bluuebunny/crossref_metadata_2025 huggingface dataset:
Clinical and biologic features predict poor prognosis in acute lymphoid leukemias in children and adolescents: A pediatric oncology group review
Authors: Crist W. , Boyett J. , Pullen J. , van Eys J. , Vietti T.
Published: February 2007

Although the prognosis of children with ALL has improved markedly, approximately one‐half of children continue to relapse. Clinical and biologic features of ALL have been studied at the time of diagnosis and many of these features have been shown to predict the risk of relapse, permitting tailoring of therapy based on relapse hazard. Results of studies of the Pediatric Oncology Group (POG) detailed here demonstrate that high white blood cell (WBC) count at diagnosis, very young or older age within childhood, and the presence of a mediastinal mass or central nervous system leukemia predict a poor prognosis within the context of certain therapies. However, not only age and WBC count, but immunophenotype of ALL as well, were especially predictive of prognosis in these POG studies. The information reviewed here has special importance for the planning of clinical trials for children with ALL.
Predictable risk factors in children with acute lymphoblastic leukemia
Authors: Sakurai Minoru , Kamiya Hitoshi , Kawai Kazuo , Kawasaki Hajime , Komada Yoshihiro , Ido Masaru , Ochiai Hitoshi , Akane Hiroyuki , Sato Kunihiko
Published: February 2007

The predictable prognostic factors were analysed among 174 children with acute lymphoblastic leukemia who were treated during the last ten years under different protocols in one institute. It was confirmed that the children under 1 year of age, or with T‐cell marker, had poor prognosis. The initial WBC of more than 50,000/mm3 was less significant as a predictable risk factor with chemotherapy of the newer protocols. Cell kinetic study was found to be of no more help than the initial WBC, but a more accurate prediction could be obtained by measuring glucocorticoid receptor of leukemic cells. The prognosis was poor among children with initial WBC of more than 50,000/mm3 and receptors of less than 20,000 sites per cell.
Age at diagnosis in childhood acute lymphoblastic leukemia
Authors: Sather Harland N.
Published: February 2007

Age at initial diagnosis in ALL has been identified in numerous studies to be an important prognostic factor. Data were examined from a large series of patients entered on ALL clinical trials of the Childrens Cancer Study Group during the period 1972‐1983. These data confirm the importance of age as a prognostic factor and show that even after adjustment for other factors by multivariate analysis, age remains a highly significant predictor of outcome. The data suggest that there are three main age groups with different prognoses: infants (< 1 year at diagnosis), the 1–9 age group, and those 10 or greater. The best prognosis is found in the intermediate age range and the worst in the infant group. While age is presumably a surrogate factor reflecting the effect of factors with more direct and explainable relationships to the disease process, analysis of data in this review and from other recent studies do not yet adequately explain the age effect in terms of other prognostic factors.
United Kingdom medical research council acute lymphoblastic leukaemia (UKALL) trials I‐VIII: Clinical features and results of treatment in four groups of children with adverse prognostic features
Authors: Lilleyman J. S. , Eden O. B.
Published: February 2007

Four groups of children with poor‐prognosis disease were selected for study from the Medical Research Council childhood ALL trials. Each group was reviewed for distinguishing clinical features and response to therapy. The four comprised those with a diagnostic white cell count over 50 × 109/1, those with a mediastinal mass, those less than a year old at diagnosis, and those presenting with meningeal involvement. They accounted for 17, 5.3, 2.6, and 3%, respectively, of unselected children with ALL.

As expected, all four categories showed significantly inferior relapse‐free survival to the rest, but only in patients with a mediastinal mass and in the under‐1‐year‐olds did a recognisable syndrome emerge clearly associated with other clinical features. These two syndromes, high‐count T‐ALL and high‐count infant null‐ALL, are logical candidates for alternative therapy. Established meningeal disease also continues to present a major therapeutic challenge.
Methotrexate infusions in poor prognosis acute lymphoblastic leukemia in children: I. The Norwegian methotrexate study in acute lymphoblastic leukemia in childhood, august 1975‐December 1980
Authors: Moe Peter Johan , Seip Martin , Finne Per H. , Kolmannskog Svein
Published: February 2007

One hundred fifty‐three children with ALL were diagnosed in Norway in the period August 1975‐December 1980. One hundred thirty‐two of them received 3 infusions of methotrexate as consolidation therapy combined with methotrexate intrathecally as CNS prophylaxis. Eleven (44%) of the total 25 methotrexate cases with WBC above 50 × 109/L were in CCR after 4 1/2‐10 years. Two more cases had discontinued therapy, while in second remission.

The event‐free survival of all diagnosed 32 children in Norway with WBC above 50 × 109/L was 37%. Seven infants below the age of 1 year are included in the 32 cases.
Medical oncology manpower supply
Authors: Kennedy B. J.
Published: February 2007

An explosion in the number of trained medical oncologists has led to concern that an overproduction will occur. A survey of 154 medical oncology training programs revealed 767 current trainees—316 first year, 307 second year, and 144 third year. Seventeen programs contained 301 persons (38.5%). After the 1985 certifying examination, 3,659 oncologists were certified. With approximately 750 eligible candidates for each examination, and assuming a 75% pass rate, by 1989 there will be 4,779 certified medical oncologists. By 1990, there will be no deficiency of clinical medical oncologists, but there appears to be a lack of academic oncologists. In order to assure the quality goals of oncology, and in an attempt to meet an adequate supply of medical oncologists, a position statement has been published by the American Society of Clinical Oncology. To avoid an overproduction of medical oncologists, an ongoing assessment of the number needed is warranted.
Treatment of hodgkin's disease stages I and II with chemotherapy alone
Authors: Bubman Itiel , Kirchhoff Louis V. , Morioka Helena , Debellis Normando
Published: February 2007

