bluuebunny/crossref_metadata_2025 huggingface dataset:
Mesenchymal hamartoma of the liver in an older child: Association with disseminated intravascular coagulation
Authors: Rao S. P. , Bhagavath S. , Chen C. K. , Tolete‐Velcek F.
Published: February 2007

A 9‐year‐old black girl underwent right hepatectomy because of a large primary tumor in the right lobe of the liver. Histopathologic examination revealed the diagnosis of mesenchymal hamartoma, a rare benign tumor occurring mostly in children below the age of 2 years. Preoperatively, the patient had laboratory evidence of mild disseminated intravascular coagulation (DIC) but had no bleeding manifestations. In preparation for the surgery, the patient received whole blood exchange transfusion and platelet transfusion which reresulted in marked improvement in hemostatic parameters. Despite what appeared to be adequate replacement of blood loss during the surgical procedure, the patient developed sudden cardiac arrest near the end of the procedure and died. The probable cause of death was hypovolemia. This case of mesenchymal hepatic hamartoma illustrates two unusual features: age of the patient and the association with DIC. The latter, to our knowledge, has not been reported before.
Endobronchial metastases from carcinoma of the breast
Authors: Ettensohn David B. , Bennett John M. , Hyde Richard W.
Published: February 2007

We report the case histories of four patients with endobronchial metastases from breast cancer, two of whom died of resultant respiratory insufficiency. To aid in earlier diagnosis and thus permit more rapid application of specific therapy, we characterized the epidemiology and presenting symptoms of these patients and 38 additional patients with endobronchial metastases from breast carcinoma that have been reported in the literature. The average age at presentation with endobronchial metastases was 55, and the average time from diagnosis of the breast primary lesion to the endobronchial metastasis was 77 months. Cough occurred in 71% of patients; wheezing and hemoptysis occurred in 25%. Segmental atelectasis occurred in 57% on chest roentgenogram. Average survival of patients from the time of diagnosis of endobronchial metastasis was 21 months; median survival was 19 months.
Tumors of unknown origin
Authors: Ringenberg Q. Scott
Published: February 2007

Tumors of unknown origin (TUOs) account for 3% of cancers and represent the eighth‐most common cancer diagnosis. Although TUOs are commonly classified by their histological presentation, examination of the location of the metastases is equally important. When the histological type and location of metastases are both known, a finite number of common clinical syndromes emerge and one can predict the most common occult primaries.
Acute cytoreduction techniques in the early treatment of hyperleukocytosis associated with childhood hematologic malignancies
Authors: Strauss Robert A. , Gloster Elizabeth S. , McCallister John A. , Jimenez Jorge F. , Neuberg Ronnie W. , Berry Daisilee H.
Published: February 2007

Early and effective Cytoreduction for high peripheral white blood cell counts in pediatric patients with acute leukemia may be helpful in preventing complications secondary to hyperviscosity. It also may be a useful adjunct to systemic chemotherapy. As an alternative to automated apheresis for this purpose, manual exchange transfusion is efficacious and does not require hemapheresis instrumentation and disposables and the related special staff. Two patients, a neonate with acute myeloblastic leukemia and a white blood cell count of 422.2 k/μl as well as a 2 1/2‐year‐old with an admission diagnosis of acute promyelocytic leukemia and a white blood cell count of 617.4 k/μl, underwent manual exchange hemotherapy for acute cytoreduction. The procedures were tolerated well, and significant leukocyte removal was achieved, with the respective leukocyte reductions being 81.1 and 68.7%. The techniques available for pediatric cytoreduction are compared, with emphasis on their efficiency and safety and appropriateness for very small children.
Serum neuron‐specific enolase in metastatic merkel cell tumors
Authors: Giannone Leonard , Johnson David H. , Grosh William W. , Davis Ben W. , Marangos Paul J. , Greco F. Anthony
Published: February 2007

Three patients with widely disseminated Merkel cell tumors of the skin are presented. In all three cases, neuron‐specific enolase (NSE) was demonstrated in neoplastic tissue by immunohistochemical staining, and serum NSE levels were also elevated in all three patients. Serum NSE may prove to be a useful tumor marker in this and other malignancies of neuroendocrine origin.
Congestive heart failure, hypertension, and hyperreninemia in bilateral Wilms' tumor: Successful medical management
Authors: Stine Kimo C. , Goertz Kenneth K. , Poisner Alan M. , Lowman James T.
Published: February 2007

Congestive heart failure is an unusual complication of the hyperreninemia of Wilms' tumors. Cases with bilateral tumors present a difficult management problem. This is a report of the successful medical management of a child with congestive heart failure secondary to hyperreninemia from bilateral Wilms' tumor. Hypertension and hyperreninemia were extensively documented. Their etiologic relation to the congestive heart failure was supported by the patient's improved cardiac function following specific reninangiotensin blockade. With unilateral tumors, surgical excision corrects the hypertension; however, with large bilateral tumors, excision is out of the question. A unique feature of this case is the ability to control the blood pressure with saralasin. With subsequent antitumor therapy, renin concentrations decreased proportional to tumor size, and renin angiotensin blocking therapy could be discontinued.
The impact of induction anthracycline on long‐term failure‐free survival in childhood acute lymphoblastic leukemia
Authors: Hitchcock‐Bryan Suzanne , Gelber Richard , Cassady J. Robert , Sallan Stephen E.
Published: February 2007

Early intensive therapy might be critical in improving failure‐free survival for children with acute lymphoblastic leukemia. Between 1973 and 1977, 107 children received vincristine and prednisone (VP) induction and 30 received the same two agents plus an anthracycline (VPA), Ninety‐nine of the VP‐treated group and all 30 of the VPA‐treated patients achieved complete remission.

