bluuebunny/crossref_metadata_2025 huggingface dataset:
Esophageal cancer in a young woman after treatment for osteosarcoma
Authors: Dewar J. M. , Courtney J. T. , Byrne M. J. , Joske R. A.
Published: February 2007

Five years after completing adjuvant chemotherapy for osteosarcoma of the fibula, a 20‐year‐old woman developed an esophageal carcinoma. The association between prior chemotherapy and radiation exposure, and the significance of genetic factors and family history are discussed. This case exemplifies the importance of continued follow‐up of the long‐term survivors of tumor management.
Concurrent development of a thymic cyst and mediastinal Hodgkin's disease
Authors: Kaesberg Paul R. , Foley David B. , Pellett John , Hafez G. Reza , Ershler William B.
Published: February 2007

A case is presented of a young adult female who presented with nodular sclerosing Hodgkin's disease and a mediastinal mass that proved to be a thymic cyst. Prior to this illness, a chest x‐ray had been ob tained that did not show a mediastinal mass. This case illustrates that thymic cysts may arise simultaneously with mediastinal Hodgkin's disease, rather than as a result of treatment or being present congenially.
Skin rash associated with Candida guilliermondii
Authors: Booth Linda V. , Collins Amanda L. , Lowes J. A. , Radford M.
Published: February 2007

We report a case in which the development of a skin rash in a neutropenic patient was associated with multiple blood culture isolates of Candida guilliermondii—an unusual isolate not previously documented to cause rashes.
Phase I/II study of bisantrene in childhood cancer: A report from the childrens cancer study group
Authors: Movassaghi Nasser , Krivit William A. , Krailo Mark D. , Hammond G. Denman
Published: February 2007

A phase I/II study of bisantrene using a 3‐week schedule was undertaken in 171 children with refractory leukemias and solid tumors. The doses ranged from 190 to 430 mg/m2. The maximum tolerated dose for children with solid tumors and acute leukemias was 280 mg/m2 and 360 mg/m2 every 3 weeks, respectively. The dose limiting toxicities were hepatic and hematologic. One patient with ALL achieved a complete remission and partial responses were observed in three patients with soft‐tissue sarcomas. The data indicate that bisantrene, at the doses and schedule used in this study, has limited antitumor activity in pretreated children with Cancer.
I‐131 metaiodobenzylguanidine: Diagnostic use in neuroblastoma patients in relapse
Authors: Heyman Sydney , Evans Audrey E. , D'Angio Giulio J.
Published: February 2007

Metaiodobenzylguanidine (MIBG) has been used for the detection and treatment of neuroectodermal tumors, including neuroblastoma. We report our experience with 131I‐MIBG used diagnostically in neuroblastoma patients with relapse. Thirty‐eight studies were performed in 26 patients. There were 24 children (range 3 months‐14 years) and two adults. While the study was found to be both sensitive and specific for the presence of disease, there are instances of discordance. False‐negative studies were found with a markedly anaplastic tumor and with two mature ganglioneuromas. A bone lesion was negative with 131I‐MIBG, but positive on bone scan. A biopsy confirmed the presence of neuroblastoma. Caution should be exercised when scanning pretreated patients, and perhaps with newly diagnosed patients as well.
Bone marrow biopsy in the initial staging of Hodgkin's disease
Authors: Doll Donald C. , Ringenberg Q. Scott , Anderson Sharon P. , Hewett John E. , Yarbro John W.
Published: February 2007

Results of bone marrow biopsy were retrospectively evaluated in 120 previously untreated patients with Hodgkin's disease. The incidence of bone marrow involvement was 13%. All patients with marrow invasion had B symptoms and/or clinically advanced disease. When patients with bone marrow involvement were compared to those without there were significant differences in the incidence of B symptoms, the clinical stage, hemoglobin levels, leukocyte counts, platelet counts, and serum levels of lactate dehydrogenase and alkaline phosphatase. None of 59 patients with clinical stage IA and IIA had evidence of marrow invasion. This study demonstrates that trephine bone marrow biopsy is of value in detecting marrow involvement in specific subgroups of untreated patients with Hodgkin's disease, i.e., those patients with constitutional symptoms and/or clinical stage III or IV. However, bone marrow biopsy adds little to the initial staging of patients with clinical stage IA and IIA. Routine use of this procedure in such patients may be unnecessary.
Wide‐field radiation therapy plus simultaneous chemotherapy for refractory Hodgkin's disease
Authors: Gomez German A. , Krishnamsetty Ramachandra M. , Han Tin , Henderson Edward S.
Published: February 2007