Ten previously untreated patients with stages I and II Hodgkin's disease were treated with MOPP chemotherapy alone. Eight of nine evaluable patients went into complete remission (CR). Six remained in CR on the average 82.7 months after induction. Two patients relapsed early and died more than 5 years after entering the study. The patient in whom CR was not achieved died as well. The estimated 10‐year survival rate is 51%. This experience adds to the very small number of reports that have appeared describing the results of treatment of patients with early stage Hodgkin's disease with MOPP alone.
Thyroid cancer following radiotherapy for hodgkin's disease: A case report and review of the literature
Authors: Moroff Saul V. , Fuks Joachim Z.
Published: February 2007

Improved survival resulting from advances in therapy in patients with Hodgkin's disease is associated with long‐term morbidity, including the potential for the development of a second solid malignancy. We report a 44‐year‐old man with an unusually aggressive course of thyroid carcinoma 15 years after treatment for Hodgkin's disease. In a review of the English‐language literature, we found 21 cases of thyroid cancer following radiotherapy for Hodgkin's disease, with latency periods ranging from 6 to 48 years. The development of secondary thyroid cancer after high‐dose neck irradiation may be related to hypothyroidism, itself a complication of radiotherapy. Thyroid function should be measured at least once a year in all patients given neck irradiation, with initiation of thyroid hormone replacement if there is evidence of sustained hypothyroidism.
Transcobalamin I as a “marker” for fibrolamellar hepatoma
Authors: Wheeler K. , Pritchard J. , Luck W. , Rossiter M.
Published: February 2007

A 12 year old girl with a localised fibrolamellar hepatoma had a raised serum unsaturated vitamin B12 binding capacity (UBBC) and transcobalamin I (TCI) prior to complete resection and chemotherapy. Regular clinical and radiological follow‐up detected no recurrence of her disease, but the UBBC and TCI slowly rose. Local recurrence and pulmonary metastases became detectable 2 1/2 years after diagnosis, 18 months after the UBBC and TCI level became elevated. Measurement of UBBC and TCI can help in the early detection of recurrence long before there is clinical or radiological evidence of recurrent fibrolamellar hepatoma.
Secondary Hodgkin's disease in childhood acute lymphoblastic leukemia
Authors: Peeters Marie A. , Smith Charles , Saunders E. Fred.
Published: February 2007

Improved survival in childhood acute lymphoblastic leukemia has led to the occurrence of second malignancies in these patients. Hodgkin's disease is very rare as a second malignancy. We report three patients with acute lymphoblastic leukemia in remission who developed Hodgkin's disease. Although all had received low‐dose irradiation, none received alkylating agents as part of their chemotherapy. Review of our cases and of 11 reported in the literature revealed unique aspects of this association. There was a short median interval of 19 months to the development of the second malignancy. Over one‐third of the patients had uncommon sites of involvement (lung, tonsil, small bowel). The distribution of histologic subtypes was unusual, as 5 of 14 cases had lymphocyte depletion or unclassifiable Hodgkin's disease. The results of therapy were excellent. Our three patients are alive, with both malignancies in continuing remission. Two patients are off all therapy for 4 and 6 years, respectively. The third remains on antileukemic treatment. Secondary Hodgkin's disease in childhood acute lymphoblastic leukemia does not appear to have a poor prognosis and long‐term survival and possible cure of both diseases may be achieved.
Massive adrenal hemorrhage secondary to metastatic lung carcinoma
Authors: Rowinsky Eric K. , Jones Richard J. , Abeloff Martin D.
Published: February 2007

The adrenal glands are frequently metastatic sites of lung and other primary neoplasms. These metastases are usually clinically occult and secondary adrenal hemorrhage is extremely rare. Two patients who developed massive adrenal hemorrhage seconday to metastatic lung carcinoma to the adrenal glands are presented.
Infections in patients with non‐small‐cell lung cancer treated with intensive induction chemotherapy
Authors: Fuks Joachim Z. , Patel Halesh , Hornedo Javier , Van Echo David A. , Moody Mark , Aisner Joseph
Published: February 2007

The records of 65 consecutive patients with non‐small‐cell lung cancer (NSCLC) treated with intensive induction chemotherapy were reviewed to study the infectious complications during therapy and to analyze the relationship of the frequency of infections to various predisposing factors. A total of 44 infectious episodes were observed among 30 of the 65 patients. Of the 44 infections, 18 were microbiologically documented and 19 clinically. Seven (16%) infections were without microbiological or clinical documentation and were categorized as “possible” infections. Among the 18 microbiologically documented infections, fifteen (83%) were caused by bacteria and three by fungi. The most frequent bacteria identified in 11 (61%) of the 18 infections were gram‐negative organisms, Escherichia coli and Klebsiella pneumoniae being the most frequent. Eight of the 44 infections were associated with bacteremia and three with microbiologically documented pneumonias. There were three drug‐related infectious deaths, two associated with bacteremia and one with possible infections. All 44 infectious episodes presented with WBC counts of <1,000/μil, and 34 (79%) had WBC counts of <500/μl. We observed that during therapy, patients with poor performance status (< 80%) are at a much higher risk to develop infectious complications than those with good performance status (>80%; p <.001).

