Treatment with nephrectomy only for small, stage I/favorable histology Wilms' tumor: a report from the National Wilms' Tumor Study Group.

PURPOSE: Children younger than 24 months with small (< 550 g), favorable histology (FH) Wilms tumors (WTs) were shown in a pilot study to have an excellent prognosis when treated with nephrectomy only. PATIENTS AND METHODS: A study of nephrectomy only for the treatment of selected children with FH WT was undertaken. Stringent stopping rules were designed to insure closure of the study if the true 2-year relapse-free survival rate was 90% or lower. RESULTS: Seventy-five previously untreated children younger than 24 months with stage I/FH WTs for which the surgical specimen weighed less than 550 g were treated with nephrectomy only. Three patients developed metachronous, contralateral WT 1.1, 1.4, and 2.3 years after nephrectomy, and eight patients relapsed 0.3 to 1.05 years after diagnosis (median, 0.4 years; mean, 0.51 years). The sites of relapse were lung (n = 5) and operative bed (n = 3). The 2-year disease-free (relapse and metachronous contralateral WT) survival rate was 86.5%. The 2-year survival rate is 100% with a median follow-up of 2.84 years. The 2-year disease-free survival rate (excluding metachronous contralateral WT) was 89.2%, and the 2-year cumulative risk of metachronous contralateral WT was 3.1%. CONCLUSION: Children younger than 24 months treated with nephrectomy only for a stage I/FH WT that weighed less than 550 g had a risk of relapse, including the development of metachronous contralateral WT, of 13.5% 2 years after diagnosis. All patients who experienced relapse on this trial are alive at this time. This approach will be re-evaluated in a clinical trial using a less conservative stopping rule.

Complete surgical excision is effective treatment for children with immature teratomas with or without malignant elements: A Pediatric Oncology Group/Children's Cancer Group Intergroup Study.

PURPOSE: To determine whether the 3-year event-free survival (EFS) of children with completely resected immature teratomas is greater than 85%. PATIENTS AND METHODS: Patients with immature teratomas treated at Pediatric Oncology Group or Children's Cancer Group institutions were eligible. Pathology was centrally reviewed to confirm diagnosis and tumor grading. Follow-up included physical examination, measurement of tumor markers (alpha fetoprotein and human chorionic gonadotropin), and imaging. All patients were monitored for events, defined as tumor recurrence, second malignancy, or death. RESULTS: Seventy-three children (median age, 7.8 years) with extracranial immature teratomas were enrolled on study. Primary tumor sites included ovarian (n = 44), testicular (n = 7), and extragonadal (n = 22). However, on review, 23 patients had foci of yolk sac tumor (n = 21) or primitive neuroectodermal tumor (n = 2), whereas 50 had pure immature teratomas. Twenty-five patients had increased alpha fetoprotein (n = 18), human chorionic gonadotropin (n = 5), or both (n = 2); nine had foci of yolk sac tumor on review. Pathology review identified 23 patients with grade 1, 29 with grade 2, and 21 with grade 3 immature teratomas. With a median follow-up of 35 months, the overall 3-year EFS was 93% (95% confidence interval, 86% to 98%), with 3-year EFS of 97.8%, 100%, and 80% for patients with ovarian, testicular, and extragonadal tumors, respectively. Only four of 23 patients with immature teratoma and malignant foci developed recurrence, suggesting that surgical resection followed by close observation are effective treatment. Overall, five patients had disease recurrence 4 to 7 months from diagnosis, and four (80%) are disease free after platinum-based therapy. The fifth patient has residual tumor after cisplatin, etoposide, and bleomycin treatment requiring further therapy. CONCLUSION: Surgical excision is safe and effective treatment for 80% to 100% of children with immature teratoma.

Surgical resection alone is effective treatment for ovarian immature teratoma in children and adolescents: a report of the pediatric oncology group and the children's cancer group.

