bluuebunny/crossref_metadata_2025 huggingface dataset:
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

Rhabdomyosarcoma 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

Cytotoxic 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

Attitudes 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

Neuroblastoma, 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

A 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

Twenty‐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

Langerhans' 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

We 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

In 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

To 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

A 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.
Testicular adhesion: A potential complication from wedge testicular biopsy in childhood leukemia
Authors: Chan Ka Wah , Wood B. J. , Johnson H. W.
Published: February 2007

Elective bilateral wedge testicular biopsy is frequently performed in childhood acute lymphoblastic leukemia. Acute and delayed complications of this procedure are rarely encountered. We report on two children with testicular adhesions 16 and 20 months post‐surgery. In each instance there were resultant diagnostic and management difficulties. The possible cause and potential side effects of testicular adhesions are discussed.
Poor‐risk non‐lymphoblastic lymphoma of childhood: Results of an intensive pilot study
Authors: Finlay Jonathan L. , Trigg Michael E. , Link Michael P. , Frierdich Sharon
Published: February 2007

Children with “poor‐risk” nonlymphoblastic lymphoma, especially those with marrow or nervous system (CNS) involvement at presentation, have fared poorly even on aggressive chemotherapy regimens. We report here the results of a pilot study of 30 children treated with a highly intensive chemotherapy regimen. This regimen includes an intensive Induction Phase consisting of three cycles of CHOP therapy (cyclophosphamide, doxorubicin, vincristine, and corticosteroids) as well as intensive intrathecal therapy with each cycle. This is followed by a CNS Consolidation Phase consisting of a single cycle of CHOP therapy with five intrathecal doses of “triple” chemotherapy (methotrexate, cytosine arabinoside, and hydrocortisone). Thereafter, a Maintenance Phase consists of alternating cycles of 1) cytosine arabinoside and 6‐thioguanine, 2) oral methotrexate and VP‐16, and 3) CHOP, for a duration that varied from 36 to 72 wk. Neither debulking surgery nor radiation therapy were recommended. There were 20 patients with Stage III disease (St. Jude's Staging System) and an additional ten patients with bone marrow and/or CNS involvement. The latter group included six patients with B‐cell leukemia, three of whom also had CNS disease at presentation. Two additional patients had CNS disease without marrow involvement.

Twenty‐nine of 30 patients achieved a complete response. Six patients died with recurrent or progressive disease. Twenty‐three patients are alive without any adverse events between 21 and 65 mo after diagnosis, with the median time of survival not yet reached (at least 32 mo). All seven adverse events occurred within 7 mo of diagnosis. Event‐free survival for all patients is 77%, for Stage III patients is 80%, and for patients with marrow and/or CNS involvement is 70%.

This pilot study offers encouragement for improvement in the prognosis of children with “poor‐risk” nonlymphoblastic lymphoma and merits evaluation in a Phase III randomized trial in the multicenter cooperative group setting.
Ovarian function following chemotherapy for childhood brain tumours
Authors: Clayton P. E. , Shalet S. M. , Price D. A. , Jones P. H. Morris
Published: February 2007

Pubertal development, basal gonadotrophin, and oestradiol levels were assessed in 21 girls who had received neuroaxis irradiation for a brain tumour followed by adjuvant chemotherapy with carmustine (BCNU) or lomustine (CCNU) and procarbazine. Thirteen received chemotherapy before the age of 11 years. Ten remained prepubertal at their last assessment, nine of whom showed biochemical evidence of primary ovarian failure. The remaining three were pubertal or adult, and although basal folliclestimulating hormone (FSH) and luteinising hormone (LH) levels were normal, all had shown abnormalities of gonadotrophin secretion previously. Eight girls received chemotherapy after 11 years of age. Only three girls exhibited an elevated basal FSH level or exaggerated FSH response to GnRH. Elevated basal FSH values had been noted previously in two of the other five girls.

All girls entered or progressed through puberty spontaneously. Seven experienced menarche at an appropriate age. However in four, gonadotrophin levels, which had been elevated, were now within the normal range. In two, menses had continued throughout with normal midfollicular oestradiol levels, whilst the other two developed secondary amenorrhoea associated with radiation‐induced gonadotrophin deficiency.

