RESUMO
Objective The intracavitary irradiation of cystic tumors has been used as a therapeutic alternative modality in the management of cystic craniopharyngiomas. In the present study, we review our experience, considering the technical issues, outcomes, and complications associated with the use of stereotactic intracavitary irradiation (SICI) with colloidal rhenium-186 (186Re) for cystic craniopharyngioma. Material and Methods The records of 33 patients with cystic craniopharyngiomas treated by SICI with colloidal 186Re were retrospectively reviewed. The median radiation dose to the cyst wall was of 408 Gy (range: 175 Gy to 500 Gy). All tumors were composed of a large cyst cavity, and 9 (27.3%) also had a solid component. The mean follow-up period was of 3.7 years. Results After SICI, 31 (93.9%) patients showed radiological evidence of cyst regression, and, in 2 (6.1%), no response was observed. An improvement in the visual deficits was observed in 8 cases (24.2%), and an improvement in endocrinogical disturbances, in 2 cases (6.1%). We observed complications in 3 patients (9.1%): diabetes insupidus in 1 case (3%), aggravation of visual acuity in 1 case (3%), and severe headache after infusion of the colloid in 1 case (3%); and 1 patient (3%) died after meningitis. Conclusion Stereotactic intracavitary irradiation with colloidal 186Re is a safe procedure, with satisfactory results in the present series, and should be considered, in the management of cystic craniopharyngiomas, the first-intention therapy or as an adjuvant to other therapeutical modalities, with acceptable morbidity and mortality rates.
Objetivo A irradiação intracavitária tem sido empregada como modalidade terapêutica alternativa no manejo dos craniofaringiomas císticos. No presente estudo, revisamos nossa experiência, considerando parâmetros técnicos, resultados e complicações associadas ao uso da irradiação estereotáxica intracavitária (IEIC) com rênio186 (186Re) coloidal em pacientes com craniofaringiomas císticos. Material e Métodos Os prontuários de 33 pacientes com craniofaringiomas císticos tratados por IEIC com 186Re coloidal foram revisados retrospectivamente. A dose média de radiação na parede do cisto foi de 408 Gy (variação: 175 Gy a 500 Gy). Todos os tumores eram compostos por uma grande porção cística, e, em 9 casos (27,3%) havia também um componente sólido. O período médio de seguimento foi de 3,7 anos. Resultados Após a IEIC, 31 (93,9%) pacientes apresentaram evidência radiológica de regressão do cisto, e em 2 (6,1%) não foi observada resposta. Melhora do déficit visual foi observada em 8 casos (24,2%), e dos distúrbios endocrinológicos, em 2 casos (6,1%). Complicações ocorreram em 3 pacientes (9,1%): diabetes insipidus em 1 caso (3%), piora da acuidade visual em 1 caso (3%), e cefaleia intensa após a infusão do coloide em 1 caso (3%); e 1 paciente (3%) faleceu após meningite. Conclusão A IEIC com 186Re coloidal é um procedimento seguro, com resultados satisfatórios nesta série, e deve ser considerada no manejo de craniofaringiomas císticos, seja como intervenção primária, seja como adjuvante a outras modalidades terapêuticas, com taxas de morbidade e mortalidade aceitáveis.
RESUMO
PURPOSE: A 10-month-old girl with a Brachmann-Cornelia de Lange syndrome and a choroid plexus papilloma of the brain was studied at the Hospital Infantil de México Federico Gómez (HIMFG) in Mexico City. METHODS AND RESULTS: Presumptive papilloma of the third ventricle was evidenced on CT and MR images and removed. Pathological analysis confirmed its origin. A posterior radiosurgery was required due to a tumor relapse. Karyotypes (GTG bands) of the patient and her parents undertaken at HIMFG were normal. Array comparative genomic hybridization (array CGH) analyses of blood DNA of the patient and her parents carried out at BlueGnome's Laboratory in Cambridge, UK, set in evidence amplification of genes SPNS2, GGT6, SMTNL2, PELP1, MYBBP1A, and ALOX15 in chromosome 17p of the patient. Since MYBBP1A is a proto-oncogene and ALOX15 participates in the development of cancer and metastases of tumors, further fluorescent in situ hybridization (FISH) analyses of these two genes were implemented at HIMFG. Amplification of the two genes was found in the tumor of the case under study but not in an unrelated papilloma of the choroid plexus. DISCUSSION: Further analyses of the association of choroid plexus papillomas with disorders of psycho-neural development and its relationship to molecular genetic modifications at chromosome 17p are now under way at HIMFG.