OBJECTIVE: In both adult women and children the potential for malignant recurrence from ovarian immature teratoma has prompted the standard use of chemotherapy after complete resection of the primary tumor. The efficacy of postoperative chemotherapy in children and adolescents with ovarian immature teratoma, however, has not been established. A pediatric intergroup trial (INT 0106) was designed to determine the need for postoperative chemotherapy in patients with ovarian immature teratoma after management with surgical resection only. STUDY DESIGN: Between 1990 and 1995, 44 patients with completely resected ovarian immature tumor and without postoperative chemotherapy, who were able to undergo assessment, were accrued. Tumor tissue was evaluated by central pathology review to confirm diagnosis and determine tumor grading of immature neural elements. Patients were followed carefully for recurrence of disease with appropriate diagnostic imaging and serum marker studies. RESULTS: Thirty-one patients had pure ovarian immature teratoma with a tumor grade of 1 (n = 17), 2 (n = 12), or 3 (n = 2). Age at diagnosis ranged between 1.5 and 15 years (median, 10). Of the 29 patients studied, the serum alpha-fetoprotein level was elevated in 10 (34%); the median level was 25 ng/ml. Thirteen patients had ovarian immature teratoma plus microscopic foci of yolk sac tumor. Tumor grade was 1, 2, or 3 in 1, 6, and 6 patients, respectively. Age ranged between 6 and 20 years (median, 12). In the 12 patients evaluated for serum alpha-fetoprotein, 10 (83%) had elevated levels; the median level was 262 ng/ml. The 4-year event-free and overall survival for the ovarian immature teratoma group and for the ovarian immature teratoma plus yolk sac tumor group was 97.7% (95% confidence interval, 84.9%-99.7%) and 100%, respectively. The only yolk sac tumor relapse occurred in a child with ovarian immature teratoma and yolk sac tumor who was then treated with chemotherapy and is alive and free of disease 57 months after recurrence. CONCLUSION: The results of this study suggest that surgery alone is curative for most children and adolescents with resected ovarian immature teratoma of any grade, even when elevated levels of serum alpha-fetoprotein or microscopic foci of yolk sac tumor are present. This experience strongly supports avoiding the long-term effects of chemotherapy in most children with ovarian immature teratoma by reserving postoperative therapy for cases with relapse.

Wilms tumor imaging: patient costs and protocol compliance.

PURPOSE: To evaluate the patient costs for imaging and compliance with imaging protocols in pediatric patients in the National Wilms Tumor Study (NWTS) IV. MATERIALS AND METHODS: The medical and imaging records of 60 patients (28 male, 32 female; aged 3 days to 12.6 years) in NWTS IV were reviewed. Initial imaging and follow-up imaging were evaluated separately. Three levels of follow-up compliance were evaluated. RESULTS: The total patient cost for imaging was $442,180: $94,212 for initial and $347,968 for follow-up studies. Many areas of potential cost savings were identified. Protocol compliance was variable. Seventy-five percent of patients underwent studies in full compliance with the initial protocol requirements. For follow-up, compliance was 0%-80% for different studies at different compliance levels. For no study was compliance 100%. CONCLUSION: Imaging costs in pediatric patients with Wilms tumor are substantial. Protocol compliance was not optimal.

Interphase in situ hybridization reveals minimal residual disease in early remission and return of the diagnostic clone in karyotypically normal relapse of acute lymphoblastic leukemia.

