bluuebunny/crossref_metadata_2025 huggingface dataset:
Efficacy of postoperative chemotherapy using cisplatin plus etoposide in young children with brain tumors
Authors: Strauss Lewis C. , Killmond Thomas M. , Carson Benjamin S. , Maria Bernard L. , Wharam Moody D. , Leventhal Brigid G.
Published: February 2007

Neuraxis radiation therapy (RT) for primary intracranial tumors is associated with major late effects if administered to very young children. To control residual tumor and to delay RT, we treated eight young children (median age 6.5 months) with primary central nervous system (CNS) tumors using combination chemotherapy: cisplatin, 20 mg/M2/day plus VP‐16, 75 mg/M2/day i.v. for 5 days, given q. 3–6 weeks for 8 cycles. The tumors were medulloblastoma (one), malignant ependymoma (two), primitive neuroectodermal tumor PNET (two), malignant glioma (two), astrocytoma (one). Six had measureable disease; three had positive cerebrospinal fluid (CSF) cytopathology. All patients with measureable tumor had initial objective responses (three) complete response [CR], one partial response [PR], two minor response [MR], including cytopathology (three CR of three) and metastatic deposits (two CR of two). One patient relapsed during chemotherapy. Median time to disease progression was 17.5 months; median survival was 34 months. Three patients, none of whom received RT, have prolonged progression‐free intervals of 47–67 months to date. Neurodevelopmental progress continued during and after chemotherapy. Chemotherapy toxicity was mild. Median neutrophil nadir was 312/mm3, platelets 72,000. Fever during neutropenia occurred in six of 61 courses. Moderate high‐frequency auditory losses were detected in three patients, and mild renal injury (GFR <70 ml/min) was detected in two of seven evaluable children. This pilot study demonstrates the apparent efficacy and mild toxicity of 5 day courses of cisplatin plus VP‐16, with delayed RT, in young children with CNS neoplasms. A POG treatment protocol that incorporates cisplatin plus VP‐16 is evaluating primary chemotherapy with delayed radiotherapy in larger numbers of pediatric brain tumor patients.
Myelofibrosis in a child suffering from a hypereosinophilic syndrome with trisomy 8: Response to corticotherapy
Authors: Michel G. , Thuret I. , Capodano A. M. , Scheiner C. , Guitard A. M. , Mozziconacci M. J. , Fossat C. , Perrimond H.
Published: February 2007

The idiopathic hypereosinophilic syndrome (IHS) is extremely rare in childhood and relationships of this syndrome with my‐eloproliferative diseases are controversial. We reported the observation of a 7‐year‐old girl suffering from an IHS with myelofibrosis. A clonal cytogenetic abnormality, trisomy 8, was detected in the bone marrow cells of this child. This is the decisive proof of a myeloproliferative disorder. IHS with myelofibrosis is usually considered as unresponsive to corticotherapy. In our case, corticotherapy resulted in a rapid, complete, and lasting disappearance of myelofibrosis. Complete remission of the disease, however, was not achieved and the trisomy 8 persisted after treatment.
Early infectious complications after peripheral blood stem cell autografts in children
Authors: Watanabe Tsutomu , Takaue Yoichi , Abe Takanori , Matsunaga Keiko , Saito Shin‐Ichi , Hirao Atsushi , Kawano Yoshifumi , Hirose Masao , Ninomiya Tsuneo , Yokobayashi Ayako , Kuroda Yasuhiro
Published: February 2007

Seventeen children underwent marrowablative high‐dose chemotherapy with peripheral blood stem cell autografts and were studied retrospectively to determine the type, frequency, and outcomes associated with infectious complications 3 months postgraft. The patients were kept in isolated rooms with a laminar air flow facility, but no decontamination procedures, such as gut sterilization with nonabsorbable antibiotics, nonmicrobial diet, and skin cleansing, were used. They were under their mothers' daily care to maintain good psychological conditions. After the completion of marrow‐ablative chemotherapy and the infusion of stem cells, the absolute granulocyte count exceeded 0.5 × 109/liter with a mean of 17.9 days (range 6–65 days), Fifteen patients developed a total of 16 febrile episodes during the first 4 week period, and the confirmed diagnoses were mucositis (12), enterocolitis (nine), septicemia (four), central venous catheter‐associated infection (three), pneumonia (one), perianal abscess (one), and possible invasive fungal infection (one). All episodes were successfully treated with parenteral antibiotic therapy, and no patient died of infectious complications. The observations suggest that high‐dose chemotherapy can be performed safely with simple and efficient patient management protocol followed by peripheral blood stem cell autografts.
Hepatocellular carcinoma following chronic delta virus hepatitis in a patient cured of leukemia
Authors: Rossetti Flavio , Zancan Lucia , Perilongo Giorgio , Zanesco Luigi
Published: February 2007

A 20‐year‐old patient with chronic delta virus hepatitis (CDVH), cured of acute lymphoblastic leukemia (ALL), developed hepatocellular carcinoma (HCC) 14 years after hepatitis B virus (HBV) infection. The association between chronic HBV infection and HCC is well known, but CDVH patients affected by HCC are rarely reported in literature. To our knowledge, the case we describe is the first HCC case reported in literature occuring in a young boy with CDVH. We could expect further similar cases, considering 1) the high prevalence of HDV infection in children affected by ALL in our series, 2) the previous ALL treatment, and 3) a possible natural predisposition to cancer.
Malignant fibrous histiocytoma in the abdominal soft tissues of a child: A case report
Authors: Earl Ursula , Lockwood Liane R. , Batcup Gillian
Published: February 2007

