While the workshop was co-sponsored by the NCI and RSS, the comments in this report are strictly the opinions of the co-authors and does not constitute endorsement of these results and/or treatments by the NCI and RSS or consensus of all the co-authors on each of the points. This report is designed to stimulate further formal research and development to explore the future clinical application of these novel therapies and not for implementation into routine clinical practice.
A retrospective review of all 115 infants less than 1 year of age with acute lymphoblastic leukemia (ALL) entered on a consecutive series of recent Children's Cancer Study Group (CCSG) leukemia protocols was undertaken to examine in detail the outcome and clinical course of a large group of similarly treated infants. In comparison to the 4,392 children older than 1 year, entered on the same studies, infants had a significantly (P = .0001) increased incidence of leukocytosis, hepatosplenomegaly, meningeal leukemia at presentation, hypogammaglobulinemia, and failure to achieve complete remission (CR) status by day 14 of induction therapy. In contrast, lymphadenopathy, non-L1 French-American-British (FAB) morphology, mediastinal mass, and T cell leukemia were not more frequently observed. Ninety percent of these infants successfully completed the induction phase of therapy. With a median follow-up of 35 months, life table estimate of disease-free survival is only 23% at 4 years. Identical disease-free survival rates for infants were observed in each of the individual studies reviewed. Excessive toxicity resulting in limitation of therapy delivered was not a causative factor for the disappointing outcome of these patients. Rather, early disease recurrence, characterized by bone marrow relapse (55%) and CNS (22%) relapse, was the major factor responsible for the extremely poor prognosis of this patient group. Identical CNS relapse rates were observed in those patients who received cranial irradiation as part of CNS prophylaxis (21.8%) and in those patients who did not receive cranial radiotherapy (24%). Results of salvage therapy for patients who experienced systemic or extramedullary relapse were dismal. Debilitating neuropsychologic sequellae, presumably related to CNS irradiation, have been observed in 50% of the small number of long-term survivors. Infants less than 1 year of age with ALL present with a constellation of features which predict a poor outcome and constitute the group of children with ALL at greatest risk for treatment failure.
Placement of ventriculo-peritoneal (VP) shunts is an established procedure for treatment of hydrocephalus. With increasing longevity following successful treatment, complications are becoming more common. The authors reviewed 350 VP shunts in 242 patients and found five uncommon complications relating to the peritoneal end of the catheter: (a) cerebrospinal fluid (CSF) pseudocyst of the lesser sac, (b) subphrenic CSF loculation, (c) bowel perforation and formation of a CSF-enteric fistula, (d) intrathoracic migration of the tip of the shunt, and (e) intractable CSF ascites.
The treatment of 21 patients with a diagnosis of chordoma seen over a 20-year period is described. The 13 men and eight women (ratio 1.6:1) ranged in age from 7 to 82 years (average, 50 years). Eleven chordomas arose from the clivus; the others originated in the sacral region (three patients), thoracic spine (one), cervical spine (three), and lumbar spine (three). Primary treatment consisted of surgery, reserving radiation therapy for recurrence in the majority of patients. Radiation doses, delivered with megavoltage equipment, ranged between 5,000 cGy delivered over 5 weeks and 6,600 cGy over 6 1/2 weeks. Irradiation produced significant tumor control, with remission of symptoms from 1 to 6 years. The combination of high-dose radiation therapy and complete or subtotal surgical resection offers the best chance for prolonged survival in this group of patients.
Objective Lattice radiotherapy (LRT) is a novel technique of delivering heterogeneous doses of radiation to voluminous tumors not amenable to surgery. Built from the conventional two-dimensional grid, LRT utilizes the power of new technology, three-dimensional radiation allowing the delivery of higher doses of radiation to small spheres, also called vertices, inside bulky tumors while limiting exposure to surrounding healthy tissue. The main goals of the study were the evaluation of tumor response and the overall safety of LRT in this cohort of patients with bulky non-small cell lung cancer. Materials and methods During a seven-year period, 10 patients with non-small cell lung cancer (NSCLC), who presented with bulky, unresectable tumors, were treated using a single fraction of LRT followed by conventionally fractionated radiation. Patients received one initial LRT fraction of 18 Gy in the vertices and 3 Gy in the periphery. After the LRT, all patients continued with conventional radiation: 25 to 29 daily fractions of 1.8 Gy to 2 Gy. Results With a median follow-up of six months (range: one to 71 months), the mean decrease in tumor volume was 42%. The overall survival of the entire group ranged from four to 86 months (mean 22, median 16). There was no mortality related to LRT. No significant acute or chronic toxicity was noted. Conclusion In this small cohort, LRT appears to be a safe and effective modality to treat bulky NSCLC. Further research is needed to establish its efficacy in the management of voluminous NSCLC.
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