The majority of girls showed evidence of primary ovarian dysfunction. This did not prejudice pubertal development or the timing of menarche. Ovarian function may return to normal in the years after treatment, indicating a potential for fertility.
Malignancy in the neonate
Authors: Crom Deborah B. , Wilimas Judith A. , Green Alexander A. , Pratt Charles B. , Jenkins Jesse J. , Behm Fred G.
Published: February 2007

From January 1962 to July 1988, 34 infants less than 29 days of age who had cancer were seen at St. Jude Children's Research Hospital (SJCRH). The malignancies in this group consisted of 19 neuroblastomas, 6 leukemias, 3 retinoblastomas, 2 Wilms' tumors, 2 melanomas, and 2 teratomas. Twenty‐three patients (68%) are alive and free of disease 2 months to 24 years after diagnosis. We reviewed the presentation and initial symptoms, pathology reports, patient population, associated anomalies, potential genetic influences, and possible perinatal factors. The most common initial symptom was an enlarging abdomen or abdominal mass. Pathological findings were occasionally difficult to interpret; five additional infants who were referred to us did not have malignancies. There was no increased incidence of associated anomalies or perinatal insults. The only genetic factor was retinoblastoma in one parent of each infant diagnosed as having retinoblastoma. The possible etiology of neonatal tumors is discussed.
Pulmonary function in childhood leukaemia survivors
Authors: Shaw N. J. , Tweeddale P. M. , Eden O. B.
Published: February 2007

Little is known of pulmonary function in survivors of acute lymphoblastic leukaemia (A.L.L.); this is despite the fact that some drugs used, most notably methotrexate, have well‐recorded pulmonary toxicity, and the most common infections during therapy in most series are of upper and lower respiratory tract.

As part of a survey of all cancer survivors attending the Royal Hospital for Sick Children in Edinburgh, 38 leukaemic patients, who had completed treatment 3 months to 14 years 6 months (median 6 years and 8 months) prior to survey were assessed with regards to their respiratory status. Each patient completed a questionnaire and had spirometry and lung volumes measured; 30 patients additionally had transfer factor for carbon monoxide (TCO) measured. There were 21 children, 11 adults, and 6 patients in the age range between child and adult. Of the 26 adults and children studied with complete data available, 17 (65%) had one or more low values for vital capacity (VC), total lung capacity (TLC), residual volume, or TCO. Mean VC, TLC and TCO were significantly lower than the mean of the predicted values (P < .001). Gas transfer per unit lung volume (KCO) was normal in all cases. Few patients had symptoms of respiratory disease. There was an increased incidence of low TCO in patients diagnosed under 8 years of age. Impairment of lung growth could be a contributing factor to the observed abnormalities in pulmonary function. Impairment of pulmonary function in survivors of A.L.L. may be of significance for them in later life.
Very late recurrence of childhood acute lymphoblastic leukemia treated with chemoimmunotherapy: A report of three cases occurring 19, 11, and 9 years after discontinuation of chemotherapy
Authors: Salloum Emile , Pico José Luis , Herait Patrice , Bayle Chantal , Ghosn Marwane , Moran Angel , Friedman Samuel , Hayat Marcel
Published: February 2007

Current therapeutic modalities for childhood acute lymphoblastic leukemia (ALL) are associated with a high cure rate, and recurrences more than 4 years after therapy cessation are very unusual. We report three cases of exceptionally late recurrences of childhood ALL after cessation of chemotherapy (CT) given for respective periods of 8, 7, and 24 months. CT was followed by maintenance immunotherapy (IMT) with Bacillus Calmette‐Guérin (BCG) and allogeneic leukemic lymphoblasts pretreated with formaldehyde or irradiated in vitro. Leukemic recurrences were observed 19, 11, and 9 years after cessation of CT and appeared morphologically similar to the original blasts. A second complete remission was easily achieved in all three patients, but two went on to repeated relapse (one has died following the fourth recurrence). We speculate that some residual leukemic cells, remaining after the inadequate, short‐term CT, were responsible for these unusual evolutions, and we question a possible delaying role of IMT in prolonging remission. Other possible etiologies are discussed.
Mediastinal germ cell tumor in a young woman
Authors: Brown Kathleen , Collins James D. , Batra Poonam , Steckel Richard J. , Kagan A. Robert
Published: February 2007