An 18‐month‐old female presented with an abdominal tumor mass which on morphological, immunohistological and ultra‐structural examination was found to be a malignant fibrous histiocytoma. This soft tissue sarcoma is rarely encountered in childhood. Treatment in this case consisted of surgical tumor debulking with pre‐ and post‐operative chemotherapy. The child is well and free of detectable tumor at 23 months after diagnosis.
Clinical trials in cancer therapy: Efforts to improve patient enrollment by community oncologists
Authors: Fisher William B. , Cohen Stuart J. , Hammond Monica K. , Turner Sandra , Loehrer Patrick J.
Published: February 2007

A prerequisite for the completion of a clinical trial is the accrual of adequate numbers of patients. It is estimated that over 90–95% of all cancer patients are now treated in their local communities by practicing oncologists and are not seen by primary investigators at teaching hospitals. One risk of this trend is decreased patient enrollment into clinical trials. The private practitioner often lacks the academic oncologist's incentives to participate in clinical research. The Hoosier Oncology Group (HOG) is composed of community medical and radiation oncologists (85%) and Indiana University faculty members (15%). In an effort to improve patient enrollment onto clinical cancer research trials, HOG has attempted to identify 1) the major obstacles to patient enrollment by community oncologists and 2) potential aids to overcome such obstacles. One hundred fourteen members were surveyed and 75 responded (66%). The major obstacles were, in descending order: time demands on both the oncologist and his staff; explaining clinical trials to patients; completing flow sheets; perceived increased cost to the patient; remembering protocols; adhering to a rigid protocol. Aids identified as potentially most helpful were, in descending order: pocket‐sized synopses of protocols; computer generated, individualized prompts; the availability of a clinical trials specialist to explain the clinical trial to patients and assist in obtaining informed consent; the availability of a video tape which could be used to explain clinical trials to patients. These aids are being implemented with the attempt to systemically study their impact on patient accrual.
Acute fatal myeloencephalopathy after combined intrathecal chemotherapy in a child with acute lymphoblastic leukemia
Authors: von der Weid Nicholas X. , de Crousaz Henriette , Beck Daniel , Deonna Thierry , Miklossy J. , Janzer R. C.
Published: February 2007

We report a case of a fatal toxic encephalomyelopathy in a 12‐year‐old girl due to prophylactic intrathecal injection of methotrexate and cytosine arabinoside, with a characteristic progressive symptomatology leading to death after 28 days. The location and type of neuropathological changes support the hypothesis of a direct toxic effect of methotrexate and/or cytosine arabinoside on structures directly exposed to the cerebrospinal fluid.
Primary subconjunctival lymphoma: An unusual presentation of childhood non‐Hodgkin's lymphoma
Authors: Karadeniz Ceyda , Bilgiç Sevgül , Ruacan Sevket , Sarialioǧlu Faik , Büyükpamukçu Münevver , Akyüz Canan , Doǧan Ahmet
Published: February 2007

Of 806 non‐Hodgkin's lymphoma cases, only one primary subconjunctival lymphoma case was observed in Hacettepe Children's Hospital over a period of 16 years. The patient was a 12 year‐old‐boy with a subconjunctival mass on the upper part of his right eye. Biopsy revealed malignant lymphoma. Extensive investigations disclosed no other site of tumor. He was given the modified LSA2‐L2 protocol. He has been in remission for 22 months. This may be the first report of a primary subconjunctival lymphoma in the childhood period.
Association of delivered drug dose and outcome for children with acute lymphoblastic leukemia and unfavorable presenting features
Authors: Gaynon Paul S. , Steinherz Peter G. , Bleyer W. Archie , Finklestein Jerry Z. , Miller Denis R. , Reaman Gregory H. , Sather Harland N. , Hammond G. Denman
Published: February 2007

Dose and dose intensity are believed critical for attaining a maximal therapeutic effect in drug‐responsive tumor systems. Childhood acute lymphoblastic leukemia may be an example of such a drug‐responsive system as it is cured with current chemotherapy in a majority of cases. Between August 1981 and May 1983, the Childrens Cancer Study Group enrolled 209 children with ALL and unfavorable presenting features on CCG‐193P, a trial based on the Berlin Frankfurt Munster 76/79 regimen. The cumulative delivered dose of each medication was recorded prospectively. Patients who completed the intensive portion of therapy in continuous complete remission were ranked by the percentage of protocol required drug delivered from the initiation of therapy to that date. No association was found between delivery of any single drug and subsequent disease‐free survival. However, when patients were ranked by the sum of the percentages of protocol vincristine, l‐asparaginase, and anthracycline delivered, children in the approximate middle and lower tertiles were 3 and 5 times more likely to have had a subsequent relapse than were those in the upper tertile (P =0.025, test for trend). Delivery of the full protocol prescribed dose of these agents may have been critical, but corroboration is certainly needed. Only prospective trials can determine if children with acute lymphoblastic leukemia and unfavorable presenting features might benefit from greater use of vincristine, l‐asparaginase, and/or anthracycline.
Analysis of N‐ras gene mutations in medulloblastomas by polymerase chain reaction and oligonucleotide probes in formalin‐fixed, paraffin‐embedded tissues
Authors: Iolascon Achille , Lania Arturo , Badiali Manuela , Pession Annalisa , Saglio Giuseppe , Giangaspero Felice , del Giudice Emanuele Miraglia , Perrotta Silverio , Cutillo Stefano
Published: February 2007

