bluuebunny/crossref_metadata_2025 huggingface dataset:
Anaplastic large cell lymphoma of true histiocytic origin in an infant: Unusual clinical, hematological, and cytogenetic features
Authors: Ferster Alina , Corazza Francis , Heimann Pierre , Dehou Marie‐Françoise , De Busscher Chantal , Devalck Christine , Sariban Eric , Vamos Esther
Published: February 2007

We report here the case of a 7‐month‐old boy who developed anaplastic large cell lymphoma of true histiocytic origin or malignant histiocytosis, with fever, bone and bone marrow infiltration. Usual clinical features were absent. The neoplastic nature of the disease was supported by the presence of clonal chromosomal abnormalities [t(6;8)(p23;P21),der(8)del(8)(q11aq13), der(22) t(11;22) (q13;13)]. Neither B nor T lineage could be demonstrated here. Morphology, ultrastructural analysis, surface antigens expression, and cytogenetics were more specific for the monocyte‐macrophage lineage.
Stereotactic external beam irradiation in previously untreated brain tumors in children and adolescents
Authors: Freeman Carolyn R. , Souhami Luis , Caron Jean‐Louis , Villemure Jean‐Guy , Olivier André , Montes José , Farmer Jean‐Pierre , Podgorsak Ervin B.
Published: February 2007

Stereotactically guided external beam irradiation may be a useful form of treatment for small, well‐circumscribed, but surgically inaccessible, primary brain tumors that are either benign or of low malignant potential. Between March 1988 and December 1991, 10 children and adolescents with previously untreated primary brain tumors were treated with stereotactic external beam irradiation (SEBI) using a linac‐based dynamic technique. Eleven lesions were treated in the 10 patients. Treatment was given using a collimator diameter of 1.5‐4 cm (median 2 cm). Single fractions of 18, 20, and 25 Gy were used for 3 lesions in 2 patients. A fractionated schedule delivering a median dose of 42 Gy in 6 fractions over 2 weeks was used in the remaining 8 patients. Morbidity related to treatment was minimal. Three patients suffered a temporary worsening of preexisting neurological symptoms and/or signs at 2, 5, and 5 months posttreatment, with subsequent recovery in all. With a median follow up post‐SEBI of 17.5 months (range 5‐47 months), improvement in neurological findings related to the lesion was noted for 5 treated lesions; 6 remained clinically stable. Seven of the 11 treated lesions improved radiologically, and only 2 showed evidence of progressive disease. Stereotactic external beam irradiation represents a potentially valuable therapeutic option for selected primary brain tumors in the pediatric and adolescent age group. Morbidity related to the treatment appears acceptable in frequency and type, and preliminary data with regard to response are encouraging. However, in order to assess the impact of such treatment on long‐term tumor control and survival, further experience with a larger cohort of patients followed for a longer period of time will be necessary. © 1994 Wiley‐Liss, Inc.
Plasma and urine levels of methotrexate and 7‐hydroxymethotrexate in children with all during maintenance therapy with weekly oral methotrexate
Authors: Skoglund Kathleen A. , Söderhäll Stefan , Beck Olof , Peterson Curt , Wennberg Mona , Hayder Salah , Björk Olle
Published: February 2007

A new high‐performance liquid chromatographic assay was used to determine methotrexate (MTX) and its main metabolite, 7‐hydroxymethotrexate (7‐OH‐MTX), in the plasma (n ‐ 17) and urine (n = 14) of children (age 3‐12 years) on maintenance therapy for acute lymphocytic leukemia (n = 14) or non‐Hodgkin's lymphoma (n = 3). Each child received oral doses of weekly MTX (4.0‐29 mg/m2) and daily 6‐mer‐captopurine (40‐111 mg/m2). Plasma samples were collected daily from two children during the 1‐week dose interval. A limited sampling strategy was designed, whereby 2 days of blood sampling were used in the other 15 patients. Morning urine samples were collected daily for 1 week following MTX intake from 14 of the children. MTX was detectable in all plasma and urine samples for the entire dose interval. The main metabolite, 7‐OH‐MTX, could be detected in plasma and urine from all patients on the first day after dose intake but only in a few patients during the whole dose interval. Interpatient variability of MTX and 7‐OH‐MTX levels was high at all points during the week. Significant correlation were found between the urinary MTX levels on days 2 and 7 and plasma MTX levels on day 2 after intake. No significant correlation was found between drug levels in plasma or urine and liver function tests in the children showing signs of mild liver injury. This assay provides a tool for further studies on the role of pharmacokinetics for the clinical effects of weekly oral low‐dose MTX given alone or in combination with 6‐mercaptopurine. © 1994 Wiley‐Liss, Inc.
Clinical relevance of in vitro drug resistance testing in childhood acute lymphoblastic leukemia: The state of the art
Authors: Pieters Rob , Kaspers Gertjan J. L. , Klumper Edwin , Veerman Anjo J.P.
Published: February 2007