Primary mediastinal germ cell malignancies are uncommon neoplasms, usually presenting in young men. We review a case of a primary mediastinal seminoma occurring in a 23‐year‐old woman. The differential diagnosis, work‐up and therapy for these lesions is discussed with emphasis on the role of magnetic resonance imaging in the evaluation of anterior mediastinal masses.
Malignant histiocytosis: Therapeutic results in 27 children treated with a single polychemotherapy regimen
Authors: Brugieres L. , Caillaud J. M. , Patte C. , Rodary C. , Bernard A. , Kalifa C. , Hartmann O. , Lemerle J.
Published: February 2007

Twenty‐seven children with histologically proven malignant histiocytosis were treated in the same institution from January, 1975 to December, 1986 with a combination chemotherapy regimen containing vincristine, cyclophosphamide, doxorubicine, and prednisone. Twenty‐two patients achieved complete remission, one partial remission, and four no remission. Eight patients relapsed and were treated with Lomustine (CCNU), vinblastine, and bleomycin. In seven cases, a second complete remission was obtained. The overall survival rate is 81% at 5 years and the relapse‐free survival rate is 54.5% at 5 years. Prognostic factors were fever and age under 10 at diagnosis, which were correlated with a higher incidence of relapse or no remission.
Phase II study of N‐methylformamide, spirogermanium, and 4‐demethoxydaunorubicin in the treatment of non‐small cell lung cancer (EST 3583): An Eastern cooperative oncology group study
Authors: Ettinger David S. , Finkelstein Dianne M. , Donehower Ross C. , Chang Alex Y. C. , Green Michael , Blum Ronald , Hahn Richard G. , Ruckdeschel John C.
Published: February 2007

One hundred forty‐four patients with non‐small cell lung cancer, the majority (72%) of whom had received previous chemotherapy, were evaluable in this randomized phase II study of N‐methylformamide (N‐MF), spirogermanium, and 4‐demethoxydaunorubicin. There were two partial responses, one each with spirogermanium and 4‐demethoxydaunorubicin. There were eight life‐threatening complications (mostly hematologic) and two lethal complications (N‐MF, hematologic; 4‐demethoxydaunorubicin, gastrointestinal). The overall survival ranged from 9 days to 533 days with a median of 17.6 weeks. The following factors were associated with poor survival: Poor initial performance status, prior weight loss, presence of liver or subcutaneous metastases.
An animal model to detect the neuropsychological toxicity of anticancer agents
Authors: Yanovski Jack A. , Packer Roger J. , Levine Joel D. , Davidson Terry L. , Micalizzi Marlynne , D'Angio Giulio
Published: February 2007

The unexpected discovery that certain chemotherapeutic agents used in the treatment of childhood cancers have neurocognitive side effects has prompted a search for techniques that identify those medications that place children at risk. An animal model for the assessment of resultant neurocognitive toxicity is described which makes use of simple classical conditioning. We have shown that rats learn about environmental events more slowly following neonatal administration of methotrexate. The changes after methotrexate exposure are not related to stimulus characteristics or to perceptual abilities, but rather to damage to the neural systems involved in acquisition, retention, or recall. Similar problems with learning have been observed in children treated with methotrexate. An effective animal model such as the one described here may help detect and avoid antineoplastic agents that produce severe cognitive defects in childhood cancer patients.
Feasibility study for neonatal neuroblastoma screening in the United States
Authors: Tuchman Mendel , Fisher Edward J. , Heisel Margaret A. , Woods William G.
Published: February 2007

