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Background Little is known about treatment outcomes for children who have end-stage renal disease (ESRD) after treatment for Wilms tumor (WT). Methods Time-to-transplant, graft failure, and survival outcomes were examined for 173 children enrolled on the National Wilms Tumor Study who developed ESRD. Results Fifty-five patients whose ESRD resulted from progressive bilateral WT (PBWT) experienced high early mortality from WT that limited their opportunity for transplant (47% at 5 years) and survival (44% at 10 years) in comparison to population controls. The 118 patients whose ESRD was due to other causes (termed 'chronic kidney disease'), many of whom had WT-associated congenital anomalies, had transplant (77% at 5 years) and survival (73% at 10 years) outcomes no worse than those for population controls. Graft failure following transplant was comparable for the two groups. Minority children had twice the median time to transplant as non-Hispanic whites and twice the mortality rates, also reflecting population trends. Conclusions In view of the continuing high mortality in patients with ESRD, and the dramatic improvement in outlook following kidney transplantation, re-evaluation of current guidelines for a 2-year delay in transplant following WT treatment may be warranted. Keywords Chronic kidney failure * Transplantation * Graft failure, Introduction The past half-century witnessed dramatic progress in the treatment and survival of children with Wilms tumor (WT) [1, 2]. Since the late 1980s, 5-year survival rates have remained consistently [...]

The study aims to evaluate the survival outcome following reduced chemotherapy in the case of patients with intermediate-risk neuroblastoma. The results indicate that reduced chemotherapy was quite effective in improving the survival rate in the case of such patients.

Byline: Harry P.W. Kozakewich (1), Antonio R. Perez-Atayde (1), Michael J. Donovan (1), Jonathan A. Fletcher (2), Judy A. Estroff (3), Robert C. Shamberger (4), Lisa Diller (5) Keywords: Key words: congenital neuroblastoma, cystic neuroblastoma, adrenal cyst, newborn, infant Abstract: Cystic neuroblastoma (CN) is an unusual variant of neuroblastoma characterized by a grossly visible cyst(s) and almost always distinctive microcysts on light microscopy. Rarely, CN will appear solid grossly, but microcystification will be present. We examined the clinical, pathologic, and biologic features of 17 cases of CN. The majority of CN had been detected by prenatal ultrasound. The tumors were favorable stage, stroma-poor, but with low or intermediate mitotic-karyorhectic indices and had favorable biologic markers reflected by aneuploidy and by an absence of N-myc amplification and chromosome 1p deletions. However, the high trk expression typically identified in good risk tumors was absent. Although the complete natural history of CN is not fully defined, our experience suggests that some tumors progress in size, whereas others may spontaneously regress or mature. The clinical outcome is excellent, as is expected in localized and stage 4S neuroblastoma in infancy. Author Affiliation: (1) Department of Pathology, Children's Hospital, and Department of Pathology, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA , US (2) Department of Pathology, Brigham and Women's Hospital, and Departments of Pediatrics and Pathology, Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA , US (3) Department of Radiology, Children's Hospital, and Department of Radiology, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA , US (4) Department of Surgery, Children's Hospital, and Department of Surgery, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA , US (5) Department of Medicine, Children's Hospital, Department of Pediatric Oncology, Dana-Farber Cancer Institute, and Department of Pediatrics, Harvard Medical School, 44 Binney Street, Boston, MA 02115, USA , US Article note: Received June 13, 1996 accepted June 19, 1997

Ultrasound prenatal diagnosis of neuroblastoma usually results in infant survival. Neuroblastoma is a cancer of the nervous system that occurs in embryos. The records of eleven infant patients with neuroblastoma identified by prenatal ultrasound in a hospital between 1982 and 1992 were reviewed. Nine of the patients had adrenal tumors and two had thoracic paraspinal tumors. All patients had their tumors surgically removed. One patient received chemotherapy when the cancer continued to progress after surgery. No patients died during follow-up periods ranging from three months to ten years. Prenatal ultrasound diagnosis, surgery after birth and chemotherapy as a last resort seems the best treatment and has excellent results., Objectives. To assess the relative frequency of, the clinical and pathological correlates in, and the prognosis of the subset of infants with neuroblastoma who were identified initially by prenatal ultrasonography. Design. Retrospective review of all patients with neuroblastoma evaluated between 1982 and 1992. Setting. Large, urban, tertiary care children's hospital in Boston, Massachusetts. Patients. Eleven infants with neuroblastoma initially detected with prenatal sonograms were identified. Results. Nine patients had adrenal tumors; two had thoracic paraspinal tumors. Typical diagnostic evidence for neuroblastoma including a palpable abdominal mass and elevations in urinary catecholamines were not commonly seen postnatally. These patients had multiple favorable prognostic indicators including low stage of disease (10/11), favorable biological markers including cellular DNA content (5/5) and N-myc oncogene copy number (5/5), and histopathology suggestive for neuroblastoma in situ (7/11). All patients were treated by surgical resection. One patient exhibited progression of disease postoperatively, but demonstrated a complete clinical response to multiagent chemotherapy. Overall survival in our population was excellent with no deaths seen at a mean follow-up of 37 months (range 3 to 120 months). Conclusions. Patients with neuroblastoma identified by prenatal ultrasonography generally, although not exclusively, follow a clinically favorable course in which surgical resection is curative. Chemotherapy is not indicated unless substantial progression of disease occurs.