Early clinical remission of acute lymphoblastic leukemia (ALL) was examined by fluorescence in situ hybridization (FISH) in bone marrow cells from eight patients with high hyperdiploid (> 50 chromosomes) clones at diagnosis. Alphoid centromeric probes to chromosomes X, 10, 17, and 18, trisomic at diagnosis, were used as appropriate. Three hematologically normal marrows were used as controls. At diagnosis, trisomic interphase cells ranged from 69.3-84.4%. One month later, trisomic and tetrasomic interphase cells were significantly increased over control frequencies in 2/7 cases tested (p < 0.05 and p < 0.001) and trisomy alone in one case (p < 0.05). At two months post-diagnosis, trisomy and tetrasomy were in the control range. Pentasomy and hexasomy, not seen in controls, were found in 5/7 samples at one month and in 1/5 samples at two months. One patient examined in chromosomally normal relapse had 34.4% trisomic cells by FISH at confirmed relapse (p < 0.001). An additional hyperdiploid case, examined at central nervous system relapse, had increased numbers of trisomic cells (p < 0.01) in a morphologically and cytogenetically normal marrow. These findings demonstrate the elimination of aberrant ploidy in most hyperdiploid cases two months from diagnosis and indicate that failure to detect the abnormal clone in relapse may result from selective mitotic activity of chromosomally normal cells, rather than relapse in a cytogenetically normal clone.

Cytogenetic analysis in relapsed childhood acute lymphoblastic leukemia.

The nature of the cytogenetic abnormalities present at relapse of childhood acute lymphoblastic leukemia (ALL) and their relationship to the disease and the karyotype at diagnosis have not been clearly defined. This report describes cytogenetic analyses of 50/51 consecutive relapsed childhood ALL patients. Evolution of the karyotype was common, with structural abnormalities particularly frequent. Rearrangements involving chromosome 1 occurred frequently, particularly in patients with greater than 50 chromosomes. In patients with less than or equal to 50 chromosomes, structural aberrations were often unbalanced, leading to loss of genetic material, but these did not show a predominance of chromosome 1 abnormalities. These differences among the cytogenetic groups of ALL are an indication that the chromosomal abnormalities occurring in ALL reflect different biological events underlying this disease, and that different biological processes are involved in the several cytogenetic groups of ALL patients not only at initiation, but also during the progression and evolution of the disease.

Assessment of ventricular function in infants and children. Response to dobutamine infusion.

A method to assess ventricular functional reserve in infants and children unable to perform dynamic exercise was evaluated. Left ventricular ejection fraction was measured by radionuclide angiocardiography at rest and during infusion of dobutamine in 5-15 micrograms/kg/min dosages. The only side effects noted were arrhythmias in two patients, who had similar ectopy documented previously. Group left ventricular ejection fraction increased from 0.40 +/- 0.21 to a maximum of 0.49 +/- 0.24 (P less than 0.001). Left ventricular ejection fraction at 5 micrograms/kg/min (0.44 +/- 0.23) was not statistically different from that measured during infusion of dobutamine at 10 micrograms/kg/min (0.48 +/- 0.24). The six patients receiving anthracyclines, who had relatively low function at rest and increased function during dobutamine administration, were continued on their anticancer regimen without deteriorating in condition. Absolute values of left ventricular ejection fraction correlated best with the prognosis in patients with idiopathic dilated cardiomyopathy or structural abnormalities; six out of ten patients whose left ventricular ejection fraction never rose above 0.40 have either died or are in transplant protocols. Thus, study of left ventricular function can be performed easily during dobutamine infusion, generating valuable prognostic information.

Effectiveness of two methods of parenteral nutrition support in improving muscle mass of children with neuroblastoma or Wilms' tumor. A randomized study.

The short-term and long-term effectiveness of central parenteral nutrition (CPN) versus peripheral parenteral nutrition (PPN) in improving muscle mass (arm muscle area [AMA]) was evaluated for 24 malnourished children with newly diagnosed Stage IV neuroblastoma (n = 14) or Stages II-V Wilms' tumor (n = 10). Patients were randomized to either CPN or PPN plus enteral nutrition (EN: intense nutrition counseling, oral foods, and supplements) for 4 weeks followed by EN until week 10. Oncologic treatment was similar for each tumor type. Dietary, anthropometric, and biochemical measurements were obtained at weeks 0, 4, and 10. During weeks 1 through 4, energy (CPN: means 100 +/- 4; PPN: means 96 +/- 4% of healthy children) and protein (CPN: means 2.5 +/- 0.1; PPN means 2.7 +/- 0.2 g/kg) intakes of the two groups did not differ. The AMA increased (P less than 0.05) with 4 weeks of CPN but not with PPN; changes thereafter with EN were not significant. Weight (P less than 0.05) and triceps skinfolds (P less than 0.01) increased with 4 weeks of PN in both groups and decreased with EN thereafter (P less than 0.01) but were higher at week 10 than diagnosis. Increases in albumin in both groups reached significance at week 10 (P less than 0.05). These data show that CPN improves AMA in malnourished children with neuroblastoma or Wilms' tumor when energy and protein intakes are adequate. The AMA gains can be maintained thereafter with EN.