Urine was obtained from infants born at two large metropolitan Minneapolis/St. Paul, Minnesota hospitals to determine whether normal levels of urinary catecholamine metabolites in neonates can be used for subsequent neuroblastoma screening as well as to determine compliance with collecting such urine in a voluntary program. The results suggest that there are wide variations in raw values of urinary creatinine, homovanillic acid, and vanillylmandelic acid through the first 4 days of age, apparently because of maternal creatinine and catecholamine metabolite influences. Such maternal influences appear negligible by 3 weeks of age. Furthermore, the study suggests that voluntary compliance in the United States with collecting urine at home without rigorous public education and/or methods for reminding parents may be very poor.
Childhood acute lymphoblastic leukemia: Results of the austrian cooperative study group with the all a 84 protocol
Authors: Gruemayer Eva Renate , Gadner Helmut , Mutz Ingomar , Urban Christian , Ausserer Bernd , Grienberger Heinz , Juergenssen Olaf , Koeller Ursula , Mueller Giudo , Panzer Simon , Ploier Robert , Stoellinger Olaf , Messner Heimo , Tulzer Wilhelm
Published: February 2007

We prospectively treated 127 children with ALL with a risk‐adapted regimen. All patients received the identical induction‐consolidation therapy. The early maintenance included intermediate dose methotrexate in patients with standard risk (n = 79) and medium risk (n = 39). In addition patients with high risk (n = 6) received high dose ARA‐C followed by L‐asparaginase. Intensification treatment and prophylactic cranial irradiation was also tailored according to the risk group. Treatment duration was 2 years. Complete remission was achieved in 97.6% of all patients. Treatment‐related toxicity accounted for one death in complete remission. The probability of event‐free survival (pEFS) for the combined group was 72% at a median follow‐up of 42 months. The pEFS was higher in patients with standard risk (SR) than in patients with medium risk (MR) (80% versus 65%; p<0.05) at 30 months, but attenuated in the follow‐up evaluation at 42 months (76% versus 63%; p<0.1). The number of high‐risk patients was too small for statistical evaluation. Relapse within the first 18 months after diagnosis indicated a poor prognosis and was more common in patients with MR than in patients with SR. The immunophenotype of the leukemic cells was not found to constitute an independent risk factor after treatment has been risk‐adapted. Patients with an initial white blood cell count of more than 50 × 109/l had a worse prognosis than patients with a lower white blood cell count (p<0.01).
Familial erythrophagocytic lymphohistiocytosis: Late relapse despite continuous high‐dose VP‐16 chemotherapy
Authors: Ware Russell , Friedman Henry S. , Kinney Thomas R. , Kurtzberg Joanne , Chaffee Sara , Falletta John M.
Published: February 2007

Familial erythrophagocytic lymphohistiocytosis (FEL) is a rare disease of infancy and early childhood. Transmitted in an autosomal recessive manner, FEL is characterized by infiltration of lymphocytes and histiocytes into visceral organs, the bone marrow, and the central nervous system (CNS) [1,2,3]. Epipodophyllotoxin (VP‐16) is currently the most effective chemotherapeutic agent against FEL; complete clinical remissions have been attained with this agent alone [4,5]. Prolonged remission has been achieved in only a few patients, usually those in whom aggressive systemic and CNS therapy was initiated early in the course of the disease [6]. For these children, the necessary duration of therapy has not been defined.
Diagnostic and therapeutic problems of soft tissue tumors other than rhabdomyosarcoma in infants under 1 year of age: A clinicopathological study of 34 cases treated at the institut gustave‐roussy
Authors: Salloum Emile , Flamant Françoise , Caillaud Jean M. , Friedman Samuel , Wacker Pierre , Coppes Max J. , Brugieres Laurence , Lemerle Jean
Published: February 2007

Thirty‐four cases of soft tissue tumors (STT) other than rhabdomyosarcoma in infants under 1 year of age were seen in our institution between 1955 and 1985. All were diagnosed initially as malignant tumors except for three cases of fibromatosis, and, thus, they received therapy appropriate at that time. During a recent pathologic review, four were seen to have had hemangioma, six hemangiopericytoma, one hamartoma, seven fibromatosis, eight fibrosarcoma, and eight unclassified sarcomas. Of these 34 cases, the initial histological diagnosis was confirmed in only 17. Sixteen patients were believed to have received inappropriately aggressive therapy; indeed, four of these cases diagnosed prior to 1975 had been benign. Major long‐term side effects were seen in 7/16 treated patients (six radiotherapy, one surgery), who, in retrospect, should have received less aggressive treatment.

