bluuebunny/crossref_metadata_2025_split huggingface dataset:
Radionuclide therapy of neural crest tumors
Authors: Voǔte P. A. , Hoefnagel C. A. , De Kraker J. , Evans A. E. , Hayes A. , Green A. 
Published: February 2007

AbstractThe potential concentration of I 131‐Metaiodo‐benzylguanidine (MIBG) in pheochromocytoma, and the successful application in diagnosis and therapy of pheochromocytoma, has led to its use in therapy in other tumours derived from the neural crest. In neuroblastoma, the concentration of MIBG is as reliable as it is in pheochromocytoma. 18 patients with a neuroblastoma were treated, leading to two complete remissions, seven partial remissions, two no change, and two progressive disease; one patient was lost for follow‐up.Six adults were treated, three with a carcinoid, two malignant pheochromocytoma and one medullary thyroid carcinoma. Although follow‐up is still short, preliminary results of therapeutic use of I 131‐MIBG indicate that this treatment modality may be effective.
Treatment of neuroblastoma with 131I‐metaiodobenzylguanidine
Authors: Troncone L. , Riccardi R. , Montemaggi P. , Rufini V. , Lasorella A. , Mastrangelo R. 
Published: February 2007

AbstractSeven patients with neuroblastoma (six children and one adult) were treated with therapeutic doses of high specific activity 131l metaiodobenzylguanidine 131l‐MIBG). Six patients were in stage IV and unresponsive to conventional treatment. One patient, in stage III, was treated at diagnosis, an approach never previously reported.Single doses of 131l‐MIBG varying from 70 to 184 mCi split into two parts were administered by slow i.v. infusion (4 to 3 hours) at 2‐to 4‐day intervals. The following results were obtained in the six evaluable patients: two patients showed transient stabilization of the disease; three had an objective response, with shrinking of the primary tumor and/or regression of the metastatic lesions. Of these three patients, two suffered relapses at 2 and 7 months, respectively, from the first course of MIBG. The third patient, in whom the residual disease almost completely disappeared following MIBG therapy, is still alive in complete remission after autologous bone marrow transplantation with a follow‐up of 14 months. The single patient treated at diagnosis showed a dramatic response to a relatively low dosage of MIBG, with histologically proved disappearance of the tumor mass.Our data indicate that MIBG may be useful in the treatment of neuroblastoma unresponsive to conventional chemotherapy. The complete response observed in the patient treated at diagnosis suggests that the full potentiallty of MIBG therapy should be explored in untreated patients.
High‐dose melphalan, vincristine, and total‐body irradiation with autologous bone marrow transplantation in children with relapsed neuroblastoma: A phase II study
Authors: Pinkerton C. R. , Philip T. , Biron P. , Frapazz D. , Phillipe N. , Zucker J. M. , Bernard J. L. , Philip I. , Kemshead J. , Favrot M. 
Published: February 2007

AbstractSeven children with neuroblastoma who had relapsed on or after conventional therapy (3 originally stage IV, 3 stage III, 1 stage II) were entered on a study of “massive therapy” with purged autologous bone marrow rescue. In 5 patients attempts were made to reinduce remission with alternative chemotherapy, and a partial or complete response was achieved in 3. The massive therapy regimen comprised melphalan, vincristine, and total‐body irradiation. Of 6 patients with measurable disease, all showed objective response to high‐dose therapy (5 partial, 1 complete remission), but the median duration of remission was only 5 months (range 1/2 to 10). One patient remains disease‐free at 18 months post graft. This patient was the only one treated in second complete remission. These data confirm the high response rate achieved by high‐dose melphalan, total‐body irradiation regimens, but it appears unlikely that a single high‐dose chemoradiotherapy procedure will cure patients after relapse, particularly if they are unresponsive to conventional salvage regimens. Such protocols may, however, have a role as consolidation in first remission. The use of double‐autograft procedures is an alternative that warrants further investigation in patients with relapsed neuroblastoma.
Long‐term sequelae of cancer treatment on the central nervous system in childhood
Authors: Packer Roger J. , Meadows Anna T. , Rorke Lucy B. , Goldwein Joel L. , D'Angio Giulio 
Published: February 2007

