Outcome of patients with localized orbital sarcoma who relapsed following treatment on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols-III and -IV, 1984-1997: a report from the Children's Oncology Group
Authors
Raney, R. BeverlyHuh, Winston W.
Hawkins, Douglas S.
Hayes-Jordan, Andrea
Million, Lynn
Rodeberg, David
Teot, Lisa
Anderson, James
Document Type
Journal ArticlePublication Date
2013-03-01Keywords
AdolescentAntineoplastic Combined Chemotherapy Protocols
Child
Child, Preschool
Clinical Trials, Phase III as Topic
Clinical Trials, Phase IV as Topic
Combined Modality Therapy
Female
History, Ancient
Humans
Male
Neoplasm Recurrence, Local
Orbital Neoplasms
Radiotherapy
Research Report
Retrospective Studies
Sarcoma
Treatment Outcome
Neoplasms
Oncology
Metadata
Show full item recordAbstract
BACKGROUND: We wanted to ascertain patterns of recurrence, re-treatment, and outcome among 188 eligible patients treated for localized orbital sarcoma on IRSG Protocols III/IV, 1984-1997. PROCEDURE: Retrospective chart review. RESULTS: Twenty-four of 188 patients (12.8%) developed local (n = 22) or distant relapse (n = 2) at 0.057-7.05 years (median, 1.58) after enrollment. Ages at study entry were 0.14-17 years (median, 5 years). Initial tumor operations included biopsy (n = 20) or gross resection with microscopic residual (n = 4). Initial tumor diameters were 0.5-7 cm (median, 3). Pathologic subtypes were embryonal rhabdomyosarcoma (ERMS, n = 19), sarcoma not otherwise specified (n = 2), and alveolar RMS, botryoid ERMS, or undifferentiated sarcoma (n = 1 each). Initial treatment included vincristine/dactinomycin (n = 24) including an alkylator (n = 4) and radiotherapy (RT, n = 21). Twenty patients responded, 14 completely, 6 partially. After recurrence, patients underwent orbital exenteration (n = 10), enucleation (2), tumor excision (3), or biopsy (1); 7 had no operation, and 1 had no data. Post-relapse chemotherapy included combinations of etoposide (n = 14 patients), doxorubicin (14), ifosfamide (12), cyclophosphamide (7), and dacarbazine (n = 1). Six patients received RT, including four previously treated and two not irradiated initially. Two patients died; one at 1.79 years after contralateral brain metastasis followed by local recurrence, and another at 2.49 years after multiple local recurrences. Twenty-two patients (91.7%) survived sarcoma-free for 0.04-17 years (median, 6.9) after relapse, and 18 of 22 (82%) were alive >/=5 years after relapse. CONCLUSION: Survival following recurrent localized orbital sarcoma appears likely after vigorous re-treatment given with curative intent.Source
Pediatr Blood Cancer. 2013 Mar;60(3):371-6. doi: 10.1002/pbc.24289. Link to article on publisher's siteDOI
10.1002/pbc.24289Permanent Link to this Item
http://hdl.handle.net/20.500.14038/47914PubMed ID
22961750Notes
This study was supported in part by Grant CA-29511 from the National Cancer Institute for the IROC Rhode Island (QARC), a quality assurance vehicle and data management service for diagnostic imaging and radiation oncology for the National Cancer Institute Clinical Trials Program. QARC is a research program within the University of Massachusetts Medical School led by Dr. Thomas (TJ) FitzGerald.
Related Resources
Link to Article in PubMedae974a485f413a2113503eed53cd6c53
10.1002/pbc.24289