bluuebunny/crossref_metadata_2025 huggingface dataset:
Intracellular ribonucleotide pools of lymphoblastic leukemic cells in untreated and relapsed all children
Authors: Kawasaki Hajime , Hori Hiroki , Higashigawa Masamune , Ookubo Toshiki , Sakurai Minoru
Published: February 2007

Intracellular ribonucleotide pools were analyzed by HPLC with leukemic blasts obtained from 20 children, including 13 untreated ALL, 5 relapsed ALL, and 2 lymphomas with hematological relapse.

Nucleotide pools were, in general, found more expanded among relapsed cases. Also, the larger mono and diphosphate ribonucleotide levels found in relapsed cells, for instance adenine nucleotide pools, were significantly larger among the relapsed patients, as measured 216.97 ± 53.30 nmol/108 cells, in contrast to 109.70 ± 39.54 nmol/108 cells with untreated children (p<0.005). Furthermore, AMP and ADP occupied only 18% of the total adenine pool in untreated children, but 34% in relapsed children. The similar pattern of nucleotide pools was observed in guanine, cytidine, and uridine nucleotide pools.

Inosine mono phosphates were measured 5.07 ± 6.02 nmol/108 cells with untreated ALL, and 3.55 ± 1.44 nmol/108 cells with relapsed ALL, but thymidine mono phosphates, as a key pyrimidine nucleotide of salvage pathway, were larger (p<0.01) among with relapsed patients measuring 31.47 ± 8.11 nmol/108 cells as compared with that of untreated ALL, 11.19 ± 12.84 nmol/108 cells.

The present results may suggest that there is a difference in nucleotide metabolism between untreated and relapsed leukemic cells, and nucleotide profiles provide more accurate information of leukemia chemotherapy.
Sudden neurologic death after intrathecal methotrexate
Authors: Shore T. , Barnett M. J. , Phillips G. L.
Published: February 2007

Methotrexate leukoencephalopathy is a chronic syndrome of ataxia and confusion which may progress to seizures, coma, and death. We report a fatal case of this syndrome in a patient who displayed no evidence of the typical prodrome of neurologic symptoms or signs. This patient suffered brain death after receiving 11 doses of intrathecal methotrexate for leukemic meningitis. Since leukoencephalopathy was not clinically suspected, this case underscores the need for a test that would reliably monitor central nervous system toxicity due to intrathecal therapy.
Cytotoxic drug‐induced fever: A report on procarbazine‐induced hyperpyrexia
Authors: Akyol Hatice , Sarialioglu Faik , BüyükpamukcCu Münevver , Akyüz Canan
Published: February 2007

A case of hyperpyrexia induced by procarbazine in a child with Hodgkin's disease, neurofibromatosis, and pectus excavatum deformity is presented. After the diagnosis of stage IIIS Hodgkin's disease, combined COPP chemotherapy was initiated. One week later she presented with high fever. After a diagnosis of infection was made, chemotherapy was stopped and antibiotics were given. Nearly the same picture recurred three times after reinstituting chemotherapy. On the fourth occasion, the patient had to be hospitalized because of hyperpyrexia and arrhythmia. There was no obvious reason for fever, and cytotoxic‐induced fever was considered. The drugs were given one at a time. When a test dose (10 mg) of procarbazine was given, she developed a high fever with severe nausea and vomiting. The reaction was controlled by antihistaminics and steroids. To our knowledge this is the second report on hyperpyrexia due to procarbazine administration.
Phase II trial of etoposide and cis‐diaminodichloro‐platinum in patients with refractory and relapsed Hodgkin's disease: Cancer and leukemia group B (CALGB) study 8353
Authors: Rybak Mary Ellen , McCarroll Kathleen , Kaplan Richard J. , Propert Kathleen J. , Budman Daniel R. , Gottlieb Arlan J.
Published: February 2007

A phase II study was conducted by the Cancer and Leukemia Group B (CALGB) in patients with refractory and relapsed Hodgkin's disease (HD) to assess the activity of the combination of etoposide and cis‐platin. Twenty‐seven patients were entered; 22 were evaluated for this report. Treatment consisted of etoposide (VP‐16), 80 mg/m2 IV over 1 hour and cis‐platin, 20 mg/m2IV over 1/2‐1 hour; both agents were given daily for 5 days and repeated every 21 days. All patients had received at least 2 prior chemotherapy regimens, had measurable disease, and most (86%) had a performance status of 0‐1. In the 22 evaluable patients, there were 4 complete responses (18%) and 4 partial responses, for an overall response rate of 36% (95% Cl: 17.2%, 59.3%). Response duration was from 2.1 to 31 months. Significant toxicity was observed with this regimen. Ten patients (45%) had leukopenia <1,000/μl, and 11 patients (50%) had thrombocytopenia <25,000/μl. Serum creatinine levels reached >2.0 in 14% of patients. Seven patients (32%) had severe nausea and vomiting. VP‐16, cis‐platin appears to be an active combination in HD; however, their combined activity is only marginally better than reported single‐agent activity for VP‐16 in the doses and schedule used. Further studies of related combinations in HD are currently under evaluation by the CALGB.
2′ deoxycoformycin (pentostatin) for refractory non‐Hodgkin's lymphoma: A calgb phase II study
Authors: Duggan D. B. , Anderson J. R. , Dillman R. , Case D. , Gottlieb A. J.
Published: February 2007

