Inadvertent vincristine sulfate overdosage was treated with folinic acid. The sequence of development of the signs and symptoms associated with known vincristine toxicity was accentuated; however, the duration of these toxicities was markedly compressed in time when compared with previous reports. The known pharmacologic and biologic activities of vincristine sulfate and presumed mechanism of counteractivity of folinic acid is presented.

Twenty-three children with CNS tumors were treated with combination chemotherapy including nitrogen mustard, vincristine sulfate, procarbazine, and prednisone (MOPP). All but one had progressive or recurrent tumors following surgery and irradiation. In addition, nine of these patients had prior chemotherapy. Seventeen out of 23 patients (73.4%) responded to MOPP chemotherapy including seven patients who had prior chemotherapy with single or multiple agents such as VCR, nitrosoureas, intrathecal methotrexate, and VM-26. Three comatose patients who were being kept on Decadron without benefit recovered from coma. At the time of this report 8 of the 17 responders are surviving without evidence of recurrence 7--30 months from the start of MOPP chemotherapy. In two of these children chemotherapy has been completely stopped.

Most patients with dyskeratosis congenita [DC or Zinsser-Cole-Engman syndrome] are males hemizygous for an X-linked recessive mutation. However, one or more autosomal form(s) of DC may exist. We have studied a 6-year-old black girl with the characteristic triad of nail dystrophy, cutaneous and mucosal pigmentary changes, and bone marrow failure. Severe microcephaly, mental retardation, cerebellar hypoplasia, and purple discoloration of the tongue were other manifestations not usually seen in DC. Comparison of our patient's phenotype with that of 5 other sporadic and 10 familial cases of DC in females showed that the autosomal and X-linked phenotypes are not distinguishable in the absence of a suggestive pedigree pattern. Additional cases of DC need to be studied to better elucidate the apparent causal heterogeneity in this syndrome.

No abstract

Combination chemotherapy with adriamycin and DTIC was used in 102 evaluable patients under 15 years of age who had previously treated metastatic solid tumors. Responses, defined as 50% or more reduction in all tumor masses, occurred in 10 out of 27 patients with neuroblastoma, 3 out of 8 patients with Wilms tumor, 7 out 15 patients with Ewing sarcoma, 2 out of 6 patients with osteosarcoma, 5 out of 13 patients with rhabdomyosarcoma, and 15 out of 33 patients with miscellaneous tumors which included a patient who had a complete regression of an extensive juvenile angiofibroma. Response rate to combination chemotherapy with adriamycin and DTIC in patients with Ewing sarcoma was significantly superior to the response rate obtained with adriamycin alone in another Southwest Oncology Group Study. Major toxicity included nausea, vomiting, myelosuppression, high incidence of pneumocystis carinii pneumonia (5 patients) and congestive heart failure (4 patients). There was 7 drug-associated deaths due to sepsis (1), pneumocystis carinii pneumonia (4), and congestive heart failure (2).