Inability to alkalinize urine in a patient at risk for tumor lysis syndrome: a case report.

This report provides a description and discussion of a 19-year-old, 65-kg male, with a large mediastinal mass, right pleural effusion, and pericardial effusion, requiring urine alkalinization during a propofol infusion. The patient required NaHCO3 boluses, urine pH, electrolyte, arterial blood gas and lactate monitoring, and discontinuation of the propofol. The authors suggest that caution be used when prescribing a propofol infusion for patients who are at risk of tumor lysis syndrome and the need for urine alkalinization.

Natural course of neuroblastoma detected by mass screening: s 5-year prospective study at a single institution.

PURPOSE: To describe various favorable courses of neuroblastoma (NBL) detected by mass screening and to present our observation program as a temporary treatment option, to be used until a final decision is made regarding the mass screening program for 6-month-old infants. PATIENTS AND METHODS: Between October 1993 and November 1999, 26 of 51 patients with NBL detected by mass screening were enrolled in our observation program. The criteria for observation included urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels less than 50 microg/mg creatinine, smaller tumor size (< 5.0 cm), preoperative status, and granted informed consent. Patients were divided into four groups according to changes in urinary VMA and HVA values and tumor size. Patients who no longer fulfilled criteria underwent surgery. RESULTS: The observation period ranged from 4 to 73 months. Urinary VMA and HVA levels decreased in 19 of 26 patients, often by age 16 months. Eighteen patients had regressing tumors, and in 10 of these cases, the tumor was undetectable or barely detectable by imaging techniques. Four patients younger than 12 months had increased tumor marker levels and tumor volume, histologically reflecting neuroblastic proliferation. The remaining three patients, all older than 18 months, had varied tumor marker levels but increased tumor volume, histologically reflecting an increase in Schwann cells. No upgrading of tumor stage or unfavorable biologic factor was noted in any patient. CONCLUSION: None of our patients showed evidence of transition from favorable to unfavorable prognosis, a finding that points to a reduction in the significance of screening as a public health measure. Until results of ongoing screening trials involving older patients have been evaluated, the observation program can be used as a temporary measure to avoid, with little risk, unnecessary surgical intervention.

Functioning paraganglioma in the posterior mediastinum.

An unusual case of a functioning paraganglioma originating from the posterior mediastinum is reported. The main indications in the 21-year-old patient were hypertension and an abnormal roentgenogram of the chest. Blood and urine catecholamine assay confirmed the diagnosis of norepinephrine-secreting tumor, which was covered by the parietal pleura and attached to the sympathetic trunk. In the literature we could find reports of 25 patients with paraganglioma arising from the sympathetic trunk in the posterior mediastinum, 8 of whom had some symptoms and only 3 of whom were assayed for catecholamines. We diagnosed the present tumor as functioning aorticosympathetic paraganglioma according to the new classification and terminology suggested by Glenner and Grimley [1].

Urinary excretion of beta 2-microglobulin and N-acetyl-beta-D-glucosaminidase in advanced neuroblastoma patients receiving cis-diamminedichloroplatinum(II).

Urinary excretion of beta 2-microglobulin and N-acetyl-beta-D-glucosaminidase (NAG) were determined in eight patients with advanced neuroblastoma receiving cis-diamminedichloroplatinum(II) (CDDP). Urinary excretion of beta 2-microglobulin was increased within 3 days after CDDP treatment and returned to its pretreatment level within the first week. Increased urinary excretion of NAG was observed until the end of the second week after CDDP administration. But the measured values on the third day after CDDP administration were not significantly correlated with the accumulated dose of CDDP administered. These results indicate that beta 2-microglobulin excretion and NAG enzymuria after CDDP treatment are sensitive parameters of acute renal tubular damage, but the long-term latent nephrotoxicity of CDDP cannot be predicted from these parameters.

Intratumorous distribution of catecholaminergic clone cells of human neuroblastoma. A catecholamine fluorescence study.

The intratumorous distribution of catecholaminergic clone cells in 23 human neuroblastomas was studied using Falck-Hillarp's method, and the findings compared with the catecholamine (CA) content within the tumour. All the specimens contained elements with CA fluorescence, and the pattern of fluorescence was classified from the distribution of CA-positive cells and neurofibrils, as diffuse cellular (DC), diffuse fibrillary (DF), sporadic (S), clustered (C), island-shaped (I), and bundled (B). The strength of CA fluorescence of both cellular and fibrillary elements correlated well with the CA content within the tumour. In addition, all tumours of urinary VMA-negative cases also contained significantly larger amounts of CA than other, non-functioning, tumours in the paediatric age group. The results of this study suggest that firstly, the ratio of CA-positive cells to CA-positive neuronal processes is proportionately higher in the poorly-differentiated neuroblastomas and that secondly, even tumours negative for urinary VMA or HVA might be polyclonal and contain catecholaminergic elements.

Neuroblastoma. Mass screening for early detection and its prognosis.

Since July 1973, the authors began developing a mass screening system using a VMA (vanilmandelic acid) spot test on 6-to-7 month-old infants for early detection of neuroblastoma in Kyoto city, Japan. Using this method, six infants with this tumor were discovered; five of the six infants were cured, and one is under treatment. These patients showed a favorable prognosis on early diagnosis. In this article, 57 neuroblastoma patients from the Department of Pediatrics, Kyoto Prefectural University of Medicine, treated during the last 20 years, from July 1962 to June 1982, are evaluated. Since the mass screening program has run smoothly since July 1974, clinical findings are compared between 35 neuroblastoma cases before mass screening from the 12-year period from July 1962 to June 1974 and 22 cases after mass screening, during the 8-year period from July 1974 to June 1982. Before mass screening, only 20% (7/35) of the patients were discovered with neuroblastoma younger than 12 months of age and 68.6% were older than 2 years of age. After mass screening, 54.6% (12/22) of the patients were younger than 12 months of age and only 31.8% (7/22) were older than 2 years of age. Before mass screening, 17.1% (6/35) survived with five of the six surviving patients being younger than 12 months of age at the time of diagnosis; 72.7% (16/22) of the patients detected after mass screening are living now. Eleven of the 16 patients have already been cured, and the remaining 5 patients are presently undergoing treatment. A marked improvement of their prognoses is dependent on the early detection of this tumor by mass screening. To date, using the VMA spot test for early detection in infancy is convenient and effective for improvement of its prognosis.

Histological, immunohistochemical, and chromosomal studies on non-adrenal neuroblastomas without increased excretion of catecholamines and their metabolites in urine.

We reviewed our records for the last 10 years and found three of the 36 patients with neuroblastoma did not excrete significantly increased quantities of catecholamine metabolites in urine. All of these tumors were histologically characterized by small round cells and possessed a few dense core granules on the electron microscopic examination. All of them were reacted with anti-neuron-specific enolase (NSE) antibodies, OKB2, PI153/3 monoclonal antibodies (MoAbs). The HNK-1, BA-1, and SJ-9A4 MoAbs reacted with two out of three. One of them demonstrated a reciprocal translocation involving the short arm deletion of chromosome 1. This multidisciplinary study has been helpful in making more accurate diagnoses for neuroblastoma without classical clinical characters.

Mass screening for neuroblastoma in Japan.

The present status of the neuroblastoma mass screening program in Japan, the first national trial in the world, is evaluated. This program, now in its fifth year, was conducted in cooperation with the infants' mothers, local health centers, screening centers, and selected hospitals. From the onset of the program in Kyoto in 1973 to the end of 1989, 337 cases were detected and analyzed. Most cases were detected at early stages and 97% are expected to be cured. Several social, technical, and clinical problems remain unresolved.