At a median observation time of 10 years, 59 of 137 children remain in continuous complete remission. Failure‐free survival was 37% for the VP group and 63% for the VPA group (p = 0.02). Failure‐free survival for boys who received VP was 28%, compared with 68% for boys who received VPA (p = 0.007). All 11 extramedullary relapses and all seven relapses occurring beyond 3.8 years from diagnosis (three testicular and four bone marrow) were observed among the VP group.

We conclude that use of an anthracycline during remission induction therapy influenced failure‐free survival and that early results of successful antileukemic therapy in children must be confirmed by follow‐up progress reports.
The enigma of testicular leukemia: A critical review
Authors: Russo Antonio , Schiliro Gino
Published: February 2007

Isolated testicular relapse (T.R.) in acute lymphoblastic leukemia (ALL) has an overall incidence of 10% and affects mainly patients off therapy. Multivariate analysis of pretreatment characteristics has shown that lymphadenopathy and splenomegaly are independently associated with increased risk of T.R. during maintenance and off therapy, respectively. Sequential biopsy studies have demonstrated that testicular biopsies are unable to detect scanty infiltrates and have no practical utility. Prophylactic gonadal irradiation produced equivocal results and should not be used because of its sterilizing effect. Intensive multidrug regimens or prolonged maintenance were unable to substantially reduce T.R. rate. On the contrary, intermediate‐dose methotrexate (IDM) early in remission has almost abolished T.R. These findings strongly support the hypothesis that testicular interstitium is a very peculiar site where blasts are partially protected from the drug action; high drug concentrations are required for the optimal cytocidal effect. There are sufficient clues of a link between the excess of late marrow relapse in male sex and the capacity of testes of harboring blasts. Therefore IDM early in remission should be routinely adopted for prevention of testicular leukemia and its potential of late spread.
Treatment of lymphohistiocytic erythrophagocytosis with VP‐16 and aziridinylbenzoquinone
Authors: Becton David L. , Kurtzberg Joanne , Kinney Thomas R. , Friedman Henry S. , Chaffee Sara , Falletta John M.
Published: February 2007

Lymphohistiocytic erythrophagocytosis (LE) is a usually fatal disease characterized by fever, organomegaly, hyperlipidemia, central nervous system involvement, and cellular immunodeficiency. Treatment with corticosteroids, cytotoxic chemotherapy, and blood exchange is unsuccessful. We have treated two children with the epipodophyllotoxin VP‐16 and with intrathecal chemotherapy. Each patient had an initial complete response, and one remains in remission 36 months after therapy began. Aziridinylbenzoquinone (AZQ) therapy induced a complete response in a patient who relapsed during VP‐16 therapy. A combination of VP‐16 and intrathecal chemotherapy appears to be the most effective therapy for LE, and further evaluation of the role of AZQ is indicated.
Improved detection of metastatic Ewing's sarcoma with the use of bone marrow scintigraphy
Authors: Jones Gary R. , Miller John H. , White Les , Laug Walter E. , Shore Nomie A.
Published: February 2007

Prognosis in Ewing's sarcoma is inversely related to the extent of the disease at the time of presentation. The most common sites of metastases are the lungs and skeleton. Bone marrow metastases may be present but clinically silent. We report the use of Technetium (Tc)‐99m bone marrow scintigraphy to detect sites of marrow involvement by metastatic Ewing's sarcoma. This method of evaluation allowed identification of sites of involvement by Ewing's sarcoma that were not available by any other method of evaluation. In several instances, information provided by this method was pivotal in the management of these patients. Based on this small series of patients, bone marrow scintigraphy appears to be a sensitive modality in the detection of metastatic disease in patients with Ewing's sarcoma. Better understanding of the role of bone marrow scanning and its correlation with other diagnostic procedures in Ewing's sarcoma will require further study.
Malignant histiocytosis in infants: Surface marker analysis of malignant cells in two cases
Authors: Ishii Eiichi , Hara Toshiro , Okamura Jun , Suda Masahiro , Takeuchi Takami , Lida Koichi , Ueda Kohji
Published: February 2007

Two infants with malignant histiocytosis, as diagnosed by morphological and cytochemical examinations of malignant cells infiltrating pleural effusion, skin, and bone marrow, are described. Surface marker analysis of the malignant cells with monoclonal antibodies showed these cells to express markers of Tlymphocytes and HLA‐DR antigen, but not those of B‐lymphocytes, granulocytes, or monocytes. These immunological findings demonstrated that the neoplastic cells in malignant histiocytosis of infancy may be of Tcell origin, rather than histiocytic or monocytic origin.
Hodgkin's disease following Wilms' tumor: A case report
Authors: Bell B. A. , Esseltine D. W. , Freeman C. R.
Published: February 2007

A patient is described who developed Hodgkin's disease 16 years after treatment of Wilms' tumor with radiation and actinomycin D. The issues relating to Hodgkin's disease as a second malignancy are reviewed. The need for long‐term surveillance of patients with malignant disease for late effects of the cancer and its treatment is emphasized.
Clinical utility of fine needle aspiration in the diagnosis and management of neuroblastoma
Authors: Ganick Dorothy J. , Silverman Jan F. , Holbrook C. Tate , Dabbs David J. , Kodroff Michael B.
Published: February 2007