The reasons for these initial errors in diagnosis included doubtful histologic features associated in some cases with alarming clinical presentation. We believe that difficulties still remain in distinguishing benign from malignant STT in this age group by histological examination. Therefore, we urge extreme caution in using aggressive therapy before the diagnosis is certain.
Role of chemotherapy in pediatric pulmonary blastoma
Authors: Ozkaynak M. Fevzi , Ortega Jorge A. , Laug Walter , Gilsanz Vicente , Isaacs Hart
Published: February 2007

Pulmonary blastoma is a rare malignant tumor of the lung that has been treated primarily with surgery. The effect of combination chemotherapy has not been systematically investigated. Two pediatric cases are reported in whom combination chemotherapy consisting of vincristine, actinomycin‐D, cyclophosphamide, cis‐platinum, and adriamycin was successfully used. The first case, a 5‐year‐old boy, underwent incomplete surgical excision of the tumor followed by a 104‐week course of combination chemotherapy. The second case is a 3‐year‐old boy who was initially treated with combination chemotherapy that resulted in an objective response; he subsequently underwent surgical excision. This intensive combination chemotherapy is effective both in inoperable tumors as initial therapy as well as a surgical adjuvant.
Can primary cancer of the liver in western countries be prevented? Pediatric point of view
Authors: Perilongo Giorgio , Pontisso Patrizia , Basso Giuseppe
Published: February 2007

The role of hepatitis B virus (HBV) in the etiology of hepatocellular carcinoma (HCC) in Western countries is uncertain in spite of the large amount of data demonstrating an etiologic relationship between HBV and HCC in endemic areas [1]. This paper will discuss the epidemiology of HBV in HCC and will raise several questions about the conventional wisdom of maintaining a connection between the two.
Pretreatment liver biopsy in 20 children with histiocytosis X: A clinicopathologic correlation
Authors: Heyn Ruth M. , Hamoudi Ala , Newton William A.
Published: February 2007

Liver biopsies were done on 20 patients with histiocytosis × (HX) as part of pretreatment evaluation prior to entry on two Childrens Cancer Study Group protocols. Seventeen patients had hepatomegaly, and seven had one or more abnormal laboratory parameters using Lahey's criteria for liver dysfunction. Nineteen of 20 specimens showed various abnormalities of the portal triads. A single biopsy revealed normal liver. Among the changes were triaditis, bile duct proliferation, variable fibrosis with histiocytic infiltrates, and cirrhosis. One patient had typical granulomas of HX within the liver parenchyma in addition to portal triaditis.

Patients with larger livers and dysfunction tended to show more marked histologic abnormalities in the portal triads. However, correlations among liver size, function, and pathology showed considerable overlap. Early death among these patients was more likely to be associated with progressive HX in other sites and/or infection. Death from cirrhosis and liver failure per se occurred in one patient 4 years after initial biopsy, but five other children had evidence of cirrhosis on biopsy or at autopsy. The majority of patients with triaditis initially did not have clinical evidence of progressive liver disease although four expired with other manifestations of HX or infection. Conversely, patients showing fibrohistiocytic changes or cirrhosis initially were likely to have continuing or progressive liver disease. Although the liver histology was not diagnostic of HX, the types of portal changes usually predicted the subsequent course of liver disease.
Systemic Castleman's disease in association with Evan's syndrome and vitiligo
Authors: Marsh Johnathan H. , Colbourn Donald S. , Donovan Virginia , Staszewski Harry
Published: February 2007

Systemic Castleman's disease is a lympho‐proliferative disorder which has been associated with autoimmune phenomena. We report a case of the plasma cell variant of Castleman's disease that was associated with autoimmune hemolytic anemia and autoimmune thrombocytopenia (Evan's syndrome), and vitiligo, the association of which has not been previously reported.
Adriamycin and cis‐platinum administered by continuous infusion preoperatively in hepatoblastoma unresectable at presentation
Authors: Langevin A. M. , Pierro A. , Liu P. , Filler R. M. , Greenberg M. L.
Published: February 2007