Although encouraging responses with intensive chemotherapy have been reported for NSCLC in several studies, a major impact of chemotherapy on the survival of patients with this disease has yet not been established. Thus, intensive chemotherapy for NSCLC should remain an experimental treatment modality and should be offered only to patients with good prognostic factors such as those defined by pretreatment performance status.
Aggressive infantile myofibromatosis: Report of a case of a clinically progressive congenital multiple fibromatosis
Authors: Molnar Peter , Olah Eva , Miko Tivadar L. , Gomba Szabolcs
Published: February 2007

We report a case of congenital multiple fibromatosis (infantile myofibromatosis) showing the typical spindle‐cellular proliferation with prominent vascularity on light microscopical observations. Electron microscopy showed the abundance of fibroblasts with conspicuous collagen and reticulin fibers together with numerous cells sharing the characteristics of both fibroblasts and smooth muscle cells (myofibroblasts). Neither visceral involvement nor ossification has been detected during the 4‐year‐long follow‐up period. However, the clinical course has shown a slow, continuous, protracted though destructive proliferation of subcutaneous myofibroblastic nodules. These findings are contrasted with previous reports that claimed that the presence of myofibroblasts indicates benign behavior and results in the regression of fibromatous lesions.
Health status of young children with cancer following discontinuation of therapy
Authors: Pastore G. , Zurlo M. G. , Acquaviva A. , Calculli G. , Castello M. , Ceci A. , Di Tullio M. L. , Gandus S. , Macchia P. , Di Montezemolo L. Cordero , Mandelli F. , Massolo F. , Nespoli L. , Paolucci G. , Rosate M. , Senesi E. , Tamaro P. , Tripoli U. , Terracini B.
Published: February 2007

This paper reports late effects and health status of 198 children who had cancer or leukemia diagnosed under 2 years of age and their therapies electively withdrawn. This series (92 neuroblastoma [NBL], 57 Wilms' tumor [WT], 46 acute lymphoblastic leukemia [ALL], and 3 non‐Hodgkin's lymphomal was followed for 1‐12 years after discontinuation of therapy. Thirty‐three children were diagnosed before 1973, 92 between 1973 and 1977, and 73 after 1977 in 16 Italian Pediatric Oncology Centers. As of December 1983, 176 children were reported to be alive and without evidence of primary cancer by physicians responsible for their care. One child died from a second primary tumor, two from late recurrences of the primary cancer, and three from other causes; eight were alive with evidence of primary cancer; and eight were lost to follow‐up. Kyphoscoliosis was found in 22 children and other musculoskeletal anomalies in 8. Neurological sequelae were observed in 8 out 35 children with ALL treated with radiotherapy (RT) and intrathecal methotrexate. All but one were in continous complete remission when they developed seizures (three cases), leukoencephalopathy (three cases), or intracerebral calcitications (two cases). One child had cardiomyopathy and subsequently died from cardiac failure: he had received doxorubicin (400 mg/m2) and mediastinal RT (13 Gy) for NBL. Growth impairments were observed in children with NBL and WT.
Randomised study of ticarcillin, cefamandole with or without tobramycin in febrile, neutropenic patients with solid tumors
Authors: Page Jonathan P. , Levi John A. , Woods Robert L. , Chan Wing K. , Bell David R. , Aroney Rodney S.
Published: February 2007

A prospective randomised study was conducted comparing the efficacy and toxicity of the antibiotics ticarcillin and cefamandole (TC) with or without tobramycin (TCT) in 100 febrile neutropenic patients with solid tumours undergoing conventional chemotherapy. In this study, neutropenia <100/μMl was noted in 31% of 106 evaluable infectious episodes and neutrophil counts <1,000/μMl persisted for a median 4 days. Infection was microbiologically documented in 42% of episodes (bacteremia 24%) with gram‐negative organisms responsible for 63% of bacterial isolates. Overall, 65% of episodes responded to TC and 76% to TCT (p>0.05). Patients with initial shock bacteremia, pulmonary infection, or gram‐negative sepsis responded relatively poorly. Neutrophil nadir and pathogen susceptibility did not influence outcome. Antibiotic toxicity was minimal with no tobramycin‐related nephrotoxicity. These results are broadly comparable to those observed with leukemic patients, but the relatively short duration of neutropenia in the solid‐tumour patients appears to minimize the need for additional antibiotics provided there is adequate antimicrobial coverage with the initial choice of antibiotics.
Number and cure rate of neuroblastoma cases detected by the mass screening program in Japan: Future aspects
Authors: Sawada Tadashi , Sugimoto Tohru , Tanaka Terufusa , Kawakatsu Hidekazu , Ishii Takashi , Matsumura Takafumi , Horii Yoshihiro
Published: February 2007

The annual incidence of neuroblastoma in Japan, a common malignancy of young children which has a very poor prognosis in progressive cases, was previouly estimated to be 8.2 cases/million children under 15 years of age. Based on the Japanese National Census of the Population for 1982, the annual number of cases of this tumor is predicted to be 220. (Sawada et al: Med Pediatr Oncol 12:101‐103, 1984). Before the mass screening program was started, one‐fourth of all neuroblastoma cases were detected before the age of one by clinical symptoms and other findings.