Eleven fine needle aspiration (FNA) biopsies were performed in five children with neuroblastoma, including one patient with peripheral neuroectodermal tumor of the thoracopulmonary region (Askin tumor). Cytologic features in conjunction with immunocytochemistry and electron microscopy on the aspirated material enabled us to make a primary diagnosis in four of the five patients and diagnose local recurrence and metastatic disease in three patients. There were no false‐positive or false‐negative cytologic diagnoses; therefore, diagnostic accuracy was 100%. FNA is an extremely useful technique for the primary diagnosis and management of neuroblastoma and excludes other small cell malignancies of children. The results of this study and literature review demonstrate that FNA cytology coupled with ancillary techniques of immunocytochemistry and electron microscopy is a rapid, safe, minimally invasive procedure which can aid in the diagnosis and management of patients with neuroblastoma without resorting to more aggressive diagnostic procedures in selective cases.
Non‐Hodgkin's lymphoma complicating acute lymphoblastic leukemia in remission
Authors: Friedman Eitan , Ben‐Bassat Isaac , Kende George , Behar Albert J. , Ramot Bracha
Published: February 2007

Second malignancies occurring in patients cured of childhood acute lymphoblastic leukemia (ALL) are rare but increasingly recognized. There are only three reported cases of non‐Hodgkin's lymphoma complicating ALL in remission. We report here another case of B‐cell‐type large‐cell lymphoma, presenting in the uterus of a 24‐year‐old woman, 16.5 years after the diagnosis of ALL was made. Although immune markers of the ALL were not available, it was thought to be unlikely that the second malignancy was a relapse of the original tumor. Due to the long latency period and the minimal exposure to alkylating agents, the role of chemotherapy seems to be insignificant in this case. The development of a second malignancy could be a manifestation of the basic susceptibility to cancer.
Prospective study of childhood acute lymphocytic leukemia: Hematologic, immunologic, and cytogenetic correlations
Authors: Michael P. M. , Carson O. M. , Ekert H. , Tauro G. , Rennie G. C. , Pilkington G. R.
Published: February 2007

We studied the karyotype in 81 consecutively diagnosed children with acute lymphocytic leukemia (ALL) treated at one institution on a randomized treatment protocol. In 75 patients (93%), a morphological cytogenetic result was obtained, and 57 (65%) were successfully G‐banded. Of the 75 patients, 46 (61%) showed abnormal chromosomes, mainly hyperdiploidy and pseudodiploidy, and 29 had no detectable abnormality. Our findings confirmed that the karyotype has prognostic significance. Duration of complete remission was 93% at 42 months for patients with high hyperdiploidy (>50). For patients with an apparently normal karyotype, it was 58%; and for patients with structural abnormalities it was 15%. The significance of these findings was confirmed by multivariate analysis, which showed age and karyotype to be the most important determinants of duration of remission.
Efficacy of mesna in preventing further cyclophosphamide‐lnduced hemorrhagic cystitis
Authors: Luce James K. , Simons Jarid A.
Published: February 2007

Mesna is a sulfhydryl compound that reacts with the metabolites of cyclophosphamide that are excreted in the urine and may produce bladder wall irritation. Mesna is converted in the blood to a biochemically inactive compound that is reduced back to mesna in the kidneys. In this way it has the potential to protect the bladder mucosa without interfering with the antineoplastic effect of cyclophosphamide. Twenty‐two patients who had developed hemorrhagic cystitis from cyclophosphamide were treated again with cyclophosphamide and mesna prophylaxis. A total of 68 cycles of cyclophosphamide were given with mesna, with a median of three cycles per patient. A recurrence of cystitis was prevented in all but one patient. Thus, mesna is effective in preventing recurrent cystitis in patients receiving further cyclophosphamide.
Phase II trial of etoposide and cisplatin for refractory small cell lung cancer: A cancer and leukemia group B study
Authors: Graziano Stephen L. , Lee Kewchang , Propert Kathleen J. , Tinsley Roger , Hayes Daniel M. , Green Mark , Comis Robert L.
Published: February 2007

A phase II trial of etoposide and cisplatin for patients with previously treated small cell lung cancer was carried out by CALGB from June 1983 to May 1984. Thirty‐five evaluable patients who had failed one prior chemotherapy regimen were treated with etoposide 80 mg/m2 and cisplatin 20 mg/m2 days 1–5 every 3 weeks. Objective responses were seen in 7 patients (20%) with 1 complete response and 6 partial responses. The median duration of response was 5.1 months. Fifteen patients (43%) had stable disease. Overall survival was 49% at 6 months and 14% at 1 year. The median survival was 6 months. Toxicity was mainly hematologic with severe and life‐threatening leukopenia and thrombocytopenia seen in 71% and 66% of patients, respectively. Two treatment‐related deaths caused by infection were seen. This cooperative group study fails to confirm the high response rates previously reported in institutional pilot studies.
Treatment of an aggressive non‐Hodgkin's lymphoma during pregnancy with MACOP‐B chemotherapy
Authors: Nantel Stephen , Parboosingh John , Poon Man‐Chiu
Published: February 2007

Non‐Hodgkin's lymphoma occurring during pregnancy is a rare event. A young woman at 18 weeks gestation with twins presented with a B‐cell immunoblastic lymphoma. Combination chemotherapy with methotrexate, DO xorubicin, cyclophosphamide, vincristine, prednisone, and bleomycin (MACOP‐B) was instituted after limited staging. There was excellent response and the 12 weekly chemotherapy protocol was completed in 13 weeks. Twin male infants were born at 28 weeks gestation by cesarean section for premature labor. There was no evidence of any congenital malformations or hematologic suppressions. We believe this to be the first report of aggressive weekly chemotherapy during pregnancy; this approach to treatment in aggressive non‐Hodgkin's lymphoma is both efficacious and safe.
Bone marrow relapse occurring as first relapse in children with acute lymphoblastic leukemia
Authors: Behrendt Henk , Van Leeuwen Eleonore F. , Schuwirth Cas , Verkes Robert J. , Hermans Jo , Van Der Does‐Van Den Berg Anna , Van Wering Elisabeth R.
Published: February 2007

In a retrospective review which covered the whole Dutch childhood population of approximately 3 million children we studied the prognosis in 164 children with acute lymphoblastic leukemia (ALL) who were initially treated between 1973 and 1983, and who had an isolated bone marrow relapse occurring as first relapse.