Nine male patients with disseminated Hodgkin's disease who had relapsed and/or were refractory to two or more non‐“cross‐resistant” programs of combination chemotherapy (range, two to five previous programs; median, three) were admitted into a study of treatment with wide‐field low‐dose radiation therapy (2,000 cGy), to all lymph node bearing areas, the liver, the spleen, and in selected cases the lungs (1,000 cGy). Simultaneous with each course of radiation therapy, chemotherapy with BCNU and procarbazine given at 50% doses of a known program of chemotherapy (BOPP) with vincristine and prednisone was given.

Treatment was completed in five patients. Three of these achieved a complete remission; the other two had a partial response. Of the other four, two died during treatment with interstitial pneumonitis (probably radiation induced), and two failed to respond. Remission durations were 18+, 38, and 44+ months. Pulmonary toxicity was severe; however, hematologic and other toxicities were acceptable. The incorporation of wide‐field low‐dose radiation should be considered in programs of rescue for patients with Hodgkin's disease who relapse or are refractory to standard combination chemotherapy.
Late relapses after treatment for acute lymphoblastic leukemia in childhood: A population‐based study from the nordic countries
Authors: Nygaard Randi , Moe Peter Johan , Brincker Hans , Clausen Niels , Nyman Ralf , Perkkiö Mikko , Eilertsen Mary Elizabeth , Johansen Ole Jakob , Väre Markus , Brinch Lorentz , Siimes Martti A.
Published: February 2007

Seven late relapses of acute lymphoblastic leukemia occurring 5.5 to 12.3 years after cessation of therapy are reported in 986 patients who had discontinued treatment for leukemia acquired before the age of 15. The study covers patients from the five Nordic countries. Of the 434 patients with ALL who had passed 5 years of follow‐up without recurrence, seven have subsequently relapsed so far; an estimated cumulative proportion of 6.9% within the 10 years. In addition, we report a girl 15.9 years old at diagnosis who relapsed 7.3 years after cessation of therapy. These findings confirm that “cure” of acute lymphoblastic leukemia treated in the 1970s cannot be considered definite, even 5 years after discontinuation of therapy.
Combination chemotherapy for adrenal carcinoma: Response in a 5 1/2‐year‐old male
Authors: Crock Patricia A. , Clark A. C. L.
Published: February 2007

Adrenocortical carcinoma is an uncommon tumor of childhood, and systematic studies of therapeutic options are not available. Children with very large tumours have a poor outlook with surgery alone, so that other treatment should be considered, but the literature is unhelpful in suggesting what this should be. In the case reported here, combination chemotherapy, including cisplatinum, gave a well‐documented good partial response, suggesting a possible role for adjuvant chemotherapy using such a drug combination.
Treatment of acute nonlymphocytic leukemia in the elderly with intermediate high‐dose cytosine arabinoside
Authors: Letendre Louis , Niedringhaus Robert D. , Therneau Terry M. , Gastineau Dennis A. , Goldberg Richard M. , Gerstner James B. , Mailliard James A. , Gundlach William J. , Kardinal Carl G. , Pierre Robert V.
Published: February 2007

Effective treatment of the elderly patients (≥ 65 years) with acute nonlymphocytic leukemia (ANLL) remains elusive and controversial. In the present study, single‐agent Cytosine Arabinoside (ARA‐C) was administered at an intermediate dose level (500 mg/m2 intravenously [I.V.] 1‐hour infusion q12 hours for 12 doses) to 30 newly diagnosed and previously untreated patients. Complete remission was achieved in seven patients after the initial cycle of treatment, in two patients after retreatment, and in one patient after delayed recovery of his peripheral count over a 6‐month period. The toxicity of this schedule was primarily hematologic, and the response rate was in keeping with that reported by other groups using aggressive multiagent regimens.
Treating childhood acute lymphoblastic leukaemia (ALL): Summary of ten years' experience in Italy
Authors: Paolucci G. , Masera G. , Vecchi V. , Marsoni S. , Pession A. , Zurlo M. G.
Published: February 2007