Hepatoblastoma deemed surgically unresectable at presentation is fatal unless conversion to resectability is attained. We report a series of six consecutive patients, ranging in age from newborn to 5.75 years with hepatoblastoma unresectable at presentation, either because of size of tumor or its anatomical boundaries, or because of metastatic disease. All were treated with preoperative continuous infusions of cis‐platinum and Adriamycin. All achieved resect‐ability, and there was only one operative death. The five surviving patients are alive and free of disease off therapy. Potential benefit may accrue from preoperative chemotherapy in most cases of hepatoblastoma.
Erythrocyte fatty acid profiles in childhood malignancy
Authors: Heard W. , Kohler A. , Postle A. D.
Published: February 2007

The erythrocyte fatty acid composition was determined for 79 children using gas chromatography. The stearic:oleic acid ratio (SOR) was significantly lower in those children with newly diagnosed malignancies than in a reference group of healthy children. However, a control group of children with chronic, non‐malignant conditions were also found to have a low SOR. These results suggest that the erythrocyte SOR cannot be used as a tumour marker in children.
Klinefelter's syndrome and primary central nervous system lymphoma
Authors: Liang R. , Woo E. , Ho F. , Collins R. , Choy D. , Ma J.
Published: February 2007

Klinefelter's syndrome is known to be associated with various malignancies including male breast cancer and germ cell tumour. We report a patient with Klinefelter's syndrome who developed a primary B‐cell lymphoma of the central nervous system (CNS). To the best of our knowledge, this is the first case of primary CNS lymphoma in Klinefelter's syndrome.
Day 7 marrow response and outcome for children with acute lymphoblastic leukemia and unfavorable presenting features
Authors: Gaynon Paul S. , Bleyer W. Archie , Steinherz Peter G. , Finklestein Jerry Z. , Littman Phillip , Miller Denis R. , Reaman Gregory , Sather Harland , Hammond G. Denman
Published: February 2007

The percent of marrow blasts on day 7 of therapy was determined for 128 children with previously untreated acute lymphoblastic leukemia and white blood count (WBC) ± 50,000/μl and/or lymphomatous features enrolled in the Childrens Cancer Study Group trial of the Berlin Frankfurt Munster 76/79 regimen (CCG‐193P). Patients received four‐drug induction therapy including vincristine, prednisone, l‐asparaginase, and daunomycin. Ninety‐seven patients had fewer than 25% marrow blasts on day 7. Of these, 94 survived and maintained remission through day 28 and were designated early responders. Thirty‐one patients had greater than 25% marrow blasts on day 7. Of these, 28 survived and achieved remission on day 28 and were designated late responders. The outcome of patients who underwent a day 7 marrow aspiration was similar to those who did not. Early responders had a 77.4% ± 4.5% (standard deviation) 3‐year estimated disease free survival, while late responders had 47.3% ± 9.8% (P<0.001). Early responders had a superior outcome both in the subset with an initial WBC<50,000/μl (P = 0.025) and in the subset with a WBC ± 50,000/μl (P = 0.01). The day 7 marrow response had prognostic value in this population of children with unfavorable presenting features who received four‐drug remission induction therapy.
Epidural spinal cord compression as the presenting manifestation of tumor of unknown origin
Authors: Doll Donald C. , Ringenberg Q. Scott , Bickel John T. , Yarbro John W.
Published: February 2007

Epidural spinal cord compression is a common complication of malignancy. In the majority of cases, the primary site is known at diagnosis or is evident following limited investigation. During the period January 1975 to December 1987 we encountered seven cases of tumor of unknown origin presenting as cord compression. Myelography detected the site of cord involvement in six cases, and computed tomography of the spine was utilized in one case. All seven patients underwent laminectomy. Histologic diagnosis was adenocarcinoma in four cases, squamous in one case, and large cell undifferentiated carcinoma in two cases. Evaluation for a primary site was unrewarding. Prognosis was poor, with a median survival of 10 weeks. Only one patient had a satisfactory response to treatment.
A successfully treated case of multicentric angiofollicular hyperplasia with oral chemotherapy (Castleman's disease)
Authors: Pavlidis Nicholas A. , Skopouli Fotini N. , Bai Mary C. , Bourantas Constantinos L.
Published: February 2007