Nowadays about two‐thirds of children with acute lymphoblastic leukemia (ALL) can be cured with chemotherapy, but one‐third die from the disease. The clinical response of leukemic cells to chemotherapy is roughly due to two factors: the effective drug levels reaching the cells and the resistance of these cells to the drugs. The clinical value of cellular drug resistance in children with ALL is not known. We developed an in vitro assay to study drug resistance in these children. In this article, the main results obtained with this MTT assay on samples from 137 children with ALL are summarized: (1) patients whose cells are resistant to several drugs at initial diagnosis have a poor prognosis; (2) relapsed leukemias show a considerable drug resistance which might partly explain the poor prognosis. Relapsed cases differ in their type and degree of resistance; (3) the poor outcome of high risk groups as defined by age and immunophenotype can partly be explained by specific patterns of drug resistance; (4) P‐glycoprotein‐mediated multidrug resistance is not an important cause of resistance in childhood ALL; and (5) no relation exists between the activities of the purine enzymes HGPRT, 5′NT, ADA, and PNP and drug resistance in childhood ALL. The conclusion is that in vitro drug resistance data have clinical relevance and can be used to develop more effective and less toxic treatment strategies in childhood ALL. © 1994 Wiley‐Liss, Inc.
Predisposition to corynebacterium jeikeium infection in acute lymphoblastic leukemia: a report of two cases in trinidad
Authors: Fosi‐Mbantenkhu Joseph , Orett F. A.
Published: February 2007

Two hospitalized patients (a 4 and 6 year old male and female, respectively) with acute lymphoblastic leukaemia (ALL), had each at different periods sustained burns with necrosis from extravasation of chemo‐therapeutic products. Pure cultures from their wound exudates and blood repeatedly revealed Corynebacterium jeikeium, an organism recognized and identified in the last decade as an opportunistic cause of life‐threatening nosocomial infections particularly in patients on long‐term multiple antimicrobial therapy, and in those who are neutropenic or have cardiac valve replacement. The organism has been found to be highly resistant to many antibiotics but sensitive to a few, including vancomycin. Both patients were successfully treated with vancomycin and are reviewed here to emphasize the importance of this group of microorganisms resistant to multiple antibiotic therapy as possible frequent cause of sub‐acute bacterial infection in immunode‐pressed conditions such as ALL, especially when external wounds are inflicted by extravasation of antileukaemic agents (notably, vinca alkaloids). A review of the literature indicates that these are the first reported cases of infection caused by these organisms in Trinidad, and it is of clinical and therapeutic interest to note that they are sensitive to norfloxacin, polymyxin, tetracycline, and particularly vancomycin in this Study. © 1994 Wiley‐Liss, Inc.
The role of fast neutron therapy in unresectable pelvic osteosarcoma: preliminary report
Authors: Carrie C. , Breteau N. , Negrier S. , Frappaz D. , Bouffet E. , Thiesse P. H. , Blay J. Y. , Clavel M. , Philip T. , Biron P. , Brunat‐Mentigny M.
Published: February 2007

Primary osteosarcoma of the pelvic bones represents less than 10% of all osteosarcoma. despite mutilating surgical procedures or high‐dose radiotherapy and neoadjuvant chemotherapy, local failures and distant metastases remain common. the 2‐year actuarial survival rate is less than 50% and the disease‐free survival is under 20% at 2 years. we report 4 cases of pelvic osteosarcoma treated by a combination of chemotherapy, photon beam and neutron beam radiotherapy. after a median follow‐up of 24 months, all 4 patients are alive and disease free. © 1994 wiley‐liss, inc.
Treatment of relapsing acute lymphoblastic leukemia in childhood. III. Experiences with 54 first bone marrow, nine isolated testicular, and eight isolated central nervous system relapses observed 1985‐1989
Authors: Weid Nicolas Von Der , Wagner Bendicht , Angst Regula , Arnet Beat , Baumgartner Christoph , Beck Daniel , Bleher Arnold , Caflisch Ueli , Delaleu Beatrice , Feldges Andreas , Frey Eva , Glanzmann Ingeborg , Hirt Andreas , Imbach Paul , Kratel Silke , Küchler Henri , Leibundgut Kurt , Meister Andreas , Morin Anne‐Marie , Nobile Luisa , Plüss Hans‐Juerg , Lüthy Annette Ridolfi , Signer Erich , Stettler Erich , Wyss Marinette , Wagner Hans‐Peter
Published: February 2007

Of 54 children with acute lymphoblastic leukemia (ALL) and first hematological recurrence observed between 1985 and 1989, 31 relapsed while still on treatment and 23 after cessation of therapy. Of the former, only one survived. Of the latter, 11 children survived after a minimum follow‐up of 25 months. During the same period, a first isolated testicular relapse was observed in nine boys, of whom six survived, and an isolated CNS relapse in eight patients, of whom three survived. As a rule, survivors of a bone marrow or testicular relapse were doing well while those surviving a CNS relapse had considerable neuropsychological sequelae. These results, compared with those of two preceding studies, suggest that with intensification of front‐line treatments, it becomes more difficult to rescue children who relapse, particularily those with a bone marrow relapse while on therapy. © 1994 Wiley‐Liss, Inc.
Cerebral irradiation causes blunted pubertal growth in girls treated for acute leukemia
Authors: Moëll C. , Marky I. , Hovi L. , Kristinsson J. , Rix M. , Moe P. J. , Garwicz S.
Published: February 2007

The improved treatment of childhood leukemia is a major achievement. The late effects of the treatment need further investigation. Growth inhibition has been demonstrated in earlier studies.

Growth and the timing of puberty were studied in 179 girls who had been treated for acute lymphoblastic leukemia (ALL) in Denmark, Finland, Norway, and Sweden. The patients were divided into two groups according to mode of CNS prophylaxis: with or without cerebral irradiation.

Longitudinal analysis of 103 patients showed no difference in prepubertal growth in irradiated and nonirradiated girls. Growth during puberty was normal in girls without irradiation and below normal in irradiated girls. There was no difference in growth between girls after 24 Gy or 20 Gy of cerebral irradiation. Irradiated girls had a final height which was one SD less than expected before puberty and menarche occurred one year earlier than in the nonirradiated girls.

