bluuebunny/crossref_metadata_2025 huggingface dataset:
Lack of bcr rearrangement in juvenile chronic myeloid leukemia
Authors: Toren Amos , Mandel Mathilda , Amariglio Ninette , Hakim Ytzhak , Brok‐Simoni Frida , Rechavi Gideon , Neumann Yoram , Ramot Bracha
Published: February 2007

Juvenile chronic myeloid leukemia (JCML) is an unusual subtype of children's leukemia, characterized by unique clinical presentation. Recent studies revealed several biological features, distinguishing from those observed in adult type chronic myeloid leukemia (ACML). The majority of ACML cases are characterized by the presence of an hybrid bcr‐abl rearranged gene. In an effort to elucidate the molecular basis of this unusual leukemia we looked for bcr rearrangement in six JCML cases. No bcr rearrangement was identified in any of the analyzed samples. Together with previous studies from JCML cases, JCML has a different mechanism of leukomogenesis from ACML.
Hyperaminoaciduria identifies patients at risk of developing renal tubular toxicity associated with ifosfamide and platinate containing regimens
Authors: Caron Hubert N. , Abeling Nico , van Gennip Albert , de Kraker Jan , Voucte P. A.
Published: February 2007

We monitored renal tubular function in 18 neuroblastoma patients treated with chemotherapeutic regimens containing Ifosfamide and platinates. The total IFO dose ranged from 30 to 48 g/m2. After each IFO course and at regular intervals during follow‐up 24 hour urinary excretion of aminoacids, qualitative excretion of protein and glucose, tubular reabsorption of phosphate, serum pH, and liver enzyme (SGOT and SGPT) were measured. The ratio of α‐amino‐Nitrogen/total‐Nitrogen (normal <2.5%) and the urinary excretion pattern were used to quantify the aminoaciduria In 12 out of 15 evaluable patients some degree of tubular toxicity occurred during treatment, slowly progressing to a Debré‐de Toni‐Fanconi syndrome (DTFS) in 7 patients. The DTFS was fully developed in most cases 3–9 months after the end of treatment and was reversible in 3 cases. Hyperaminoaciduria (HAA) occurred in all patients during treatment, preceding other signs of tubular toxicity. The maximum ratio measured before development of a DTFS was significantly higher in the patients with severe toxicity (P < .01). HAA characterized by a ratio >10% predicts the development of a DTFS with a sensitivity of 71.4% and a specificity of 87.4%.
Acute nonlymphoblastic leukemia in children treated for acute lymphoblastic leukemia with an intensive regimen including teniposide
Authors: Verdeguer Amparo , Ruiz Jose Gabriel , Ferris Josep , Esquembre Carlos , Tasso Maria Jesus , Fernandez Jose Maria , Prieto Felix , Castel Victoria
Published: February 2007

Some cases of conversion from acute lymphoblastic leukemia (ALL) to acute nonlymphoblastic leukemia (ANLL) at relapse have been reported recently. We report three cases initially diagnosed as having ALL and showing morphological, cytochemical, and immunophenotypic features of ANLL at relapse (lineage switch). Conversion was observed among 14 patients who developed bone marrow relapse while undergoing intensive treatment with our ALL protocol, which includes teniposide, and that had been administered to 62 patients. The three cases converted at first relapse, with a mean time of 20 months (13–29 months). Clinical and immunologic characteristics of T‐cell leukemia were present in one patient. Changes documented in cytogenetic studies are discussed. The underlying mechanisms for the lineage switch remain unclear as does its relation with mixed lineage leukemias, but we believe that drugs employed in our therapy protocol could have had an influence on this conversion.
Successful management of inferior vena cava and right atrial nephroblastoma tumor thrombus with preoperative chemotherapy
Authors: Oberholzer Hendrik F. , Falkson Geoffrey , de Jager Lourens C.
Published: February 2007

Involvement of inferior vena cava (IVC) by tumor thrombus occurs in up to 10% of patients with nephroblastoma. Right atrial involvement by tumor thrombus is much less frequent. Four patients presenting with advanced nephroblastoma were diagnosed as having IVC involvement with tumor thrombus. Two of these patients had in addition thrombus extending up to the right atrium. All 4 patients were treated with preoperative chemotherapy (vincristine, actinomycin D, 4‐epi‐Adriamycin). Nephrectomy was subsequently performed without undue difficulty on all 4 patients. The intravascular tumor thrombus had completely cleared in all 4 patients and most of the primary renal tumor was necrotic. The results obtained with preoperative chemotherapy as given to these patients mediates strongly against difficult surgery being undertaken as primary treatment for such patients.
Malignant melanoma: Primary presentation in bone marrow and lymph node
Authors: Basile Myrtha , Moskowitz Bruce , Harris James , Blumberg Neil , Bennett John M.
Published: February 2007

We describe an unusual presentation of malignant melanoma with simultaneous lymph node and bone marrow metastasis. In addition the disease was associated with immune‐mediated thrombocytopenia. A brief remission was obtained with combination chemotherapy.
Hepatocellular carcinoma associated with arteriohepatic dysplasia in a 4‐year‐old girl
Authors: Békássy Albert N. , Garwicz Stanislaw , Wiebe Thomas , Hägerstrand Inga , Jensen Ove A.
Published: February 2007