Integration of nutrition support into oncologic treatment protocols for high and low nutritional risk children with Wilms' tumor. A prospective randomized study.

Benefits and risks of nutrition support were evaluated in 31 malnourished children with newly diagnosed Wilms' tumor managed according to the third National Wilms' Tumor Study protocol. Patients were classified at diagnosis as being at high nutritional risk (HNR, n = 19) or low nutritional risk (LNR, n = 12). Ten HNR patients were randomized to central parenteral nutrition (CPN) and nine HNR patients were randomized to peripheral parenteral nutrition (PPN) plus enteral nutrition (EN) for 4 weeks of initial intense treatment and EN (nutritional counseling, oral foods and supplements) thereafter. Thirteen HNR patients (seven CPN, six PPN) completed the protocol. Twelve LNR patients received EN; 11 Stage I malnourished patients were randomized to 10 or 26 weeks of chemotherapy. Dietary, anthropometric, and biochemical data were determined for HNR patients at weeks 0-4, 6, 13, 19, and 26 and for LNR patients at weeks 1, 2, 5, and 26. In HNR patients, adequate parenteral nutrition support reversed protein energy malnutrition (PEM), and prevented chemotherapy and radiotherapy delays due to granulocytopenia. CPN was superior to PPN in reversing PEM: energy intake, weight gain, and retinol binding protein were higher (P less than 0.05). LNR patients lost weight and fat reserves in the first 2 weeks of treatment; depletion persisted at week 5, and 25% had chemotherapy delays. Thereafter, EN reversed PEM in patients with both chemotherapy regimens. These data suggest that CPN is preferable during initial intense treatment for HNR patients, and that, although EN is ineffective in preventing depletion and treatment delays in the first 5 weeks of treatment for LNR patients, it is effective thereafter.

Chest wall resection and reconstruction for malignant conditions in childhood.

This report evaluates the efficacy of extensive chest wall resection and prosthetic reconstruction in 15 children with chest wall malignancies. There were nine boys and six girls, with a mean age of 9.6 years. Eleven patients had primary chest wall tumors including Ewing's sarcoma (ES), six; rhabdomyosarcoma (RH), two; chondrosarcoma (CS), one; Askin's malignant neuroectodermal tumor, one; and mesenchymal sarcoma, one. Four children had metastases to chest wall and lung from Wilms' tumor (WT), two; osteogenic sarcoma (OS), one; and neuroblastoma (NB), one. Chest wall resection of two to six ribs and reconstruction with Marlex mesh (seven), lattisimus flap (two), prolene mesh (one), and more recently, a Gortex patch (five), was performed. Eight of the patients required concomitant en-bloc pulmonary resection (wedge, five; lobectomy, two; pneumonectomy, one) and two required resection of diaphragm. Fourteen received adjunctive therapy (chemotherapy, 14; irradiation, eight [preoperative, five; postoperative, three]. Six patients had second-look resections after chemotherapy. There was no operative mortality. Early pulmonary function was normal; however, pulmonary restrictive disease and scoliosis occurred with growth. One ES patient developed a radiation-induced second malignant tumor at age 10 and one ES child died at age 6 (no evidence of disease) of meningitis. Average survival length for ES patients was 77 months (range, 18 to 132 months.) Currently, eight patients are alive and five are free of disease. Extensive chest wall resection and reconstruction is useful in the treatment of primary chest wall tumors, but is palliative in metastatic cases. The Gortex patch is the current prosthetic of choice.