Since 1974, a neuroblastoma mass screening program for 6‐month‐old infants by means of a qualitative Vanilmandelic Acid (VMA) test has been in effect in Kyoto, Japan, for the prognostic improvement of neuroblastoma patients (Sawada et al: Am J Dis Child 136:710‐712, 1982). A Neuroblastoma Mass Screening Study Group (NBMSSG) was organized in 1981 (Sawada et al: Lancet ii:271‐272, 1984), and the mass screening program has grown to encompass eight selected areas. This group discovered 15 cases of neuroblastoma among 247,500 6‐month‐old infants, 1 of every 16,500, until the end of 1983, and 23 cases among 434,970, 1 of every 18,900, until the end of 1984. All cases were asymptomatic. As the incidence of infantile neuroblastoma detected by this program is projected to be 52.9‐60.6 cases/million 6‐month‐old infants, it could be predicted that 80‐91 neuroblastoma cases will be discovered annually in Japan by mass screening. The cure rate of 25 cases discovered by the mass screening and followed up over 20 months was 92% (Sawada: Lancet i:377, 1986).

In Japan, it should be possible to detect 135‐146 cases‐[symptomatic 55 = 220 × 1/4] + [80‐91 cases detected by mass screening]‐(61.4‐66.4% of the total neuroblastoma cases) in infants under 1 year of age annually, and the cure rate of all neuroblastoma cases in Japan is expected to reach 60% or more.
Chronic myeloid leukemia: Manifesting as spontaneous splenic rupture and terminating in megakaryoblastic transformation
Authors: Shih Lee‐Yung , Su Ih‐Jen
Published: February 2007

We report a case of chronic myeloid leukemia with spontaneous splenic rupture as the initial presenting feature; there was a successful surgical outcome, but this case terminated in megakaryoblastic transformation. Results are reported based on morphological, immunological, cytochemical, ultrastructural, immunocytochemical studies, and in vitro liquid culture studies. The megakaryocytic nature of the blast cells was identified through the demonstration of platelet peroxidase activity by ultrastructural cytochemistry and the presence of platelet and megakaryocyte‐specific antigen using monoclonal antibody, as well as the anti‐factor VIII antibody by immunocy‐tochemical technique.
Transient myeloproliferative disorder in chromosomally normal newborn infant
Authors: Kalousek D. K. , Chan K. W.
Published: February 2007

Transient Myeloproliferative disorder is a condition clinically resembling congenital acute myelogenous leukemia. As in acute leukemias the blast cell population in this disorder may have either normal or abnormal chromosomal complement. Transient myeloproliferative disorder is well recognized in neonates with a complete or mosaic trisomy 21 (Down's syndrome). We report a phenotypically and cytogenetically normal infant with this syndrome in whom only blast cells showed trisomy 21. We postulate that the pathogenesis of the transient myeloproliferative disorder in both Down's syndrome infants and those with a normal chromosomal complement is related to abnormal prenatal production of placental regulatory hemopoietic factors caused by chromosomal defect(s) confined to placental tissues.
Salvage chemotherapy for advanced Hodgkin's disease
Authors: Garbes Isosceles D. , Gomez German A. , Han Tin , Henderson Edward S.
Published: February 2007

Eleven patients with Hodgkin's disease refractory to chemotherapy were treated with six cycles of intermediate‐dose methotrexate with calcium leucovorin rescue, followed by cyclophosphamide, doxorubicin, vincristine, and prednisone (MTX‐CHOP). Three other patients were treated with a similar program of treatment minus doxorubicin (MTX‐COP). The overall response rate was 57%, with four (29%) patients achieving a complete response and four patients achieving a partial response. None of the three patients treated with MTX‐COP had a complete remission (CR); thus the complete remission rate with MTX‐CHOP was somewhat higher (36%). Only one of the CR's is in continuous complete remission and free of disease at 99+ months. One patient died of overwhelming sepsis and pancytopenia during treatment. Hematologic toxicity in the other patients was acceptable. The overall median survival was 18 months. The search for an effective treatment program for this group of patients remains a major challenge.
Siblings of paediatric cancer patients: A population at risk
Authors: Carr‐Gregg Michael , White Les
Published: February 2007

The psychosocial care of children with cancer has received increasing and welcome emphasis in the medical literature. However, the current and future status of siblings of such patients has been an underestimated area of concern. We examine the general importance of sibling relationships and present the literature regarding siblings of paediatric oncology patients. Donors and recipients of bone marrow transplants are considered specifically. Preliminary results of original research, supported by available published studies, suggest important disturbances in the mental health of siblings. Implications for paediatricians and other health professionals as well as prevention and intervention strategies are indicated.
Neuroblastoma overview—1986
Authors: D'Angio Giulio J.
Published: February 2007

Understanding of the biology of neuroblastoma has advanced dramatically in the last several decades. Misunderstood and misdiagnosed in the early years of this century, neuroblastoma is now the prototypic model for research in the molecular biology and genetics of the transformed cell. It is through these advances in knowledge of the malignant process, and of neuroblastoma itself, that exquisitely targeted therapies will shortly evolve. These include not only immunologic approaches through monoclonal antibodies, I but also the exploitation of metabolic pathways. Radioactive meta‐iodobenzylguanidine (MIBG) is among the most promising of the latter methods. It is deservedly undergoing clinical tests for its diagnostic and therapeutic applications in the management of neuroblastoma patients.
Radiochemistry, biochemistry, and kinetics of 131I‐metaiodobenzylguanidine (MIBG) and 123I‐MIBG: Clinical implications of the use of 123I‐MIBG
Authors: Shapiro B. , Gross M. D.
Published: February 2007

Metaiodobenzylguanidine (MIBG) is actively concentrated in adrenergic neuroendocrine tissues and tumors by an active, energyand sodium‐dependent, high‐affinity, saturable mechanism. This has proved successful, when labeled with 131‐l or 123‐l, in scintigraphically depicting pheochromocytomas and neuroblastomas.