Between 1976 and 1986, 2,093 children with ALL were enrolled in three consecutive generations of trials conducted by the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP). A 50% event‐free survival at 5 years was achieved overall in this population, approximately accounting for more than 50% of the entire childhood ALL population in Italy. Participation in the group protocols increased from the original seven founding centers to the current 37 institutions. Results in the standard population (non‐T immunophenotype, non‐FAB L3, and < 50,000 white blood cells [WBC]/ml) were considerably better with more recent, more aggressive protocols. The two major results in this population (N = 540) were a relatively low incidence (8% at 5 years) of central nervous system (CNS) relapse in the “good”‐risk population (< 10,000 WBC, ages 3–6 years, and FAB L1), without the use of cranial irradiation, and a projected 4‐year disease‐free interval for bone‐marrow relapse of 80% in the “average”‐risk group, where a three‐drug reinduction program was adopted after consolidation. Overall, the event‐free survival of the most recent generation (protocol 82, median follow‐up time of 38 months) is 66% at 4 years (95% confidence limits [CL] 61–71). Based on these 10 years of experience, the general strategy of the group for the 90s is outlined and discussed.
Etoposide (VP16) in the treatment of multisystem langerhans cell histiocytosis (histiocytosis X)
Authors: Broadbent V. , Pritchard J. , Yeomans E.
Published: February 2007

Ten children with Langerhans cell histiocytosis (histiocytosis X) either resistant to or intolerant of corticosteroids received etoposide (VP16). Nine responded. In one instance, partial diabetes insipidus was temporarily reversed. There was no major toxicity. Etoposide should be seriously considered as therapy for patients with LCH in whom the toxicity/benefit ratio of steroid therapy is unacceptably high.
Soft tissue sarcomas in infants younger than 1 year of age: A report of the German soft tissue sarcoma study group (CWS‐81)
Authors: Koscielniak E. , Harms D. , Schmidt D. , Ritter J. , Keim M. , Riehm H. , Treuner J.
Published: February 2007

Of 357 patients with soft tissue sarcomas (STSs) who were registered between 1981 and 1986 in the CWS‐81 study, 21 (5.8%) were younger than 1 year of age. Six cases were probably of congenital origin since they were diagnosed in the 1st month of life. There was no difference in clinical grouping (staging and primary site) between infants and older patients. The major histological subsets comprised 12 patients with embryonal rhabdomyosarcoma (57% vs. 44% in older patients) and five patients with fibrosarcoma (23% vs. 2% in older patients). Of 21 patients, 17 received chemotherapy. The response rate (tumor reduction more than 1/3) to exclusive cytostatics therapy within 7–9 weeks, of ten evaluable patients, was 100% (vs. 86% in older patients). Eight of the responders achieved complete remission after 16 weeks of therapy and are in continuous complete remission (CCR). Most patients received 20–50% of the recommended chemotherapy dose for older children, which was calculated using body surface area. No fatal or life‐threatening complications were observed. The eventfree survival rate for children younger than 1 year of age with rhabdomyosarcoma was 70% vs. 47% in older children (no significant difference). Now, 2–5 years after therapy all five fibrosarcoma patients are alive and free of disease.

Our data indicate that children under 1 year of age with rhabdomyosarcoma have a slightly better prognosis than older children, when therapy modalities are adapted to age.
Preservation of anaplastic features in Wilms' Tumors after preoperative chemotherapy
Authors: Alvarez‐Silvan Ana M. , Carrasco F. Gavilan , Cuevas G. Pineda , Becerra E. Andrade , Anguita M. L. , Caro A. Martinez , Valles M. C. Gracia , Arcega M. A. Maillo
Published: February 2007

In 4 of 35 childhood Wilms' tumors on record in the Hospital Infantil Virgen de Rocio de Sevilla, it was possible to identify diffuse anaplasia despite the fact these children had received cytoreductive chemotherapy preoperatively.
Leukemic infiltration of the eye: Results of therapy in a retrospective multicentric study
Authors: Lo Curto M. , Zingone A. , Acquaviva A. , Bagnulo S. , Calculli L. , Cristiani L. , Dini G. , Di Tullio M. T. , Guazzelli C. , Jancovic M. , Masera G. , Massolo F. , Nespoli L. , Rosati D. , Russo A. , Werne B. , Zanesco L.
Published: February 2007