Prophylactic cerebral irradiation is the most important factor for subnormal growth after treatment for ALL. There is no short‐term influence on growth but the effects of irradiation become apparent several years after therapy when girls enter puberty somewhat early and have a subnormal pubertal growth. Growth and growth hormone (GH) levels should be evaluated several years after CNS irradiation, and treatment with GH and/or luteinizing hormone releasing hormone (LHRH) analogues may be considered. © 1994 Wiley‐Liss, Inc.
Somatostatin therapy in l‐asparaginase‐induced pancreatitis
Authors: Cheung Y. F. , Lee C. W. , Chan C. F. , Chan K. L. , Lau Y. L. , Yeung C. Y.
Published: February 2007

L‐Asparaginase‐induced pancreatitis is an uncommon but potentially lethal complication. An 8‐year‐old girl with acute lymphoblastic leukaemia developed acute pancreatitis following treatment with asparaginase. Clinical and laboratory improvements were evident after treatment with somatostatin, with no complications of pancreatitis. Induction therapy for the leukaemia was able to be continued and complete remission was documented during the course of pancreatitis and somatostatin treatment, suggesting a beneficial role of somatostatin in the management of asparaginase‐induced pancreatitis. © 1994 Wiley‐Liss, inc.
Leukemic ophthalmopathy: A report of 21 pediatric cases
Authors: Curto M. Lo , D'Angelo P. , Lumia F. , Provenzano G. , Zingone A. , Bachelot C. , Bagnulo S. , Behrendt H. , Jankovic M. , Masera G. , Mann J. , Rosito P. , Schaison G. , Schuler D.
Published: February 2007

A multicentric retrospective study on leukemic ophthalmopathy (LO) is reported. It includes 21 patients, 16 males and 5 females, with acute leukemia (AL) observed in 10 SIOP centers. LO developed in three patients at the time of diagnosis of AL; five patients were in first complete remission (three off therapy); four patients were in second or third remission; and nine were in combined relapse. Most frequent symptoms were blurred vision, photophobia, and ocular pain. Two patients with acute nonlymphoblastic leukemia died before treatment; another underwent bone marrow transplantation; one patient with B‐cell acute lymphoblastic leukemia (B‐ALL) treated with chemotherapy and radiotherapy died 4 months after LO; the remaining 17 children were treated according to different schedules with (10) or without (7) radiotherapy on the affected eye. Twelve patients achieved ocular remission and four of these had a second ocular relapse. Complete remission after LO treatment lasting for more than 3, 7, 24, 29 months was observed in four patients. The authors conclude that cure is possible in patients who had LO in first complete remission treated with chemotherapy and radiotherapy at high dose on the affected eye. © 1994 Wiley‐Liss, Inc.
Renal cell carcinoma in children: A single institution's experience
Authors: Eckschlager Tomáš , Kodet Roman
Published: February 2007

Seven patients with renal cell carcinomas (RCC) were treated at the Department of Pediatric Oncology in Prague over a period of 15 years. The average age at diagnosis was 8 years 9 months. Histology review showed five tumors to be of clear cell type with mixed tubular, papillary, and solid patterns; one tumor was purely papillary, eosinophilic type and one was composed of clear and eosinophilic cells. Five patients received chemotherapy and four of them also received radiotherapy. Four patients are without evidence of disease with a minimal follow‐up period of 2 years. These four were all clinical stage I or II. Two stage III patients died of metastatic disease. One patient died of tumor emboli to the lungs at operation. Tumor diagnosis and treatment of the patients in the lower clinical stages seem to have a favorable prognostic impact in childhood RCC. Combined chemotherapy with vinblastine as one of the agents, and possibly additional immunotherapy, appears to have promising results, although the number of patients treated thus far is limited. © Wiley‐Liss, Inc.
Granular cell tumor of the biliary system
Authors: Mackenzie Douglas J. , Klapper Ellen , Gordon Leo A. , Silberman Allan W.
Published: February 2007

Granular cell tumors (GCT) are rare benign neoplasms of Schwann cell origin which have been found in virtually every location in the body. Their location in the biliary system is unusual, and typically occurs in black females during the fourth decade of life. Forty‐eight cases have been reported since 1952. We present two patients treated for obstructive jaundice caused by GCT of the extrahepatic biliary tree. The literature on biliary GCT is reviewed and their management is outlined. Local excision with Roux‐y‐hepaticojejunostomy was performed in one patient with GCT obstructing the common bile duct at the level of the cystic duct. Pancreaticoduodenectomy was performed on one patient for GCT of the common bile duct involving the ampulla and adjacent pancreas. GCT of the extrahepatic biliary system are rare but should be considered in black females who present with obstructive jaundice in the fourth decade of life. Local excision with cholecystectomy and reconstruction of the biliary remnant is indicated. Primary biliary anastomosis is desired but choledochojejunostomy or hepaticojejunostomy may be necessary. Rarely, pancreaticoduodenectomy may be indicated in GCT of the distal common bile duct. © 1994 Wiley‐Liss, Inc.
Synovial sarcoma of the larynx in a child: Case report and histological appearances
Authors: Morland Bruce , Cox Graham , Randall Chris , Ramsay Alan , Radford Martin
Published: February 2007

Synovial sarcoma of the larynx is extremely rare having been reported only six times previously in the literature. We add another case report, which to our knowledge is the first recorded case in a child. We discuss the alternative approach of combination chemotherapy and radiotherapy which in this case led to a remission lasting about 3 years. The immunohistological and ultrastructural characteristics of the tumour are also presented. © 1994 Wiley‐Liss, Inc.
Acute leukemia in association with langerhans cell histiocytosis
Authors: Egeler R. Maarten , Neglia Joseph P. , Aricò Maurizio , Favara Blaise E. , Heitger Andreas , Nesbit Mark E.
Published: February 2007

Langerhans cell histiocytosis (LCH) and malignancy occurring in the same individual is unusual and has generally been the subject of isolated case reports. To better define the occurrence of these events a registry of cases with synchronous or asynchronous LCH and malignancy was developed with the cooperation of the Histiocyte Society.