For imaging purposes 123‐l has multiple advantages over 131‐l; the gamma ray energy is ideal for modern instruments the decay by electron capture limits the particulate emissions, and the short half‐life reduces the radiation burden. It is thus possible to use doses of 123‐I‐MIBC 20 times as large as doses of 131‐I‐MIBG with equivalent absorbed radiation doses. Disadvantages of 123‐I include the cost and difficulties in the regular delivery of this short‐lived radionuclide.

For most imaging purposes 123‐I‐MIBC is the optimal agent if logistical problems in its sup ply can be overcome.
Treatment of neuroblastoma with 131I‐MIBG: Dosimetric problems and perspectives
Authors: Beierwaltes William H.
Published: February 2007

Between 7/3/80 and 5/7/86 we gave 32 of our neuroblastoma patients 62 diagnostic doses of metaiodobenzylguanidine (MIBG) and 12 patients 20 treatment doses. Our conclusion from our diagnostic dose studies is that MIBG should be used for staging the extent of neu‐roblastoma before therapy is started, because it may change the proposed staging and therapy. In MIBG therapy for neuroblastoma, our criteria for agreeing to treat a patient are based on calculations from a 4‐day tracer dose study that assures that the patient will receive from his first therapy dose a tumor dose of at least 2,000 rads/100mCi, with a total body dose of not greater than 200 rads. Under these circumstances in children, the blood dose has been about 50 rads. The platelet count falls routinely with a 150‐rad whole‐body dose but never to dangerous levels. We have delivered tumor doses of 7,000‐34,600 rads on the first dose using 150‐215 mCi. We have had objective regressions (as shown by before and after CAT scans) of 30‐59% in volume of the principal tumor mass in 3 of the first 12 patients treated. All patients had Grade IV neuroblastoma with extensive previous surgery, radiation, and chemotherapy, with and without previous bone marrow transplants. MIBG therapy was most effective in patients with slower‐growing tumors for whom initial treatment doses were 200 mCi or more.
Therapeutic use of 131I‐metaiodobenzylguanidine (MIBG) in neuroblastoma: A phase II study in nine patients
Authors: Hartmann O. , Lumbroso J. , Lemerle J. , Schlumberger M. , Ricard M. , Aubert B. , Coonaert S. , Merline L. , Olive D. , De Lumley L. , Parmentier C.
Published: February 2007

Effects of high activities of I 131 meta‐iodo‐benzylguanidine (mIBG) were evaluated in nine children with advanced neuroblastoma. All patients had been previously heavily treated and had either primarily refractory disease or resistant relapse. Twenty‐two doses of mIBG labeled with 1.3 to 4 GBq (35‐108 mCi) of iodine 131 were administered. Three subjective effects, especially relief of pain, and two objective effects were observed. Transient blood pressure increase was observed once and did not recur after prolongation of the infusion time to 6 hours. A major side effect was bone marrow toxicity, essentially marked by thrombopenia, particularly severe in previously bone‐marrow‐transplanted patients.
Considerations on 131I‐metaiodobenzylguanidine therapy of six children with neuroblastoma
Authors: Sanguineti Michele
Published: February 2007

Six children affected by neuroblastoma at stages III and IV were treated with high‐specific‐activity 131I‐metaiodobenzylguanidine (MIBG). After 131I‐MIBG treatment three patients died at 12, 10, and 12 weeks, respectively; the other three were still living at 21, 16, and 24 weeks, respectively.

Although the assumptions for this therapy were propitious, the results obtained do not correspond to those expected. It is supposed that large tumor volume and previous chemotherapy and/or radiotherapy may impair the effectiveness of 131I‐MIBG therapy. Consequently, 131I‐MIBG therapy is recommended even if the spread of disease is not proved—only, however, when the tumor is small.
131I‐metaiodobenzylguanidine treatment in neuroblastoma: Report of two cases
Authors: Cottino F. , Mussa G. C. , Madon E. , Favero A. , Silvestro L. , Grazia G.
Published: February 2007

Poor results with 131I‐meta‐iodobenzylguanidine (MIBG) therapy have been obtained in two children with stage IV neuroblastoma treated after partial surgery and unsatisfactory combination chemotherapy. Both patients' response to treatment (four and three 1‐month‐spaced courses, respectively; cumulative administration of 11.9 and 9.2 GBq) has been characterized by a low isotope concentration in the primary tumor and in the multiple bone metastases and by bone marrow uptake with final severe hematological toxicity. A slight decrease in the primary tumor's volume was observed in one patient at a cumulative dose of 85 Gy; there was no change in the other's at 42 Gy. At an initial, greater isotope concentration delivering 103 Gy, some bone metastases displayed a sharp decrease in uptake that persisted in the successive courses. For both patients a progressive spreading of new tumor localisation in the bones and finally in the soft tissues was observed.
Cytoreductive procedures in the early management in cases of leukemia and hyperleukocytosis in children
Authors: Bunin Nancy J. , Kunkel Keith , Callihan Thomas R.
Published: February 2007

