentation. Treatment consisted of radiation alone in 11%, radiation and surgery in 18%, radiation and chemotherapy in 34%, and all three modalities in 37%. With a median follow-up of 10.5 years, the 10-year actuarial disease-free and overall survival rates were 41% and 40%, respectively. The 10-year actuarial local, lymph node, and metastatic control rates were 75%, 82%, and 53%, respectively. The major determinant of metastatic control and survival was primary tumor size (≤ 5 vs. > 5 cm). Local control was satisfactory (10-year rate of 87%) for sites other than parameningeal (50% at 10 years). Patients whose disease responded to chemotherapy had a significantly better metastasis free period (72% at 10 years) than those whose disease failed to respond (19% at 10 years). CONCLUSIONS. Adult RMS is a highly malignant tumor with a significant incidence of metastatic recurrence. Continuing investigation of new and potentially more effective chemotherapy is crucial. Local control is satisfactory for sites other than parameningeal where new radiation technologies such as intensity-modulated therapy may be necessary to safely deliver adequate doses.

AB - BACKGROUND. Childhood rhabdomyosarcoma (RMS) has a relatively good prognosis. Outcome for adults with this disease is poorly documented due to its rarity. METHODS. The clinicopathologic features, treatment methods, and disease outcome were reviewed retrospectively for 82 adults with locoregional RMS treated between 1960 and 1998. Patients with distant metastasis at diagnosis were excluded. Actuarial univariate and multivariate statistical methods were used to evaluate outcome. RESULTS. Patient ages ranged from 17 to 84 years (median, 27 years). Histologic subtypes were embryonal (34%), pleomorphic (43%), and alveolar (23%). Anatomic sites of origin were head and neck (52%), trunk (26%), and extremity (7%). Tumor size was 5 cm or smaller in 51% of patients. Regional lymph node metastasis was present in 33% of patients at presentation. Treatment consisted of radiation alone in 11%, radiation and surgery in 18%, radiation and chemotherapy in 34%, and all three modalities in 37%. With a median follow-up of 10.5 years, the 10-year actuarial disease-free and overall survival rates were 41% and 40%, respectively. The 10-year actuarial local, lymph node, and metastatic control rates were 75%, 82%, and 53%, respectively. The major determinant of metastatic control and survival was primary tumor size (≤ 5 vs. > 5 cm). Local control was satisfactory (10-year rate of 87%) for sites other than parameningeal (50% at 10 years). Patients whose disease responded to chemotherapy had a significantly better metastasis free period (72% at 10 years) than those whose disease failed to respond (19% at 10 years). CONCLUSIONS. Adult RMS is a highly malignant tumor with a significant incidence of metastatic recurrence. Continuing investigation of new and potentially more effective chemotherapy is crucial. Local control is satisfactory for sites other than parameningeal where new radiation technologies such as intensity-modulated therapy may be necessary to safely deliver adequate doses.

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