Until their first relapse, the patients were initially treated according to standard protocols, while treatment for relapse was heterogeneous, and not intensive. Second complete remission (CR) was attained by 78% of the patients. The median duration of second CR was 9 months, the median survival 13 months. Multivariate analysis showed that the duration of the first CR was the most significant variable with regard to prognosis. None of the patients who developed their bone marrow relapse during initial treatment, i.e., within 24 months from diagnosis, survived. Among the 73 patients who relapsed after cessation of the initial treatment there were 19 long‐term disease‐free survivors, 14 of whom had not developed subsequent relapses after 48+‐125+ months.

From this study we conclude that treatment in children with first bone marrow relapse has to be intensified.
Comparative study on the pharmacokinetics of 7‐hydroxy‐methotrexate after administration of methotrexate in the dose range of 0.5‐33.6 g/m2 to children with acute lymphoblastic leukemia
Authors: Borsi Joseph D. , Sagen Erling , Romslo Inge , Moe Peter J.
Published: February 2007

Concentrations of 7‐hydroxy‐methotrexate (7‐OH‐MTX) were determined in serum samples obtained after 266 infusions of methotrexate administered to 58 children with acute lymphoblastic leukemia. The dose of methotrexate (MTX) was in the range of 0.5‐33.6 g/m2. Pharmacokinetic parameters (metabolic index, drug/metabolite ratio, half‐life) of 7‐OH‐MTX and their relationship to the kinetics of methotrexate were analyzed. A great variability was observed in the extent and time‐course of the metabolite formation. The concentration of the metabolite was higher than that of the parent compound at any examined time after the end of the 24 hours' infusion. The increase of 7‐OH‐MTX levels at the end of the methotrexate infusion was found to be proportionate to the increase of the dose of MTX. Males had significantly higher metabolite levels than did females (P<0.01) in the dose range of 0.5‐8.0 g/m2. The age of the patients also significantly influenced the rate of the metabolite formation. The serial number of the treatment courses did not have effect on the metabolism of MTX. Dose dependency of the elimination half‐life of the metabolite was found. Although a tendency was observed that patients in continuous complete remission had higher metabolite levels than those who relapsed, the difference was not significant. Further studies are needed to determine the clinical importance of 7‐OH‐MT.
Renal dysfunction and hyperuricemia at presentation and relapse of acute lymphoblastic leukemia
Authors: Jones Deborah P. , Stapleton F. Bruder , Kalwinsky David , McKay Charles P. , Kellie Stewart J. , Pui Ching‐Hon
Published: February 2007

Hyperuricemia is an unusual presenting feature of acute lymphoblastic leukemia (ALL) and is generally associated with a large leukemic cell burden. We describe three children with T‐cell ALL who presented with acute renal failure and very high serum uric acid concentrations, despite a relatively small leukemic cell burden. Two of the three patients had normal complete blood counts without circulating blasts or other physical evidence of leukemia. An isolated renal relapse in one case was associated with hyperuricemia, increased renal excretion of uric acid, and renal dysfunction. An unusually high rate of purine catabolism of the lymphoblasts may cause hyperuricemia in these cases. Unexplained hyperuricemia should prompt a search for occult malignancy.
Poor prognosis infantile fibrosarcoma with pathologic features of malignant fibrous histiocytoma after local recurrence
Authors: Salloum Emile , Caillaud Jean M. , Flamant Françoise , Landman Judith , Lemerle Jean
Published: February 2007

In a retrospective study of infants under 1 year of age treated at our institution over a 30‐year period for soft tissue tumors, eight fibrosarcomas (FS) were seen, six of which were congenital. Therapy consisted of local excision (n = 3), radiotherapy (n = 1), surgery + radiotherapy (n = 1), surgery + chemotherapy (n = 1), and surgery + chemotherapy + radiotherapy (n = 2). Among these eight patients, four are alive in first complete remission (CR) with 13, 17, 23, and 27 years of follow‐up. Of the remaining four patients, two had local recurrences and are still alive in CR after re‐excision of the tumor, while the other two had both local and distant relapses and died. Interestingly, in the two patients who developed distant metastases, the pathologic pattern was that of malignant fibrous histiocytoma (MFH) at the time of local recurrence. To our knowledge, no similar cases of transitions between infantile FS known for its favorable outcome and MFH have been reported in this age group. The relevance of such transitions is difficult to assess. However, given the known metastatic potential of MFH, we believe that chemotherapy regimens currently used in the management of childhood soft tissue sarcomas should be used in similar cases.
Phase I study of a 120‐hour continuous intravenous infusion of 5‐fluorouracil in pediatric patients with recurrent solid tumors: A pediatric oncology group study
Authors: Green Daniel M. , Krischer Jeffrey P. , Bell Beverly , Brecher Martin L. , Cushing Barbara , Whitehead V. Michael
Published: February 2007