Cytoreduction for hyperleukocytosis before the initiation of primary therapy may reduce morbidity and mortality from blast cell lysis in children with acute lymphoblastic leukemia (ALL) and from leukostasis in children with acute nonlymphoblastic leukemia (ANLL) or chronic myelogenous leukemia (CML). The clinical features of 35 children (23 with ALL, 5 with ANLL, and 7 with CML) who underwent cytoreduction before the institution of definitive therapy were studied. Twelve children had exchange transfusions and 23 underwent leukaphereses. The cytoreductive procedures were equally effective in removing peripheral leukocytes (median decrease, 60%) and produced no complications. Ten children required additional cytoreduction because of further leukocyte increase before chemotherapy became effective. Three children with ALL who had renal insufficiency and metabolic derangement prior to leukapheresis sub‐sequently required additional therapeutic measures. Three children with respiratory symptoms attributable to leukostasis improved after cytoreducation, and there were no episodes of intracerebral hemorrhage. These observations demonstrate the safety and efficiency of exchange transfusion and leukapheresis, and provide support for the role of cytoreduction in the early management of cases of hyperleukostasis and leukemia in children.
Long‐term survival in patients with Ewing's sarcoma relapsing after completing therapy
Authors: Hayes F. A. , Thompson E. I. , Kumar M. , Hustu H. O.
Published: February 2007

While the proportion of patients with Ewing's sarcoma attaining and maintaining long‐term remission has markedly improved, a proportion of patients suffer relapse of the tumor. In our experience relapses may occur late in the course of the disease, approximately 20% of those at risk beyond 5 years having recurrence of tumor.

Review of our data indicates that for patients relapsing after therapy has been discontinued, the probability of attaining second remission is high (0.84) and that a significant proportion of these patients attaining second complete remission will survive their disease (0.60).
Leiomyosarcoma of the kidney: Report of a patient with favorable response to doxorubicin and cisplatin suspended in a lipid contrast medium and cyclophosphamide
Authors: Taniguchi Hiroki , Takahashi Toshio , Fujita Yoshihiro , Sakita Masakazu , Ogita Shuhei , Sawai Kiyoshi , Yamaguchi Toshiharu , Yokota Takashi , Nakagawa Noboru , Shimotsuma Masataka , Kondo Shinji
Published: February 2007

A man who had unresectable leiomyosarcoma of the left kidney with skin and lung metastases was treated with intra‐arterial infusion chemotherapy consisting of cisplatin and doxorubicin suspended in lipid contrast medium, lipiodol, and oral administration of cyclophosphamide. The tumor responded well to this treatment three times. He is alive and well in remission, the renal tumor decreased in size, and lung metastases became unclear more than 50 weeks after treatment was completed.
Long‐term follow‐up of children born to mothers with acute leukemia during pregnancy
Authors: Avilés Agustin , Niz José
Published: February 2007

Seventeen children born to mothers with acute leukemia who received chemotherapy during pregnancy were examined for physical health, growth, and development. The hematologic and neurologic status and school performance were also evaluated. Chromosomal studies were done in the long‐term survivors ranging in age from 4 to 22 years. The children had thorough history and physical examinations to detect any abnormal symptoms or signs. The mothers' previous treatment was documented. In each child growth and development, school performance, intelligence testing, neurologic examination, and hematologic evaluation including bone marrow were normal. Bone marrow cytogenetic studies were also normal. Chemotherapy was given during the pregnancy in each case, including 11 cases during the first trimester. No fetal malformations were found and no late side effects could be demonstrated. The results of this study indicate that pregnancy is not a counterindication for treatment of patients with acute leukemia, and in the cases described here chemotherapy is not associated with excessive risk to the fetus.
Epidemiological features of Wilms' tumor: Results of studies by the international society of paediatric oncology (SIOP)
Authors: Pastore Guido , Carli Modesto , Lemerle Jean , Tournade Marie F. , Voute Paul A. , Rey Annie , Burgers J. Marion V. , Zucker Jean M. , Burger Dietrich , De Kraker Jan , Delemarre Jan F. M.
Published: February 2007

This descriptive epidemiology study of 1,040 children with Wilms' tumor (WT) registered in the International Society of Paediatric Oncology (SIOP) clinical trials confirms the findings reported by the National Wilms' Tumor Study. The male:female rate was 0.89:1. The mean age at diagnosis of the 43 bilateral cases was significantly younger than children with unilateral renal involvement (32.4 vs 45 months). However, the mean ages at diagnosis for unilateral multicentric and for unicen‐tric WT were very similar. On the other hand, the mean age at diagnosis of children with sporadic aniridia and hypospadias was younger than the mean age of patients with or without other congenital malformations. Thus aniridia as well as hypospadias could be indices of the first mutation, according to the Knudson and Strong hypothesis. WT was reported in two members of each of five families. However, these familial cases were comparable in terms of demographic and clinical features to the nonfamilial ones. These data suggest that the heritable fraction of WT is relatively small and that genetic and environmental factors interact in the development of WT.
Cyclic combination chemotherapy for acute lymphoblastic leukemia recurring after elective cessation of therapy
Authors: Pui Ching‐Hon , Bowman W. Paul , Ochs Judith , Dodge Richard K. , Rivera Gaston K.
Published: February 2007