A multicentric retrospective study on leukemic ophthalmopaty (LO) is reported, including 38 patients (21 males, 17 females) with acute leukemia (AL) observed from 1976 to 1985. LO developed in four patients at the time of diagnosis of AL; ten were in first complete remission (eight off therapy), 12 in second remission, and 12 in combined relapse. The children were treated according to different schedules of systemic and intrathecal chemotherapy with or without radiotherapy (RT) of the affected eye. Ocular remission occurred in 32 of 38 patients, but with subsequent ocular relapse in six of the 32. Complete remission after LO treatment lasting for more than 24, 30, 40, and 78 months was observed in four of the ten children with isolated LO in first AL marrow remission. The authors concluded that systemic and intrathecal chemotherapy probably is associated with RT (at least 30 Gy to the affected eye). Aggressive treatment is justified because children with isolated ocular relapse can still be cured.
Case report of successful use of VP‐16 in nasopharyngeal carcinoma
Authors: Daghistani Doured , Davis Joanna , Toledano Stuart
Published: February 2007

We report the use of VP‐16 as a single agent for the treatment of nasopharyngeal carcinoma. Our patient, a 16‐year‐old Black Haitian male, had complete clinical and radiographic response to VP‐16. The remission continued for 14 months. The patient experienced minimal toxicity. We believe that the good response, excellent compliance, and minimal toxicity experienced by our patient should encourage more trials of VP‐16 as upfront therapy for patients with nasopharyngeal carcinoma.
Testicular relapse in adult acute lymphocytic leukemia: A case report and literature review
Authors: Vukelja Svetislava J. , Swanson S. John , Knight Robert D. , Weiss Raymond B.
Published: February 2007

In the pediatric age group, acute lymphocytic leukemia (ALL) may involve the testicles during complete remission of the disease in the marrow. We report a case of testicular involvement with ALL in an adult and review 11 cases of this phenomenon reported previously, to draw attention to the fact that it can occur at any age. Recognition of this fact is important, because testicular involvement may be the first manifestation of systemic relapse. The treatment of choice is irradiation to both testicles and combination chemotherapy.
Nasopharyngeal Hodgkin's disease with intracranial extension in a child
Authors: Mallouh Ahmad A.
Published: February 2007

Childhood Hodgkin's Disease rarely involves the nasopharynx or the brain. This is a report of a 12‐year‐old boy who presented with a 3‐month history of headache, diplopia, dizziness, and early morning vomiting. Computerized axial tomography (CT) scan revealed a nasopharyngeal mass with intracranial extension through the skull base. Biopsy of the nasopharyngeal mass and an upper cervical lymph node was consistent with Hodgkin's disease of mixed cellularity. This, to the author's knowledge, is the first report of a child having the combination of nasopharyngeal and intracranial involvement in Hodgkin's disease.
Patients', parents', and oncologists' perceptions of informed consent for bone marrow transplantation
Authors: Lesko Lynna M. , Dermatis Helen , Penman Doris , Holland Jimmie C.
Published: February 2007

Bone marrow transplantation (BMT) is gaining increasing acceptance as a therapeutic treatment modality and is being offered to patients even in the early stages of disease in the presence of minimal debilitating symptoms. Despite this, little is known regarding patients' and physicians' perceptions of the process in which informed consent for this controversial and potentially lethal procedure is obtained. Thirty‐nine adult BMT patients and the parents of 61 children undergoing BMT and each of their physicians completed a questionnaire concerning their perceptions of the discussion in which consent for BMT was obtained and their evaluation of the consent document. In addition, the factors influencing patients' and parents' decision to accept BMT and the nature and amount of BMT information retained by patients and parents were assessed. The results indicate that on the whole patients and parents evaluated the BMT consultation and consent document favorably, were motivated by their trust in the physician and their belief in BMT as a cure, retained information regarding major points of informed consent from both the consent document and physician discussion, and had considerable difficulty with recall of the specific toxic side effects associated with BMT. Physicians' perceptions, on the other hand, reflected a less positive view of the extent to which patients and parents were actively involved in the consent process and the readability of the consent document. Perceptions of the informed consent process on the part of oncologist‐investigators and patients which could impede the goals of informed consent and implications for facilitating the process are discussed.
An attempt to use a common staging system in rhabdomyosarcoma: A report of an international workshop initiated by the international society of pediatric oncology (SIOP)
Authors: Rodary C. , Flamant F. , Donaldson S. S.
Published: February 2007