AbstractIncreasing numbers of children with cancer, including those with acute lymphocytic leukemia and medulloblastoma, are experiencing long‐term disease control. As survival increases, so does the recognition that the treatment used to prolong survival may have significant detrimental effects on the central nervous system (CNS). Because of the slow replication rate of most constituents of the CNS, these effects tend to be delayed. Radiotherapy, and to a lesser extent, chemotherapy (primarily methotrexate) have been implicated in the causation of such sequelae. The pathogenesis of CNS damage is only partially understood and evidence suggests that direct effects on intracranial endothelial cells and brain white matter and immunologic mechanism play a role. A spectrum of clinical syndromes may occur, including radionecrosis, necrotizing leukoencephalopathy, mineralizing microangiopathy with dystrophic calcification, cerebellar sclerosis and spinal cord dysfunction. The two most common forms of sequelae are neuropsychological and neuroendocrinologic damage. The frequency, degree of and etiology of neurocognitive dysfunction is less than completely elucidated. Radiotherapy has been implicated as the major cause of damage, but the relationship between radiotherapy and the type of damage caused and the volume and dose of radiotherapy and degree of cognitive damage is unclear. Cognitive deficits are progressive in nature. Younger children are more likely to suffer the severest damage; but no patient of any age is free of risk of damage. Growth hormone impairment is the most common form of neuroendocrinologic dysfunction. There is increasing evidence that children with cancer who are long‐term survivors are at increased risk for the development of secondary CNS tumors; possibly due, in part, to previous treatment. Much work needs to be done to characterize the sequelae which may occur, develop means of earlier detection, investigate ways to ameliorate sequelae and devise less toxic treatment.
Unusual skin rash following withdrawal of oral 6‐mercaptopurine in children with leukemia
Authors: Kirk Judy A. , Rogers Maureen , Menser Margaret A. , Bergin Mary , Dalla‐Pozza Luciano , Stevens Michael M. 
Published: February 2007

AbstractSixteen episodes of a distinctive, papular rash occurred in eight patients following withdrawal of 6‐mercaptopurine (6MP) and methotrexate (MTX) used as maintenance therapy for acute lymphoblastic leukemia (ALL). The rash also developed in one of the eight patients when only 6MP was discontinued. The eruption occurred mainly on the face, and in this site resembled the perioral dermatitis seen following withdrawal of topical fluorinated steroids. The rash generally began within 3 weeks of stopping 6MP and lasted 3 to 4 weeks. It failed to improve with the use of topical corticosteroid. We conclude that this rash is caused by the withdrawal of oral 6MP.
Hematologic and clinical features of patients with chromosome 5 monosomy or deletion (5q)
Authors: Brusamolino Ercole , Orlandi Ester , Morra Enrica , Bernasconi Paolo , Pagnucco Guido , Colombo Anna , Lazzarino Mario , Bernasconi Carlo 
Published: February 2007

AbstractThis paper analyzes the hematologic features and outcome of 13 patients with chromosome 5 abnormalities (monosomy 5 or deletion of 5q), either isolated or with additional anomalies. Among four patients with isolated del (5q), two had a stable refractory macrocytic anemia with thrombocytosis (5q‐syndrome). All nine patients with complex karyotypes had acute leukemia or refractory anemia with excess of blasts in acute transformation; two cases were TdT‐positive, with a lymphoid or a mixed phenotype. In seven patients, preleukemia preceded overt leukemia, and in six, a prior therapeutic, or occupational exposure to mutagens/carcinogens had occurred. Additional chromosome 7 abnormalities were seen in four cases. The median survival of patients with complex karyotypes was 19 months from the time of diagnosis of the hematologic disorder and 5 months from the time of identification of the chromosome 5 abnormality. Pathogenetic implications of the chromosome 5 monosomy or del (5q) through a proto‐oncogene activation and the putative hemopoietic stem cell involvement in a clonal disease are discussed.
Fine‐needle aspiration biopsy in children: Experience in 70 cases
Authors: Verdeguer Amparo , Castel Victoria , Torres Vicente , Olagüe Ramon , Ferris Josep , Esquembre Carlos , Vallcanera Amparo , Muro M. Dolores 
Published: February 2007