To determine the maximum tolerated dose of 5‐fluorouracil administered as a 120‐hour continuous intravenous infusion to pediatric patients, we performed a phase I study using a starting dosage of 900 mg/m2/day. The maximum tolerated dosage (MTD) was 1,100 mg/m2/day. At this dosage level 40% of courses were complicated by grade 3 mucositis. Three additional patients were treated at the dosage level of 1,000 mg/m2/day after the MTD was determined. We recommend the dosage level of 1,000 mg/m2/day for phase II studies of 5‐fluorouracil administered as a 120‐hour continuous intravenous infusion to pediatric patients.
Vascular events in patients receiving high‐dose infusional 5‐fluorouracil‐based chemotherapy: The University of Chicago experience
Authors: Gradishar W. , Vokes E. , Schilsky R. , Weichselbaum R. , Panje W.
Published: February 2007

In a retrospective review, 22 of a total of 244 patients (9%) patients with head and neck or gastrointestinal cancer who were treated with infusional 5‐fluorouracil (5‐FU)‐based chemotherapy regimens experienced thrombotic vascular events. These events occurred concurrently with treatment or several months following the completion of therapy and consisted mainly of cardiac arrhythmias, myocardial infarction, sudden death, and thromboembolism. The temporal relationship of these vascular events to the time of treatment suggests that infusional 5‐FU may have been the underlying cause.
Renal cell carcinoma in children: Histology, immunohistochemistry, and follow‐up of 10 cases
Authors: Leuschner Ivo , Harms Dieter , Schmidt Dietmar
Published: February 2007

Ten renal cell carcinomas in children under 15 years were investigated. The average age was 122.5 months and the girls predominated in our cases (7 girls, 3 boys). By using the classification of Thoenes et al., Pathol Res Pract 181:125–143, 1986 [18] a predominance of clear cell‐eosinophilic tumor cell type and of the tubulopapillary growth pattern was found. Immunohistochemistry revealed a heterogeneity of cytokeratin expression. By using the monoclonal antibodies Cam 5.2 and KL 1, cytokeratins were found in 7 cases each. The other 4 cytokeratin antibodies used were less sensitive. The expression of cytokeratin 13 in 3 cases suggested a more complex histogenesis than assumed. Vimentin was found in 3 tumors, but an association to a higher grade (G) of malignancy was not found in these cases. One tumor expressed the Tamm‐Horsfall‐protein, which is predominantly found in the distal tubule of the normal kidney. In summary the results of immunohistochemistry characterized the great heterogeneity of these tumors.

Follow‐up information was available in 9 cases. All patients with G I‐ and G II‐ tumors were free of disease after an average time of 39.6 months (mean 27 months). Two of the 3 cases with G III‐tumors died after 9 and 15 months, despite additional chemo‐ or radiotherapy. Therefore tumors of grade I and II of the Thoenes classification [18] seem to have a good prognosis.
Results of a changing treatment philosophy for children with stage I Hodgkin's disease: A 35‐year experience
Authors: Garden Adam S. , Woo Shiao Y. , Fuller Lillian M. , Sullivan Margaret P. , Ramirez Irma
Published: February 2007

Over the last four decades, significant changes have occurred in the management of childhood stage I Hodgkin's disease. Between 1949 and 1984, 50 children, ages 4 to 16 years, were treated for stage I Hodgkin's disease at The University of Texas M. D. Anderson Cancer Center. Nineteen children had clinically staged (CS) disease. Thirty‐one patients were pathologically staged (PS). Thirty‐four children were treated with radiotherapy only, 12 were treated with both radiotherapy and chemotherapy, and 3 patients were treated with combination chemotherapy alone. All patients were followed from 32 to 311 months (median 170 months). Five‐, 10‐, and 15‐year actuarial survival rates for all patients were 94, 89, and 84%, respectively. The corresponding freedom from relapse (FFR) rates were 76, 69, and 69% respectively. The 10‐year actuarial survival and FFR rates for CS patients were 79 and 42%. The corresponding rates for PS patients were 97 and 86%. In patients with PSI disease, actuarial 10‐year FFR rates of 100% were obtained either with regional radiotherapy alone or with combination chemotherapy and involved field radiotherapy. The following delayed adverse effects of treatment were observed: growth abnormalities in 17, aspermia in 3, thyroid abnormalities in 11 (two carcinomas), and second malignancies beyond the radiotherapy fields in 2. We conclude with a recommendation of combined chemotherapy and involved field radiation for children who have not fulfilled their growth potential, to achieve high cure rates, while minimizing morbidity.
Nonmetastatic thoracic neuroblastomas: A review of 40 cases
Authors: Rubie Hervé , Hartmann Olivier , Giron Alain , Lemoine Georges , Gruner Max , Brugieres Laurence , Valteau‐Couanet Dominique , Robert Alain , Lemerle Jean
Published: February 2007

From 1982 to 1987, 40 children with nonmetastatic thoracic neuroblastoma were treated with the same therapeutic regimen. According to TNM staging, there were 11 CS I,19 CS II, and 10 CS III. All patients underwent surgery; 30 had primary surgical excision; in 10 whose tumors were deemed unresectable, surgery was delayed until after a trial of chemotherapy. Operation was completed by several courses of chemotherapy in case of microscopic residual disease or regional lymph node involvement; radiotherapy was delivered in case of gross residual disease. Using this therapeutic approach, EFS is 92% with a median follow‐up of 40 months. Severe complications were rare and sequellae appear to be related to the disease, i.e., neurologic consequences of cord compression.
Continuous‐infusion 5‐fluorouracil combined with doxorubicin and cyclophosphamide: Feasibility study
Authors: Saphner Thomas , Tormey Douglass C. , Carey Patrick
Published: February 2007