One hundred medical records of children with rhabdomyosarcoma were reviewed independently by ten European and American specialists primarily concerned with pediatric cancer patients. Each specialist assessed the stage of disease using a pre‐treatment staging system (International Society of Pediatric Oncology—Union Internationale Contre le Cancer TNM [SIOP—UICC TNM] and a post‐surgical staging system (Intergroup Rhabdomyosarcoma [IRS] grouping system). These two prognostic systems are based on different criteria, and there is no simple correspondence between them. The reliability of the two systems, based on patients' characteristics extracted from medical records, was similar and satisfactory (Kappa index: 0.78 and 0.82 with the TNM system and with the IRS system, respectively). Nevertheless, the IRS grouping system, which considers a primary method of treatment: Surgery, is dependent upon the surgeon, the institution, and the current concept of treatment. We believe that the TNM system provides the best description of extent of disease and should be used for determining therapeutic options and comparing therapeutic results.
Is androgen substitution necessary in hypogonadal patients when they are treated with chemotherapy for malignant diseases?
Authors: Pavlidis Nicholas , Nikolaou Nikitas , Tolis Christos , Orfanos Stylianos , Tolis George
Published: February 2007

A patient with hypogonadotropic hypogonadism owing to endogenous gonadotropin releasing hormone deficiency, who developed Hodgkin's disease is described. Chemotherapy administration caused prolonged and life‐threatening myelosuppression; androgen substitution seemed to reverse bone marrow function and to maintain normal peripheral blood counts.

Whether or not androgens are a necessary substitution in hypogonadal patients suffering from cancer and undergoing chemotherapy is discussed.
Adult‐type T‐cell lymphoma in an adolescent with human T‐lymphotropic virus type 1 seropositivity
Authors: Fort John A. , Graham‐Pole John , Mottshaw Gail
Published: February 2007

Recently, a form of T‐cell lymphoma associated with seropositivity for human T‐lymphotrop virus type 1 (HTLV‐1) antibody has been described in the adult population. The lymphoma frequently presents with massive hepatosplenomegaly, lymphadenopathy and circulating malignant cells, with a normal bone marrow. HTLV‐1‐associated lymphoma has been seen most frequently inadults from Japan, the Caribbean, and the southeastern United States. We report the first known case of a child with a lymphoma having the clinical and serological features of an HTLV‐1‐associated T‐cell lymphoma.
Daily profiles of plasma phenylalanine and tyrosine in patients with osteogenic sarcoma during treatment with high‐dose methotrexate‐citrovorum rescue
Authors: Hilton Mary A. , Bertolone Salvatore , Patel Chandrakant C.
Published: February 2007

Three adolescents and one child with osteosarcoma were studied during multiple courses of high‐dose methotrexate, citrovorum factor rescue (HDMTX‐CFR), with one adolescent treated intermittently over a period of 6 years. Plasma phenylalanine (Phe) and tyrosine (Tyr) were measured immediately before the infusion of MTX and then daily until serum MTX fell below 10−7 M. At 24 hours, all showed marked increases in Phe and in the Phe/Tyr ratio. This suggests inhibition of dihydropteridine reductase (DHPR) which, in association with hepatic Phe hydroxylase, controls plasma concentrations of Phe. Inhibition of this enzyme system is not relieved by CFR. In the adolescent patients, although MTX levels in plasma declined steadily, Phe concentrations, which fell between 24 and 48 hours, rose to a new peak at 4–7 days. Possible reasons for this secondary increase are discussed. The patient with the longest exposure to HDMTX showed an increase in pretreatment Phe/Tyr ratios with time, suggesting damage to liver parenchymal cells not indicated by standard tests of liver function. Evaluation of plasma Phe during the course of HDMTX‐CFR may permit assessment of intracellular concentrations of MTX or its metabolites in the liver without interference by CFR.
Enlargement of mediastinal masses on simulation films: A radiotherapeutic problem in the management of patients with Hodgkin's disease
Authors: Vukelja Svetislava J. , Andejeski Yvonne , Giguere Jeffrey K. , Yuen Albert , Diehl Louis F.
Published: February 2007