AbstractResults of 70 fine‐needle aspiration biopsies (FNAB) were evaluated retrospectively in 61 pediatric patients. Over a period of 9 months all mass lesions suspected being malignant were aspirated. Twelve of the 70 aspirations were performed in children having known tumours, in order to exclude recurrence or metastasis. The others were carried out to obtain a diagnosis. Satisfactory specimens were obtained from 58 (83%). There were 21 benign diagnoses, 36 malignant diagnoses, and 1 with suspected malignancy. Correlation of histologic and cytologic diagnoses was possible in 45 cases. The diagnostic sensitivity and specificity were 95% and 80%, respectively. We have found FNAB more accurate in the diagnosis of malignancies than in benign lesions. The results suggest that this is a useful technique for obtaining a first diagnosis of malignancy, as well as for excluding recurrence or metastatic disease.
Heterogeneity of immunological patterns in Langerhan's histiocytosis and response to crude calf thymic extract in 11 patients
Authors: Ceci Adriana , De Terlizzi Marino , Tbma Maria Grazia , Calculli Giovanni , Caputo Ruggero , Castello Manuel , Indolfi Paolo , Rosati Domenico 
Published: February 2007

AbstractResponse to treatment with daily intramuscularly administered crude calf thymic extract (Suppressin) in 11 patients with Langerhan's histiocytosis (L.H.) is reported.In ten patients, T‐lymphocytic subsets were studied before starting immunotherapy: OKT3 positive and OKT4 positive cells were reduced in four patients; OKT8 positive cells were reduced in two patients; three patients were normal. After immunotherapy, one patient entered complete remission, four patients had stationary disease, and six had marked clinical progression.Subsequently eight patients underwent conventional chemotherapy, and only three entered complete remission.This study has demonstrated the heterogeneity of immunological patterns in L.H. and justifies the necessity for investigations on the immunoregulatory mechanism of L.H.
Rhabdomyosarcoma presenting with diffuse bone marrow involvement, hypercalcemia and renal failure
Authors: De La Serna Francisco Javier , Martinez Miguel Angel , Valdes Maria Dolores , Hornedo Javier , Mestre Maria Jose , Morales Jose Maria 
Published: February 2007

AbstractRhabdomyosarcoma and other small round‐cell neoplasms of infancy and childhood frequently involve the bone marrow. An unusual clinical presentation of diffuse bone marrow involvement as the sole manifestation of the disease represents a difficult diagnostic challenge. We report the case of an 18‐year‐old boy presenting with lower back pain, sternum tenderness, anemia, thrombocytopenia, leukoerythroblastic blood film, hypercalcemia, and renal failure. No solid tumor was found.A diagnosis of rhabdomyoblastic bone marrow metastatic involvement was initially suggested by the morphological picture, and later confirmed by the ultrastructural findings. Hypercalcemia and renal failure were controlled, initially, with furosemide and calcitonin. The patient was treated with intensive chemotherapy, including vincristine, actinomycin D, cyclophosphamide, and doxorubicin. A remission was achieved, lasting 5 months until relapse.
The application of a two‐stage design for clinical trials in patients with recurrent head and neck cancer
Authors: Eisenberger Mario A. , Ellenberg Susan , Leyland‐Jones Brian , Friedman Michael 
Published: February 2007

AbstractCytotoxic chemotherapy produces modest benefits for patients with recurrent and metastatic squamous cell carcinoma of the head and neck (SCCHN). Prospective randomized clinical trials have failed to demonstrate unequivocal superiority of aggressive multidrug regimens over single agents. Despite this, phase II trials frequently result in encouraging preliminary observations that compare favorably to historical single‐agent data. While providing for a useful method of screening for anti‐tumor activity, phase II studies have limited use in determining the relative value of a new treatment program. Results of phase II studies are considerably influenced by patient selection factors and criteria used to establish therapeutic benefits (responses). Furthermore, estimations of true levels of efficacy (response rates) are dependent on sample sizes, which are usually limited in such trials.We propose that newly developed combinations containing at least one known active agent in this disease should be tested in a controlled setting after their toxicity pattern has been well established. The conduct of the usual phase II study in these situations will probably not provide useful new information, since responses are likely to be observed. We describe a two‐stage design applied to terminate a trial if at the first stage there is no evidence of improvement over the control arm. This method allows for early termination of studies involving relatively inefficient treatment regimens and, at the same time, continuation of those with a high likelihood to result in significant therapeutic improvements over a control arm. Loss of power is negligible and sample sizes can be reduced significantly. The rationale behind this method and its simplicity are attractive features for a widespread application for new drug development strategies in this and other diseases.
Medical students' attitudes towards cancer: Influence of the type of clerkship experience
Authors: Hays Daniel M. , Hoffman Kaaren I. , Williams Kenneth O. , Siegel Stuart E. , Miller Robert 
Published: February 2007

