RESUMO
Malignant lymphoma of the central nervous system in a thirteen-year-old boy with immotile cilia syndrome (ICS) is reported. He had frequent upper respiratory tract infections, chronic sinusitis and pneumonia during in childhood. Bronchiectasis was demonstrated by bronchography. The diagnosis of ICS was confirmed by the lack of dynein arms of cila in the nasal mucosa with electronmicroscopy. In 1987, he complained of headache and vomiting and a space occupied mass lesion in the left frontoparietal lobe was found by head CT scan, which was subtotally resected. Histological studies showed large cell type non-Hodgikin lymphoma of B-cell phenotype. He received radiotherapy (41Gy) to the whole brain and systemic chemotherapy consisting of adriamycin, cyclophosphamide, vincristine, prednisolone, L-asparaginase and intrathecal methotrexate, and the patient maintained complete remission for eight months. However, relapse occurred and the patient died twelve months after the initiation of treatment.
Assuntos
Neoplasias Encefálicas/complicações , Transtornos da Motilidade Ciliar/complicações , Linfoma não Hodgkin/complicações , Adolescente , Humanos , MasculinoRESUMO
The therapeutic results obtained in eight cases of childhood malignant soft-tissue sarcoma are summarized. Male to female ratio was 4:4, four were rhabdomyosarcoma and 5 were defined as Group I/II. Adjuvant combination chemotherapy was administered soon after biopsy (3 cases), or after partial or total resection (3 cases) of the primary tumor. In one case, irradiation was employed and in the remaining case, no further therapy was given after surgery. The three patients who underwent total removal of the tumor followed by adjuvant chemotherapy including adriamycin, and a patient with a partially resected intra-orbital tumor which responded well to a similar form of chemotherapy are currently alive after 26+ to 71+ months. By contrast, four patients whose tumors were too bulky to be resected responded poorly to chemotherapy and died. It must be emphasized that adjuvant chemotherapy is of limited value in the treatment of advanced childhood soft-tissue sarcoma.
