title: Orbital sarcoma with metastases at diagnosis: a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.
abstract: We reviewed clinicopathologic features and treatment outcomes in seven patients diagnosed with Stage 4/Group IV orbital sarcoma and treated on IRSG protocols I-III. Three patients had embryonal rhabdomyosarcoma (RMS), and two patients each had alveolar RMS or unclassified sarcoma. Median age at diagnosis was 1.8 years (range 0.2-6.9 years). All patients had bone marrow involvement, including six with normal complete blood count at diagnosis. Cerebrospinal fluid was normal in six patients. Three patients survived >5 years, including one with local recurrence. In conclusion, further study is needed to determine necessity of bone marrow and CSF examination in orbital sarcoma patients.
authors.affiliation: Division of Pediatrics, The University of Texas M.D. Anderson Cancer Center, Houston, Texas 77030, USA. whuh@mdanderson.org
authors.forename: Winston W
authors.initials: WW
authors.lastname: Huh
authors.forename: James R
authors.initials: JR
authors.lastname: Anderson
authors.forename: David
authors.initials: D
authors.lastname: Rodeberg
authors.forename: Lisa
authors.initials: L
authors.lastname: Teot
authors.forename: Torunn
authors.initials: T
authors.lastname: Yock
authors.forename: R Beverly
authors.initials: RB
authors.lastname: Raney
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003609:Dactinomycin / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005047:Etoposide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male; D009362:Neoplasm Metastasis / Q000188:drug therapy / Q000473:pathology*; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D009367:Neoplasm Staging; D009918:Orbital Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D011878:Radiotherapy; D012509:Sarcoma / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D002945:Cisplatin