bluuebunny/crossref_metadata_2025_split huggingface dataset:
Fatal varicella infection in a child associated with thymoma and immunodeficiency (Good's syndrome)
Authors: Watts Raymond G. , Kelly David R. 
Published: February 2007

AbstractAn 8‐year‐old male underwent excision of an encapsulated thymoma. Four months later he presented with disseminated, fatal varicella. Evaluation of his immune status during the terminal illness revealed hypogammaglobulinemia and lymphopenia consistent with a diagnosis of Good's syndrome (immunodeficiency with thymoma). This is the first case of Good's syndrome reported in a child and the first case of fatal varicella associated with Good's syndrome. The combination of this rare pediatric tumor and immunodeficiency is discussed. Despite specific antiviral therapy, varicella remains a deadly disease in the immunocompromised host.
Hodgkin's disease in children: Treatment with MOPP and low‐dose, extended field irradiation without laparotomy late results and toxicity
Authors: Jenkin Derek , Doyle John , Berry Martin , Blanchette Victor , Chan Helen , Doherty Mary , Freedman Melvin , Greenberg Mark , Panzarella Tony , Saunders Frederick , Sonley Marilyn , Weitzman Sheila , Zipursky Alvin 
Published: February 2007

AbstractThe 10 year results of a trial of bimodal treatment of Hodgkin's disease in children with 6 cycles of MOPP and low‐dose extended field irradiation, without staging laparotomy, were for 57 children in all stages as follows: survival 85%, relapse‐free survival 80%, and survival‐free of second relapse 86%. There were three fatal toxic events, two due to viral infection and one to a second malignant tumor (NHL). Three other patients developed a second malignant tumour, and one developed a thyroid adenoma. No patient developed acute leukemia.These results are compared with the results of treatment of surgically staged children by extended field irradiation alone, with bimodal treatment reserved for relapse or advanced disease at diagnosis. Initial bimodal treatment improved the overall 10 year survival free from a second relapse rate by 20% (86% vs. 66%). No major difference in treatment toxicity between these two groups has emerged during the first 10 years of follow‐up.We conclude that, except for favourable CS‐1 presentations, children with Hodgkin's disease confined to the lymphatic system should be given bimodal treatment, but that the least morbid effective combination remains to be determined.
Postoperative chemotherapy for primary intracranial germ cell tumor
Authors: Castañeda Victoria L. , Parmley Richard T. , Geiser Clementina F. , Saldivar Victor A. , Mullins Judith K. , Marlin Arthur E. 
Published: February 2007

AbstractTwo children with primary intracranial mixed germ cell tumors are described who were successfully treated by partial resection of the tumor followed by sequential combination chemotherapy without radiation therapy. The chemotherapy consisting of VP‐16 and cisplatin alternating with vincristine, methotrexate, and bleomycin resulted in apparent complete response after 6 to 7 months of treatment. Disease‐free remission has continued 30–34 months off therapy. A small residual mass in one patient continues to decrease in size on magnetic resonance imaging and is presumed to represent postsurgical change rather than malignant tumor. This report demonstrates that chemotherapy may be effective in primary germ cell tumors of the suprasellar and pineal regions and could be considered for primary treatment instead of radiotherapy.
Long‐term sequelae after pediatric brain tumors: Their effect on disability and quality of life
Authors: Lannering Birgitta , Marky Ildiko , Lundberg Anita , Olsson Elisabeth 
Published: February 2007

AbstractIn an unselected series of pediatric brain tumors, 56 of 60 long‐term survivors—craniopharyngiomas and pituitary tumors excluded–were investigated and interviewed × = 10 (5–16) years after diagnosis. After this time, sequelae were stable and included cognitive (38%), motor (25%), visual (20%), hormonal (20%), and psychological‐emotional (14%) dysfunction. Memory dysfunction was found in 22% of patients with normal intelligence. Moderate or severe disability, from combinations of these impairments, was found in 34%. Sixty‐six percent had no or mild disability compatible with active life and employment. However, these patients less often were married or had children compared with a control group of healthy subjects. Moderate and severe disability was found in 48% of supra‐ and in 21% of infratentorial tumors, after radiotherapy (RT) in 55% vs. without RT in 18%. RT before 6 years of age caused subnormal IQ in all cases. The self‐reported quality of life was not related to degree of disability. Patients with psychological‐emotional sequelae self‐evaluated their quality of life lower than did patients with other types of long‐term sequelae.
Diethylstilbestrol revisited in advanced breast cancer management
Authors: Boyer Michael J. , Tattersall Martin H. N. 
Published: February 2007