Precise data on the incidence of transforming ras oncogenes in pediatric tumors and the correlations with the histopathological properties of the tumors are very limited. Additionally the presence of ras activation in medulloblastomas has not been investigated so far. Using a combination of techniques including in vitro gene amplification by polymerase chain reaction (PCR) and detection of single base mutations by sequence‐specific oligonucleotides we studied N‐ras activation (mutations at codon 12, 13, and 61) in 32 medulloblastomas. DNA was isolated from 20 μm sections of formalin‐fixed paraffin‐embedded tissue. Mutations were found in 3 out of 32 examined medulloblastomas. In all cases only mutations of codon 61 were found: two of three mutations were C to A mutations at position 1 of the codon 61 (leading to a substitution of a glutamine residue for a lysine) and one was A to T mutation at position 3 in the same codon (glutamine‐histidine). Our results indicate 10% incidence N‐ras mutation in medulloblastoma, higher than in other CNS tumors studied so far. The main advantages of the procedure described are its greatly improved sensitivity, the increased speed with which tumor samples can be analyzed, and the possibility of using paraffin‐embedded sections to analyze various rare tumors in retrospect.
EBV genome and immunoglobulin gene rearrangement in the differential diagnosis of nasopharyngeal carcinoma and lymphoma
Authors: Vonsover Ami , Rechavi Gideon , Kesler Alexander , Rapaport Yoram , Brok‐Simoni Frida , Mileguir Fernando , Ramot Bracha , Gotlieb‐Stematsky Tamar
Published: February 2007

Head and neck tumors include nasopharyngeal carcinoma (NPC) and lymphoma. The differential diagnosis of these tumors is based on histology, immunocytochemical staining, and EBV serology. In rare cases it might be difficult to distinguish between NPC and lymphoma in HE section or biopsies. DNA hybridization with cloned EBV and human Immunoglobulin gene fragments allows the detection of EBV‐related sequences and immunoglobulin gene rearrangements. The presence of EBV genome supports the diagnosis of NPC or EBV related BL, while rearrangement of immunoglobulin genes points to B‐cell lymphoma.

The diagnosis in 11 patients suspected of head and neck tumors was carried out by hybridization of DNA extracted from the tumors and assayed with cloned EBV and IgHCJ DNA probes.

One patient proved to have EBV‐associated BL based on positive hybridization with EBV probes and immunoglobulin rearrangement, presenting a unique hybridization with cloned EBV DNA BamHI W fragment, with bands of 3.2 and 3.9 kb. BL was confirmed in this patient by demonstration of c‐myc rearrangement. A second patient was negative in hybridization with EBV, and positive for immunoglobulin rearrangement, and therefore was diagnosed as having B‐cell lymphoma. In seven patients NPC was confirmed by hybridization with EBV‐DNA probes. In two patients, both NPC and B‐cell lymphomas were excluded.
A longitudinal study of growth and growth hormone secretion in children during treatment for acute lymphoblastic leukemia
Authors: Màrky Idikó , Mellander Lotta , Lannering Birgitta , Albertsson‐Wikland Kerstin
Published: February 2007

Diminished growth rate during treatment for acute lymphoblastic leukemia (ALL) is of the multifactorial etiology. Effects on GH secretion have been shown after discontinuation of treatment including prophylactic CNS irradiation. Seventeen children treated for ALL with three different CNS preventive schedules were followed longitudinally with repeated estimations of the spontaneous GH secretion during a 24‐month period. No difference was found in GH secretion during this time between patients who had received no radiotherapy and those who had received 18 or 24 Gy as CNS prophylaxis. During dexamethasone treatment the GH secretion was completely suppressed, which can be a mediator for the diminished growth rate during the first 2 years of ALL treatment. We conclude that there is no clinical reason to perform GH analysis within the first 24 months of treatment for ALL.
A phase I trial of α‐interferon in combination with pentostatin in hematologic malignancies
Authors: Bernard Stephen , Gill Parkash , Rosen Peter , Gavigan Molly , Steagall Ann , Ellingham Elizabeth , Morgan Tim , Janic Gracie , Ozer Howard
Published: February 2007

Pentostatin, a novel inhibitor of adenosine deaminase, has shown activity in various lymphoid malignancies of both the T and B cell lineage. This agent has unique side effects and in general myelosuppression has been mild. Interferon has both antiviral and antineoplastic properties. This agent has shown activity in hairy cell leukemia, chronic granulocytic leukemia, low grade lymphoma, and myeloma. Side effects from interferon are in general dissimilar to those that have been seen with pentostatin and in particular myelosuppression has not been a major toxicity with low doses of interferon. This current trial explored the combination of pentostatin and interferon in hematologic malignancies. Fifteen patients were enrolled in this phase I trial at a fixed dose of pentostatin of 4 mg/m2 biweekly and interferon at doses of 0.5, 1, 2, or 4 million units/m2 of interferon. At the first three dose levels of interferon nausea and vomiting were the predominant toxicity and appeared to worsen with time on study. Fatigue also was seen at the lowest level of interferon and was severe enough to cause two individuals to discontinue the study medications. At higher dose levels of interferon, myelosuppression, nausea and vomiting, and fatigue were the predominant toxicities. One patient with hairy cell leukemia had a complete response and a second patient with T cell cutaneous lymphoma had a partial response which lasted for 6 to 7 weeks. The maximum tolerated dose of interferon with pentostatin in this patient population was four million units/m2.
Etoposide in the treatment of six children with langerhans cell histiocytosis (histiocytosis X)
Authors: Viana Marcos Borato , Oliveira Benigna Maria , Silva Celia Maria , Leite Virginia Hora Rios
Published: February 2007