Hepatocellular carcinoma and obliterated hepatic bile duct were found at postmortem examination in a 4‐year‐old girl with arteriohepatic dysplasia (Alagille's syndrome). AFP level was extremely high. Liver cirrhosis was present on percutaneous needle biopsy 9 months before she succumbed in progressive liver failure. Episodes of repeated gastrointestinal, life‐threatening hemorrhages occurred during the last 6 months of her life. Histopathologic findings of the eyes were documented at autopsy.
Extrapulmonary and pulmonary small‐cell carcinoma: Tumor biology, therapy, and outcome
Authors: Remick Scot C. , Ruckdeschel John C.
Published: February 2007

Extrapulmonary small‐cell cancer is a distinct clinicopathological entity from smallcell anaplastic carcinoma of the lung. Approximately 1,000 cases have been projected annually in the United States, which represents an overall incidence of between 0.1% and 0.4% of all cancer. Not surprisingly then, little information is available regarding the treatment of this disease, which presents a challenge to the clinician when it is regionally confined. The majority of patients with extrapulmonary small‐cell neoplasms have only been treated with local modalities of therapy, surgery, radiation, or a combination of both. Prolonged survival is not infrequent, which is in contrast to the experience for small‐cell lung cancer and surprising given our current systemic approach to patients with this disease.

This report will summarize the similarities and differences in biology, natural history, and clinical characteristics of patients with extrapulmonary small‐cell cancer and smallcell anaplastic carcinoma of the lung. The histogenesis of small‐cell cancer is briefly reviewed. A general therapeutic approach to patients with small‐cell lung cancer is reported. Lastly, recommendations for therapy of patients with regionally confined extrapulmonary small‐cell cancer by primary site are outlined.
High‐dose methylprednisolone sodium succinate as a single agent in relapsed childhood acute lymphoblastic leukaemia
Authors: Ryalls Michael R. , Pinkerton C. Ross , Meller Simon T. , Talbot Denis , McElwain Timothy J.
Published: February 2007

Twenty children received methylprednisolone (1 g/m2), daily for 5 to 8 days, as initial single agent therapy for relapsed acute lymphoblastic leukaemia. Bone marrow blasts were reduced to <5% in 2 and 5–10% in 3 of 12 patients with bone marrow relapses. In 3/9 with central nervous system relapses the cerebrospinal fluid (CSF) blasts completely cleared and were reduced in 4 others. In two patients with testicular relapses there was shrinkage of tumour and one patient with a navicular bone relapse became pain free. Toxicity was minimal. These results indicate high‐dose methylprednisolone is an effective agent, particularly in the treatment of established central nervous system (CNS) disease and could contribute to early CNS directed therapy in acute lymphoblastic leukaemia.
The unique association of malignant histiocytosis and a primary gonadal germ cell tumor
Authors: Margolin Kim , Traweek Thomas
Published: February 2007

Previous reports of the association between hematologic malignancies and germ cell tumors have been limited to patients with nonseminomatous tumors, exclusively of mediastinal origin. Among the various hematologic disorders, a large proportion have involved histiocytic tumors, either acute monocytic leukemia or malignant histiocytosis. We now report the first case of simultaneously occurring malignant histiocytosis and testicular embryonal carcinoma. The patient, an 18‐year‐old male, presented with hepatosplenomegaly due to malignant histiocytosis and was found on further evaluation to have a stage I testicular cancer consisting of teratocarcinoma with endodermal sinus elements. Despite aggressive chemotherapy, the patient died of malignant histiocytosis 6 months after the original diagnosis. The autopsy revealed widespread organ involvement with malignant histiocytosis and no evidence of residual germ cell tumor. This case demonstrates that germ cell tumors associated with hematologic malignancy do not arise solely in extragonadal sites.
Carcinoid syndrome in the absence of liver metastasis: A case report and review of literature
Authors: Haq Amin U. , Yook Clara R. , Hiremath Vijay , Kasimis Basil S.
Published: February 2007

A patient with carcinoid tumor of the head of pancreas and carcinoid syndrome presented without liver metastasis. The patient had retroperitoneal lymphadenopathy. He had symptoms of flushing, diarrhea and abdominal pain. 5‐Hydroxyindoleacetic acid (5‐HIAA) was elevated. Absence of liver metastasis was documented not only by the negative computed tomography (CT) scan and liver/spleen scan, but also by autopsy. Except for carcinoid arising from ovary, testis, or bronchi, the other carcinoid tumors rarely cause carcinoid syndrome without liver metastasis. The literature was reviewed, and the findings are presented. © 1992 Wiley‐Liss, Inc.
Partial response after intensive chemotherapy for adrenal cortical carcinoma in a child
Authors: Aricò Maurizio , Bossi Grazia , Livieri Chiara , Raiteri Emanuela , Severi Francesca
Published: February 2007