Previous studies have demonstrated continuous‐infusion 5‐fluorouracil (Cl 5‐FU) to be an active single‐agent treatment for breast cancer without significant myelotoxicity. These qualities made Cl 5‐FU an attractive agent for combination with other effective but myelosuppressive agents. In this study we attempted to determine the maximal doses of Cl 5‐FU that could be added to a combination of agents known to be dose limited by myelotoxicity, doxorubicin 50 mg/m2 day 2 and cyclophosphamide 150 mg/m2 days 3–12 of a 28‐day cycle. Patients who received doxorubicin and cyclophosphamide alone developed significant myelotoxicity but did not develop stomatitis. The addition of 5–7 days of Cl 5‐FU at 200–300 mg/m2 was associated more closely with increased stomatitis (P = .11) than with increased granulocytopenia (P = .57). The stomatitis observed for low doses of Cl 5‐FU given with doxorubicin and cyclophosphamide would not have been expected for these low doses of Cl 5‐FU given as a single agent. We conclude that the addition of Cl 5‐FU to myelotoxic doses of doxorubicin and cyclophosphamide is not a promising therapeutic strategy for significantly increasing the effectiveness of this combination of agents. © 1992 Wiley‐Liss, Inc.
Serum biological markers and paraneoplastic syndromes in Wilms tumor
Authors: Coppes Max J.
Published: February 2007

Various tumors secrete tumor‐specific substances capable of producing signs and symptoms in host organs not caused by direct tumor invasion or organ destruction. These symptoms are collectively referred to as “remote effects” or “paraneplastic syndromes” of malignancy. Paraneoplastic syndromes are uncommon in childhood cancer. In Wilms tumor several distinct paraneoplastic syndromes have been reported: hypertension, erythrocytosis, hypercalcemia, Cushing syndrome, and acquired Von Willebrand disease. In addition some tumor‐specific substances are known to be elevated in patients with a malignancy without causing specific symptoms. These so called “tumor markers” can be used to detect early recurrence in previously treated patients, or in the evaluation of patients undergoing adjuvant therapy. Five of particular interest are erythropoietin, neuron‐specific enolase (NSE), hyaluronic acid (HA), hyaluronic acid‐stimulating activity (HSA), and hyaluronidase. © 1993 Wiley‐Liss, Inc.
Prognostic significance of myeloid‐associated antigen expression on blast cells in children with acute lymphoblastic leukemia
Authors: Fink Franz‐Martin , Köller Ursula , Mayer Hannes , Haas Oskar A. , Grümayer‐Panzer E. Renate , Urban Christian , Dengg Kathrin , Mutz Ingomar , Tüchler Heinz , Gatterer‐Menz Irmgard , Knapp Walter , Gadner Helmut
Published: February 2007

The prognostic significance of expression of myeloid‐associated antigens in childhood acute lymphoblastic leukemia (myA + ALL) was evaluated. From 1984 to 1990, 251 children with immunologically verified ALL were treated in two prospective consecutive Austrian studies. Complete immunopheno‐typing was performed in 206 cases (82%). Out of these 175 cases were classified as B‐cell precursor ALL, 31 cases as T‐ALL. Expression of myeloid‐associated antigens was demonstrated in 23 cases (13.1%) of childhood B‐cell precursor ALL, particularly in immature (CD10 negative) forms (P< .0001), and in 1 case (3.2%) of T‐ALL. CDw65 was expressed most frequently (12 cases), followed by CD13 and CD15 (5 cases each), CD33 (4 cases), and blood‐group H (3 cases). Compared to myA‐ ALL prognosis of children with myA+ B‐cell precursor ALL was poor, despite intensive multiagent chemotherapy according to BFM protocols. Remission rates were not impaired, but pEFS was 74.6% for myA‐ ALL, and only 37.8% for myA+ ALL (P= .0001). As demonstrated by multivariate analysis the expression of myeloid‐associated antigens was the most important prognostic variable for EFS in B‐cell precursor ALL, whether or not CD10 was expressed. © 1993 Wiley‐Liss, Inc.
Low dose continuous infusion doxorubicin in children and young adults
Authors: Kynaston J. Anne , Pearson Andrew D. J. , Kernahan Jennifer , Lucraft Helen , Barstow Diane , Thompson Anne , Craft Alan W.
Published: February 2007

Ten patients (age range 3.2–26.3 yrs) with relapsed or resistant malignancies received a total of 20 courses of low dose continuous infusion doxorubicin (3 mg/m2/day for 28 days) delivered by portable Graseby infusion pumps via central venous catheters. Each patient received a median dose of 144 mg/m2 (range 87–261). Four patients responded to treatment (1 complete response (CR) and 3 partial response (PR)) and performance status improved in seven patients. Overall toxicity was minimal: WHO Grade 4 anaemia in 2/18 courses, Grade 4 neutropenia in 1/18, Grade 3–4 thrombocytopenia in 3/18, nausea and vomiting of Grades 2 and 4 in 4/20 and 1/20 respectively, and mucositis of Grades 2 and 4 in 2/20 courses each. Cardiac toxicity was assessed using echocardi‐ography, and fractional shortening remained within normal limits in all patients. Low dose continuous infusion doxorubicin is a feasible, well tolerated, ambulatory therapy in children and may be an effective way of delivering doxorubicin with less toxicity, thus enabling the development of more dose intensive regimens. © 1993 Wiley‐Liss, Inc.
Nutritional status of children with leukemia
Authors: Yu Lolie C. , Kuvibidila Solo , Ducos Rafael , Warrier R. P.
Published: February 2007