In 1991 the Histiocyte Society surveyed its members requesting information on cases in which LCH was associated with malignancy. The questionnaire was mailed to all members of the society and specifically requested information on the clinical and laboratory features of the cases, disease evolution, and response to therapy. Retrospective reporting was allowed. With this initial data, an ongoing registry of LCH patients with associated malignancy was begun of such cases, including evolution and response to therapy.

Twenty‐seven patients were enrolled during the first year of the registry, of whom 4 patients had the association of LCH with a malignant lymphoma and 10 cases had an association of LCH with other types of solid tumor. The remaining 13 patients had the association of LCH with acute leukemia. In five cases, LCH was associated with acute lymphoblastic leukemia FAB L1 (ALL). In four cases the ALL preceded the LCH by 6 months to 1 year. In four of five patients the LCH was localized; in two instances the LCH was treated with chemotherapy. In all cases the leukemia was treated according to local standard ALL protocols and in one case autologous bone marrow transplantation (ABMT) was performed at relapse. Three patients are free of leukemia, one of whom has persistent localized LCH of the skin. Two patients died of the ALL, one of whom was free of the LCH at the time of death. In eight instances LCH was reported in association with acute myeloid leukemia (AML). Six of these patients had a generalized form of LCH. In seven the diagnosis of LCH preceded the diagnosis of leukemia by more than 2 years (median 4 years). In the remaining patient both diagnoses were made concurrently. In all seven cases in whom LCH was the initial diagnosis the treatment consisted of chemotherapy and/or radiotherapy. Seven patients died from the AML, five without evidence of LCH.

The temporal patterns of the LCH‐ALL and LCH‐AML associations are distinct with ALL usually preceding the diagnosis of LCH and AML succeeding it. Such a pattern is suggestive that in cases of ALL the LCH may be a reactive process while in cases of AML occurring after LCH the primary LCH therapy may play an inductive role in the leukemia. © 1994 Wiley‐Liss, Inc.
Acute myeloid leukemia in patients treated for rhabdomyosarcoma with cyclophosphamide and low‐dose etoposide on intergroup rhabdomyosarcoma study III: An interim report
Authors: Heyn Ruth , Khan Fareed , Ensign Lisa G. , Donaldson Sarah S. , Ruymann Frederick , Smith Malcolm A. , Vietti Teresa , Maurer Harold M.
Published: February 2007

The early occurrence of five cases of acute myeloid leukemia (AML) in children treated for primary rhabdomyosarcoma on the Intergroup Rhabdomyosarcoma Study III (IRS III) has prompted this report. These patients received cyclophosphamide and four received etoposide in addition to other agents.

There were 1,062 eligible patients entered on IRS III between 1984 and 1991. Following surgery, treatment consisted of multiagent chemotherapy and radiotherapy in select clinical groups. Median follow‐up time is 3.7 years (range 0–7.4 years). Incidence densities and odds ratios for AML were calculated for various treatment groups.

Five cases of secondary AML have been reported through August 1992. A single case of osteogenic sarcoma was reported in the same period and a patient with myelodys plastic syndrome has occurred since that time. Median time to development of AML was 39 months. Incidence density of AML for patients receiving neither cyclophosphamide nor etoposide was 0, for those receiving cyclophosphamide but no etoposide it was 7.6, and when both agents were given, it was 51.6. The odds ratios of AML for the latter two groups indicated a risk of AML which was seven times higher in the patients who received both agents. A history of breast cancer was present in all five families of patients with AML and several other cancers had occurred in three families.

Preliminary analysis suggests a possible causal role for low‐dose etoposide in addition to that assumed for cyclophosphamide in the early development of AML among pediatric patients treated for rhabdomyosarcoma. © 1994 Wiley‐Liss, Inc.
Negative 123‐meta‐lodobenzyl‐guanidine imaging in Ewing's sarcoma of bone
Authors: Oberlin O. , Lumbroso J. , Terrier P. H. , Hartmann O. , Lemerle J. , Parmentier C.
Published: February 2007

The role of radiolabeled meta‐iodobenzyl‐guanidine (mlBC) is established in the detection and the staging of neuroblastoma. We designed a study of mlBG scanning for patients with Ewing's sarcoma, a tumor for which a neuroectodermic origin has been proposed.

We explored 15 children with round cell sarcoma of bone by a whole body scan carried out 24 hours after injection of 123‐1 mlBG. No patient demonstrated significant uptake either at the site of the primary or at the sites of metastases.

