Multidisciplinary treatment of primary orbital rhabdomyosarcoma. A single-institution experience

Abstract

Orbital rhabdomyosarcoma accounts for one-fourth of the primary tumors in the head and neck region. Modern treatment modalities have led to a 2-year survival rate of about 90% in these patients. However, new therapeutic trials are designed to reduce complications and salvage more than 90% of orbital cases. Between 1979 and 1990, 12 children affected by primary orbital rhabdomyosarcoma have been diagnosed and treated at the University of Naples. Ten of them have been uniformly treated by biopsy, followed by immediate radiation and combined chemotherapy. All 12 patients are alive and free of detectable disease, from a minimum of 7 months to a maximum of 123 months after diagnosis. In all children, ocular structures have been spared and the complications observed until now have been few. The above results suggest that the association of immediate radiation therapy and chemotherapy might represent an optimal tool for treatment of orbital rhabdomyosarcoma.

Rhabdomyosarcoma in Children: A SEER Population Based Study

“…Our analysis revealed a 20% survival advantage of embryonal versus non-embryonal subtypes of RMS. This substantial difference in prognosis between RMS types may be due to well-known distinct genetic alterations that putatively play a role in the pathogenesis of these tumors and their response to treatment [25]. When specifically looking at SEER data, embryonal RMS presented most often with localized disease, at favorable sites, and in children younger than 10 y of age.…”

Les cancers de l’orbite etude retrospective à propos de 31 cas

“…Rhabdomyosarcome embryonnaire 3/6 (50%) 3/6 (50%) 1/6 (1 6%) (4). Pour les rhabdomyosarcomes, certains auteurs préconisent un traitement radical avec une exentération orbitaire large suivi de RT (7), d'autres préconisent une radiochimiothérapie qui permet d'obtenir un bon contrôle local de la tumeur mais avec un taux élevé de récidive (7,8,9,10). Le traitement de choix des lymphomes repose sur l'association radio-chimiothérapie (11).…”

Rhabdomyosarcoma in children

“…Patients with tumors expressing the PAX-FKHR fusion tend to be younger and more likely to present with an extremity lesion, suggesting a distinct clinical phenotype. PCR assays are now available that allow for confirmation of the diagnosis of alveolar RMS based on the presence of these fusion genes [14][15][16][17].…”