Children with cancer represent a high‐risk group for protein‐energy malnutrition due to side effects associated with treatment. Assessment of nutritional status at the time of diagnosis and during treatment is, therefore, essential for planning nutritional intervention. We studied the nutritional status of 25 children with leukemia [9 newly diagnosed/relapsed (D/R) leukemic patients and 16 children with leukemia in remission (REM)], Plasma proteins (prealbumin, PA; albumin, Alb; transferrin, Tr; retinol‐binding protein, RBP) and acute phase‐reactant proteins (α1‐acid glycoprotein, AGP; C‐reactive protein, CRP; ceruloplasmin, CER) were measured by radial immunodiffusion. Results show that there were no significant deficits in anthropometric measurements among leukemic children. In contrast, the mean levels of all plasma proteins, especially PA (P < 0.005), were significantly lower in the D/R group than in the REM group. All D/R children, compared to 59% of those in remission, had PA levels < 20 mg/dl. Only the D/R group had abnormal levels of RBP, Tr, and Alb. Children who were treated with prednisone had significantly higher mean levels of PA, RBP, and AGP than those who were not receiving prednisone. The mean levels of acute phase‐reactant proteins in these leukemic children were comparable to those of healthy children. We conclude that mild/moderate malnutrition is common in leukemic patients at D/R and that PA seems to be the most sensitive indicator of visceral protein Status. © 1994 Wiley‐Liss, Inc.
What Is the prognostic value of osteomedullary uptake on MIBG scan in neuroblastoma patients under one year of age?
Authors: De Cervens Claire Labreveux , Hartmann Olivier , Bonnin François , Couanet Dominique , Valteau‐Couanet Dominique , Lumbroso Jean , Behar Catherine , Martelli Hélène , Lemerle Jean
Published: February 2007

Twenty‐seven infants under one year of age with metastatic neuroblastoma were studied for osteomedullary metastases. They were evaluated by at least two imaging procedures: X‐ray films and MIBG scan. Taking into account the results of these investigations, 3 groups were defined: osteomedullary metastases were detected in 8 infants by both X‐ray and MIBG scan, no osteomedulary sites were detected by either technique in 13 patients, and 6 had osteomedullary foci with positive MIBG uptake but no radiological abnormality. These three groups were apparently different in terms of median age, response to chemotherapy, and long‐term survival. The third group, which has not been previously described, appears to have a better prognosis than patients with radiologically detectable bone lesions. Nevertheless it was necessary to use intensive chemotherapy to obtain remission in these particular patients. It should be mandatory to specify the investigatory technique used to describe osteomedullary metastases in infants and treatment should be adapted according to this grouping system. © 1994 Wiley‐Liss, inc.
Stage III abdominal non‐Hodgkin's lymphoma in costa rican children: Comparison of two consecutive trials of treatment
Authors: Lobo‐Sanahuja Francisco , García Ivette , Barrantes José C. , Barrantes Max , Jiménez Rafael , Argüello Ana
Published: February 2007

Seventy‐three patients with Stage III abdominal non‐Hodgkin's lymphoma were prospectively treated following two sequential protocols (P): L278 P (group A, 33 patients) (1978‐1983) and L384 P (group B, 40 patients), (1984‐1991). No patient received radiotherapy. The L278 P included 7 drugs: cyclophosphamide, vincristine (VCR), adriamycin (ADR), prednisone, methotrexate (MTX), dexamethasone, and 6‐mercaptopurine, given for remission induction, maintenance, and CNS prophylaxis. In the L384 P we introduced a consolidation phase consisting of intravenous MTX and citrovorum factor rescue, and IV cytosine arabinoside. VCR was also added to the monthly doses and the maintenance phase was reduced from 18 to 15 months. From January 1988 we changed ADR for epirubicin in the same doses. Prophylactic treatment of the CNS, in the L384 P, was intensified by increasing the number of doses of MTX IT in the remission, induction, and consolidation phases, and with the use of ara‐C IT. Laparotomy in 50 patients allowed partial resection in 16, and second‐look laparotomy was performed in 27 patients. Viable tumor was found in four patients. Three patients (G‐A) died from metabolic complications and another 4 (2 G‐A and 2 G‐B) failed to attain CR and died. A total of 28 (85%) of 33 children of G‐A and 38 (95%) of 40 children in G‐B achieved CR. Five children died in remission (2 G‐A, 3 G‐B). Three patients (G‐A) relapsed in the CNS and one (G‐B) relapsed in the abdomen and died. Disease‐free survival at 120 months was 70% in G‐A and 84% in G‐B.
Long‐term disease‐free survival in a child with refractory metastatic malignant germ cell tumor treated by high‐dose chemotherapy with autologous bone marrow rescues
Authors: Devalck Christine , Tempels Didier , Ferster Alina , De Laet Marc‐Henri , Bujan Willem , Heiman Pierre , Sariban Eric
Published: February 2007

We report the case of a 8‐month‐old girl who presented with a metastatic malignant germ cell tumor (MGCT) that proved to be resistant to chemotherapy, including salvage platinum‐based combination therapy, surgery, and metastatectomy. The child was then treated with high‐dose carboplatin, etoposide, and cyclophosphamide with autologous bone marrow rescue (ABMR) for two courses, since the first course was well tolerated and resulted in serum α‐f etoprotein normalisation. The child remains in complete remission 3 years post‐ABMR and 4 years post‐diagnosis, with no detectable treatment‐related sequelae. Thus, as already reported in adults, dose‐intensive chemotherapy with ABMR may have curative potential in children with refractory MGCT.
Carney's triad: Apropos of a new case
Authors: Acha Tomás , Picazo Beatriz , García‐Martín Francisco J. , Urda Antonio , Campos Joaquín
Published: February 2007

Carney's triad is defined by the coexistence of at least two of three rare disorders, including gastric epithelioid leiomyosarcoma (malignant leiomyoblastoma), pulmonary chondroma, and paraganglioma, most often extra‐adrenal and functioning.