Six children received etoposide as the single agent for treatment of Langerhans cell histiocytosis (LCH; histiocytosis X). Five were less than 2 years old at diagnosis. All had multiorgan involvement; one had liver and pulmonary dysfunction. Two infants also had clinical signs of immune deficiency. Complete response was observed in five. There was no major toxicity. Although three of four evaluable patients relapsed, the drug was considered useful in moving the children from a symptomatic to an asymptomatic clinical status. Etoposide may become a “first‐line” drug in the treatment of systemic LCH, especially when the side effects of steroid therapy are considered unacceptable.
Neuroblastoma stage IV‐S: A heterogeneous disease
Authors: Wilson Peter C. G. , Coppes Max J. , Solh Hassan , Chan Helen S. L. , Jenkin Derek , Greenberg Mark L. , Weitzman Sheila
Published: February 2007

Eighteen patients were diagnosed and treated for Stage IV‐S neuroblastoma at The Hospital for Sick Children, Toronto between January 1971 and December 1988. All patients were 6 months of age or younger at diagnosis. Nine patients (50%) have remained disease free with a mean follow‐up of 9.3 years. Of the seven patients under 6 weeks of age at presentation, four presented in the early neonatal period and died, three due to mechanical complications related to progressive disease, and one due to late recurrence. The remaining three patients under 6 weeks of age, two of whom had skin involvement at diagnosis, are alive and disease free. Six of the 11 patients over 6 weeks of age at presentation survived, combined modality therapy (CMT) being more effective than single modality treatment. N‐myc was studied from tumor tissue at diagnosis in four patients and was amplified in three (25x, 25x, 100x), all of whom had late disease progression and died. The patient with a single gene copy has no evidence of disease 24 months following diagnosis. Our study confirms the heterogeneity described in this clinically defined group of patients. Because of it, management of Stage IV‐S neuroblastoma cannot be uniform and until further development of a subclassification, or a reclassification based on molecular biologic markers is developed, pediatric oncologists will regularly be confronted with a decision whether or not to treat a newly presenting patient that fits into the clinical classification IV‐S.
Intellectual function in long‐term survivors of childhood acute lymphoblastic leukemia: Protective effect of pre‐irradiation methotrexate a childrens cancer study group study
Authors: Balsom William R. , Bleyer W. Archie , Robison Leslie L. , Heyn Ruth M. , Meadows Anna T. , Sitarz Anneliese , Blatt Julie , Sather Harland N. , Hammond G. Denman
Published: February 2007

Having demonstrated in a laboratory model that the neurotoxicity of CNS irradiation can be ameliorated with pre‐irradiation methotrexate, we retrospectively compared two methods of CNS prophylaxis in childhood acute lymphoblastic leukemia which differed only in the timing of intrathecal methotrexate and radiotherapy. The results of standard IQ tests conducted 2–11 years after 24 Gy of cranial radiotherapy were obtained in 72 patients, of whom 27 had pre‐irradiation methotrexate and 45 did not (control group). The two groups were otherwise comparable. In girls, the full‐, performance‐, and verbal‐scale IQ scores were consistently higher in the pre‐irradiation methotrexate group than in the corresponding control group (P<0.025). Among girls <5 years of age when irradiated, the mean IQ scores were 25–29 points higher after pre‐irradiation methotrexate than after the control treatment (P<0.0007). These results suggest that pre‐irradiation methotrexate may help prevent CNS radiotoxicity in children, and that the benefit is dependent on patient age and gender.
Malignant thymoma associated with peripheral T‐cell lymphocytosis
Authors: Doll Donald C. , Landreneau Rodney J. , List Alan F.
Published: February 2007

Three cases of peripheral T‐cell lympho‐cytosis associated with thymoma are described. Each patient had a malignant, predominantly lymphocytic thymoma with resolution of lymphocytosis upon treatment of the epithelial neoplasm. These findings suggest that lymphocytosis complicating thymoma is a paraneoplastic phenomenon and is not part of the neoplastic process.
Complications associated with indwelling catheters
Authors: Simmons Jeané R. , Buzdar Aman U. , Ota David M. , Marts Kathy , Hortobagyi Gabriel N.
Published: February 2007

Between 1983 and 1985, 170 consecutive patients received doxorubicin‐containing adjuvant chemotherapy through central venous catheters, and four via a long indwelling catheter in the antecubital fossa. The objective of this retrospective study is to determine the acute and chronic complications associated with indwelling catheters. Ninety‐four (56%) patients did not experience any complications. The incidence of acute complications was 2%, which included three pneumothorax. Twenty‐nine (17%) patients experienced chronic complications. Of those, 20 (12%) developed infectious complications, and 9 (19%) developed thrombus. In addition, 12 (7%) developed multiple complications, and 32 (19%) patients had other local complications at the catheter site, which included allergic reactions and catheter breakage. Each of these complications was resolved with appropriate treatment. None of the patients died from complications of the indwelling catheter.
Bone marrow necrosis and thrombotic complications in childhood acute lymphoblastic leukemia
Authors: Eguiguren José M. , Pui Ching‐Hon
Published: February 2007

Two children with bone marrow necrosis at diagnosis or at relapse of acute lymphoblastic leukemia (ALL) had thrombotic complications 15 and 17 days after starting remission induction therapy including prednisone, vincristine, and L‐asparaginase. The close temporal relationship of these two relatively rare events suggests that bone marrow necrosis is a predisposing factor to the development of thrombosis.
Isolated central nervous system relapse of testicular cancer
Authors: Perry James J. , Jelinek James S.
Published: February 2007