AbstractAttitudes of third‐year medical students relative to patients with cancer were assessed prior to and following three different 6‐week pediatric clerkships. Clerkship I (N = 139) was conducted in a public hospital where student contact with all patients occurred in wards, supervised by pediatricians in all subspecial‐ties. Clerkship II (N = 146) was in a pediatric hospital which is a cancer center. Contact with patients with cancer was directed by oncologists and nononcologists. Clerkship III (N = 31) was similar to II, with an exposure to an oncology ward. A 51‐item instrument, employing a visual analog scale, which included general, adult, and pediatric items, was used (pre‐ & postclerkship) to evaluate attitudes in all 316 students and 49 faculty instructors associated with the clerkships.Factor analysis of student responses to pediatric items resulted in four factors: a) early diagnosis, b) aggressive therapy, c) long‐range surveillance, and d) psychosocial support in reference to patients with cancer. Post‐test differences between clerkship groups in items a and b were noted, with increasing positivity correlated with increased exposure to oncology and oncologists. Attitudes of faculty non‐oncologists differed significantly from those of oncologists irrespective of institutional affiliation.
Diagnosis of neuroblastoma metastasis in bone marrow with a panel of monoclonal antibodies
Authors: Sugimoto Tohru , Sawada Tadashi , Matsumura Takafumi , Kemshead John T. , Horii Yoshihiro , Saida Tohru , Imashuku Shinsaku , Takeuchi Yoshihiro 
Published: February 2007

AbstractNeuroblastoma, along with rhabdomyosarcoma, Ewing's sarcoma, and acute lympho‐blastic leukemia/lymphoma, is one of the small, round‐cell tumors of childhood. All of these malignancies show a propensity to me‐tastasize to bone marrow. Occasionally when the clinical picture is unclear and the tumor is particularly anaplastic, it can be difficult to arrive at a diagnosis by conventional histological and biochemical procedures.In the present study, a panel of nine monoclonal antibodies was used to undertake a detailed analysis of seven bone marrows contaminated with tumor cells: six cases of stage IV neuroblastoma, and one case of stage IV‐S neuroblastoma. The antibody profiles obtained were compared with those deduced from the studies of over 20 marrows from patients with acute lymphoblastic leukemia. A comparison of these data with those obtained from the studies of rhabdomyosar‐coma and Ewing's sarcoma cell lines and tissues suggests that when high levels of tumor cells are present in the marrow, it is possible to obtain a confident diagnosis of either neuroblastoma or acute lymphoblastic leukemia. In addition, the immunocytological identification of neuroblasts in bone marrow enables accurate staging without histological examination.
The syndrome of inappropriate antidiuretic hormone secretion with carcinoma of the tongue
Authors: Abdi Ehtesham A. , Bishop Sandra 
Published: February 2007

AbstractA 64‐year‐old male developed symptomatic recurrent syndrome of inappropriate ADH secretion in association with locally advanced squamous cell carcinoma of the tongue. His SIADH was managed successfully with saline infusion, frusemide, and maintenance treatment with demeclocycline.
Prepubertal endocrine follow‐up in subjects with Wilms' tumor
Authors: Perrone Laura , Sinisi Antonio A. , Sicuranza Romolo , Di Tullio Maria T. , Indolfi Paolo , Giuliano Maria G. , Bellastella Antonio , Faggiano Michelangelo 
Published: February 2007

AbstractTwenty‐three prepubertal subjects treated for Wilms' tumor (10 males and 13 females) were endocrinologically evaluated off therapy from 0.5 to 4.08 years. They were divided into two groups: 11 subjects (6M, 5F) who had received chemotherapy only (group 1) and 12 (4M, 8F) who had in addition received abdominal radiation (1,500–3,000 rads) (group 2). Follicle‐stimulating hormone (FSH), luteinizing hormone (LH), prolactin (PRL), thyroid‐stimulating hormone (TSH), free thyroxine (FT4), free tri‐iodo thyronine (FT3), testosterone (T), estradiol‐17β (E2), and cortisol (F) were measured by radioimmunoassay (RIA). Plasma levels of TSH, PRL, FT4, FT3, and F were normal in both groups, as were FSH, LH, T, and E2 in group 1. In group 2, female subjects showed FSH levels significantly higher than controls, while LH and E2 were normal; male subjects showed significantly higher LH levels, while FSH and T levels were normal. These results indicate that in the treatment protocol used by us for Wilms' tumor (WT), chemotherapy does not affect endocrine function, whereas abdominal radiation seems to damage gonadal function directly. The present findings indicate that gonadal damage may be revealed in WT before puberty not only in females, as has been previously reported, but also in male subjects.
Radiation therapy in soft‐tissue lesions in histiocytosis X (Langerhans' cell histiocytosis)
Authors: Gramatovici Razvan , D'Angio Giulio J. 
Published: February 2007