Seventy‐six eligible patients with relapsed or refractory non‐Hodgkin's lymphoma (NHL) were treated with 2′‐deoxycoformycin (pentostatin) at a dose of 4 mg/m2 intravenously weekly for three weeks and then every other week for a minimum of five total treatments. All patients had measurable disease, near normal hematologic, renal, and hepatic function, and a performance status of 0 or 1. Severe hematologic toxicity was observed in 13% of patients; severe renal or neurologic toxicity was observed in less than 5% of patients. There were no treatment‐related deaths. Objective therapeutic responses were seen in 16% of patients (five complete response [CR] and seven partial response [PR]). However, in three of the patients achieving CR and one patient achieving PR, dexamethasone was employed as an anti‐emetic, making the response of these patients to pentostatin difficult to evaluate. There were eight responses (3 CR) in patients with diffuse histologies and four responses (2 CR) in patients with nodular or mixed histologies. Three responses were in patients with a T‐cell phenotype. Three of five patients with diffuse well‐differentiated lymphoma (IWF A) responded. We conclude that 2′ deoxycoformycin is only minimally active at this dose and schedule against refractory or relapsed NHL. The possibility that low grade B‐ and T‐cell malignancies are more sensitive to 2′ deoxycoformycin deserves further investigation.
Turcot's syndrome with intestinal lymphoma in a child: An unusual case of triple tumor
Authors: Aricó Maurizio , Parigi Gian Battista , Locatelli Davide , Bragheri Romano , Lombardi Fabrizio , Zangrandi Adriano
Published: February 2007

Turcot's syndrome, the association of brain tumor (usually glioblastoma, medullo‐blastoma, or astrocytoma) and colonic polyps, is a very rare condition of which about 20 cases have been reported. It has been described only once previously with cancer in a third organ system. In this paper, we report a child affected with colonic polyposis and astrocytoma (i.e., Turcot's syndrome) associated with intestinal non‐Hodgkin's lymphoma.
Severe acute encephalopathy following inadvertent intrathecal doxorubicin administration
Authors: Aricó Maurizio , Nespoli Luigi , Porta Fulvio , Caselli Dèsirèe , Raiteri Emanuela , Burgio G. Roberto
Published: February 2007

In a female with acute lymphoblastic leukemia, inadvertent doxorubicin administration intrathecally caused severe, life‐threatening, acute encephalopathy with high‐pressure hydrocephalus. Ventriculoperitoneal shunting led to complete reversal of hydrocephalus with progressive disappearance of the acute encephalopathy.
Acute nonlymphocytic leukemia after transient myeloproliferative disorder in a patient with down syndrome
Authors: Barnett P. L. J. , Clark A. C. L. , Garson A. C. M.
Published: February 2007

An infant with Down syndrome (DS) and RH isoimmunization developed transient myeloproliferative disorder (TMD) during the neonatal period. At 16 months she presented with acute nonlymphocytic leukemia (ANLL). Cytogenetic studies during TMD showed trisomy 21 only but new abnormalities emerged during ANLL. She is now in complete remission 5 years after diagnosis. Patients with TMD have either trisomy 21 or mosaic 21 in blood and bone marrow but in phenotypically normal children this cell line disappears with resolution of the TMD. A review of the literature indicates that there are no clinical, hematological, or cytogenetic differences between DS children with TMD who subsequently develop acute leukemia and those who do not. However, the leukemia in the former group may differ in presentation, type, and possibly survival time from other DS children who develop leukemia de novo.
Childhood malignant lymphoma of bone
Authors: Coppes Max J. , Patte Catherine , Couanet Dominique , Caillaud Jean‐Michel , Salloum Emile , Brugières Laurence , Hartmann Olivier , Kalifa Chantal , Bernard Alain , Lemerle Jean
Published: February 2007

From 1974 to 1987, 450 children with non‐Hodgkins' lymphoma (NHL) were seen at the Institut Gustave Roussy (IGR); 14 children had malignant lymphoma of bone (MLB). Eleven of the 14 were newly diagnosed, whereas three presented in relapse. Nine patients presented with multifocal bone involvement. The median age of these eight girls and six boys was 9.5 years (range 1.25–15 years). Bone pain was present in all patients as the initial symptom. Evaluation included physical examination, routine serum chemistries, complete blood count, chest roentgenography, skeletal survey, radionuclide bone scan, lumbar puncture, bone marrow aspiration, and intravenous pyelography, and/or abdominal ultrasonography. Hypercalcemia was found in six patients. Biopsy was performed in 12 patients, revealing high‐grade lymphoblastic lymphomas in all. In two patients diagnosis was made on cytological examination of bone marrow aspirate. Immunophenotyping in four cases, demonstrated non‐B, non‐T cell origin in three and pre‐B cell origin in one. Three patients were treated prior to 1982 with Cyclophosphamide/Oncovin/Prednisone/ADriamycin (COPAD) and seven patients, seen after 1982, were treated with a modified LSA2L2 protocol (LMT). None of the previously untreated patients received radiotherapy. All patients treated with CO‐PAD have died, whereas four out of seven treated with LMT are alive with a median follow up of 51 months (range 36–82 months). One child treated on a pilot study died. One of the three children seen at relapse is disease‐free with a follow‐up of 98 months after high‐dose chemotherapy followed by autologous bone marrow transplantation (ABMT). Five out of six patients presenting with hypercalcemia have died. Results with LMT are encouraging and together with published results suggest that sufficiently intensive chemotherapy can result in complete remission and cure in MLB. Radiotherapy does not seem to be necessary, avoiding possible serious long‐term effects. Hypercalcemia is a bad prognostic feature.
Infant leukemia in Japan: Clinical and biological analysis of 48 cases
Authors: Ishii Eiichi , Okamura Jun , Tsuchida Masahiro , Kobayashi Masao , Akiyama Yuichi , Nakahata Tatsutoshi , Kojima Seiji , Hanada Ryoji , Horibe Keizo , Sato Takeyuki , Komada Yoshihiro , Kimura Ken , Sakaguchi Chiaki , Hosoya Ryota , Oda Megumu , Tsunematsu Yukiko , Kawa Keisei , Konishi Shozaburo , Tsukimoto Ichiro , Matsuyama Kohji , Ueda Kazuhiro
Published: February 2007