Central nervous system involvement with testicular cancer usually occurs with advanced systemic disease. Isolated CNS disease at relapse is rare. We report a patient who developed a solitary brain metastasis with no other systemic disease after having achieved a complete response to frontline therapy. After combined modality therapy for the CNS disease, the patient has remained disease‐free for more than 3 years. The literature regarding brain metastases in relapsed testicular cancer is reviewed, including nine cases of isolated brain metastases. The CNS can be a “sanctuary” site for testicular cancer, and in the unusual subset of patients with isolated brain relapse, long‐term remission is possible with aggressive therapy.
Secondary acute non‐lymphoblastic leukemia in two children following treatment with a cis‐diamminedichloroplatinum‐II‐based regimen for osteosarcoma
Authors: Jeha Sima , Jaffe Norman , Robertson Resa
Published: February 2007

Acute nonlymphocytic leukemia (ANLL) developed in 2 of 142 pediatric patients with osteosarcoma treated with a cis‐diamminedichloroplatinum‐II (CDP)‐based regimen: acute monomyelogenous leukemia (M4) with a normal female karyotype in one and acute myelogenous leukemia (M2) with t (8,21) in the other. The ANLLs occurred, respectively, in each patient 3 and 4 years after the initial diagnosis of osteosarcoma. In contrast to most of the adult experience and consistent with the majority of reported ANLL in children, the disease was characterized by an absence of the smoldering phase and cytogenetic findings similar to those seen in de novo ANLL.
Determination of the maximum tolerated dose of idarubicin when used in a combination chemotherapy program of reinduction of childhood ALL at first marrow relapse and a preliminary assessment of toxicity compared to that of daunorubicin: A report from the childrens cancer study group
Authors: Feig Stephen A. , Krailo Mark D. , Harris Richard E. , Baum Edward , Holcenberg John S. , Kaizer Herbert , Steinherz Laurel , Pendergrass Thomas W. , Saunders E. Frederick , Warkentin Phyllis L. , Bleyer W. Archie , Hammond G. Denman
Published: February 2007

An escalating‐dose trial of idarubicin, used weekly for 3 doses in combination with vincristine, prednisone, and L‐asparaginase (VPLI), to reinduce remission of childhood ALL at first bone marrow relapse was conducted by the Childrens Cancer Study Group (CCSG). The maximum tolerated dose (MTD) of idarubicin, used in the manner, was determined to be 12.5 mg/m2/dose. Twelve of 16 (75%) evaluable patients in first marrow relapse of ALL treated at a dose of 10 or 12.5 mg/m2 entered a second complete remission, compared to 41 of 69 evaluable patients (59%) treated in a comparable way with daunorubicin (30 mg/m2) (VPLD). Prolonged myelosuppression was observed in both groups, but the frequency of documented bacterial sepsis and the duration of required hospitalization were greater among patients treated with idarubicin. No additional toxicity, specifically attributable to idarubicin, was observed at these doses.
Children with cancer: Special considerations in the discontinuation of life‐sustaining treatment
Authors: Freyer David R.
Published: February 2007

Every year in the United States, over 2,100 children die of progressive cancer, or of complications related to the disease or its treatment. Physicians, other clinicians, and parents caring for these children are often faced with decisions about the continuation or termination of life‐sustaining treatment (LST). In adults, a consensus has emerged which holds that LST may be ethically discontinued if the burdens of continued treatment outweigh its benefits for the patient. While this standard is also applicable to LST decisions in pediatric oncology, its appropriate use must address several medical and ethical issues characteristic of children with cancer. These special considerations, which are the subject of this discussion, include the extensive medical experience of children with cancer, the nature of modern oncology treatment, the unpredictable patterns of response to treatment, the parent and/or physician biases which may threaten the child's well‐being, the distinction between being incurably ill and imminently dying, the need for effective palliative care, and the variable levels of cognitive and emotional development which determine a child's capacity for participating in an LST decision. Consideration of these factors facilitates a consistent approach to these difficult decisions which is both compatible with current ethical guidelines and responsive to the particular needs of these patients.
Benign cystic mesothelioma of the peritoneum: The occurrence of an adult entity in a child
Authors: Hanukoglu Aaron , Gewurtz Gabriella , Zaidel Liliana , Krispin Mayer , Fried Daniel
Published: February 2007

Benign cystic mesothelioma of the peritoneum is considered a disease of adulthood occurring predominantly in women. We report a case of benign cystic mesothelioma of the peritoneum in an 11‐year‐old boy. The innumerable cystic lesions that varied in size occupied the entire peritoneal cavity. He underwent two laparotomies in a period of 15 months during which a partial removal of the tumor was performed. Four years after the second operation the child is well and active, despite significant tumor tissue left behind after the first two operations. The management of this disease is discussed, and the importance of a conservative approach minimizing the number of laparotomies and avoiding radical surgical intervention especially in childhood is emphasized.
Neuropsychological status of children treated for brain tumors: A critical review and integrative analysis
Authors: Mulhern Raymond K. , Hancock Janet , Fairclough Diane , Kun Larry
Published: February 2007