These results suggest that despite its neural histogenesis Ewing's sarcoma is a member of the nonneuroblastoma neural crest tumors, which does not produce or store adrenergic metabolites. © 1994 Wiley‐Liss, Inc.
Primary cardiac angiosarcoma: A case report and review of therapeutic options
Authors: Stein Moshe , Deitling Freddy , Cantor Angel , Perner Yvonne , Bezwoda Werner
Published: February 2007

A 45‐year‐old black patient presented with right‐heart failure. Echocardiography showed a large tumor mass in the right atrium and a debulking procedure was performed. Histological and immunohistochemical studies revealed a high grade angiosarcoma infiltrating the myocardium and extending to the pericardial surface. Metastatic work‐up showed no evidence of dissemination. The patient made an uneventful postsurgical recovery and was treated with chemotherapy, consisting of epidoxorubicin and dacarbazin. Following chemotherapy, echocardiographic re‐evaluation failed to show any residual tumor in the right atrium.

At present, one year following diagnosis, the patient is alive with no evidence of disease. Therapeutic options of primary cardiac angiosarcoma are reviewed. © 1994 Wiley‐Liss, Inc.
Primary osteosarcoma of the cervical spine: A pediatric case report and review of the literature
Authors: Kebudi Rejin , Ayan Inci , Darendeliler Emin , Aǧaoǧlu Leyla , Bayindir Çiçek , Turantan Inan , Kinay MÜNir , Bilge Nijad
Published: February 2007

A 4 1/2‐year‐old girl with a primary osteosarcoma of the seventh cervical vertebra, treated by surgery and chemotherapy, and who is off therapy for 24 months with no evidence of disease is presented and the literature reviewed. © 1994 Wiley‐Liss, Inc.
Bone marrow transplantation for high risk neuroblastoma at the children's hospital of philadelphia: An update
Authors: Evans Audrey E. , August Charles S. , Kamani Naynesh , Bunin Nancy , Goldwein Joel , Ross Arthur J. , D'Angio Giulio J.
Published: February 2007

A bone marrow transplant (BMT) protocol including surgical excision, local and total body irradiation, and high dose multiagent chemotherapy based on melphalan and bone marrow rescue has been in effect for children with high risk or relapsed neuroblastoma at the Children's Hospital of Philadelphia since 1979. The initial results were reported in 1984 [August et al.: J Clin Oncol 2:609–616, 1984]. This report updates the initial results and those that followed changes in the original conditioning regimen. Forty‐two patients were treated between May 1979 and November 1987, and included 27 whose disease had relapsed and 15 who received BMT as part of primary treatment. Allogeneic marrow was given to 12 and autologous marrow to 30; in 7 of these 30, the marrow was purged with monoclonal antibodies and magnetic beads. The 4‐year actuarial survival rate is 29%. Ten patients died of early treatment‐related complications, 18 died of progressive disease, and 2 died of late complications (1 AIDS and 1 acute myelogenous leukemia). Censoring the two late complications the actuarial 4‐year relapse‐free survival rate becomes 32%. The longest interval after BMT to relapse was 20 months. There was no significant difference in the survival for patients transplanted following relapse or in first remission. The better survival for patients rescued with autologous marrow (30%) is not statistically significantly different from the result with allogeneic marrow (17%). © 1994 Wiley‐Liss, Inc.
Pediatric renal cell carcinoma: A complete response to recombinant interleukin‐2 in a child with metastatic disease at diagnosis
Authors: Macarthur Craig A. , Isaacs Hart , Miller John H. , Ozkaynak Fevzi
Published: February 2007

Renal cell carcinoma is a rare pediatric malignancy that appears to have a similar clinical outcome in children and adults. We review the experience of Childrens Hospital Los Angeles and compare it with the published pediatric series, reporting on seven cases from 1954 to the present. As in earlier pediatric series, we find that Stage I/II patients do well (five of five complete responses with prolonged disease‐free survival) with surgical resection. As in other pediatric series, our only Stage III patient died of disease.

We also report on a recent case of renal cell carcinoma, metastatic to lymph nodes and lung parenchyma at diagnosis (Stage IV). This patient was treated with high dose continuous infusion recombinant interleukin‐2 and had a partial response. The patient attained a complete response following a second laparotomy and two subsequent cycles of recombinant interleukin‐2. He is presently well, without evidence of disease, 3 1/2 years after diagnosis. The significance of this form of therapy to advanced renal cell carcinoma in childhood is discussed. © 1994 Wiley‐Liss, Inc.
Primary central nervous system Burkitt's lymphoma presenting as Guillain‐Barré syndrome
Authors: Toren A. , Mandel M. , Shahar E. , Rimmoni E. , Roizin H. , Neuman Y. , Brok‐Simoni F. , Mark Z. , Biniaminov M. , Rosenthal E. , Segal M. , Frand M. , Ben‐Bassat I. , Rechavi G.
Published: February 2007

A rare case of CNS Burkitt's lymphoma presenting as acute Guillain‐Barré syndrome is presented. A 6‐year‐old previously healthy female presented with acute onset of limb and truncal weakness, involvement of ocular and bulbar nerves, and areflexia. The clinical diagnosis of Guillain‐Barré syndrome prompted treatment with intravenous gammaglobulin with no response. A lumbar puncture following revealed marked pleocytosis, elevated protein, and decreased glucose. Immunological, cytological, and molecular studies of these cells confirmed the diagnosis of Burkitt's lymphoma IgM, kappa with t(8;14) and rearrangement of the J and kappa immunoglobulin chains. Aggressive systemic and intrathecal chemotherapy were started and within 5 days remission was achieved. The child is in complete remission 2 years from diagnosis. Although very rare, CNS lymphoma should be taken into account in every patient presenting with the clinical features of acute polyneuropathy. © 1994 Wiley‐Liss, Inc.
High‐dose chemotherapy with marrow reinfusion and hyperfractionated irradiation for children with high‐risk brain tumors
Authors: Kedar Amos , Maria Bernard L. , Graham‐Pole John , Ringdahl Deborah M. , Quisling Ronald G. , Mickle J. Parker , Mendenhall Nancy P. , Marcus Robert B. , Gross Samuel
Published: February 2007