Adrenocortical carcinoma (ACC) in childhood is a rare tumor with high fatality rate. Available reports provide event free survival rates ranging between 10 to 50%. Optimal treatment has not yet been established; surgery plays a major role, and the value of adjuvant chemotherapy needs to be evaluated further, especially in children who develop recurrent disease and those with metastases at diagnosis. Optimal therapy of ACC has not been established. Surgery has been curative after complete tumor resection. Children with inoperable, recurrent and metastatic ACC have been treated with O, P'DDD, with response rates ranging from 10 to 60% in different series [7,11–20]. Radiotherapy [21] and other anti‐cancer drugs have been used [4–22] but their efficacy has not been established. Combination chemotherapy containing oncovin, cisPlatinum, epipodophyllotoxin and cyclophosphamide (OPEC) produced regression of metastatic ACC in a 5‐year‐old male [23].

We report one girl with relapsed disseminated ACC who showed good, even if temporary, control of the disease, with disappearance of lung, liver and spleen metastases, and marked reduction of the adrenal mass, following combined chemotherapy according to the “eight‐drugs‐in‐one‐day” protocol. © 1992 Wiley‐Liss, Inc.
Inadvertent intrathecal injection of daunorubicin with fatal outcome
Authors: Mortensen Mary Ellen , Cecalupo Anthony J. , Lo Warren D. , Egorin Merrill J. , Batley Rosalind
Published: February 2007

We report the first known case of daunorubicin administered directly into the human central nervous system. A 3½‐year‐old female with pneumonia and otitis media was diagnosed with acute lymphoblastic leukemia and was admitted for antibiotics and chemotherapy. On the first day she inadvertently received a 17 mg intrathecal (IT) injection of daunorubicin. When the error was recognized about 1 hour later, her cerebrospinal fluid (CSF) was exchanged with sterile saline by barbotage, IT hydrocortisone was given, a subarachnoid catheter was inserted, and the CSF was allowed to drain for 36 hours. Only 5.6 mg (33%) of the dose was recovered from CSF, 2.7 mg as daunorubicin and 2.9 mg as the metabolite, daunorubicinol. Initially she was asymptomatic and induction therapy continued with vincristine, 1‐asparaginase, prednisone, and IT methotrexate. One week after the daunorubicin injection she developed headache and irritability; CSF protein was 3.2 gm/dl. On the 12th day, she developed fungal sepsis and worsening pneumonia. On the 15th day, she became comatose with a flaccid paraparesis, areflexia, and an ascending progressive bulbar palsy. A series of computerized tomography scans over 6 weeks showed increasing diffuse cerebral atrophy. Nerve conduction velocity studies were consistent with an axonal neuropathy. Despite her multiple concurrent medical problems, the timing and characteristics of neurologic damage suggest that IT daunorubicin caused progressive destruction of the nervous system. © 1992 Wiley‐Liss, Inc.
Cranial computed tomographic findings in children with newly diagnosed acute lymphoblastic leukemia: A prospective follow‐up study during treatment
Authors: Vainionpää Leena , Laitinen Juhani , Lanning Marjatta
Published: February 2007

Cranial computed tomography (CT) was performed on 40 consecutive children with newly diagnosed acute lymphoblastic leukemia (ALL) on admission before any chemotherapy, 5 months after CNS therapy (n = 39) and after 2 to 3 years of therapy (n = 31). Changes related to leukemia were found in only 10% of the patients at the time of diagnosis (4/40). These initial changes, two intracranial hemorrhages, one dural thickening and one contrast enhancement, all disappeared during therapy. The findings which persisted unchanged in the next two CT scans were thought to be normal variations or caused by earlier disorders.

CNS therapy consisted of intrathecally and intravenously administered methotrexate in 20 standard risk (SR) patients and cranial irradiation in addition to chemotherapy in 19 intermediate risk (IR) or high risk (HR) patients. Four SR patients developed changes during therapy. Three had enlarged cerebrospinal fluid (CSF) spaces and one developed a focal low density area suggesting disturbances in brain blood circulation and also experienced disturbances in level of consciousness. Of the 19 IR or HR patients, eight developed changes related to the therapy, including four with white matter hypodensity areas, of whom three also had enlarged CSF spaces, and four others who developed enlarged CSF spaces. The medians of the widths of the cortical sulci (P < .001), insular cisterns (P < .01), third ventricles (P < .01), and frontal horns (P < .05), and also of Evans' ratios (P < .05) increased significantly after CNS therapy as compared with the findings at diagnosis in the patients who had received cranial irradiation. Most of these changes persisted during the follow‐up. We conclude that the clinical value of CT scanning during therapy for ALL is restricted to patients with neurological symptoms or those who have undergone CNS irradiation. © 1992 Wiley‐Liss, Inc.
“Shuttle sheet”: A patient‐held medical record for pediatric oncology families
Authors: Stevens Michael M.
Published: February 2007