The literature on the neuropsychological status of children with primary brain tumors was reviewed to identify English‐language publications reporting the results of standardized, quantitative measures of patient function. The 22 studies that met these review criteria, representing 544 patients, were evaluated to assess the relationship between traditional risk factors (age at diagnosis, tumor location, radiation therapy, and time since completion of treatment), as well as subsequent intellectual development, academic achievement, psychosocial adjustment, and neuropsychological status. The impact of other potentially salient factors, such as seizures and sensory and motor deficits, was evaluated when possible. Despite inconsistent reporting of demographic and treatment‐related effects across studies which precluded formal meta‐analysis, we were able to confirm the primary importance of radiation therapy volume and age at treatment on IQ. No effects were detected for tumor location. Younger children treated with cranial (whole brain) irradiation showed a 14‐point deficit in IQ as compared with their older counterparts. No significant differences were noted between older children receiving lòcal or cranial irradiation, although both groups had IQ levels 12–14 points lower than those not irradiated. The high‐risk groups identified in this study require increased clinical surveillance. Definitive evaluation of potential risk factors for neuropsychological impairment will depend on more complete reporting of relevant patient characteristics and interinstitutional studies. © 1992 Wiley‐Liss, Inc.
Nasopharyngeal rhabdomyosarcoma and multiple lentigines syndrome: A case report
Authors: Heney David , Lockwood Liane , Allibone Eleanor B. , Bailey Clifford C.
Published: February 2007

An 8‐year‐old boy with multiple lentigines syndrome presented with a nasopharyngeal rhabdomyosarcoma. The tumour failed to respond to chemotherapy. This is a further association of rhabdomyosarcoma with a congenital syndrome. © 1992 Wiley‐Liss, Inc.
Genetics and epidemiology of Wilms' tumor: The French Wilms' tumor study
Authors: Bonaïti‐Pellié Catherine , Chompret Agnès , Tournade Marie‐France , Hochez Joelle , Moutou Céline , Zucker Jean‐Michel , Steschenko Dominique , Brunat‐Mentigny Maud , Roché Henri , Tron Philippe , Frappaz Didier , Munzer Martine , Bachelot Colette , Dusol Francois , Sommelet‐Olive Danielle , Lemerle Jean
Published: February 2007

A complete family history was obtained for 501 patients with Wilms' tumor, treated in departments of pediatric oncology in whole France. The information was collected by self‐questionnaire and/or by interview of parents. The proportion of bilateral cases is 4.6% and there are 12 patients (2.4%) with a positive family history of Wilms' tumor. The affected relatives are most often distant and no first degree relative was affected. Apart from the well‐known associations with aniridia, hemihypertrophy, genitourinary anomalies, Beckwith‐Wiedeemann, and Drash syndromes, there is also a significant excess of congenital heart defects (P = .008) which remains to be explained. Several findings support the bimutational hypothesis such as earlier diagnosis and increased parental age in bilateral cases. No particular anomalies and no increased frequency of childhood cancer were found in patients' relatives. The frequency of Wilms' tumor in relatives was estimated to be less than 0.4% in sibs, 0.06% in unclesand aunts, and 0.04% in first cousins. These figures are very different from those found in retinoblastoma and suggest that the mechanism may be more complex in Wilms' tumor. This conclusion is in agreement with molecular biology studies in tumors and linkage analysis in multiple case families which suggest that more than one locus is involved. © 1992 Wiley‐Liss, Inc.
N‐myc Gene Amplification in Neuroblastoma: A Clinical Approach Using Ultrasound Guided Cutting Needle Biopsies Collected at Diagnosis
Authors: Hedborg Fredrik , Lindgren Per G. , Johansson Irja , Kogner Per , Samuelsson Bengt‐Olof , Bekassy Albert N. , Olsen Leif , Kreuger Anders , Påhlman Sven
Published: February 2007

N‐myc gene amplification was studied in a consecutive series of neuroblastomas and ganglioneuromas, representing all of such tumours diagnosed in Sweden over a 4‐year period. Both frozen and formalin fixed specimens were used for Southern blot analysis. Thirty‐seven of 46 neuroblastomas and 7 of 9 ganglioneuromas were analyzed. Seven neuroblastomas and none of the ganglioneuromas showed N‐myc gene amplification. All children with amplified tumours, including three infants, had advanced disease at diagnosis and aggressive course of disease. However, follow‐up time was short for the two cases still alive. The use of an ultrasound guided cutting needle biopsy technique for obtaining the required tissue at diagnosis was evaluated in some cases. This technique appeared to be safe and clinically useful since early prognostic information was obtained. Using an imprint from the needle biopsy, the representativity could be confirmed. Ultrasound guided cutting needle biopsies can thus be used routinely to obtain N‐myc gene amplification data prior to initiation of therapy in neuro‐blastoma. © 1992 Wiley‐Liss, Inc.
Causes of death in a paediatric oncology unit
Authors: Al‐Asiri R. H. , Mott Martin G. , Oakhill A.
Published: February 2007

The records of 101 patients (64 males and 37 females) registered at Bristol Children's Hospital who died between January 1986 and December 1989 were reviewed to determine the cause of death. Nineteen patients (19%) died without obtaining remission and 6 (6%) in first remission. Seventy‐six (75%) died after relapse; three during re‐incluction and two in second remission. The causes of death were active disease in 85 patients (84%), active disease and infection (4%), active disease and other factors (4%), infection only (3%), toxic cardiomyopathy (2%), graft versus host disease (2%), and second malignancy (1%). © 1992 Wiley‐Liss, Inc.
Phase II trial of ifosfamide/mesna and mitoxantrone in previously treated patients with non‐Hodgkin's lymphoma: Cancer and leukemia group B study 8753
Authors: Case Delvyn C. , Santarelli Maria Teresa , Carey Robert W. , Anderson James , Gottlieb Arlan
Published: February 2007