Between November 1990 and March 1993, nine pediatric patients with newly diagnosed brain tumors having a high risk of failure with standard treatment received high‐dose thiotepa/cyclophosphamide chemotherapy followed by autologous bone marrow infusion and involved‐field hyperfractionated radiation therapy. The presenting diagnoses were brainstem glioma (BSG) [6], parietal mixed high‐grade oligodendro‐glioma‐astrocytoma [1], thalamic anaplastic astrocytoma [1], and high‐grade parietal glioma [1]. Following chemotherapy there were two partial responses, one minor response, three with stable disease, and one with progressive disease. Responses were not evaluated in two patients who had toxic deaths. Following radiation two patients, one with brainstem glioma and one with anaplastic mixed glioma, achieved complete remission. The overall survival is no better than conventional therapy. © 1994 Wiley‐Liss, Inc.
Cisplatin protein binding in pregnancy and the neonatal period
Authors: Zemlickis Donna , Klein Julia , Moselhy Galina , Koren Gideon
Published: February 2007

The most effective combination regimes for ovarian cancer contain cisplatin, but there is little knowledge about cisplatin use during pregnancy. The high protein binding of cisplatin means that small changes in protein binding result in large changes in the fraction of free drug. It is the free form of cisplatin that crosses the placenta and may contribute to fetal toxicity.

The purpose of the present study was to compare protein binding of cisplatin in pregnant women, non‐pregnant women, and newborn infants. We found that babies and pregnant women had significantly lower concentrations of both protein and albumin compared to non‐pregnant women. Analysis of variance found overall significant differences in protein binding among the three groups over time (P < .05). Babies had statistically less cisplatin protein binding than non‐pregnant women at 80 minutes and all time points thereafter (P < .05). In contrast, pregnant women had statistically less cisplatin protein binding than non‐pregnant women at 3.3 and 8 hours (P < .05). Of interest, at 75.2 hours, the percentage of free cisplatin was 15% in babies as compared with 9% in non‐pregnant women and 10% in pregnant women. This means that the fetus is exposed to 50% higher platinum levels at equal total concentration. Cisplatin protein binding significantly correlated with albumin concentrations at 3.3, 8, and 24 hours (P < .01).

Our analysis reveals that pregnancy and fetal changes in cisplatin protein binding are caused in large part by lower albumin levels. The resulting higher levels of free drug in the mother and fetus may increase the risk of toxicity in both. © 1994 Wiley‐Liss, Inc.
U.S. childhood cancer survival, 1973–1987
Authors: Novakovic Biljana
Published: February 2007

The surveillance, epidemiology, and end‐results (SEER) data on 5‐year relative survival rates (1973‐1987) for the most common pediatric tumors (ages 0–14) were analyzed. The SEER data are population based, so the observed progress in survival from childhood cancer represents the real impact that development in cancer treatment had on the population followed by the registry.

The greatest increase in survival rate from 1973 until 1987 has been achieved in hematopoietic tumors such as acute lymphocytic leukemia (ALL), in which survival increased from 47.6% (1973–1977) to 60.8% (1983–1987), and Burkitt's lymphoma in which survival increased from 27.6% (1973–1977) to 68.7% (1983–1987). Solid tumors showed a less steep, but steady increase in survival rates.

Flattening in the survival rates since 1978–1982 has been observed for acute leukemia, astrocytoma, medulloblastoma, and osteosarcoma. Females have better survival rates for most pediatric tumors, except Hodgkin's disease. Analysis of race of childhood leukemia confirmed that black children have worse survival than white.

When solid tumors were analyzed by stage at presentation, there was no indication that diagnosis in earlier stages of disease accounted for the improved survival.

Observed flattening in the survival rates since 1978–1982 of leukemia and some solid tumors warrants further follow‐up. © 1994 Wiley‐Liss, Inc.
Methotrexate osteopathy in infants with tumors of the central nervous system
Authors: Meister Bernhard , Gaßner Ingmar , Streif Werner , Dengg Kathrin , Fink Franz‐Martin
Published: February 2007

Methotrexate osteopathy, previously reported as a complication of maintenance‐therapy for acute lymphoblastic leukemia, is characterized by osteopenia, particularly involving the lower extremities, thick, dense provisional zones of calcification, growth arrest lines, and corner fractures resembling scurvy. In attempts to postpone radiotherapy in infants under three years of age, the multicentric German therapy protocol for childhood central nervous system tumors (HIT‐89 protocol) has employed high cumulative methotrexate doses. Here we describe osteopathy in three patients as a toxic side effect after administration of cumulative methotrexate doses of 20 g/m2, 80 g/m2 and 135 g/m2. The high prevalence of this adverse effect in infants with tumors of the central nervous system may be attributed to the long‐term therapy with high cumulative methotrexate‐doses. Both factors may favor intracellular accumulation of methotrexate and formation of methotrexate‐polyglutamates and may be responsible for bone toxicity. Apparently the susceptibility of the rapidly growing skeletal structures of infants under three years of age to this toxic side effect of methotrexate is remarkably high. © 1994 Wiley‐Liss, Inc.
A giant congenital orbital tumor: An unusual presentation of retinoblastoma
Authors: Zwaan C. M. , de Waal F. C. , Koole F. D. , Menko F. H. , van der Valk P. , Slater R. M. , Scheffer H. , van Waveren G. , Moll A. C. , Meeteren A. Y. N. Schouten‐van , Tan K. E. W. P.
Published: February 2007

We report a case of an unusual giant congenital tumor presenting in a newborn infant as a large exophytic mass emerging from the left orbit. After enucleation orbital recurrence developed within 14 days. No anti‐tumor treatment was given and the child died at the age of 4 weeks.