Forty‐eight Japanese infants with acute lymphoid leukemia (ALL) (n = 24) and acute nonlymphoid leukemia (ANLL) (n = 24) were analyzed on the basis of clinical and laboratory data. Morphologically, 20 of the 24 infants with ALL were of the FAB L1 type, and 20 of the 24 infants with ANLL were of the M4 or M5 type. Markedly enlarged liver and spleen, and hyperleukocytosis (more than 50,000/μl) were seen in 9, 12, and 14 infants with ALL and 10, 11, and 10 infants with ANLL, respectively. Initial CNS leukemia was evident in 2 infants. Chromosome studies of the leukemic cells showed abnormal karyotypes in 9 of the 21 infants with ALL and 19 of the 22 infants with ANLL, consisting mainly of translocation 11, 12, and inversion 16. By surface marker analysis, only 7 of the 22 infants with ALL (32%) were diagnosed as having common ALL (HLA‐DR+, CD19+, CD10+). Of the 15 infants with ANLL, 12 and 5 infants also showed reactivity to HLA‐DR and CD19, respectively. All of the 5 ANLL infants with lymphoid markers showed different leukemic cell features at the time of relapse. Twelve of the 19 infants with ALL (63%) who achieved a complete remission relapsed within the first 2 years; 8 of the 21 with ANLL (38%) relapsed within the first year. Analysis of event‐free survival shows that the ALL infants with hyperleukocytosis have a poorer prognosis than those without hyperleukocytosis (p < 0.05). Infant leukemia originates in a multipotent cell with lymphoid and myeloid features, and intensive multiagent chemotherapy is necessary for the treatment of infants with acute leukemia.
Prognostic factors in 951 nonmetastatic rhabdomyosarcoma in children: A report from the international rhabdomyosarcoma workshop
Authors: Rodary Chantal , Gehan Edmund A. , Flamant Françoise , Treuner JÖRn , Carli Modesto , Auquier Ariane , Maurer Harold
Published: February 2007

A total of 951 newly diagnosed patients with nonmetastatic rhabdomyosarcoma (RMS) under 21 years of age from four different cooperative study groups were analyzed to identify the most important pretreatment tumor characteristics in predicting survival. The patient characteristics considered were tumor invasiveness (T), tumor size, status of locoregional lymph nodes, and primary site. The cooperative groups and studies analyzed were: SIOP [RMS‐75 (1975–1984)], the intergroup Rhabdomyosarcoma Study [IRS‐II (1978–1982)], Federal Republic of Germany [CWS‐81 (1981–1986)] and Italy [RMS‐79 (1979–1986)], each study involving a different therapeutic protocol. Patient characteristics identified as having a significant relationship to survival by univariate analysis were (favorable features given in brackets): tumor invasiveness [T1], tumor size [ ⩽ 5 cm], status of locoregional lymph nodes [clinically negative], and primary site [orbit and genitourinary nonbladder prostate (GU‐non‐BP) sites]. T2tumors tended to have large size (>5 cm), to be associated with positive nodes, and to have a different distribution by primary site than T1, tumors. A multivariate Cox regression analysis of the pooled data identified tumor invasiveness (T), primary site, and the interaction between T and primary site as significant predictive factors for survival. The prognosis of orbital tumors was consistently favorable regardless of T status, whereas the prognosis for “other sites” was consistently unfavorable. International cooperation in this study has permitted the identification of good and poor risk patient subgroups, permitting the focusing of therapeutic protocols to particular risk groups and setting the stage for common future therapeutic protocols for rare subgroups of patients.
Rhabdomyosarcoma of the larynx in children: A series of five patients treated in the Institut Gustave Roussy (Villejuif, France)
Authors: Kato Mara A. D. P. , Flamant Françoise , Terrier‐Lacombe Marie José , Habrand Jean‐Louis , Schwaab Guy , Luboinski Bernard , Valteau‐Couanet Dominique , Lemerle Jean
Published: February 2007

Rhabdomyosarcoma of the larynx is a malignant disease rerely seen in children. We present five cases (3 males, 2 females) observed among 126 patients with rhabdomyosarcoma of the head and neck (excluding the orbit) treated in the Institut Custave‐Roussy between 1955 and 1981.

The patients were aged between 51/2 years and 131/2 years at the time of diagnosis. The presenting symptoms were dysphonia and/or dyspnea; tracheotomy was required in two cases. All five patients received chemotherapy consisting of vincristine, cyclophosphamide, actinomycin D + procarbazine or doxorubicine. Four patients also received radiotherapy (45 Gy during 5 weeks). Laryngectomy was not performed. Treatment sequelae included regional hypoplasia, huskiness, and subclinical thyroid insufficiency; two patients developed a benign thyroid adenoma. One patient presented a bilateral carotid stenosis subsequent to radiotherapy performed on “wide fields” portal designed to provided prophylactic treatment on uninvolved lymph nodes. All the patients are alive and relapse‐free with a follow‐up of between 13 and 17 years.