The size of the mediastinal mass on standard posterior‐anterior chest radiograph in stage I and stage II Hodgkin's disease has both prognostic and therapeutic importance. But the actual treatment is based on the anterior‐posterior supine simulation film. Problems arise when the prognosis (whether all the disease can be effectively contained in an irradiation port) and toxicity (more lung and heart irradiated or chemotherapy required) are changed when the mass is markedly enlarged on the radiation simulation film. We report two patients with small mediastinal masses on standard posterior‐anterior chest radiographs that appeared to enlarge markedly on radiation simulation films. Differences in the direction of the X‐ray beam, distance from the subject, and patient position are shown to increase artificially the size of a mediastinal mass on simulation films. The staging and therapeutic implications are discussed.
Papillary adenocarcinoma of the renal pelvis in a child: Case report and brief review of the literature
Authors: Moncino Mark D. , Friedman Henry S. , Kurtzberg Joanne , Pizzo Salvatore V.
Published: February 2007

Tumors of the renal pelvis are not common in adults and are extremely rare in children. Forty‐eight cases of adenocarcinoma of the renal pelvis have been reported, with only one patient under 32 years of age. We report the youngest patient (an 11 year old boy) diagnosed with this tumor and discuss our approach to therapy. We also review the literature with respect to the characteristics and histogenesis of this rare neoplasm.
Pharmacokinetics of cytosine arabinoside in cerebrospinal fluid and of its metabolite in leukemic cells
Authors: Békássy Albert N. , Liliemark Jan , Garwicz Stanislaw , Wiebe Thomas , Gulliksson Hans , Peterson Curt
Published: February 2007

Concentrations of ara‐CTP in leukemic cells isolated from CSF and of ara‐C in lumbar CSF were measured following intraventricular ara‐C administration in two girls with refractory meningeal leukemia. CSF samples were collected with a permanent intrathecal‐lumbar catheter. In contrast to the comparatively short retention of ara‐C in the CSF (t1/2 1.8 to 2.9 hours), there was a high accumulation and an extremely long retention of ara‐CTP in the leukemic cells (t1/2 8.1 to 36 hours). The patients included in this study had an ara‐C‐resistant disease. No obvious relationship was seen between concentrations of ara‐C in the CSF and of ara‐CTP in the leukemic cells. Similar studies were performed after simultaneous intraventricular administration of hydrocortison and ara‐C. Hydrocortison did not increase ara‐CTP retention in the leukemic cells, nor did it effect CSF pleocytosis.
Radiotherapy enhances the toxicity of aminoglutethimide
Authors: Vanek Natalie , Hortobagyi Gabriel N. , Buzdar Aman U.
Published: February 2007

We report a case of radiotherapy‐enhanced aminoglutethimide skin toxicity in a patient with metastatic breast cancer. This patient was started on aminoglutethimide 6 days prior to radiation therapy, for painful bone metastasis. On day 7 of radiation therapy, she developed an extensive erythematous maculopapular rash over her face, trunk, and extremities. The rash was confluent over the radiation ports, both anteriorly and posteriorly. Aminoglutethimide was discontinued until completion of radiotherapy, and the rash resolved. Concomitant irradiation apparently enhanced the skin toxicity of aminoglutethimide or possibly aminoglutethimide had a radiosensitizing role in this patient.
Malignant mesothelioma with bone metastases
Authors: Cheng Wai‐Fung , Berkman Aron W.
Published: February 2007