A patient‐held medical record used by a pediatric oncology unit is described. Each patient's family is provided with a folder containing detailed information about the patient's current treatment. The record helps orient all health care workers involved in the patient's management to the treatment schedule, including exact doses of drugs. Details of blood counts and treatment given are entered by health care workers at each consultation, but the record is always retained by the family. The benefits of this record are that it saves time, reduces the likelihood of errors in scheduling and doses of cytotoxic drugs, and facilitates continuation of therapy at locations away from the supervising oncology unit. It reduces the amount of correspondence required for patient care, is a useful diary and treatment planner for the patient's family and hospital staff, and is an educational resource for the patient's family. It is a useful adjunct to the hospital's medical record for clinical trial data, is easily replaced if lost, and is inexpensive. It also assists surveillance of longterm survivors. The record is particularly valuable when used with patients who are being treated or assessed at more than one institution or by multiple health care workers. The record is in its 12th year of service and is used by more than 95% of patients on therapy attending the unit. © 1992 Wiley‐Liss, Inc.
Management of testicular seminoma following organ transplantation
Authors: Villalona‐Calero Miguel A. , Ducker Thomas , Holasek Maureen , Najarian John , Kennedy B. J.
Published: February 2007

An increased incidence of malignancies has occurred in recipients of organ transplantation who are immunosuppressed. Although testicular cancers have been uncommon, seminomas are extremely rare. Two patients with long‐standing diabetes mellitus and renal transplants developed clinical stage I seminoma of the testis. These patients posed a therapeutic problem with respect to the use of radiation therapy. In one, none was given because of a combination of kidney rejection and antibiotic‐induced renal damage. The second patient received radiation therapy with shielding of the transplanted kidney. The surgical distortion of lymph node architecture increases the problems in the use of radiation therapy. Individual factors need to be considered in the use of postorchiectomy radiation therapy for seminoma in transplant patients. © 1992 Wiley‐Liss, Inc.
Minimal requirements for the diagnosis, classification, and evaluation of the treatment of childhood acute lymphoblastic leukemia (ALL) in the “BFM family” cooperative group
Authors: van der Does‐Van den Berg Anna , Bartram Claus R. , Basso Giuseppe , Benoit Yves C. M. , Biondi Andrea , Debatin Klaus‐Michael , Haas Oskar A. , Harbott Jochen , Kamps Willem A. , Köller Ursula , Lampert Fritz , Ludwig Wolf‐Dieter , Niemeyer Charlotte M. , van Wering Elisabeth R.
Published: February 2007

Minimal requirements and their rationale for the diagnosis and the response to treatment in childhood acute lymphoblastic leukemia (ALL) were defined in the recently instituted “BFM‐Family”‐Group, in which the German, Austrian, Dutch, Italian, Belgian, French and Hungarian childhood leukemia study groups cooperate. ALL is defined as ≥ 25% lymphoblasts in the bone marrow; for confirmation of the diagnosis and classification the criteria of the French‐American‐British (FAB) criteria are retained. For determination of the extent of the disease at diagnosis or relapse the criteria by the Rome Workshop [1986] are recommended: An obligatory panel of monoclonal antibodies for immunophenotyping was defined, as well as criteria for precursor B‐ALL and T‐ALL. Cytogenetic studies may support the diagnosis and subtyping, and are essential to identify certain patients with a high risk of treatment failure (f.i. t(9;22), t(4;11)). The role of molecular genetics for the diagnosis and the characterization of leukemia and the value of its clinical application needs further elucidation. Relapse was defined as recurrence of evident leukemia in the blood, bone marrow (≥ 25% lymphoblasts) or at any other site (to be confirmed by histological examination). Bone marrow involvement combined with extramedullary relapse was defined as ≥ 5% lymphoblasts in the bone marrow. © 1992 Wiley‐Liss, Inc.
Adverse prognostic features in 251 children treated for acute myeloid leukemia
Authors: Hurwitz Craig A. , Schell Michael J. , Pui Ching‐Hon , Crist William M. , Behm Fdrederick , Mirro Joseph
Published: February 2007

Potential predictors of event‐free survival (EFS) were assessed in 251 consecutively diagnosed children treated for acute myeloid leukemia (AM) on three successive clinical trials. The lack of significant differences in 4‐year EFS for these studies (20% ± 4%, 29% ± 4%, and 20% ± 7%) permitted combined analysis of presenting features. Splenomegaly (P = .002), coagulation abnormalities (P = .001), leukocyte count ⩾10 × 109/L (P = .002), and age >14 years (P = .01) were statistically significant predictors of a poorer EFS by univariate analysis and retained significance in multivariate analysis. Age <2 years and monocytic leukemias (often cited as adverse factors in AML) showed no prognostic influence in this study. The estimated relative risk of failure for a child with a single adverse feature at diagnosis was at least 1.4 times greater than that for a patient with no adverse features. For children with two or more adverse features, the relative risk increased by more than threefold. These clinical variables, alone or in combination, may identify important subgroups of patients with AML at high risk for failure and for whom improved or alternative therapies are especially important. © 1993 Wiley‐Liss, Inc.
Thirty‐year population‐based review of childhood renal tumours with an assessment of prognostic features including tumour DNA characteristics
Authors: Barrantes Jose C. , Muir Kenneth R. , Toyn Caroline E. , Parkes Sheila E. , Cameron A. Hugh , Marsden H. Basil , Raafat Farrokh , Mann Jillian R.
Published: February 2007

We have reviewed all paediatric kidney tumours seen in the West Midlands Health Authority Region over a 30‐year period. There were 205 cases confirmed after a review of the pathology by three paediatric pathologists. Seven were cases of bone metastasising renal tumour (clear cell sarcoma), 5 were rhabdoid tumours, 2 were renal cell carcinomas, and 13 were mesoblastic nephromas. In 3 cases, it was not possible to define further the histological diagnosis. The remaining 175 cases were considered to be Wilms' tumour (86%), which is equivalent to an incidence of 5.7/106/year.