This small series of RMS of the larynx shows that patients can be cured without laryngectomy, although long‐term sequelae associated with radiotherapy are observed.
Phase II trial of bisantrene for metastatic melanoma: An illinois cancer council study
Authors: Stiff Patrick J. , Sweet Donald , Kilton Lary J. , Johnson Carole M. , Muntean Betty , Blough Richard R.
Published: February 2007

Sixteen patients with metastatic melanoma entered this phase II study of the efficacy of monthly cycles of Bisantrene. Toxicity was characterized by leukopenia, resulting in the hospitalization of one patient for a febrile incident, and superficial phlebitis. The results were similar to those of previous studies, in that among the 13 patients evaluable for response (six previously untreated with chemotherapy) there were no responses.
Lower incidence of meningeal leukemia when prednisone is replaced by dexamethasone in the treatment of acute lymphocytic leukemia
Authors: Jones Barbara , Freeman Arnold I. , Shuster Jonathan J. , Jacquillat Claude , Weil Marise , Pochedly Carl , Sinks Lucius , Chevalier Louise , Maurer Harold M. , Koch Kjell , Falkson Geoffrey , Patterson Richard , Seligman Barbara , Sartorius Jurg , Kung Faith , Haurani Farid , Stuart Marie , Burgert E. Omer , Ruymann Frederick , Sawitsky Arthur , Forman Edwin , Pluess Hansjuerg , Truman John , Hakami Nasrollah , Glidewell Oliver , Glicksman Arvin S. , Holland James F.
Published: February 2007

In 1971, Cancer and Leukemia Group B (CALGB) mounted a study of acute lymphocytic leukemia (ALL) that compared the effects of the two steroid hormones dexa‐methasome and prednisone. Six‐hundred‐forty‐six children and adolescents with ALL were randomized to receive either prednisone or dexamethasone as part of their remission induction therapy. The 493 evaluable patients who achieved complete remission received the same steroid as pulses throughout remission. Specific central nervous system (CNS) therapy was randomized to either six injections of intrathecal methotrexate (IT MTX) alone or to six injections of IT MTX with cranial radiation (2,400 cGy). Both cranial radiation and dexamethasone offered increased protection against CNS relapse as the first site of failure over IT MTX alone. There were 30 CNS relapses among 238 patients (12.6%) receiving cranial radiation plus IT MTX, whereas there were 70 CNS relapses among 225 (P < 0.001) (22.5%) in those who received IT MTX alone. Similarly, there were 33 CNS relapses among 231 (14.3%) children treated with dexamethasone, whereas there were 67 CNS relapses among 262 (25.6%) treated with prednisone (P = 0.017). Both steroids appeared equal in protecting the bone marrow. Recent national studies have shown significant improvements in preventing CNS relapse over the results in the present report. However, this finding warrants further investigation and, with further documentation, could lead to the substitution of prednisone by dexamethasone to aid further in preventing CNS relapse. This may be particularly important in patients at higher risk for CNS relapse.
Cisplatin ototoxicity in children: A practical grading system
Authors: Brock Penelope R. , Bellman Susan C. , Yeomans Elizabeth C. , Pinkerton C. Ross , Pritchard Jon
Published: February 2007

A long‐term follow‐up study was carried out to assess ototoxicity in children who had been treated for a malignant tumour with “standard dose” cisplatin (60–;100 mg/m2 per course), and were at least 2 years from stopping treatment. The median age at diagnosis was 2 years 2 months (range 1 month to 13.5 years). On the basis of hearing assessment by pure‐tone audiometry, a practical grading system of hearing loss from 0 to 4 is proposed. Moderate to severe high‐frequency hearing loss (grade 2–4) was found in half the children and 10 require appropriate hearing aids. The risk of developing ototoxicity increased significantly with the cumulative cisplatin dose (P = 0.027), although there was considerable individual susceptibility. Serial follow‐up testing, to a median of 4 years after completion of cisplatin treatment, showed no recovery of hearing in any of these children. We suggest careful monitoring of young children by a consultant audiological physician, throughout treatment with cisplatin, particularly when doses of 400 mg/m2 and over have been reached. Alternative chemotherapy should be discussed if grade 2 ototoxicity develops.
Functionally active ganglioneuroma with increased plasma and urinary catecholamines and positive iodine 131‐meta‐iodobenzylguanidine scintigraphy
Authors: Clerico Anna , Jenkner Alessandro , Castello Manuel A. , Ciofetta Gianclaudio , Lucarelli Claudio , Codini Michela
Published: February 2007

Ganglioneuromas are usually considered not to be functionally active. Studies of their catecholamine excretory pattern and of their imaging by means of the adrenergic tracing agent 131‐l‐MIBG have been therefore sparse.

We report on a case of secretory ganglioneuroma, as demonstrated by the increased urinary excretion of the catecholamine metabolites HVA and VMA, increased plasma dopamine and epinephrine levels, and positive 131‐l‐MIBG scintigraphy.

We must therefore be aware that a functionally active tumor is not necessarily a neuroblastoma, and that the diagnosis should be biopsy proven.
Midazolam premedication for pediatric bone marrow aspiration and lumbar puncture
Authors: Friedman Alice G. , Mulhern Raymond K. , Fairclough Diane , Ward Pamela M. , Baker Donald , Mirro Joseph , Rivera Gaston K.
Published: February 2007

The ability of midazolam, a benzodiaz‐epine, to reduce the distress associated with lumbar puncture and bone marrow aspiration was examined in 23 children with acute lymphocytic leukemia. Patients were randomized to receive 0.2 mg/kg midazolam HCI or placebo intravenously 3‐5 min before the procedures, under double‐blind conditions. Based on prior experiences, children in both groups anticipated severe pain from these procedures. Postprocedure pain ratings by patients were markedly reduced in the midazolam but not the placebo group. Both physicians and parents judged the midazolam group as significantly less distressed than controls during and after the procedures. Trained observers recorded significantly fewer pain‐ and anxiety‐related behaviors in the midazolam group immediately before and after, but not during the procedures. The amnestic effects of midazolam, confirmed in a visual recall/recognition test, appear to account for the decreased pain ratings since the behavioral manifestations were similar in the two groups. There were no adverse drug reactions or significant changes in vital signs. Midazolam warrants further investigation as a premedication for painful diagnostic and treatment procedures in children with cancer.
Basal cell carcinoma in young patients after irradiation for childhood malignancy
Authors: Dinehart Scott M. , Anthony John L. , Pollack Sheldon V.
Published: February 2007