Cyclic combination chemotherapy was administered to 26 patients with acute lymphoblastic leukemia who had relapsed in the bone marrow ≥ 6 months after elective cessation of therapy. Each patient had been in initial continuous complete remission for 36‐111 months (median, 47 months). Prednisone, vincristine, and doxorubicin induced second complete remissions in all patients within 1 month. Continuation therapy consisted of alternating 6‐week courses of 6‐mercaptopurine/methotrexate and vincristine/cyclo‐phosphamide with intervening reinforcement courses of prednisone/doxorubicin, for a total of 18 months. All patients received 4 weeks of late intensification therapy with the same agents used for remission reinduction. Periodic intrathecal methotrexate was given as reprophylaxis for subclinical central nervous system leukemia. The estimated rate of continuous failure‐free survival at 5 years is 31% ± 17% (2 SE). Eight patients remain free of leukemia for 42+ to 65+ months after completing therapy a second time. Adverse second events included 11 hematologic, 1 testicular, and 3 meningeal relapses. Patients who relapsed at more than 12 months after the completion of initial treatment have had significantly longer second remissions than patients whose first remissions were shorter (p = .04). None of the other six factors we analyzed showed predictive strength. These end results indicate that intensive cyclic continuation chemotherapy, as described here, will secure durable second remissions in approximately one‐third of the children with late bone marrow relapses.
Phase II study of recombinant beta interferon in patients with advanced non‐small‐cell lung carcinoma
Authors: Ettinger David S. , Harwood Kerry
Published: February 2007

Eighteen patients with advanced non‐small‐cell lung cancer (NSCLC) received recombinant beta interferon, 90 million units three times weekly. No complete or partial responses were seen. Five patients had stable disease for several months. Most patients experienced some toxicity, most commonly fever and chills. No dose reduction of interferon had to be made due to toxicity. Tachyphylaxis to symptoms of fever and chills occurred in most patients. While 33% of patients developed beta‐interferon‐binding antibodies, the incidence of interferon‐neutralizing activity was 0%. This dose and schedule of beta interferon did not result in significant anti‐tumor effects in advanced NSCLC. Stabilization of disease for up to 9 months in patients with previously progressive disease was of interest. Tachyphylaxis of symptoms should allow for dose escalation within patients. A dose‐escalating study has been initiated to evaluate the efficacy of beta interferon when given at each patient's maximally tolerated dose.
Referral of pediatric oncology patients for marrow transplantation and the process of informed consent
Authors: Chauvenet Allen R. , Smith Nancy M.
Published: February 2007

A survey of 24 pediatric oncologists from 21 institutions not performing bone marrow transplants found that 156 patients were referred for transplants in the years 1984/85; only 10 of these patients were not transplanted. No patient in good clinical condition whose disease was under control was denied transplantation after evaluation at a transplant center. The decision for transplantation was made by the families and the referring pediatric oncologists, almost always before the patient was seen at a transplant center. The fact that almost all patient and family evaluations at transplant centers take place after a family has decided to have a marrow transplant has obvious implications regarding informed consent. Referring pediatric oncologists must attempt to provide the best possible information to families when decisions regarding marrow transplantation are actually being made.
Trends in the incidence of childhood and adolescent cancer in connecticut, 1935–1979
Authors: Van Hoff Jack , Schymura Maria J. , McCrea Curnen Mary G.
Published: February 2007

Trends in the incidence of childhood cancer in Connecticut are reported and analyzed for the period 1935‐1979 by 5‐year age groups (0–4, 5–9, 10–14, 15–19 years), using a log linear model method. A threefold increase (P < .001) in the incidence of ALL in males 0–4 years of age was observed, with significant increases of smaller magnitude seen in males aged 5–9 and 15–19 and females aged 0–4 and 5–9. The incidence of central nervous system cancers also increased in several age groups for both sexes with the largest increase seen in males 0–4 years old. Significant increases in incidence of large magnitude were also observed for Hodgkin's disease, in males aged 15–19 years and females aged 10–19 years, for neuroblastoma in both sexes at ages 0–4 years, and for testis and ovarian cancer at ages 15–19 years. This study of trends in incidence of childhood cancers by 5‐year age groups has revealed significant changes, which would not have been as apparent if broader age groups had been used. These results provide relevant data for investigating the etiology of cancer during infancy, childhood, and adolescence. Trends in Connecticut are compared with findings from other registries in the United States and other countries.
Cytogenetic evidence for involvement of erythroid and granulocyte/macrophage progenitors in an infant with monosomy 7 syndrome presenting de novo
Authors: Tachibana N. , Raimondi S. C. , Santana V. M. , Dow L. W.
Published: February 2007

Monosomy 7 presenting as a myelodysplastic syndrome following radiation and chemotherapy has been reported to involve a stem cell capable of both erythroid and granulocyte/macrophage differentiation. To determine if monosomy 7 presenting de novo also involves a multipotential stem cell, we examined mitoses from individual colony‐forming units (CFU)‐CM and burst‐forming units (BFU)‐E colonies derived from semisolid cultures of marrow from an infant with this disorder. Direct cytogenetic analysis of bone marrow cells disclosed the characteristic 45,XY,‐7 karyo‐type in 32 of 35 abnormal metaphases.

Metaphases were obtained from 63 (73%) of 85 CFU‐GM and BFU‐E colonies (median metaphases per colony = 4, range = 1–21), with well‐banded analyzable chromosome spreads available for 15 of the colonies with metaphases. The monosomy 7 karyotype was present in all 14 metaphases from ten BFU‐E colonies and in all seven metaphases from five CFU‐GM colonies. These results indicate that the monosomy 7 karyotype can originate in haematopoietic stem cells with both erythroid and granulocyte/macrophage differentiative potential.
Phase II trial of ifosfamide and mesna in previously treated patients with non‐Hodgkin's lymphoma: Cancer and leukemia group B study 8552
Authors: Case Delvyn C. , Anderson James , Ervin Thomas J. , Gottlieb Arlan
Published: February 2007