In the cases of Wilms' tumour, there were 91 boys and 84 girls (1.1:1). The majority of patients were Caucasian with only 7% of non‐Caucasian origin. At presentation, 78% of the patients were less than 5 years old. All of these patients except 9 had surgery as part of their treatment, 154 children had total nephrectomy, 3 had partial nephrectomy, and 9 had other surgical procedures. The majority also received chemotherapy and radiotherapy. Sex, chemotherapy, and stage all had prognostic significance in univariate analysis. The actuarial survival at 10 years increased from 17% for patients treated in the first decade of the study to 78% for patients treated in the third.

DNA characteristics were investigated using flow cytometry in paraffin‐embedded material and adequate information was obtained in 73 cases of Wilms' tumour. Only 7 had aneuploid tumours. Univariate survival analysis of these 73 results showed that stage, sex, the percentage of cells in the synthetic phase and the proliferative index from the DNA investigations had predictive value. © 1993 Wiley‐Liss, Inc.
Allogeneic bone marrow transplantation for myelodysplastic syndromes of childhood: Report of three children with refractory anemia with excess of blasts in transformation and review of the literature
Authors: Uderzo Cornelio , Locasciulli Anna , Rajnoldi Angelo Cantù , Mozzana Ruggero , Lambertenghi‐Deliliers Giorgio , Masera Giuseppe
Published: February 2007

Myelodisplastic syndromes (MDS) in childhood deserve a negative prognosis even though disease‐free survival has been obtained in 20% of cases by using aggressive chemotherapy. We describe three children with refractory anemia with excess of blasts in transformation (RAEB‐T) who underwent bone marrow transplantation (BMT). We also reviewed 21 additional cases (median age was 8 years) with primary MDS recently reported in the literature with the aim of clarifying the role of BMT in treating these patients.

Twelve of the 24 children were long‐term survivors and free from disease at a median time of 1,320 days (range 302–2,340). There were five relapses, two graft failures, two early deaths (one VOD, one severe GVHD), and three late deaths (two respiratory diseases, one severe GVHD). We didn't find any correlation between karyotype and outcome.

In conclusion, so far BMT seems to be the most valid treatment of childhood primary MDS. However, since the major causes of failure were regimen‐related toxicity or recurrence of the disease after BMT, it must be pointed out that, when a compatible donor even unrelated is available, BMT for childhood MDS should be given as soon as possible or at any rate prior to blastic crisis. © 1993 Wiley‐Liss, Inc.
Clostridium cadaveris bacteremia in the immunocompromised host
Authors: Gucalp Rasim , Motyl Mary , Carlisle Penny , Dutcher Janice , Fuks Joachim , Wiernik Peter H.
Published: February 2007

Clostridium cadaveris, usually considered a non‐pathogen, was isolated from blood cultures of two febrile patients with cancer. The bacteremias appeared to have originated from the abdomen. This organism has not been previously reported as the etiological agent in this setting. © 1993 Wiley‐Liss, Inc.
Congenital juvenile chronic myelogenous leukemia: Therapeutic trial with interferon alpha‐2
Authors: Hazani Anna , Barak Yigal , Berant Moshe , Bar‐Maor Adoniram
Published: February 2007

A newborn with congenital juvenile chronic myelogenous leukemia (JCML) is described. The diagnosis was suggested by the characteristic clinical and hematologic presentation, and was confirmed by the results of in‐vitro cultures of the hematopoietic progenitors, which showed excessive proliferation of monocytic colonies, with and without the addition of exogenous granulocyte‐macrophage colony stimulating factor (GM‐CSF). Based on published in‐vitro response of JCML cells to alpha interferon‐2 (α IFN 2), we treated this child for 17 weeks with subcutaneous alpha interferon, 1,000,000 units per day. In contrast to previous in vitro results, treatment of this patient affected neither the clinical course of the disease, nor the in vitro growth of the peripheral blood‐derived monocytic colonies. © 1993 Wiley‐Liss, Inc.
Intracerebral recurrence of primary intrathoracic rhabdomyosarcoma
Authors: Micallef‐Eynaud Paul D. , Goulden Nicholas T. , Langdale‐Brown Barbara , Eden Tim B. , Steers James W.
Published: February 2007