Ifosfamide (2.0 g/m2 intravenously/day × 3) with mesna (400 mg/m2 intravenously q 4 h daily × 3) was combined with mitoxantrone (14 mg/m2 intravenously × 1) and given on a 3 week schedule to patients with previously treated non‐Hodkgin's lymphoma. In 45 eligible/evaluable patients, a 47% response rate (95% confidence interval: 32%, 62%) was achieved of which 25% were complete responses and 22% were partial responses. Median duration of remission was 10 months with 42% of patients in remission at 18 months. Hematologic toxicity (granulocytopenia) was the dose‐limiting toxicity with severe or life‐threatening granulocytopenia in 98% of patients at full protocol doses. Nausea/vomiting was seen in 73% of patients but was usually mild/moderate. Three patients had significant hematuria secondary to ifosfamide. Two patients had severe changes in the left ventricular ejection fraction (LVEF) secondary to mitoxantrone; and one patient had acute neurologic dysfunction secondary to ifosfamide.

Ifosfamide/mesna can be safely combined with mitoxantrone at full doses in previously treated patients with non‐Hodkgin's lymphoma. This combination should be considered for other trials in lymphoma. © 1992 Wiley‐Liss, Inc.
Neuroendocrine carcinoma in a patient with hairy cell leukemia: A case report
Authors: Ballen Karen K. , Canoso Rosa , Seiler Marcel , Neish Andrew , Bauer Kenneth A.
Published: February 2007

Second malignancies are common in hairy cell leukemia. We report a case of a neuroendocrine carcinoma arising in a patientwho had been diagnosed with hairy cell leukemia 6 years earlier. This case is the first report of these two tumors' occurring together. The pathogenetic basis for the presence of these two uncommon tumors in our patient is discussed. © 1992 Wiley‐Liss, Inc.
Cutaneous angiosarcoma as a second malignant neoplasm after peripheral primitive neuroectodermal tumor
Authors: Coffin Cheryl M. , Vietti Teresa J. , Land Vita J. , Kraybill William G. , Dehner Louis P.
Published: February 2007

Second malignant neoplasms (SMN) in late childhood or young adulthood in individuals who have been successfully treated for an initial malignancy have emerged as a late effect of therapy in survivors of childhood cancer. Although radiation therapy is frequently implicated, chemotherapy with alkylating agents and antimetabolites has also been associated with SMN. Soft tissue sarcomas are among the most frequent primary malignancies complicated by a SMN and account for a majority of nonhematolymphoid SMN. We present the clinical and pathologic findings in a patient who had a peripheral neuroepithelioma (primitive neuroectodermal tumor, PNET) of the soft tissues diagnosed at 17 years of age, was treated with high‐dose irradiation and multidrug chemotherapy, and developed an angiosarcoma 14 years later. This case represents an uncommon combination of mesenchymal malignancies in a young patient with an unusually favorable clinical course following the diagnosis of PNET. © 1992 Wiley‐Liss, Inc.
Recombinant human granulocyte‐macrophage colony‐stimulating factor in children with chemotherapy‐induced neutropenia
Authors: Saarinen Ulla M. , Hovi Liisa , Riikonen Pekka , Pihkala Jaana , Juvonen Eeva
Published: February 2007

Twenty children 1–17 (median, 5.5) years of age received GM‐CSF during chemotherapy‐induced neutropenia at the dose of 5 μg/kg/day, continued until the absolute neutrophil count (ANC) exceeded 500 × 106/liter. Twelve children with solid tumors received GM‐CSF after courses of conventional chemotherapy (VP‐16 + ifosfamide or “6 in 1”). One course followed by GM‐CSF was compared to identical courses without GM‐CSF in the same patients. Eight children with recurrent/poor risk malignancies received GM‐CSF after marrow‐ablative therapy and autologous bone marrow transplantation (ABMT). Their engraftment data were compared to matched historical controls. In both groups GM‐CSF accelerated myeloid recovery, which was preceded by the appearance of immature myeloid elements in bone marrow. The ANC levels of 200, 500, and 1,000 × 106/liter were exceeded 2, 3 (P<0.05), and 6 (P<0.005) days earlier with GM‐CSF in the conventional chemotherapy group, and 6, 10 (P<0.05), and 9 days earlier in the ABMT group, as compared to the controls. All adverse effects observed were mild, including skin rashes, nasal stuffiness, general achiness, nausea, and fever. We conclude that GM‐CSF is well tolerated in children and accelerates myeloid recovery in chemotherapy‐induced neutropenia. © 1992 Wiley‐Liss, Inc.
Recurrent cerebrovascular accident with L‐asparaginase rechallenge
Authors: Foreman Nicholas K. , Mahmoud Hazem H. , Rivera Gaston K. , Crist William M.
Published: February 2007

We report a 15‐year‐old boy diagnosed with acute lymphoblastic leukemia (ALL) in 1983. Induction therapy included L‐asparaginase. After the second dose of L‐asparaginase, he had a left sided focal seizure and computed tomography (CT) scan of the head showed a right frontal infarct. No further L‐asparaginase was given. Complete remission was achieved and he successfully completed therapy in 1986. Eight months later he had an isolated bone marrow relapse. Reinduction therapy included L‐asparaginase. After the fourth dose of L‐asparaginase, he presented with severe headache and a CT scan showed a right temporal infarct. Repeat infarction on rechallenge with L‐asparaginase has not been previously reported. Prophylactic therapy, such as fresh frozen plasma, should be considered before patients, with a previous cerebral insult, are rechallenged with L‐asparaginase. However the effectiveness of such therapy has not been established. © 1992 Wiley‐Liss, Inc.
Wilms' tumor with transient dermolysis of the newborn: Recurrence of skin lesions during chemotherapy
Authors: Patrizi Annalisa , Pauluzzi Paolo , Vecchi Vico , Mancini Antonia Francesca
Published: February 2007