A case of angiofollicular lymph node hyperplasia of plasma cell type in a 60‐year‐old woman is reported. The patient presented with lymphadenopathy, splenomegaly, and fever. She responded dramatically to chlorambucil and corticosteroids and has remained free of disease for the last 30 months. The authors review and discuss the systemic cytotoxic treatment of this clinicopathologic entity.
Prognostic significance of regional lymph node involvement in childhood extremity rhabdomyosarcoma
Authors: Mandell Lynda , Ghavimi Fereshteh , Laquaglia Michael , Exelby Philip
Published: February 2007

Thirty‐four patients with extremity rhabdomyosarcoma were treated at Memorial Sloan‐Kettering Cancer Center during the years 1970 to 1987. The regional lymph node status at diagnosis was histologically confirmed in 27 patients. Of these 27 patients, there were 20 males and 7 females, ranging in age from 6 months to 22 years (median 10 years). The primary tumor was located in the upper and lower extremity in 11 and 16 patients, respectively. The histologic subtypes included embryonal in 13 patients and alveolar in 14 patients. Fourteen patients had no evidence of regional lymph node involvement (N0) and 13 patients had lymphatic metastases (N1). All patients were further staged according to the extent of surgical resection, the local invasiveness and size of the primary tumor, and the presence (M1) or absence (M0) of distant metastases.

With a median follow‐up time from diagnosis of 9.2 years (minimum 5.3 years), the overall survival was 48% (13/27): N0, 12/14 and N1, 1/13 (P=.0002). Excluding those patients with M1 disease, the survival rate was: N0, 11/12; N1, 1/10 (P=.0001). The first site of relapse in the M0 patients was distant metastases in 8/10 cases.

Our results suggest that regional lymph node involvement at diagnosis is an important prognostic factor in extremity RMS. Its presence appears to be associated with a high incidence of relapse in metastatic sites and poor survival outcome.
Superior vena cava syndrome associated with childhood malignancy: Analysis of 24 cases
Authors: Ingram Lewis , Rivera Gaston K. , Shapiro David N.
Published: February 2007

Twenty‐four children with superior vena cava obstruction at initial presentation or associated with disease recurrence were treated at St. Jude Children's Research Hospital from 1973 to 1988. Of the 16 patients with superior vena cava syndrome at presentation, eight had non‐Hodgkin's lymphoma, four had acute lymphoblastic leukemia, two had Hodgkin's disease, one had neuroblastoma, and one had a yolk sac tumor. Their clinical condition at presentation was often critical and required rapid treatment. In all cases, histopathologic diagnosis was obtained without complication by either bone marrow aspiration, lymph node biopsy, thoracentesis, or thoracotomy prior to the initiation of definitive therapy.

Eight children had superior vena cava syndrome as a late complication during the course of their therapy. None had an antecedent history of superior vena cava obstruction. In contrast to the patients with superior vena cava obstruction at presentation, this group was composed predominantly of patients with recurrent solid tumors. Other causes included disseminated candidiasis and superior vena cava thrombosis, thus underscoring the importance of recognizing the etiology of superior vena cava syndrome to facilitate proper treatment.
Non‐randomised study comparing toxicity of escherichia coli and erwinia asparaginase in children with leukaemia
Authors: Eden O. B. , Shaw M. P. , Lilleyman J. S. , Richards S.
Published: February 2007

Seven hundred fifty‐eight unselected children entered into the United Kingdom Medical Research Council acute lymphoblastic leukaemia UKALL VIII Study and Trial were studied for differences in early treatment‐related toxicity according to the type of intramuscular L‐asparaginase received.