We present two cases of intracerebral recurrence of primary intrathoracic rhabdomyosarcoma, occurring within 2 months of each other. Both had embryonal histological features. One (case 1) almost certainly arose within the lung adjacent to a developmental cyst; the intrathoracic site of origin of the other (case 2) was difficult to ascertain. Both were treated on SIOP MMT protocols. The intracerebral deposits became clinically apparent soon after completing chemotherapy. They were removed surgically and second‐line chemotherapy was given both systemically and intrathecally. Neither received radiotherapy. The disease recurred in both children whilst on therapy and they died as a consequence of raised intracranial pressure. © 1993 Wiley‐Liss, Inc.
Translocation t(11;19) (q23;p13) in a child with myelomonocytic leukemia following 2 years after chemotherapy for pneumoblastoma
Authors: Rialland Xavier , Pein François , Saint‐Andre Jean‐Paul , Flamant Françoise
Published: February 2007

A child, who received successful chemotherapy for pneumoblastoma, developed a myelomonocytic leukemia with translocation t(11;19)(q23;p13) 2 years later. Although this leukemia did not present with features generally associated with therapy‐related leukemias, we think this hematologic disease to be secondary to previous chemotherapy. Involvement of chromosome 11q23 is common in acute leukemia and seems to be more significantly related to ANLL in young children (M4 and M5 FAB classification). However, our observation and previous reports suggest that this chromosomal abnormality might be more frequent in therapy‐related leukemias. The responsibility of etoposide and cisplatin may be discussed in such secondary malignancies. © 1993 Wiley‐Liss, Inc.
Leukemic blasts with markers of four cell lineages in Down's syndrome (“megakaryoblastic leukemia”)
Authors: Slørdahl Sofie H. , Smeland Erlend B. , Holte Harald , Grønn Morten , Lie Sverre O. , Seip Martin
Published: February 2007

Among 16 patients with Down's syndrome (DS) and acute leukemia admitted to our department during a ten year period, 6 were diagnosed as acute megakaryoblastic leukemia (AMkL). The diagnosis was based on clinical and hematologic criteria, confirmed in three patients with the use of monoclonal antibodies (MoAb) specific for megakaryocytic antigens. In these three, and in a fourth patient, the leukemic blasts were positive for other myeloid, lymphoid and erythroid markers in MoAb testing. We suggest that AMkL in DS is a mixed lineage leukemia with blasts presenting a variety of cell surface antigens, indicating origin from an early progenitor cell with the capability of megakaryocytic differentiation. Of the 6 patients with AMkL, 4 treated with standard AML protocols are in complete continuing remission (CCR) with observation periods from 57+ to 148+ months. © 1993 Wiley‐Liss, Inc.
Cytosine‐arabinoside, vincristine, and prednisolone in the treatment of children with disseminated langerhans cell histiocytosis with organ dysfunction: Experience at a single institution
Authors: Egeler R. Maarten , De Kraker Jan , Voǔte P. A.
Published: February 2007

Fifty‐two pediatric patients with Langerhans cell histiocytosis (LCH) were diagnosed at the Emma Kinderziekenhuis (EKZ) in Amsterdam over a 20‐year period. Eight patients with multiorgan involvement with organ dysfunction and ten patients with multiorgan involvement without organ dysfunction received chemotherapy containing cytosine‐arabinoside, vincristine, and prednisolone as part of their treatment. Five of the 8 patients (63%) with organ dysfunction and eight of the 10 (80%) without the organ dysfunction who needed chemotherapy because of the deteriorating of symptoms despite conventional therapy are presently in complete clinical remission. Two of those with organ dysfunction have died. Four of the total 18 patients developed diabetes insipidus either as an initial symptom or during the course of the disease. These results in both groups compare satisfactorily with other chemotherapeutic regimens, and since this combination is only mildly toxic, it has been well tolerated. Therefore we would recommend wider experience using this regimen in patients with widespread LCH with organ dysfunction as well as in patients with disseminated LCH in which chemotherapy proved to be necessary. © 1993 Wiley‐Liss, Inc.
Malignant germ cell tumours in two siblings
Authors: Blake Kathryn I. , Gerrard Mary P.
Published: February 2007

Familial germ cell tumours are well recognised in kinship with gonadal dysgenesis, but their occurrence in siblings with normal chromosomes is rare. We report two sisters who presented within a 4 month period with malignant ovarian germ cell tumours; one a dysgerminoma and the other a mixed tumour with marked choriocarcinomatous elements. Both children had a normal female constitutional karyotype and normal phenotype. © 1993 Wiley‐Liss, Inc.
Comparison of serial PET and MRI scans in a pediatric patient with a brainstem glioma
Authors: Bruggers Carol S. , Friedman Henry S. , Fuller Gregory N. , Tien Robert D. , Marks Lawrence B. , Halperin Edward C. , Hockenberger Beverly , Oakes W. Jerry , Hoffman John M.
Published: February 2007