Transient bullous dermolysis of the new‐born consists of congenital skin defects and a tendency for blistering of the skin and mucous membranes during the neonatal period. A case of transient bullous dermolysis of the newborn associated with Wilms' tumor is reported. Transient bullous dermolysis of the newborn does not represent an obstacle to administering appropriate chemotherapy when simple precautions are taken.
Longitudinal study of bone age in acute lymphoblastic leukaemia
Authors: Tamminga Rienk Y. J. , Zweens Marjan , Kamps Willem , Drayer Nick
Published: February 2007

Bone age was assessed in children with acute lymphoblastic leukaemia (ALL) using the Tanner‐Whitehouse II method. X‐rays of the left hand (wrist) were made at diagnosis and annually until 5 years after diagnosis. A total of 164 X‐rays from 40 patients was available. During treatment bone age development as well as height growth was retarded in relation to calendar age. A catch‐up of bone age and height was observed in the 2 years after cessation of treatment. No significant differences could be found between patients with and without cranial irradiation as central nervous system treatment. Therefore cranial irradiation alone is not responsible for the growth retardation of children with ALL; the causative role of cytostatic drug therapy and/or corticosteroids has to be investigated. © 1993 Wiley‐Liss, Inc.
Burkitt's lymphoma between African and American types in Turkish children: Clinical, viral (EBV), and molecular studies
Authors: Çavdar Ayhan O. , Gözdaşoǧlu Sevgi , Yavuz Gülsan , Babacan Emel , Ünal Emel , Uluoǧclu Ömer , Yücesan Selçuk , Magrath I. T. , Akar Nejat
Published: February 2007

Seventy‐two Turkish children with Burkitt's lymphoma (BL) observed during a period of 22 years (1968–1990) have been analysed retrospectively. The diagnosis was established histologically according to WHO criteria. BL represented 50% of NHL in this series. The patients were staged according to Ziegler's system. The median age of patients was 5.5 years with a sex (M/F) ratio of 2.1/1. The most common primary site of tumor involvement was the abdomen (69.4%), which was followed by facial tumors, in particular the jaw and orbit (49.9%). There were 21 cases with jaw (29.1%) and 15 cases with orbital involvement (20.8%) at initial presentation. The majority of the patients (84.4%) were in advanced stages (C and D) at initial diagnosis. Facial tumors observed in Turkish children with BL were more similar to African Burkitt's lymphoma than American or European cases. High titers of antibodies against VCA and EA of EBV were also seen in our recent cases of BL. Two main treatment regimens, namely, single agent chemotherapy with cyclophosphamide (CYX) (1968–1974) and three drug (COM) combination chemotherapy, were used consecutively (1974–1988). COM has been shown to produce better results than single agent therapy. The clinical presentation, mean age, and high antibodies (lgG) to EBV and preliminary molecular studies revealed that BL appears to be in between African and non‐African types in Turkish children. This will be further elucidated by direct examination of tumor cells for EBV and investigation of the molecular characteristics of Turkish tumors. Such studies are presently under way. © 1993 Wiley‐Liss, Inc.
Massive hematuria due to extramedullary plasmacytoma invading the bladder
Authors: Shpilberg Ofer , Raviv Gil , Ramon Jacob , Goldwasser Benad , Ben‐Bassat Isaac , Ramot Bracha
Published: February 2007

Bladder involvement by extramedullary plasmacytoma is a rare condition, A 46‐year‐old woman with known lgA‐λ multiple myeloma who developed this condition is described. The patient suffered from massive hematuria, which led to hypovolemic shock. Prompt supportive treatment by blood transfusions, fulguration of the bleeding mucosa and continuous bladder irrigation stopped the bleeding. Subsequent bladder irradiation lead only to partial response of the tumor. © 1993 Wiley‐Liss, Inc.
Increasing values of serum acid phosphatase in a child with Mycoplasma pneumoniae‐associated hemophagocytic histiocytic syndrome
Authors: Abrahamsen Tore G. , Bentsen Beint S. , Brandtzaeg Per
Published: February 2007

We describe a 3 1/2‐year‐old boy with disseminated histiocytic disease probably induced by Mycoplasma pneumoniae. In this patient, acid phosphatase was elevated in serum and was also detected histochemically in the infiltrating histiocytes. The serum acid phosphatase levels increased as his his tiocytosis progressed, apparently mirroring the activity of the disease. This observation suggests that serum acid phosphatase levels should be evaluated further to determine whether they will be a useful indicator of disease in children with different histiocytosis syndromes. © 1993 Wiley‐Liss, Inc.
Multifocal synchronous osteosarcoma: The Scottish bone tumour registry experience
Authors: Jones Richard David , Reid Robin , Balakrishnan G. , Barrett Ann
Published: February 2007

We report 3 cases of multifocal synchronous osteosarcoma constituting 1% of osteosarcoma cases in the Scottish Bone Tumour Registry over 30 years. All were children and had a median age of 10 years. They all presented with multiple bony lesions in the absence of demonstrable pulmonary metastases. Treatment was with palliative radiotherapy in two and combination chemotherapy and radiotherapy in the most recent case. Survival was poor in all cases, with a median of 7 months. A proposal is made for genetic studies in future cases to help elucidate the origin of these tumours. The multicentric and metastatic hypotheses are discussed. © 1993 Wiley‐Liss, Inc.