Three patients underwent cranial irradiation as part of treatment for childhood malignancy. Two of these patients had acute lymphocytic leukemia and one had an astro‐cytoma. All had longlasting remissions from their childhood malignancy but 8–15 years later developed a basal cell carcinoma of the scalp within previous radiation portals. The basal cell carcinomas were all treated successfully using Mohs micrographic surgery. Follow‐up of patients receiving irradiation for childhood malignancy should include periodic cutaneous examinations of previously irradiated sites.
Behavioral effects of corticosteroids in children with acute lymphoblastic leukemia
Authors: Drigan Rosemary , Spirito Anthony , Gelber Richard D.
Published: February 2007

We evaluated the behavior of 38 children with standard‐risk and high‐risk acute lymphoblastic leukemia who received corticosteroids as part of their antileukemia chemotherapy. Each patient was assessed on two occasions: 16 weeks following remission and 1 year thereafter. Parent reports on emotional lability, attention span/hyperactivity, sleep disturbance, listlessness, peer relations, and depressed mood were obtained for 4 consecutive weeks: week before, week during, and 2 weeks after treatment administration. At the 16 week evaluation, standard‐risk (N = 17) and high‐risk (N = 21) treatment differed by steroid dose and systemic chemotherapy, while at the 1 year testing, treatment differed only by steroid dose (prednisone 40 mg/m2 vs. 120 mg/m2). Statistically significant changes in all measures were observed during treatment as compared with before and after treatment for both risk groups and assessment times. High‐risk patients exhibited greater behavioral effects than standard‐risk patients only for emotional lability, listlessness, and depressed mood at the 16 week testing, when both steroid dose and chemotherapy differed. Girls had slightly greater behavioral effects than boys, while no influence of age was observed. At the doses tested, steroid dose per se does not appear to be the primary variable affecting behavioral changes.
Serum‐soluble interleukin‐2 receptors in B‐cell lymphoproliferative malignancies
Authors: Pavlidis Nicholas A. , Manoussakis Menelaus N. , Germanidis George S. , Moutsopoulos Haralampos M.
Published: February 2007

The levels of soluble interleukin‐2 receptors (sIL‐2R) were determined in the serum of 53 patients with B‐cell lymphoproliferative malignancies, including 31 patients with non‐Hodgkin lymphomas (NHL), 16 with chronic lymphocytic leukemia (CLL), and 6 with multiple myeloma. In addition, serum samples from 40 patients with various solid tumors as well as from 53 healthy individuals were used as controls. It was found that the mean serum levels of sIL‐2R were significantly increased (P < 0.001) in NHL (mean ± standard error of the mean 2,327 ± 320 units/ml) and CLL patients (2517 ± 451 units/ml) as compared to normal controls (207 ± 17 units/ml). No such difference was observed when the serum sIL‐2R levels of patients with multiple myeloma or solid tumors were analyzed. Serum sIL‐2R levels were closely related to the clinical stage, the presence of B‐symptoms, and the disease activity of patients with NHL and CLL. In fact, response to chemotherapy was followed by marked decrease or normalization of sIL‐2R levels, while in a number of patients sIL‐2R values were even able to predict disease relapse. Finally, no association with histologic grade in NHL patients, could be demonstrated.

We conclude that serum sIL‐2R (1) are increased only in B‐NHL and B‐CLL but not in myeloma patients, (2) are related to the tumor burden, and (3) can serve as a valuable tumor marker for the monitoring of patients treatment.
Single institution experience with high‐dose cyclophosphamide, continuous infusion vincristine, escalating doses of VP‐16‐213, and total body irradiation with unpurged bone marrow rescue in children with neuroblastoma
Authors: Kletzel Morris , Becton David L. , Berry D. H.
Published: February 2007

Seven consecutive autologous bone marrow transplants were performed in children with neuroblastoma with very good partial remission (VGPR). A combination of cyclophosphamide, escalating doses of VP‐16‐213, continuous infusion vincristine, and total body irradiation followed by infusion with unpurged bone marrow was used. The dose‐limiting toxicity in this regimen was mucositis which occurred when the total dose of VP‐16‐213 was 2,400 mg/m2. The response rate to this regimen was 4/7 (‐CR 48+, 21+, 21+, 35+ mo) 3/7 had a CR/PR post transplant with progressive disease between 1 and 4 months later (mean 2.6 mo). We conclude that this regimen is well tolerated when the maximum dose of VP‐16‐213 does not exceed 1,800 mg/m2. Further evaluation will be necessary with this regimen to determine its therapeutic value in a larger number of patients with neuroblastoma.
Survival in Hodgkin's disease by stage and age
Authors: Kennedy B. J. , Loeb Virgil , Peterson Vida , Donegan William , Natarajan N. , Mettlin Curtis
Published: February 2007

Patterns of care for Hodgkin's disease in the United States were surveyed through voluntary audits of hospitals with cancer programs nonapproved and approved by the Cancer Commission of the American College of Surgeons. Four hundred and seventy‐three hospitals reported 6,345 patients diagnosed immediately preceding December 31, 1975. The survival rates varied with age, being better at younger ages and worse in the elderly. By pathologic stage, the younger patients faired better than the elderly in each stage grouping. Histologic type was not a factor in this poor prognosis. Hodgkin's disease in elderly patients has a different biologic behavior than in younger patients.
Seizures and cortical dysfunction following high‐dose cisplatin administration in children
Authors: Highley Martin , Meller Simon T. , Pinkerton C. Ross
Published: February 2007