Profound clinical deficits may be associated with insults to the brainstem, making management of patients with brainstem gliomas very complex. Small changes in the radiographic appearance of a brainstem tumor may be associated with significant clinical deterioration. Furthermore, both magnetic resonance imaging and computed tomography are frequently unable to differentiate between therapy‐related tissue reactions and progressive tumor. Two clinical scenarios particularly difficult to resolve include: (1) transient radiographic and clinical deterioration following hyperfractionated radiotherapy, and (2) clinical deterioration in a patient who has failed initial therapy, but has stable radiographic findings following a second therapy. We report a child with a pontine glioma whose tumor progression was demonstrated more convincingly with a 18F‐deoxyglucose positron emission scan than with magnetic resonance imaging. PET scans may be helpful in confirming that tumor progression is responsible for clinical deterioration in a patient whose MRI scans remain stable. © 1993 Wiley‐Liss, Inc.
Ten‐year survey of the intellectual deficits in children with acute lymphoblastic leukemia receiving chemoimmunotherapy
Authors: Kato Mitsuko , Azuma Eiichi , Ido Masaru , Ito Masahiro , Nii Ritsue , Higuchi Kazuo , Thara Toshiaki , Kamiya Hitoshi , Sakurai Minoru
Published: February 2007

Effects of chemoimmunotherapy, including cranial irradiation for central nervous system (CNS)‐directed therapy, on children with acute lymphoblastic leukemia (ALL) were investigated. Fifty‐five children with ALL in continuous complete remission (<5 yr) and without evidence of current or past CNS diseases were evaluated in this retrospective study. Using standard measures of intelligence (IQ), we repeatedly (1–4 times/person; mean 2.1 times) evaluated IQ in the cohort of patients for the mean follow‐up time of 9.7 yr, ranging from 5.4 to 15.8 yr. Fifty‐five patients received the total number of 118 IQ testings and 40 patients received them more than twice. Patients were examined periodically at intervals of 1.4 to 10.0 yr (mean 4.8 yr) following diagnosis. Most of the published studies dealt with single IQ testing, and long‐term follow‐ups were not enough to assess the consequent IQ change. This report confirms and extends the previous findings: decreased IQ was related to age at diagnosis and irradiation (<5 yr of age at diagnosis), irradiation‐examination interval, and female sex. Further long‐term follow‐up study will be needed in these groups, since their IQs are still on the decline even after 10 yr of diagnosis. © 1993 Wiley‐Liss, Inc.
Phase II trial of etoposide and cisplatin in patients with refractory and relapsed non‐Hodgkin's lymphoma: Cancer and leukemia group B study 8351
Authors: Rybak Mary Ellen , Anderson James , Kaplan Richard , Budman Daniel R. , Vinciguerra Vincent , Gottlieb Arlan J.
Published: February 2007

A phase‐II study was conducted by the Cancer and Leukemia Group B (CALGB) in patients with refractory and relapsed non‐Hodgkin's lymphoma (NHL) to assess the activity of the combination of etoposide and cisplatin. Sixty‐five patients were entered on study, and 51 patients were evaluated for this report. The treatment regimen consisted of etoposide, 80 mg/m2 IV daily times 5 and cisplatin 20 mg/m2 IV daily times 5, repeated every 21 days. All patients had failed 1–3 prior chemotherapeutic regimens, had measurable disease, and had a performance status of 0–2. In the 51 evaluable patients, there were 4 complete responses (8%) and 12 partial responses (23%), for an overall response rate of 31% (95% Cl: 19%, 46%). In addition, 15 patients (29%) had some improvement in disease and 6 (12%) had stable disease. Failure‐free survival for the 51 eligible patients was 40% at 3 months, 23% at 6 months, and 15% at 1 year. Significant toxicity was observed with this regimen. Severe neutropenia occurred in 20 patients (39%), severe anemia in 8 patients (16%), and severe thrombocytopenia in 18 patients (35%). One patient died of infection. Severe neurotoxicity (1) and hemorrhage (3) were also seen.

The etoposide, cisplatin combination is active in NHL; however, in this dose and schedule their combined activity is only minimally better than published reports of etoposide alone. Further studies of related combinations are under evaluation by the CALGB. © 1993 Wiley‐Liss, Inc.
Acute lymphoblastic leukemia in Noonan syndrome: Report of two cases
Authors: Piombo Margherita , Rosanda Cristina , Pasino Mirella , Marasini Maurizio , Cerruti Paola , Comelli Adele
Published: February 2007

Only two cases of Noonan Syndrome (NS) associated with tumor have previously been reported. The authors describe two new cases of NS with acute lymphoblastic leukemia (ALL), which were part of a series of 370 consecutive ALL untreated patients. © 1993 Wiley‐Liss, Inc.
Superior prognosis in combined compared to isolated bone marrow relapses in salvage therapy of childhood acute lymphoblastic leukemia
Authors: Bührer Christoph , Hartmann Reinhard , Fengler Rüdiger , Dopfer Roland , Gadner Helmut , Gerein Valentin , Gobel Ulrich , Reiter Alfred , Ritter Jörg , Henze Günter
Published: February 2007