Two hundred seventy‐five received a product obtained from Escherichia coli and 483 the enzyme from Erwinia chrysanthemi. The E. coli patients had a significantly higher incidence of neurotoxicity, pancreatitis, and life‐threatening sepsis (4%, 2%, and 20%, respectively) when compared with the Erwinia group (2%, 0%, and 18%). Severe hypersensitivity was seen in one patient from both groups and the incidence of glucose intolerance was not significantly different. These findings indicate that E. coli asparaginase may be more toxic. With a minimum follow up of 41/2 years there is no evidence that either product has made a significantly different contribution to disease‐free survival.
Treatment patterns in low‐grade non‐Hodgkin's lymphomas: A single institution study
Authors: Liesveld Jane L. , Raubertas Richard F. , Bennett John M.
Published: February 2007

Ninety‐two cases of low‐grade non‐Hodgkin's lymphoma were examined retrospectively to assess to what extent treatment deferral is practiced at a single university hospital with a local referral base. Clinical characteristics of this patient group were also analyzed; 70% of these cases were of nodular lymphocytic, poorly differentiated (NLPD) histology, and 74% were stage III or IV. Twenty‐two cases were not treated initially. Nine eventually required treatment at a median of 16 months. Survival did not differ based on whether treatment was initially deferred. Nodal progression was the most common reason for beginning treatment after initial deferral, and most cases of advanced stage disease required treatment because of nodal involvement or B‐type symptoms at diagnosis. Presence of intra‐abdominal adenopathy often prompted initial treatment even in asymptomatic cases. In this series, as compared to previously reported series of treatment‐deferred cases, more patients were judged to require treatment at disease outset, and the median time to treatment in those in whom treatment was initially deferred but then instituted was shorter. Despite this, for the entire population, 69% of total follow‐up time was spent off therapy.
A child with esthesioneuroblastoma with metastases to the spinal cord and the bone marrow
Authors: Perkkiö Mikko , Majuri Salme , Hänninen Auli , Paljärvi Leo
Published: February 2007

Esthesioneuroblastoma is a rare tumor in children, and the correct diagnosis may be difficult, as is demonstrated in this case report. A 5‐year‐old girl was diagnosed with this tumor, which was incurable and behaved like a neuroblastoma, sending metastases to the bone marrow and invading the cranium and the spinal canal.
An exploratory study of environmental and medical factors potentially related to childhood cancer
Authors: Schwartzbaum Judith A. , George Stephen L. , Pratt Charles B. , Davis Bertha
Published: February 2007

To determine whether a general‐purpose epidemiologic questionnaire can be used in childhood cancer hospitals to identify associations between environmental factors and the major types of childhood cancer, we report the results of the analysis of the data obtained from such a questionnaire. On admission to St. Jude Children's Research Hospital in Memphis, Tennessee, between 1979 and 1986 a questionnaire was administered to 1,270 mothers of patients diagnosed with childhood cancer. Approximately onehalf of the children had acute leukemias (n = 629); the remainder had lymphomas (n = 237) or solid tumors (n = 404). Responses to questions regarding the patients' and parents' environmental and medical histories were compared across nine diagnostic categories. Only 5 of 232 variables remained nominally statistically significant (P < 0.05) after adjusting for confounding by patient or maternal age, year of birth or diagnosis, patient's race, and social class. The variables identified were length of time the patient was breast‐fed (χ2 = 16.1, P = 0.04); having a garden with fertilizers, herbicides, and pesticides (χ2 = 17.2, P = 0.03); maternal use of sex hormones during the year before the patient's birth (χ2 = 18.2, P = 0.02); maternal cigarette consumption (χ2 = 18.0, P = 0.02); and patient contact with persons with cancer (χ2 = 20.7, P = 0.01). Despite the large number of patients studied, we identified fewer significant variables than would be expected, on the average, under the null hypothesis. We conclude that the data obtained from a general‐purpose epidemiologic questionnaire do not provide a useful overview of the association between exposure to environmental factors and several types of childhood cancer.
Hepatocellular carcinoma with central nervous system metastasis: A case report and literature review
Authors: Friedman Henry Dan
Published: February 2007

In the United States and western Europe, hepatocellular carcinoma is not generally regarded as a distantly metastasizing tumor. The ability of hepatocellular carcinoma to metastasize to the brain is illustrated by the example of a 65‐year‐old white American man with no identifiable risk factors for primary liver cancer. Though hepatocellular carcinoma rarely metastasizes to the central nervous sytem, such metastases are reported to have led to tumor diagnosis or signaled tumor relapse.