Four of eight children with advanced neuroblastoma treated with a rapid delivery high dose intensity cisplatin based regimen developed acute neurological toxicity. Three had seizures and one developed transient blindness. In the absence of other causes it seems probable that high dose cisplatin (200 mg/m2) as a continuous infusion over five days followed 10 days later by 80 mg/m2 infused over 48 hours was responsible. Other risk factors included an associated deterioration in renal function and neutropenia related fever.
Neuroblastoma with intracranial involvement: An ENSG study
Authors: Shaw Peter J. , Eden Tim
Published: February 2007

We report the experience of the European Neuroblastoma Study Group (ENSG) with central nervous system (CNS) involvement of neuroblastoma. Among this series of intensively treated patients, CNS neuroblastoma was diagnosed by computerised tomography (CT) scanning, rather than by autopsy. Cranial disease occurred in 5% of ENSG patients. Of 11 patients with intracranial disease, 4 had disease in the posterior fossa, a site rarely reported previously. Furthermore, 5 cases had CNS metastases at a time when there was no detectable disease elsewhere, rather than as part of extensive relapse. The pattern of disease we observed, at least for those with parenchymal disease, is in keeping with arterial spread. Although CT scanning is the optimal modality for identifying CNS disease, 2 cases had normal head CT scans prior to the onset of CNS disease. As most patients had symptoms of raised intracranial pressure (RICP) at the time the CNS disease was diagnosed, there does not seem to be any indication for routine CT scanning of the head at diagnosis, but this should be performed as soon as any symptoms or signs appear. With patients living longer with their disease, vigilance must be maintained during follow‐up.
Isolated breast relapse after allogeneic bone marrow transplantation for childhood acute lymphoblastic leukemia
Authors: Conter Valentino , D'Angelo Paolo , Rovelli Attilio , Uderzo Cornelio , Jankovic Momcilo , Bratina Giorgio , Masera Giuseppe
Published: February 2007

Unusual sites of relapses following bone marrow transplantation (BMT) for childhood acute lymphoblastic leukemia (ALL) are rarely reported. We report the case of a 16‐year‐old girl who presented with an isolated right breast relapse 8 months after allogeneic BMT for ALL in second remission. Biopsy showed an ALL infiltrate. Bone marrow and CSF were normal. The girl never showed before extramedullary involvement. She was treated with local radiotherapy and mild systemic chemotherapy. Nine months after breast relapse, she presented an isolated central nervous system relapse. The treatment of isolated extramedullary relapses following BMT is still controversial.
Expression of myeloid differentiation antigens in acute nonlymphocytic leukemia: Increased concentration of CD33 antigen predicts poor outcome—A report from the childrens cancer study group
Authors: Dinndorf Patricia A. , Buckley Jonathan D. , Nesbit Mark E. , Lampkin Beatrice C. , Piomelli Sergio , Feig Stephen A. , Kersey John H. , Hammond G. Denman , Bernstein Irwin D.
Published: February 2007

Ninety‐eight cryopreserved specimens of acute nonlymphocytic leukemia (ANLL) cells obtained at initial diagnosis of children enrolled on the Childrens Cancer Study Group 251 protocol (CCG 251) were examined by indirect immunofluorescence using four monoclonal antibodies to myeloid differentiation antigens. The relationship between the level of differentiation of ANLL cells as determined by their antigen phenotype and the clinical outcome of treatment, including complete remission (CR) rate, survival, and event‐free survival, was evaluated. Most leukemic specimens were determined to express the CD33 antigen (L4F3), a 67‐kD protein. Because the level of differentiation of normal myeloid cells is reflected by the concentration of the CD33 antigen expressed, samples were categorized as CD33‐bright (immature) versus CD33‐dull (mature). Patients with CD33‐bright leukemic blasts had a marginally inferior CR rate to those with CD33‐dull blasts (P = 0.08). With respect to survival and event‐free survival, there was a significantly inferior outcome in the CD33‐bright patients (P = 0.04 and P = 0.06, respectively). Reactions of ANLL with anti‐CD15 antibody (1G10), anti‐CD36 antibody (5F1), or anti‐CD17 antibody (T5A7) did not predict clinical outcome. This study indicates that patients whose ANLL blasts displayed the CD33 antigen in an amount associated with immature myeloid cells experienced a worse outcome than patients with ANLL blasts that expressed a phenotype associated with more mature cells. © 1992 Wiley‐Liss, Inc.
Metastatic rhabdomyosarcoma and histologically similar tumors in childhood: A retrospective european multi‐center analysis
Authors: Koscielniak Ewa , Rodary Chantal , Flamant Francoise , Carli Modesto , Treuner Jörn , Pinkerton C. Ross , Grotto Paolo
Published: February 2007

This is a retrospective analysis of children with metastatic soft tissue sarcoma (STS) registered in the major European STS studies: the SIOP MMT 75, the German CWS 1981 and 1986 studies, the Italian STS study, and the United Kingdom Children's Cancer Study Group centres in Britain. One hundred forty‐six patients out of 164 were evaluable in this analysis. The median age was 7 years (1–18) and the male/female ratio was 1.3:1. The median follow‐up was 80 months (7–171). The most common site of the primary tumor was the extremity (28%), which correlated well with the high preponderance (39%) of the alveolar type of RMS (aRMS). There was no dominant combination of metastatic sites and the most common single organ with metastasis was the lung (41%). Since the therapy depended on the country and period in which the patient was treated, we did not analyse the outcome and therapy together. Complete remission was achieved in 50% of the cases. Those with aRMS had a good chance of achieving CR (61%) but the majority of these patients relapsed (78%). The median time needed to relapse was 243 days (53 days to 47 months) for all patients. Analysis of the site of the primary tumor showed that the CR rate was best in the GU non‐BP site (62%) and worst in PM cases (35%). The overall survival and DFS rate were 18% and 15%, respectively. The GU non‐BP patients had a DFS rate of 50%, while the rate for all other sites varied between 12% and 18%. A total of 24 patients remained in CCR. The majority of them had embryonal type of RMS and metastases in only one anatomic site. Our analysis indicates that the best chance of prolonged survival was in metastatic GU non‐BP cases, patients with embryonal type RMS, and those with metastases in only one organ. To try to improve the treatment of metastatic STS, a prospective European cooperative study was started in 1989. © 1992 Wiley‐Liss, Inc.
Methylated catecholamine metabolites for diagnosis of neuroblastoma
Authors: Candito Mirande , Thyss Antoine , Albertini M. , Deville Anne , Politano Sabine , Mariani Roger , Chambon Pierre
Published: February 2007