AbstractPrior to the introduction of tamoxifen, diethylstilbestrol (DES) was widely used as the first‐line endocrine therapy in postmenopausal women with advanced breast cancer. Since randomized trials reported that tamoxifen has a similar response rate but fewer side effects than DES, its use has declined markedly. We administered DES in a dose of 10–20 mg daily to 11 postmenopausal women with advanced breast cancer, all of whom had received previous endocrine and some cytotoxic therapy also. Four women showed tumour responses to DES (1 complete and 3 partial), 5 had stable disease, and 2 progressive disease. Amongst the patients who responded, 2 had previously been unresponsive to other endocrine treatments. Of the women with stable disease, 3 had prolonged relief of symptoms. No withdrawal responses were noted. The major side effects were nausea (severe in 2 patients, mild in 1) and cardiac failure (2 patients). We conclude that DES remains a useful, active agent in the management of advanced breast cancer in postmenopausal women, even in patients with tumours unresponsive to other endocrine therapy.
Cytotoxicity of lymphokine‐activated killer cells against human neuroblastoma cells: Modulation by neuroblast differentiation
Authors: Duan Dah‐Shuhn , Farmer Diana , Rayner Anthony A. , Sadee Wolfgang 
Published: February 2007

AbstractThe cytolytic activity of lymphokine‐activated killer (LAK) cells against human neuroblastoma (NB) cells was investigated using the continuous NB cell lines, IMR‐32, Kelly, and two subclones of SK‐N‐SH, SH‐SY5Y (neuroblastic phenotype), and SH‐EP (non‐neuronal phenotype). NB cells were found to be sensitive targets of LAK. Of the SK‐N‐SH subclones, the neuroblasts, SH‐SY5Y, were more susceptible to LAK killing than were the non‐neuronal cells, SH‐EP. Pretreatment of the targets SH‐SY5Y and SH‐EP with the differentiating agents, retinoic acid (RA, 10 μM), herbimycin A (236 nM), or nerve growth factor (10 ng/ml), did not substantially alter LAK killing. Furthermore, these differentiating agents did not measurably affect LAK activity during the cytolysis assay or with 1‐h preincubation of the LAK effectors. However, co‐incubation of the LAK cultures over the 3‐day activation period with RA (1 μM) or PGE2(1 μM) inhibited cytolysis by 80%, suggesting that these agents interfere with an early activation step of LAK. These results support the potential use of LAK treatment for neuroblastoma, in combination with differentiation agents that do not affect neuroblastoma sensitivities toward LAK cells. However, some differentiation agents, (e.g., RA) and endogenous prostaglandins (e.g., PGE2) may interfere with LAK activation.
Isolated CNS involvement in Ewing's sarcoma
Authors: Yu L. , Craver R. , Baliga M. , Ducos R. , Warrier R. , Ward K. , Nadell J. 
Published: February 2007

AbstractEwing's sarcoma, an uncommon malignant neoplasm of bone, represents about 10% of all the malignant primary bone tumors. The assumption that subclinical metastases are already present in patients with apparently localized tumor indicates the need for systemic therapy as an integral part of primary treatment. The usual sites of metastases are the lungs and skeletal system. Central nervous system (CNS) involvement is rare and is usually seen only in disseminated and fairly advanced disease. We report two patients in whom, after aggressive adjuvant chemotherapy, disease developed in the CNS without local recurrence. In one patient, meningeal involvement with malignant cells was identifiable in the cerebrospinal fluid; in the second patient, who presented with a space‐occupying lesion, a diagnosis of arachnoid involvement was made histologically.
Treatment of refractory and relapsed Hodgkin's disease: Intensive chemotherapy and autologous bone marrow or peripheral blood stem cell support
Authors: Hurd David D. , Haake Robert J. , Lasky Larry C. , Christiansen Neal P. , McGlave Philip B. , Bostrom Bruce , Levine Ellis G. , Weisdorf Daniel J. , Kim Tae H. , Peterson Bruce A. , Bloomfield Clara D. 
Published: February 2007

AbstractThirty‐three patients with recurrent or refractory Hodgkin's disease were treated with high‐dose cyclophosphamide, BCNU, and etoposide and supported with either autologous bone marrow or peripheral blood stem cells or both. Peripheral blood stem cells were comparable to bone marrow in supporting the recovery of hematopoiesis. Twenty‐five patients (76%) were in complete remission following this therapy of whom 13 have subsequently relapsed. Twelve remain alive and disease free from 10 to 47 months. The Kaplan‐Meier estimate of disease‐free survival at 28 months for the entire 33 patients is 32% (95% confidence interval, 13–50%). Poor outcome in six patients was associated with bone marrow involvement by Hodgkin's disease at the time of peripheral blood stem cell collection. These six patients′ survival, disease‐free survival, the duration of complete remission were all significantly worse than for the 27 patients who were supported with bone marrow (n = 23), peripheral blood stem cells (n = 2), or both(n = 2), and whose marrows were free of disease at the time of stem cell collection. These data demonstrate that intensive therapy with autologous transplantation can produce extended disease‐free survival for some patients with advanced Hodgkin's disease and that peripheral blood stem cell support can effectively be used for hematopoietic reconstitution. However, our observations also suggest that with this preparative regimen, bone marrow involvement at the time of peripheral blood stem cell collection is predictive for a poor outcome and alternate approaches to treatment should be considered for this subset of patients.
Autografting with bone marrow exposed to multiple courses of very high dose cyclophosphamide in vivo and to 4‐hydroperoxycyclophosphamide in vitro
Authors: Kushner Brian H. , Gulati Subhash C. , O′Reilly Richard J. , Heller Glenn , Cheung Nai‐Kong V. 
Published: February 2007