The relationship of various aspects of surgical management to outcome in childhood nonmetastatic osteosarcoma: a report from the Childrens Cancer Study Group.

Recently, much attention has been focused on the role of adjuvant chemotherapy in the treatment of osteosarcoma. Surgery, however, remains the primary modality for the ablation of this disease. In this report, we examine the relationship of various aspects of surgical management of osteosarcoma to prognosis for disease-free survival (DFS) in a randomized study of 234 pediatric patients. Attention is restricted to 166 patients with nonmetastatic disease confined to an extremity and who were randomized to receive one of two chemotherapeutic regimens. No advantage with respect to DFS was attributable to the various aspects of surgical management considered: interval from first symptoms to definitive surgery, surgical sequence, and type of surgery. Only two local recurrences were seen. One occurred in an above knee amputation stump and the other occurred in a patient receiving a tibial allograft. One of these patients died of pulmonary metastases within 6 months of recurrence; the other patient is alive without evidence of disease at last contact after resection of the recurrence followed by chemotherapy.

A randomized study comparing high-dose methotrexate with moderate-dose methotrexate as components of adjuvant chemotherapy in childhood nonmetastatic osteosarcoma: a report from the Childrens Cancer Study Group.

Methotrexate (MTX) has demonstrated significant activity against relapsed and metastatic osteosarcoma. However, there is little published data to indicate the appropriate dose for MTX when given as a component of a multidrug regimen for the treatment of osteosarcoma. Therefore, the investigators at the Childrens Cancer Study Group undertook a randomized clinical trial that compared Adriamycin and vincristine given with either high-dose methotrexate or moderate-dose methotrexate as postoperation chemotherapy in the treatment of childhood osteosarcoma. We report here the results for 166 patients with completely resected nonmetastatic disease of an extremity. The two therapies demonstrated equivalent disease-free survival (DFS). Further, no therapy prejudices survival after relapse. Approximately 38% of patients remain disease free 4 years after diagnosis. Two relapses occurred in patients free of disease at least 36 months after initiation of treatment. Some factors found by other investigators to be prognostic of poorer DFS, namely, male sex, primary tumor in the humerus or femur, and larger primary tumors, demonstrated similar though not statistically significant trends. The presence of spontaneous necrosis in the tumor sample from the definitive surgery was associated with poor prognosis for DFS. We postulate that this feature represents rapidly growing tumors with increased potential for metastases.

Advances in nutrition care of children with neoplastic diseases: a review of treatment, research, and application.

Within the last decade, significant advances have been made both in treating children with cancer and in providing proper nutrition support. Oncologic treatment and nutrition research and their application to the nutrition care of children with cancer are reviewed. Quality nutrition care is now possible because of an improved understanding of (a) the prevalence and significance of protein-energy malnutrition (PEM) in high-risk groups, (b) the staging and assessment of nutritional status, and (c) the efficacy and limitations of nutrition support options. Nutrition staging, assessment, and support should be integrated into treatment protocols for children with neoplastic diseases. Common risk factors for the development of PEM have been identified from serial monitoring of newly diagnosed children with a variety of tumors. Certain tumor types and their treatment can be classified within either low or high nutritional risk groups. A comprehensive nutrition program (intense nutrition counseling, favorite nutritious foods) is preferred for low nutritional risk groups but is ineffective in preventing or reversing PEM in high-risk groups. For high-risk patients, central parenteral nutrition (CPN) is the method of choice as a relatively short-term but important support measure that allows children to withstand long intervals of intense treatment during periods of growth and development. Current data suggest that bone marrow suppression may be attenuated and treatment tolerance improved with the use of CPN in selected children with advanced cancer (e.g., acute nonlymphocytic leukemia or advanced neuroblastoma).

Nutritional support of children with neoplastic diseases.