Assays of urinary catecholamines and their metabolites (HVA, VMA, dopamine) permit biochemical diagnosis of neuroblastoma in approximately 80% of patients. The urinary methylated catecholamine metabolites normetanephrine (NMN), metanephrine (MN), and 3‐methoxytyramine (3‐MT) were analyzed in 18 patients with neuroblastoma and compared with reference values established for 69 healthy pediatric controls. All 18 neuroblastoma patients had raised urinary excretion of at least one of the three commonly assayed metabolites (HVA, VMA, dopamine). Similarly, raised urinary excretion of a methylated metabolite was noted in all but one of the neuroblastoma patients. The 3‐MT level was pathologic in 16 of the 18 patients (89%). In this series, 3‐MT assay sensitivity was sufficient to warrant trials on a larger population including comparison with patients considered nonsecretors by routine assay procedures. © 1992 Wiley‐Liss, Inc.
Recovery from widespread bone marrow necrosis occurring after chemotherapy for adult acute monocytic leukemia
Authors: Sporn Jonathan R. , Fallon Margaret A.
Published: February 2007

A patient with acute monocytic leukemia who developed bone marrow necrosis following induction chemotherapy is presented. Although the bone marrow necrosis was extensive, recovery occurred, along with complete remission of leukemia. Severe bone marrow necrosis in this setting may be reversible, and continued vigorous supportive care for these patients should be strongly considered. © 1992 Wiley‐Liss, Inc.
Reversal of radiation‐induced neutropenia by granulocyte colony‐stimulating factor
Authors: Marks Lawrence B. , Friedman Henry S. , Kurtzberg Joanne , Oakes W. Jerry , Hockenberger Beverly M.
Published: February 2007

Myelosuppression is a common sequelae of radiotherapy, occasionally delaying the completion of treatment. In this report, we describe successful reversal of radiation‐induced neutropenia in a child receiving craniospinal irradiation by granulocyte colonystimulating factor (G‐CSF). We suggest that G‐CSF be considered as supportive care in patients in whom neutropenia develops during radiotherapy. © 1992 Wiley‐Liss, Inc.
Reversible hypophosphatemic rickets following ifosfamide treatment
Authors: Van Gool Stefaan , Brock Penelope , Wijndaele Godelieve , van de Casseye Willy , Kruger Marianne , Proesmans Willem , van Daele Maria Casteels
Published: February 2007

A 7‐year‐old boy developed renal tubular dysfunction and hypophosphatemic rickets following treatment for relapsed embryonal rhabdomyosarcoma. Multi‐agent chemotherapy included ifosfamide; the child received a total of 108 g/m2. The complete Fanconi syndrome which ensued, including excessive loss of calcium, resolved spontaneously and progressively 18 months after the last dose of ifosfamide. The patient had no further symptoms of rickets and radiological signs had almost completely normalized. Further follow‐up was not possible as, despite further treatment, the child died of progressive disease. © 1992 Wiley‐Liss, Inc.
Lymphoma in Sjogren's syndrome
Authors: Pavlidis Nicholas A. , Drosos Alexandros A. , Papadimitriou Constantinos , Talal Norman , Moutsopoulos Haralampos M.
Published: February 2007

Sjogren's syndrome is an autoimmune disease with a known predisposition for lymphoma development. Eight of 120 patients with primary Sjogren's syndrome followed at the University of loannina over the past 7 years developed non‐Hodgkin's lymphoma diagnosed according to the Kiel classification. The lymphomas differed by location and grading. Six were called low grade (immunocytoma) and two intermediate grade non‐Hodgkin's lymphomas. Five of the immunocytomas involved the minor salivary or lacrimal glands. Immunoperoxidase staining for light chains revealed monoclonal populations. Two patients showed spontaneous regression not previously reported in Sjogren's syndrome. Thus, in Sjogren's syndrome, low grade non‐Hodgkin's lymphomas and especially immunocytomas are the most common lymphomas. These lymphomas tend to evolve very slowly and may regress spontaneously. Given these facts, a conservative approach to treatment is indicated in those patients with only localized disease. © 1992 Wiley‐Liss, Inc.
Smoking habits in survivors of childhood and adolescent cancer
Authors: Haupt Riccardo , Byrne Julianne , Connelly Roger R. , Mostow Eliot N. , Austin Donald F. , Holmes Grace R. , Holmes Frederick F. , Latourette Howard B. , Teta M. Jane , Strong Louise C. , Myers Max H. , Mulvihill John J.
Published: February 2007

Because of their increased risk for second cancers, childhood cancer survivors are people who really should not smoke, but available evidence suggests that they do. We studied the smoking habits of long‐term childhood cancer survivors in data collected from 1289 adult survivors of childhood cancer and 1930 of their sibling controls. Survivors were diagnosed with cancer between 1945 and 1974 when they were less than 20 years old. Using matched analyses that controlled for the influence of family, survivors were 8% less likely than controls to be current smokers, 13% less likely to be eversmokers, but 12% less likely to have quit smoking; these differences were not statistically significant. In a logistic regression analysis there was a significant difference by year of diagnosis for current smoking rate ratios (RR); survivors were less likely to be current smokers if diagnosed in recent years (RR = 0.76; 95% confidence intervals = 0.58–0.98, between 1965–74) and quite similar to controls if diagnosed in earlier years (RR = 1.05 between 1945 and 1954).