Ifosfamide (1.25 g/m2 intravenously/day x 5) with mesna (20% of the ifosfamide dose x six doses on each day of ifosfamide therapy) was administered to 46 previously treated patients with non‐Hodgkin's lymphoma of which 31 were eligible and evaluable. A 29% response rate (9/31) was observed (2 CR and 7 PR) with a median duration of response of 2.5 months. Myelosuppression was dose‐limiting. Hemorrhagic cystitis was observed in three patients (10%). Nausea and vomiting was generally mild or moderate. One patient developed transient neurotoxic symptoms with confusion and disorientation. An additional patient developed an anaphylactic‐type reaction with shortness of breath and respiratory strider during the fourth course of therapy. Ifosfamide, as a single agent, produces remissions of limited duration in non‐Hodgkin's lymphoma in patients in second or third relapse.
Minimal change disease as the etiology of the nephrotic syndrome in a patient with angioimmunoblastic lymphadenopathy
Authors: Staszewski Harry , Kumar Ganesh , Mishriki Yousri
Published: February 2007

Glomerular lesions have rarely been reported with angioimmunoblastic lymphadenopathy (AILD). A patient with histologically documented AILD developed the nephrotic syndrome. Renal biopsy demonstrated minimal change disease. Both the lymphadenopathy and the nephrotic syndrome spontaneously remitted in this patient. Prior reports of renal disease in AILD and atypical lympho‐proliferative disorders are discussed. An association between the minimal change lesions and AILD is suggested.
Immunoblastic peripheral T‐cell lymphoma confined to bone marrow in an infant presenting with aspergillosis
Authors: Nuss Rachelle , Ribeiro Raul C. , Bunin Nancy , Behm Frederick , Jenkins Jesse , Berard Costan , Murphy Sharon B.
Published: February 2007

A 16‐month‐old female infant presenting with pancytopenia and fever was found to have pulmonary aspergillosis and large‐cell immunoblastic non‐Hodgkin's lymphoma of peripheral post‐thymic origin isolated to bone marrow. Extensive noninvasive evaluations failed to demonstrate the disease in other extramedullary sites. The malignant cells were large and polymorphous; lacked terminal transferase; expressed surface CD‐2, CD‐3.

CD‐8, and HLA‐DR antigens, and showed rearrangements of the T‐cell‐receptor β‐chain gene. To our knowledge, this type of lymphoma in an infant has not been reported before. Furthermore, aspergillosis is a rare presenting feature in patients with lymphoproliferative disease. In our case, it may reflect an underlying immune deficiency associated with the transformation and proliferation of a suppressor T cell.
Cytogenetic study of 130 childhood acute nonlymphocytic leukemias
Authors: Leverger Guy , Bernheim Alain , Daniel Marie‐Thérèse , Flandrin Georges , Schaison Gérard , Berger Roland
Published: February 2007

Cytogenetic studies performed on 130 consecutive childhood acute nonlymphocytic leukemias (ANLL) investigated in the same center between 1977 and 1986 are reported. The incidence of clonal chromosomal abnormalities was 68.5% with uneven distribution among the groups of the FAB nomenclature. The high incidence of t(8;21) translocation cases which was 58.6% of M2 ANLL cases was remarkable. Complete remission rate was lower (P<0.05) in ANLL with all karyotypically abnormal metaphases (AA) than in the other ANLL (NN with only normal metaphases and AN with a mixture of normal and abnormal metaphases). Median survival was also shorter in AA ANLL than in AN and NN cases (P<0.01). Median survival was different according to karyotype abnormalities: 11q anomalies and t(15;17) were not associated with a good prognosis and the t(8;21) is not associated with a particularly long median survival (16 months) when compared with other ANLL as opposed to the results of others. The longest survival (26 months) was observed in patients with acute myelomonocytic leukemia with bone marrow eosinophilia. It may be concluded that chromosome studies have a prognostic value in childhood ANLL.
Chemotherapy side effects in pediatric oncology patients: Drugs, age, and sex as risk factors
Authors: Lebaron Samuel , Zeltzer Lonnie K. , Lebaron Christine , Scott Shannon E. , Zeltzer Paul M.
Published: February 2007

Nausea, vomiting, and the extent to which chemotherapy‐bothered children were assessed by patient and parent ratings for 31 children (65 courses) receiving combination chemotherapy with either highdose cyclophosphamide or doxorubicin /daunorubicin. Patients and parents both reported more severe vomiting with cyclophosphamide than with the anthracyclines. The use of antiemetics did not affect emesis for the former drug; for the anthracyclines, there was more severe emesis for courses with antiemetics than for those with none. Adolescents reported more severe nausea than children, and females reported both more nausea and bother than males. There were no significant age or sex findings for parent reports. The findings suggest that chemotherapy‐related nausea and vomiting in children is a complex phenomenon not accounted for by drugs alone.
Phase II study of bisantrene administered by continuous 72‐hour infusion for advanced pancreatic adenocarcinoma
Authors: Marschke Robert F. , Kvols Larry K. , Cullinan Stephen A. , Laurie John A. , Mailliard James A. , Tschetter Loren K. , O'Connell Michael J.
Published: February 2007

Eighteen ambulatory patients who had proven metastatic adenocarcinoma of the pancreas and measurable disease but no previous chemotherapy were treated with bisantrene given by constant central intravenous infusion over 72 hours at a total dose of 300 mg/m2 repeated every 3 to 4 weeks. No objective regression was seen. The median interval to progression was 6 weeks; the median survival was 14 weeks. Primary toxic reactions were nausea, vomiting, and leukopenia. In no instance were these life‐threatening. When administered by the method we used, bisantrene cannot be recommended for treatment of advanced pancreatic adenocarcinoma.