Malignant mesothelioma has rarely been reported to metastasize to bone. This is a report of a 71‐year‐old man who presented with pleural mesothelioma. After a course of chemotherapy, the patient developed lower back pain. Plain X‐ray and bone scan were negative for metastases, but magnetic resonance imaging (MRI) revealed multiple metastatic lesions to the thoracolumbar vertebrae. Subsequent lumbar spine biopsy confirmed the presence of malignant mesothelioma. The patient then received radiation therapy. This report illustrates an unusual presentation for malignant mesothelioma and shows that, in addition, when bone metastases are clinically suspected, MRI may be a useful adjunct to more conventional imaging approach.
Gonadal function after 12‐Gy testicular irradiation in childhood acute lymphoblastic leukaemia
Authors: Castillo L. A. , Craft W. , Kernahan J. , Evans R. G. B. , Aynsley‐Green A.
Published: February 2007

Gonadal function was assessed in 15 boys with acute lymphoblastic leukaemia (ALL) who had received testicular irradiation. The dose to the testes was 12 Gy in 12, 15 Gy in 1, and 24 Gy in 2 cases. All of those who had received 12 or 15 Gy had normal Leydig cell function, although high levels of gonadotropins suggest subclinical Leydig cell damage. The 2 who had 24 Gy had Leydig cell failure. All who were old enough to produce a semen specimen were azoospermic.
Platinum‐based combination chemotherapy in advanced non‐small cell lung cancer: A randomized phase II trial of the cancer and leukemia group B
Authors: Green Mark R. , Kramar Andrew , Schilsky Richard , Stoopler Marc , Zimmer Bonnie , Ii Frederick Richards , Skarin Arthur , Anderson James R.
Published: February 2007

The Cancer and Leukemia Group B (CALGB) utilized a randomized phase II trial design to evaluate two cisplatin‐based combinations, cisplatin‐cytarabine (ara‐C) and cisplatin‐vinblastine, in 151 patients with advanced non‐small cell lung cancer. Patients entered on study had not received prior chemotherapy. Platinum doses were equivalent in the two treatment programs. The total response rate (complete response, partial response, regression of evaluable disease) for the cisplatin‐vinblastine group was 22% (16/73). Failure‐free survival at six months for this group was 41%, survival at six months was 63%. The cisplatin‐ara‐C group had a total response rate of 9% (7/78) with a failure‐free survival at six months of 14% and a six‐month survival of 47%. Severe or life‐threatening toxicity was seen in 73% of cisplatin‐vinblastine cases and 59% of cisplatin‐ara‐C patients. Neither regimen is active enough to warrant designation as “standard therapy” for patients with M1 disease.
Clear cell sarcoma of the kidney: Accuracy of imaging methods for detecting bone metastases. Report from the national Wilms' tumor study
Authors: Feusner James H. , Beckwith J. Bruce , D'Angio Giulio J.
Published: February 2007

To evaluate the accuracy of various imaging methods to screen for bone metastases in patients with clear cell sarcoma of the kidney (CCSK), all such cases reported on the National Wilms' Tumor studies from 1969–1983 were reviewed. Eighty‐two CCSK children were identified, 19 of whom (23%) sustained bone metastases sometime during their course. Thirteen metastatic sites were evaluated by both radiography and bone scanning. In seven instances both modalities were positive; in four the radiograph‐was positive but not the scan; and in only two (15%) was the scan positive in light of a negative radiograph. We recommend both imaging modalities in evaluating children with CCSK in order to detect all metastatic sites in all patients, and to further our observation that “hot” lesions on scintigraphy are more amenable to treatment than “cold” lesions.
Brain tumor as a second malignant neoplasm following neuroblastoma stage IV S
Authors: Ben‐Arush Myriam Weyl , Doron Yaffa , Braun Jacob , Mendelsson Eliane , Dar Hanna , Robinson Eliezer
Published: February 2007

A rare brain tumor (spongioblastoma polare) occurring 7 years after treatment of neuroblastoma stage IV S is reported. The literature concerning the occurrence of a second cancer in children exposed to mutagenic therapy for their initial tumor is reviewed, and genetic and environmental factors are discussed. Diminishing aggressiveness of the treatment in childhood cancer with good prognosis should be considered. Continuous follow‐up of children cured of cancer is warranted.
Fatal varicella infection in a child associated with thymoma and immunodeficiency (Good's syndrome)
Authors: Watts Raymond G. , Kelly David R.
Published: February 2007

An 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

The 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

Two 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

In 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

Prior 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

The 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

Ewing'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

Thirty‐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

Intensive 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

To 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

In 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.