In our group of long‐term cancer survivors, the reduction in current smoking came about because survivors were more inclined never to start smoking than controls. Once addicted to tobacco, they were less likely to quit. While the fact that survivors are less likely to start smoking is encouraging, the persistence of smoking habits strongly suggests the need for continuing efforts to prevent smoking in this most vulnerable group. © 1992 Wiley‐Liss, Inc.
Secretory breast cancer in childhood and adolescence: Report of a case and review of the literature
Authors: Serour Francis , Gilad Adi , Kopolovic Juri , Krispin Mayer
Published: February 2007

A 17‐year‐old boy underwent a wide partial mastectomy with axillary dissection for secretory breast cancer, followed by low grade irradiation (30 Gy). There were no axillary lymph node metastases. Hormone receptor assay of the tumor was positive for progesterone and negative for estrogen. There was no local recurrence or sign of dissemination after nearly 5 years.

Literature search revealed 21 cases of secretory breast cancer in childhood and adolescence; in only 2 cases was the hormonal status studied. We report an additional case and review the literature. © 1992 Wiley‐Liss, Inc.
Primary skeletal non‐Hodgkin's lymphoma in the pediatric age group
Authors: Wollner Norma , Lane Joseph M. , Marcove Ralph C. , Winchester Patricia , Brill Paula , Mandell Lynda , Filippa Daniel , Lieberman Philip , McGowan Noreen
Published: February 2007

The authors discuss rare primary skeletal non‐Hodgkin's lymphoma in 16 patients treated from 1973 to 1989. The symptoms of these patients related to bone lesions in 95% of the cases. These bone lesions were monostotic or polyostotic, with or without regional and distant metastases. The locations of these lesions were long bones in 13 patients, pelvic bones in seven patients, and skull and vertebral bodies in two patients. The anatomical locations of these lesions in the bones were diaphysis alone in one patient, epiphysis in two patients, metaphysis in three patients, and a combination of diaphyseal, epiphyseal, and metaphyseal lesions in seven patients. Extraskeletal involvement was present in nine patients; extraskeletal sites included regional or distant lymph node involvement in seven cases, the mediastinum in two, lung nodules in two patients, the skin and subcutaneous regions in four patients; bone marrow in three patients, and peripheral nervous system (PNS) in one patient. Two patients had stage I disease, three had stage II disease, eight had stage III disease, and three had stage IV disease. The majority of patients had large noncleaved cell diffuse lymphomas or DHL by Rappaport classification. All patients were treated with the LSA2‐L2 protocol; six patients received radiation therapy to the affected bone, and ten patients received no radiation therapy. Three patients failed on treatment within the first 4 months of therapy. Two patients developed a second tumor, one in the radiation therapy field and the other in a patient who received no radiation therapy. Eleven patients are alive without evidence of disease, with a median observation time of 6.5 years. The event‐free survival for the 16 patients receiving LSA2‐L2 was 73%, and the lymphoma‐free survival was 81%. Extension of disease to multiple sites or location of the primary site was not of prognostic significance, but the finding of central nervous system (CNS), PNS, or bone marrow disease at diagnosis was. Whether the skeletal lesions were uni‐ or multifocal, with regional or distant metastases, the prognosis for lymphoma‐free survival was good. The role of radiation therapy for all patients and intensive treatment for earlystage disease is discussed. The authors conclude that chemotherapy is the most important modality of treatment for any stage, histology, or location of the tumor. This chemotherapy shou ld include drugs that have been shown to be effective in the treatment of pediatric lymphoma, such as cyclophosphamide, cytosine arabinoside, vincristine, methotrexate, and daunomycin, in dosages sufficient to produce marrow suppression and early recovery, allowing for continuous or intermittent therapy. Radiation therapy should be used if there is no response or progression of disease (demonstrated either clinically or by scans, confirmed by a biopsy) within the first few months of treatment. © 1992 Wiley‐Liss, Inc.
Treatment of childhood acute myeloid leukaemia using the BFM‐83 protocol
Authors: Sartori Patricia C. E. , Taylor Mary H. , Stevens Michael C. G. , Darbyshire Philip J. , Mann Jillian R.
Published: February 2007

Thirty children presenting with acute nonlymphoblastic leukaemia from June 1984 to December 1989 were treated at one UK centre using a West German protocol, AML‐BFM‐83. This consisted of induction, an intensive outpatient‐based Consolidation regimen with seven different drugs and cranial irradiation, and Continuation therapy with thioguanine and cytosine arabinoside for 2 years with additional Adriamycin in the first year. Twenty‐five children achieved complete remission (83%). There were two early deaths from haemorrhage and infection and three from Induction failure. After a median follow‐up time of 60 months, nine relapses have occurred, all in the bone marrow. Life table analysis revealed a probability for survival at 5 years of 47%, event‐free survival 43%, and event‐free interval 50%. Median bed occupancy for chemotherapy and toxicity was 41 days, with median hospital stays of 29 days for Induction, 11 days for Consolidation and less than 1 day for Continuation. This data suggests that long‐term remissions can be achieved in just under half of children with acute nonlymphoblastic leukaemia while creating only modest demands on inpatient resources. © 1993 Wiley‐Liss, Inc.