There are numerous factors promoting the development of PEM in the child with cancer. Some of these factors are related to the tumor, many to the treatment itself, and some to failure of recognition of PEM. Not all children with cancer are at great risk for the development of PEM. These patients must be monitored and supported with comprehensive enteral programs. Children who have developed or are at risk for PEM must be identified and supported with CPN or PPN plus CEN during early intensive periods of treatment and during the later phases of abdominal radiotherapy, operative resection of tumor, or relapse. The decision to institute CPN must be based not only on the child's current nutritional status but also on the nature of the therapy he or she is soon to receive and the likelihood that he or she will be able to maintain an adequate intake during that therapy. Realistic goals must be set for nutritional support. The value of nutritional intervention lies in its ability to correct or prevent the development of adverse effects related to PEM. This support is hoped to contribute to improved tolerance of therapy, increased energy to complete normal day-to-day activities, and an improved sense of well-being for the child. If these goals have been accomplished, then the nutritional therapy has been successful.

The value of nutrition support in children with cancer.

A positive stance towards nutrition support of the child with cancer assures potential for normal growth, development, and quality of life during extended oncologic treatment. Data from recent studies of children with cancer (advanced neuroblastoma, Wilms' tumor) demonstrate the importance of integrating nutrition staging, assessment, and support into treatment protocols. Patients with solid tumors and lymphomas who are malnourished at diagnosis have a poor outcome when compared to nourished counterparts. Enteral nutrition (intensive nutrition counseling and favorite, nutritious foods) is effective in low nutritional risk groups but ineffective in preventing or reversing protein-energy malnutrition in high nutritional risk groups. For high-risk groups, central parenteral nutrition is a relatively short-term, but important, support measure which allows children to grow despite extended periods of intense oncologic treatment. The patient's nutritional course may affect bone marrow suppression and the ability to tolerate aggressive chemotherapeutic treatment. Although treatment tolerance may be improved with nutrition support, adequacy of primary oncologic treatment outweighs other supportive factors as a determinant of ultimate survival.

Efficacy of magnetic resonance imaging in 139 children with tumors.

One hundred thirty-nine children with neoplasms were studied using magnetic resonance imaging (MRI). This procedure was as accurate as computed tomography in predicting tumor histology, except that MRI was unable to detect small areas of tumor calcification. Magnetic resonance imaging could accurately identify the organ of origin of tumor masses and differentiate soft tissue from fat, fluid, or hemorrhage. In addition, MRI was helpful in planning surgery in many cases: It was better than computed tomography in defining the size and extent of soft-tissue tumor masses. It was accurate in defining the extent of the spread of bone sarcomas in the bone marrow. Without requiring the injection of intravenous contrast agents, it accurately defined displacement, encasement, or invasion of major abdominal blood vessels by Wilms' tumors and neuroblastomas. As a means of evaluating pediatric neoplasms, MRI is noninvasive, painless, and well tolerated by children, and it uses no radiation.

MRI of Wilms' tumor: promise as the primary imaging method.

The magnetic resonance appearance of Wilms' tumor in 14 patients is described and its clinical utility is evaluated. In all cases, magnetic resonance was correlated with surgical and pathologic findings to assess accuracy. Magnetic resonance accurately identified the primary tumor and its renal origin in all cases, and tumor margins and local extension were accurately demonstrated. Tumor margins were smooth and well defined in nine of 12 cases. Local extension and size were accurately assessed, but because capsular invasion could not be predicted, four surgically proven instances of capsular invasion were missed. Metastatic spread into the liver and inferior vena cava was well documented in four cases and excluded in 10. Magnetic resonance was sensitive for identifying lymph-node enlargement in five of 14 cases, but could not predict the etiology of the enlargement. All Wilms' tumors had signal intensities consistent with prolonged T1 and T2 relaxation times. Signal intensity was highly variable, mainly because of necrosis and hemorrhage within the tumor. Magnetic resonance based on signal intensity could not distinguish Wilms' tumor from other solid renal tumors. Magnetic resonance has the potential for providing the same information as computed tomography, sonography, liver spleen radionuclide scanning, and excretory urography. Although expensive, magnetic resonance will be cost-effective if it can replace all the above techniques. This limited study indicates that magnetic resonance has promise as the primary imaging technique for Wilms' tumors.