We report a new case in a 10‐year‐old girl. The paraganglioma, although nonfunctioning, was detected after it was searched for, as Carney's triad was suspected. Unrelated seems the development of breast fibroadenomas in the same patient.

Whenever a patient with one component of the triad is encountered, the possibility of this syndrome should be considered and the other two components sought.
Choroid plexus carcinoma: Report of one case with favourable response to treatment
Authors: Aricáog Maurizio , Raiteri Emanuela , Bossi Grazia , Giordana Maria Teresa , Corbella Franco , Locatelli Davide , Pezzotta Stefano
Published: February 2007

Choroid plexus carcinoma (CPC) is a rare tumor with usually severe prognosis, whose optimal treatment has not yet been established. The exact role of complete surgical resection, chemotherapy, and radiotherapy has been debated but not clarified. We report one girl with CPC diagnosed at age 3 months and apparently cured with minimal surgical resection, chemotherapy, and delayed irradiation. At the age of 8 years, she is well, with minor psychomotor retardation and growth hormone deficiency as the only sequelae. © 1994 Wiley‐Liss, Inc.
Hypertensive crises induced by treatment of malignant pheochromocytoma with a combination of cyclophosphamide, vincristine, and dacarbazine
Authors: Wu Li‐Teh , Dicpinigaitis Peter , Bruckner Howard , Manger William , Averbuch Steven
Published: February 2007

Combination chemotherapy with cyclophosphamide, vincristine, and dacarbazine (CVD) is an effective treatment regimen for malignant pheochromocytoma. There have not been any significant acute cardiovascular effects reported following CVD treatment. Among seven patients with malignant pheochromocytoma treated with CVD at our institution, two patients with labile hypertension developed hypertensive crises following CVD treatment. The marked increase in blood pressure correlated with an increase in urinary excretion of catecholamine metabolites in one patient. Further hypertensive crises following sebsequent CVD treatments were avoided by optimizing each patient's antiadrenergic therapy. Similar to the approach used preoperatively for patients with resectable pheochromocytoma, maximal antiadrenergic therapy is essential in preventing hypertensive crises in patients with malignant pheochromocytoma undergoing CVD treatment. © 1994 wiley‐Liss, inc.
Peritoneal metastases in two patients with pineoblastoma and ventriculo‐peritoneal shunts
Authors: Gururangan Sridharan , Heideman Richard L. , Kovnar Edward H. , Sanford Robert A. , Kun Larry E.
Published: February 2007

We report the uncommon occurrence of ventriculo‐peritoneal shunt‐associated peritoneal metastases in two patients with pineoblastoma. In one patient, the peritoneal cavity was the only site of recurrence; there was no evidence of disease recurrence in the central nervous system. One other patient with recurrent intracranial disease had synchronous, but asymptomatic, peritoneal metastases which were detected on an elective ultrasound. Although rare, peritoneal metastases appear to respond well to systemic chemotherapy. Ultrasound surveillance of the abdomen should be considered as a part of the routine follow‐up evaluation in patients with embryonal central nervous system tumors and ventriculo‐peritoneal shunts. © 1994 Wiley‐Liss, Inc.
Acute tumour lysis syndrome complicating treatment of stage IVS neuroblastoma in infants under six months old
Authors: Hain Richard D. W. , Rayner Lisa , Weitzman Sheila , Lorenzana Adonis
Published: February 2007

Acute tumour lysis syndrome (ATLS) is a common complication of the treatment of haematopoeitic malignancies. It is also well recognized in many nonhaematopoeitic malignancies of adults. There are very few reports of the syndrome occurring during therapy for the nonhaematopoeitic malignancies of childhood, and none has previously been reported in the treatment of neuroblastoma. We report the cases of four patients presenting to The Hospital for Sick Children (HSC) between 1985 and 1992 who developed ATLS during treatment for stage IVS neuroblastoma. ATLS is a significant risk in patients undergoing therapy for stage IVS neuroblastoma, particularly where this has been delayed. © 1994 Wiley‐Liss, Inc.
Isolated bone relapse during hematologic remission in childhood acute lymphoblastic leukemia: Report of a metatarsal relapse and review of the literature
Authors: Murray Jeffrey C. , Gmoser Dean J. , Barnes Douglas A. , Oshman Daniel , Hawkins Hal K. , Gresik Mary V. , Dreyer Zoann E.
Published: February 2007

An 11‐year‐old boy with prior bone marrow and testicular relapses of his acute lymphoblastic leukemia (ALL) developed an isolated metatarsal bone relapse during complete hematologic remission 10 months after completion of chemotherapy. Although there was no radiographic or histologic evidence of additional occult leukemia, the polymerase chain reaction (PCR) technique detected a leukemic clone in both his bone marrow and metatarsal.

A literature survey revealed only 10 reported cases of isolated bone relapse occurring during complete bone marrow remission in childhood ALL. Most of these patients had prior bone marrow or extramedullary relapses. The majority experienced subsequent relapses after their isolated bone recurrence. We report a case of isolated bone recurrence, review all previously reported cases, and suggest that PCR elucidation of clonal disease may provide a better understanding of these extremely rare extramedullary events. © 1994 Wiley‐Liss, Inc.