AbstractLangerhans' cell histiocytosis (LCH) (previously histiocytosis X) is an infrequent disease with protean clinical manifestations and an unpredictable course.The role of radiotherapy in the soft tissue complications of LCH was evaluated in this retrospective study of 40 patients seen in two major Philadelphia institutions between 1970 and 1984. The patients were divided into two groups according to the extent of disease: unifocal versus multifocal. There was a higher rate of complete response in the unifocal form (4 of 12 patients), but this was associated with a high rate of spontaneous remissìons (6 out of 12 patients). Multifocal LCH has a more aggressive course, and the major organs involved (lung, liver, and spleen) did not respond to radiotherapy. Eight patients had diabetes insipidus, and none of them responded to radiotherapy or chemotherapy. Autopsies were performed in two patients with diabetes insipidus who died of disease. There were no pathologic changes in the pituitary gland or stalk, hypothalamus, or supraoptic nuclei.
Poland's syndrome with acute lymphoblastic leukemia in an adult
Authors: Parikh P. M. , Karandikar S. M. , Koppikar S. , Pahuja R. , Charak B. S. , Saikia T. , Gopal R. , Advani S. H. 
Published: February 2007

AbstractWe report a patient with Poland's syndrome who developed acute lymphoblastic leukemia (ALL) at 28 years of age. This is the first time that such an association has been reported from outside the United States or in an adult. The relevant literature is reviewed.
Educational achievement of long‐term survivors of childhood and adolescent cancer
Authors: Kelaghan Joseph , Myers Max H. , Mulvihill John J. , Byrne Julianne , Connelly Roger R. , Austin Donald F. , Strong Louise C. , Meigs J. Wister , Latourette Howard B. , Holmes Grace F. , Holmes Frederick F. 
Published: February 2007

AbstractIn a retrospective cohort study, the level of education attained by 2,283 long‐term survivors of childhood and adolescent cancer was investigated and compared with that of 3,270 sibling controls. Survivors of central nervous system tumors were significantly less likely than controls to complete eight grades of school or, if they completed high school, to enter college. No significant differences in educational achievement were found for survivors of non‐central nervous system cancers. The educational deficit of survivors of brain tumors was especially striking for tumors of the ventricles or cerebral hemispheres, and the deficit was more severe for those treated with radiation therapy than by surgery alone. Early age at diagnosis of a central nervous system tumor was associated with a larger educational deficit than late age at diagnosis. These findings are reassuring for the majority of long‐term survivors of childhood and adolescent cancers given therapies used prior to 1975.
Marginal analysis applied to the dose‐response curve
Authors: Silber Jeffrey H. , Kaizer Herbert 
Published: February 2007

AbstractTo improve on drug dosage decisions, the method of “marginal analysis,” traditionally found in the economics literature, is applied to the dose‐response curve. Marginal analysis is first defined and explained, next the technique is redefined for the clinical setting of dose determination subject to drug efficacy and side effects, and finally an example using adjuvant adriamycin chemotherapy in osteo sarcoma is presented. By not using “marginal analysis,” clinicians may not be maximizing overall therapeutic success or patient survival. Implications for future clinical trials are discussed, and a suggestion for estimating dose‐response is proposed. The technique described should be readily applicable to many dosing problems, and need not be restricted to chemotherapy dosing decisions.
Infantile myofibromatosis causing biliary and pancreatic obstruction: A case report
Authors: Marks M. K. , Dewan P. A. , Stokes K. B. , Smith A. L. , McKelvie P. 
Published: February 2007

AbstractA case of obstructive jaundice caused by a solitary myofibroma in an 18‐week‐old infant is presented. Preoperative investigations demonstrated total biliary and partial pancreatic obstruction, although no obstructing lesion was identified radiologically. At operation a 3‐cm solitary myofibroma was found in the head of the pancreas. Because of the morbidity associated with resection at this site, a bypass procedure was performed in the hope that the lesion will regress.