The histopathological and cytogenetical analysis of the tumor is presented. The tumor was diagnosed as a retinoblastoma but we could not exclude the possibility of a neuroblastoma. © 1994 Wiley‐Liss, Inc.
Primary bronchogenic squamous cell carcinoma in children: Report of a case and review of the literature
Authors: Keita Olivia , Lagrange Jean‐Léon , Michiels Jean‐Franéois , Soler Christine , Garcia Jean , Valla Jean‐Stéphane , Thyss Antoine
Published: February 2007

The authors report a case of primary bronchogenic squamous cell carcinoma (SCC) revealed by recurrent pneumonia in an 11‐year‐old boy. Four years after complete surgical resection followed by external radiotherapy, the patient is currently relapse‐ and metastasis‐free. To our knowledge, this is only the sixth pediatric case of primary bronchogenic SCC reported to date. © 1995 Wiley‐Liss, Inc.
Severe hyperphosphatemia following acute tumor lysis syndrome
Authors: Vachvanichsanong Prayong , Maipang Matinee , Dissaneewate Pornsak , Wongchanchailert Malai , Laosombat Vichai
Published: February 2007

We report on a 14‐year‐old boy with acute lymphoblastic leukemia (lymphomaleukemia) who had two episodes of acute tumor lysis syndrome during induction of remission with oral prednisolone alone and oral prednisolone, intravenous vincristine, and doxorubicin, respectively. Subsequently he had severe hyperphosphatemia (29.3 and 14.1 mg/dl; 9.46 and 4.55 mmol/L), hypocalcemia, hyperuricemia, hyperkalemia, and azotemia. Multiple stones and tumor cells infiltration were demonstrated in both kidneys. He responded favorably to hemodialysis. © 1995 Wiley‐Liss, Inc.
In situ breast carcinoma after treatment during adolescence for thyroid cancer with radioiodine
Authors: Green Daniel M. , Edge Stephen B. , Penetrante Remedios B. , Bakshi Suraj , Shedd Donald , Zevon Michael A.
Published: February 2007

We reviewed the courses of patients treated during childhood or adolescence for thyroid cancer to estimate the frequency of, and to identify possible risk factors for, the occurrence of second malignant tumors in this population.

We identified all patients treated for thyroid cancer in a cohort of 1,406 pediatric cancer patients who were diagnosed prior to 20 years of age during the period January 1, 1960 through December 31, 1988 and who were treated at Roswell Park Cancer Institute.

Twelve patients were treated for thyroid cancer, of whom nine were women. In situ breast carcinoma was diagnosed 25 and 26 years after diagnosis of thyroid cancer in two of four women treated with radioiodine. No new cancers were diagnosed in the five women treated with thyroidectomy only.

Two of four women treated for thyroid cancer during adolescence with radioiodine, which is concentrated in the breast as well as other organs, developed in situ breast carcinoma. Review of a large cohort of adolescent female thyroid cancer patients treated with radioiodine is necessary to provide an accurate estimate of their risk of developing breast cancer. These patients must remain under medical surveillance throughout their lifetimes to facilitate prompt diagnosis of and early intervention for new conditions, such as the occurrence of breast cancer. © 1995 Wiley‐Liss, Inc.
Recommendations of the brain tumor subcommittee for the reporting of trials
Authors: Gnekow A. K.
Published: February 2007

Since its constitution during the 22nd annual meeting of the International Society of Pediatric Oncology in Rome in 1990, the Brain Tumor Subcommittee has worked to arrive at a consensus for reporting criteria that should be adopted when brain tumor trials are presented. This consensus is presented here concerning minimum requirements for diagnostic procedures and a systematic approach to define the extent of surgically achieved resection by a radiodiagnostic classification aided by the surgical report as well as response and remission criteria. © 1995 Wiley‐Liss, Inc.
Screening for wilms' tumour in patients with aniridia, Beckwith syndrome, or hemihypertrophy
Authors: Craft Alan W. , Parker Louise , Stiller Charles , Msc Michael Cole
Published: February 2007

The role of screening for early detection of Wilms' tumour (WT) in patients with aniridia (A), Beckwith‐Weidemann syndrome (BWS) and hemihypertrophy (HH) has been explored. Of the 1,622 Wilms' tumour patients registered with the National Childhood Cancer Registry from 1971 to 1991, 41 were recorded as having A, BWS or HH. Twenty‐eight of these had their anomaly diagnosed before the WT and 13 had screening procedures carried out, mainly abdominal ultrasound. In 8 patients the screening procedure detected the WT. There was no significant difference in stage distribution or outcome for any of the three subgroups who were not screened, screen‐positive or screen‐negative. We conclude that regular screening with abdominal ultrasound is not of proven value. Parents should be taught abdominal palpation and advised to seek appropriate advice for untoward symptoms. © 1995 Wi1ey‐Liss Inc.
Vincristine disposition in children with acute lymphoblastic leukemia
Authors: De Graaf Siebold S. N. , Bloemhof Henk , Vendrig Desiree E. M. M. , Uges Donald R. A.
Published: February 2007

Vincristine (VCR) has been widely used to treat childhood malignancies for over thirty years, but its plasma disposition has not yet been well‐defined. Therefore, we conducted a pharmacokinetic study of VCR in 17 children with acute lymphoblastic leukemia (ALL) receiving the first dose of VCR. A new high‐performance liquid chromatographic assay was used for the measurement of VCR in plasma. A two‐compartment pharmacokinetic model was fit to the data by nonlinear least‐squares regression. Estimated pharmacokinetic parameters were highly variable; mean (S.D.) volume of distribution at steady‐state was 360 (176) L.m−2; total body clearance was 431 (238) ml. min−1.m−2, and elimination half‐life was 823 (390) min. These results were compared to data from eight adults with lung cancer. Mean volume of distribution in adults and children were similar, but VCR clearance was significantly larger in children (P = 0.01), resulting in a significantly longer elimination half‐life in the adults (P < 0.01).

