Improved prognosis with intensive treatment of children with cranial soft tissue sarcomas arising in nonorbital parameningeal sites. A report from the intergroup rhabdomyosarcoma study

  title={Improved prognosis with intensive treatment of children with cranial soft tissue sarcomas arising in nonorbital parameningeal sites. A report from the intergroup rhabdomyosarcoma study},
  author={Richard Beverly Raney and Melvin Tefft and William A. Newton and Abdelsalam H. Ragab and Walter Lawrence and Edmund A. Gehan and Harold M. Maurer},
In the first Intergroup Rhabdomyosarcoma Study (IRS‐I), it was found that, of 57 patients with nonorbital cranial parameningeal sarcoma treated from 1972 to 1976, 20 patients (35%) developed meningeal sarcoma and 90% (18/20) died of this complication. Since about half of these 20 patients had received inadequate radiation therapy and all had radiation therapy delayed until week 6, the treatment program was modified in December 1977 to begin radiation therapy on day 0 to the entire neuraxis and… 
Treatment of children and adolescents with localized parameningeal sarcoma: experience of the Intergroup Rhabdomyosarcoma Study Group protocols IRS-II through -IV, 1978-1997.
Biopsy, XRT to the target volume, and systemic chemotherapy are successful treatments for the large majority of patients with localized parameningeal sarcoma and aggressive initial surgical management of these patients is unnecessary.
Late complications of therapy in 213 children with localized, nonorbital soft-tissue sarcoma of the head and neck: A descriptive report from the Intergroup Rhabdomyosarcoma Studies (IRS)-II and - III
Background. This review of children and adolescents with nonorbital soft-tissue sarcoma of the head and neck was undertaken to describe late sequelae of treatment, as manifested primarily by problems
Ascending myelitis after intensive chemotherapy and radiation therapy in children with cranial parameningeal sarcoma
No additional cases have been reported to the IRS since the protocol was revised in 1987 to reduce the doses of the IT drugs and to limit them to four courses each, and the exact cause of this complication is unclear.
Treatment of nonmetastatic cranial parameningeal rhabdomyosarcoma in children younger than 3 years old: results from international society of pediatric oncology studies MMT 89 and 95.
Despite concerns about the late effects of its use in young children, cure of PM RMS remains unlikely without systematic use of RT, so accurate prediction of the small subset of patients who achieve local control without RT and who do not experience relapse would provide an opportunity for a minority of patients to avoid RT.
Local-regional non-rhabdomyosarcomatous soft tissue sarcomas of the head and neck.
Late effects of therapy in 94 patients with localized rhabdomyosarcoma of the orbit: Report from the Intergroup Rhabdomyosarcoma Study (IRS)-III, 1984-1991.
The overall survival rate was 96% and the eye was preserved in 86% of the patients, but vision was impaired in 70% of them, and the current IRS-V study recommends decreasing the dose of irradiation and using conformal techniques in an attempt to minimize these complications.
Primary intraspinal soft-tissue sarcoma in childhood: report of two cases with a review of the literature.
Two young children who presented with lower spinal cord dysfunction manifested by bilateral leg weakness and urinary retention were diagnosed with intraspinal soft-tissue sarcoma and died within 6 months of diagnosis, despite intrathecal chemotherapy and irradiation for one and very high-dose intravenous methotrexate for the other.
Long-term effects in children treated with radiotherapy for head and neck rhabdomyosarcoma.


Incidence of meningeal involvement by rhabdomyosarcoma of the head and neck in children. A report of the intergroup rhabdomyosarcoma study (IRS)
Recommendations for managing patients with embryonal rhabdomyosarcoma include earlier use of radiation and increased coverage of adjacent meninges by radiation including total craniospinal axis radiation when brain meningeal involvement exists.
Rhabdomyosarcoma of the ear in childhood. A report from the intergroup rhabdomyosarcoma study‐I
Outcome was influenced by the presence of diagnosis of signs indicating meningeal extension; death rates were 5 of 5 patients with intracranial tumor, 3 of 4 with petrous bone erosion, 2 of 6 with facial nerve palsy, and 3 of 9 with no initial evidence of meningealing extension.
Parameningeal rhabdomyosarcoma in the young
It is considered that primary tumor radiation treatment parameters may be critical in determining the incidence of subsequent meningeal relapse.
Childhood rhabdomyosarcoma with meningeal extension: Results of combined therapy including central nervous system prophylaxis
The higher doses of radiotherapy delivered to the primary tumor in the second series prevent the conclusion that the improved results are attributable entirely to the addition of central nervous system (CNS) prophylaxis.
Meningeal seeding from rhabdomyosarcoma of the head and neck with base of the skull invasion: recognition of the clinical evolution and suggestions for management.
Patients with erosion of the base of the skull or abnormal cranial nerve findings seem to have a risk of seeding the meninges with tumor, and tentative treatment guidelines are offered.
Multidisciplinary treatment of embryonal rhabdomyosarcoma in children
Twenty‐nine children under 15 years of age with embryonal rhabdomyosarcoma treated according to a multidisciplinary protocol are alive with no evidence of disease for 4+ to 42+ months, superior to those achieved between 1960–1970 among 108 children treated at Memorial Sloan‐Kettering Cancer Center.
Evaluation of intensified chemotherapy in children with advanced rhabdomyosarcoma (clinical groups III and IV). The Intergroup Rhabdomyosarcoma Study (IRS) Committee of the Cancer and Leukemia Group B Children's Cancer Study Group, Southwest Oncology Group.
Twenty-seven previously untreated children with gross residual or metastatic rhabdomyosarcoma were treated with pulse-VAC and radiotherapy and severe toxicity disappeared with drug dose reduction in subsequent courses.
Rhabdomyosarcoma of the head and neck. An appraisal of the biologic behavior in 170 cases.
The largest number of head and neck rhabdomyosarcomas observed and treated at any one center is that reported from the Memorial Center for Cancer and Allied Diseases, New York City, and Stobbe and Dargeon and Moore and Grossi reported a total of 52 cases over a 25-year period.
Rhabdomyosarcoma of head and neck in children
Combined systematic therapy offers the best chance of cure for children with rhabdomyosarcoma originating in the head and neck, and late recurrences or complications require further follow‐up.