AbstractIntensive chemotherapy prior to harvesting autologous bone marrow may be advantageous for cancers that infiltrate the bone marrow. This approach, however, may deplete hemopoietic reserves or damage the hemopoietic microenvironment and thereby jeopardize posttransplant engraftment; in vitro treatment of bone marrow to ensure a tumor‐free state may enhance the risk of nonengraftment. We addressed these concerns in a pilot study of patients with disseminated neuroblastoma. Bone marrow was harvested after initial intensive therapy that included 1) three to six courses of very high dose cyclophosphamide (CPM) (100–140 mg/kg in five patients, 140–160 mg/kg in nine patients), plus doxorubicin and vincristine, followed by 2) a median of three strongly myelosuppressive courses of cisplatin/VP16. The median interval between courses was 23 days. The marrow was treated in vitro with the CPM congener 4‐hydroperoxycyclophosphamide (4‐HC) and cryopreserved. It was infused after a massive cytoreduction regimen of melphalan 180 mg/m2 (n = 7) or thiotepa 900 mg/m2 (n = 7), plus cisplatin, BCNU, VP16, and local radiation. All 14 patients reconstituted hemopoiesis (median of 37 days to neutrophils ±500/μl and of 63 days to platelets >50,000/μl). The number per kilogram body weight of hemopoietic progenitor cells (CFU‐GM, BFU‐E) in the autografts correlated significantly with the rate of hematologic recovery. Preharvest CPM dosage, however, did not. The use of thiotepa, as opposed to melphalan, was associated with a significantly slower posttransplant platelet recovery, suggesting a possible adverse effect of high‐dose thiotepa on posttransplant hemopoiesis. In sum, while reinforcing evidence of a stem cell sparing effect of CPM, this pilot study more importantly delineates the degree to which high‐dose alkylator therapy can be exploited to attain maximal dose intensity beginning at diagnosis. This aggressive treatment approach may have wide applicability, since CPM is among the most active agents against a spectrum of pediatric cancers.
Phase II study of moplace chemotherapy for patients with previously treated Hodgkin's disease: A calgb study
Authors: Schulman Philip , McCarroll Kathleen , Robert Cooper M. , Norton Larry , Barcos Maurice , Gottlieb Arlan J. 
Published: February 2007

AbstractTo further evaluate possible non‐cross‐resistant regimens in Hodgkin's disease, a phase II trial utilizing antimetabolites and etoposide was initiated by the Cancer and Leukemia Group B (CALGB). Etoposide was included because of its known efficacy in relapsed Hodgkin's disease and to evaluate for synergy with an alkylating agent and vincristine. Cytosine arabinoside and methotrexate were included to evaluate their effectiveness in rapidly growing resistant disease. Forty‐two patients with previously treated Hodgkin's disease were entered, of which 37 are evaluable for response and toxicity. All patients had at least 2 prior regimens of chemotherapy and 59% had additional radiation therapy. Complete and partial response was observed in 61%; there were 32% complete responders. Duration of complete response was a median of 8 months (range 2–28+ months). Duration of partial response was 7 months (range 1–17 months). Three patients remain in complete remission at 19, 19, and 28 months. Major toxicity was hematologic with severe or life‐threatening toxicity in 54%. There was one patient with a fatal infection. Non‐hematologic toxicity, save for nausea and vomiting, was mild and uncommon. There were two fatal and one severe pulmonary toxicities reported in patients who had previous exposure to bleomycin and mediastinal radiation. Three had interstitial pneumonitis and one pulmonary emboli. The interstitial pneumonitis was thought to be drug related. Survival of the entire group is estimated at 61% at 12 months. We conclude that MOPLACE is an effective regimen with an appreciable complete response rate in this heavily pretreated group of patients. Hematologic and pulmonary toxicities are severe and may necessitate dose modifications. The use of etoposide containing combinations requires further study as primary therapy in untreated patients.
Phase ii trial of etoposide and doxorubicin in advanced head and neck cancer
Authors: Gradishar William J. , Vokes Everett E. , Kies Merrill S. 
Published: February 2007

AbstractIn an effort to improve the response rate of patients with advanced head and neck cancer, a clinical trial combining two infrequently studied drugs was conducted. Twelve patients with squamous cell carcinoma of the head and neck were treated with 3‐day continuous infusions of etoposide (90 mg/m2/day) and doxorubicin (15 mg/m2/day). All patients had been heavily pre‐treated with surgery, radiation therapy, and/or chemotherapy. No objective responses were observed in 9 evaluable patients. One patient maintained stable disease state for 4 months. The major toxicity was Grade 3 or 4 myelosuppression which occurred in 42% of administered cycles. Although single‐agent etoposide and doxorubicin have shown response rates of 10%‐40% in patients with squamous cell carcinoma of the head and neck, we were disappointed to find that the combination of etoposide and doxorubicin has little or no activity in patients with advanced head and neck cancer. The toxicity encountered with this combination was significant.
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

AbstractNeuraxis 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

AbstractThe 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

AbstractSeventeen 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

AbstractA 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

AbstractAn 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.