We conclude that administration of a standard dosage of VCR to children with ALL results in a highly variable systemic drug exposure, which may have implications for the oncolytic effect and/or toxicity in individual patients. Comparison of data from children and adults suggests that VCR elimination rate is a function of age; this could account for more severe neurotoxicity in older patients. However, it cannot be excluded that differences between the children and adults may be due to other variables than age. Future studies should focus on the possible influence of multidrug resistance modulating agents on VCR pharmacokinetics and on pharmacokinetic‐pharmacodynamic relationships in individual patients. © 1995 Wi1ey‐Liss Inc.
Dose escalation trial of cyclophosphamide with sargramostim in the treatment of central nervous system (CNS) neoplasms
Authors: Lachance Daniel H. , Oette Dagmar , Schold S. Clifford , Brown Mark , Kurtzberg Joanne , Graham Michael L. , Tien Robert , Felsberg Gary , Colvin O. Michael , Moghrabi Albert , Browning Iley , Hockenberger Beverly , Stewart Elizabeth , Ferrell Lee , Kerby Tracy , Duncan‐Brown Margaret , Golembe Barry , Fuchs Herb , Fredericks Ruth , Hayes Frances Ann , Rubin Abbe Sue , Bigner Darell D. , Friedman Henry S.
Published: February 2007

We conducted a dose escalation trial of cyclophosphamide plus Sargramostim in the therapy of patients with newly diagnosed or recurrent central nervous system tumors. Cyclophosphamide was administered at doses ranging between 1.0 and 2.5 g/m2 daily for two doses. Sargramostim was administered at a fixed dose of 250 μg/m2 subcutaneously twice a day beginning 24 hours after the second cyclophosphamide dose and continuing through the leukocyte nadir until the absolute neutrophil count (ANC) was >1,000 cells/μl for two consecutive days. The MTD for patients who had not received any prior chemotherapy and who had received either no radiotherapy or radiotherapy confined to the cranium was 2.0 g/m2 daily for two doses. The MTD for patients previously treated with chemotherapy or neuraxis radiotherapy was also 2.0 g/m2 daily for two doses. Responses were seen in patients with medulloblastoma (8/9), glioblastoma multiforme (2/13), germinoma (1/1), and pineoblastoma (1/2). © 1995 Wi1ey‐Liss Inc.
Bone Marrow Metastasis of Neuroblastoma Analyzed by MRI and Its Influence on Prognosis
Authors: Tanabe Masahiro , Ohnuma Naomi , Iwai Jun , Yoshida Hideo , Takahashi Hideyo , Maie Masahiko , Etoh Takao , Kawamura Kenji
Published: February 2007

Bone marrow metastases (BM mets) of neuroblastoma were analyzed by magnetic resonance imaging (MRI). BM mets were demonstrated in 45 femurs of 23 patients with neuroblastoma by MRI. They were classified into two patterns, nodular (N) type and diffuse (D) type, on MRI, with an incidence of 16% (7/45) and 84% (38/45), respectively. No bone metastases (B mets) were accompanied by N type BM mets, whereas all of them were accompanied by D type BM mets.

The rate of disappearance of N type BM mets after chemotherapy was 100% (7/7) and was significantly higher than that (26%, 10/38) of D type BM mets. The rate of disappearance (58%, 11/19) of BM mets in bones without associated B mets was also significantly higher than that (23%, 6/26) of BM mets in bones with associated B mets. BM mets present in bones with B mets tended to persist after chemotherapy. The survival rate of patients with residual BM mets after chemotherapy was significantly lower than that of patients without residual BM mets. This suggests that powerful systematic chemotherapy and additional targeted therapy to lesions remaining after chemotherapy are needed to improve the cure rate of patients with advanced neuroblastoma. © 1995 Wi1ey‐Liss, Inc.
Developmental Defects of Teeth in Survivors of Childhood ALL Are Related to the Therapy and Age at Diagnosis
Authors: Pajari Ulla , Lanning Marjatta
Published: February 2007

The relation of the therapy used and age at diagnosis to developmental defects of dental enamel and root was analyzed in 45 survivors of childhood acute lymphoblastic leukemia (ALL) and compared to that of age‐and sex‐matched healthy controls. Chemotherapy alone increased the number of enamel defects, but it did not affect the roots. The scattered irradiation of 0.72–1.44 Gy to the dental arches during central nervous system (CNS) irradiation caused an increased number of developmental defects both in enamel and root. Age at diagnosis is an important factor and it showed that the irradiation of 10 Gy did not cause enamel defects if amelogenesis was complete. In conclusion, the therapy is related to developmental defects of the teeth but the most important factor is age, both at diagnosis and during the therapy. © 1995 Wi1ey‐Liss, Inc.