Hodgkin disease and non-Hodgkin lymphomas in children: utilization of radiological modalities.

If costs of medical care are to be reduced, the choice of which imaging modality to use must be made as carefully as possible. This study was done to show how radiological modalities were used to evaluate patients with Hodgkin disease and non-Hodgkin lymphoma. We kept a record of every radiological study performed on 66 children with both diseases seen in the past 6 1/3 years. The results of these studies were analyzed to see which areas of the body were studied, which imaging modality was used, how frequently the studies were repeated, and how frequently the studies gave abnormal results. Our findings disclosed that radiological studies have been appropriately performed in anatomic regions of the body in which disease is present. New imaging modalities have been introduced, and the use of some of the older modalities has been decreased. With some modalities, such as skeletal survey, liver/spleen scan, whole-lung tomography, contrast studies of the bowel, and excretory urography, utilization is higher than it ought to be in view of the fact that the yield of positive results is low and the information is obtainable in many cases from other more sensitive procedures. These studies should not be performed as a routine on initial evaluation or follow-up of all patients with Hodgkin or non-Hodgkin lymphomas. On initial presentation all patients should undergo chest radiography and CT scanning of both chest and abdomen. A problem area is that the timing of follow-up studies has been somewhat erratic, with some inappropriate studies particularly 3 or 4 years after diagnosis. Too many imaging procedures have probably been done in follow-up of our patients.

Congenital monoblastic leukemia cutis. A case report with chromosomal abnormality: del (10p).

Congenital monoblastic leukemia cutis is a rare disorder. We report an infant who developed infiltrative skin lesions by 2 weeks of age, which, when biopsied at 4 1/2 months of age revealed a monoblastic infiltrate. Blasts in the peripheral blood were not seen until 1 week before her death at 8 months of age. Chromosomal analyses of her bone marrow showed an abnormal clone of cells with a 46,XX,del(10)(p12) karyotype. Although chromosome 10 is rarely involved in hematologic malignancies, abnormalities of this chromosome within the region 10p11-10p13 have now been shown in four of 10 reported cases of congenital monoblastic leukemia.

Short- and long-term effectiveness of enteral and parenteral nutrition in reversing or preventing protein-energy malnutrition in advanced neuroblastoma. A prospective randomized study.

The effectiveness of enteral and parenteral nutrition regimens in preventing or reversing protein-energy malnutrition (PEM) and in preventing treatment delays was evaluated in 32 children receiving treatment for newly diagnosed Stage III (3 patients) and IV (29 patients) neuroblastoma. Ten of 18 malnourished patients were randomized to central parenteral nutrition (CPN) and 8 to peripheral parenteral nutrition (PPN) plus enteral nutrition for 4 weeks and then received enteral nutrition (EN: intense nutrition counselling, oral foods and supplements) for weeks 5 through 10. Ten of 14 nourished patients received EN and 4 CPN for 4 weeks and EN thereafter. Dietary, anthropometric and biochemical measurements were determined for weeks 0, 1, 2, 3, 4, 7, and 10 for 24 patients who completed the protocols. In malnourished patients, both CPN (seven patients) and PPN (seven patients) were effective in reversing PEM in the first 4 weeks; thereafter, EN effectively maintained nutritional gains in both groups. In nourished patients, EN (seven patients) was not as effective as CPN (three patients) in preventing PEM during the first 4 weeks; afterwards, EN maintained gains in the CPN group but did not promote needed increases in weight nor fat reserves in the EN group. Patients supported by parenteral nutrition (PN, weeks 1-4) had fewer treatment delays (2/17, 12%) than EN patients (4/7, 57%, P less than 0.05). These data indicate that PN reverses or prevents PEM and prevents treatment delays during the first 4 weeks of intense oncologic treatment and provides nutritional benefits which can be maintained with EN thereafter.