Three hundred twenty‐six children with bone marrow (BM) relapse of non‐B acute lymphoblastic leukemia (ALL) were stratified according to the time of relapse in three consecutive multicenter trials—ALL‐REZ BFM 83, 85, and 87. Employing an intensive polychemotherapy regimen, extramedullary involvement appeared to be predictive of superior outcome in both strata as well as in the whole group (probability of 7‐year event free survival (EFS) 42% in combined vs. 15% in isolated BM relapse, P = 0.015). Children with combined BM relapse occurring later than 6 months after completion of front‐line therapy reached EFS estimates of 60%. We conclude that results of conventional poly‐chemotherapy with BFM relapse protocols are equivalent to those achieved by bone marrow transplantation in children with late combined BM relapse. © 1993 Wiley‐Liss, Inc.
Cisplatin‐resistant metastatic hepatoblastoma: Complete response to carboplatin, etoposide, and liver transplantation
Authors: Lockwood Liane , Heney David , Giles Geoffrey R. , Lewis Ian J. , Bailey Clifford C.
Published: February 2007

A child with stage 4 hepatoblastoma failed to respond to treatment with cisplatin and adriamycin. She then showed a response to carboplatin with complete clearing of pulmonary metastases. Bilobar liver disease persisted, although significantly reduced in size. A liver transplant was subsequently performed and she remains in complete remission 36 months later. After the first course of carboplatin, there was a dramatic rise in alpha‐feto protein which then fell exponentially. Carboplatin warrants further study in hepatoblastoma. © 1993 Wiley‐Liss, Inc.
SIOP working committee on psychosocial issues in pediatric oncology
Authors: Masera Giuseppe , Spinetta John J. , D'Angio Giulio J. , Green Daniel M. , Marky Ildiko , Jankovic Momcilo , Karamoschoglou Loumidi D. , Mor Wolfgang , Jones Patricia Morris , Stocker Martina , Postma Agneta , Nesbit Mark E. , Schuler Dezso , Stevens Michael , Wilbur Jordan
Published: February 2007

Recognizing the importance of psycho‐social issues in the care and cure of the child with cancer, the board of the International Society of Pediatric Oncology (SIOP) in 1991 constituted a Working Committee on Psychosocial Issues in Pediatric Oncology, with Giuseppe Masera as chair and John Spinetta as co‐chair. This committee met for the first time in Rhodes, Greece, in October 1991. The committee discussed various psychosocial issues and developed a document on Aims and Recommendations, summarizing the experiences of major centers. This document was approved by the SIOP board, which recommended diffusion of the document to the pediatric oncology community. © 1993 Wiley‐Liss, Inc.
Primitive malignant nonepithelial hepatic tumors in children
Authors: Babin‐Boilletot Annie , Flamant Françoise , Terrier‐Lacombe Marie‐José , Marsden H. Basil , van Unnik Adriaan , Deméocq François , Zucker Jean‐Michel , Voúte Paul A. , Otten Jacques , Behar Catherine , Valayer Jacques
Published: February 2007

Primary intrahepatic malignant nonepithelial tumors are very rare in children and account for 2% of all malignant mesenchymatous tumors under the age of 15 years. Clinical presentation, radiologic features, and histologic types are not unequivocal. The predominant role of surgery takes place either initially in small localized tumors or later, after initial reductive chemotherapy. In all cases, complete resection is the necessary but not sufficient condition for cure. Additional radiotherapy seems ineffective. High‐dose chemotherapy and/or liver transplantation can be proposed for resistant cases. The disease‐free survival rate is 37% at 2 years for the whole series. © 1993 Wiley‐Liss, Inc.
Carboplatin‐induced regression of an optic pathway tumor in a child with neurofibromatosis
Authors: Charrow Joel , Listernick Robert , Greenwald Mark J. , Das Lakshmi , Radkowski Mary Ann
Published: February 2007

Optic pathway tumors are common in children with neurofibromatosis‐1 (NF‐1). The optimal management of these tumors is unknown, particularly when the optic chiasm and other brain structures are involved. We report the dramatic response to carboplatin in a 10‐year‐old girl with NF‐1 and a progressive optic pathway tumor. Tumor shrinkage was accompanied by striking improvement in visual fields, return of color discrimination, and marked improvement in visual acuity. No significant toxicity was observed. One year following completion of chemotherapy the glioma remains as small or smaller than it was at the conclusion of therapy, and there has been no deterioration of vision. Carboplatin is a promising agent for the treatment of optic pathway tumors in children with NF‐1. © 1993 Wiley‐Liss, Inc.
Non‐midline endodermal sinus tumor in the head and neck region: A case report
Authors: Kebudi Rejin , Ayan Inci , Darendeliler Emin , Agaoglu Leyla , Kunay Munir , Olgac Vakur , Bilge Nijad
Published: February 2007

Germ cell tumors, in particular teratomas, are some of the most commonly found tumors in childhood. Six percent of all germ cell tumors are located in the head and neck region. Endodermal sinus tumors (yolk sactumors) of the head and neck, exclusive of the central nervous system, are rare. This study reports a 20‐month‐old girl with a mass of 5 × 5 cm on the left temporal area and with bone destruction on CT. The histopathological examination of the excised mass revealed an endodermal sinus tumor. The serum AFP and the LDH levels were elevated at diagnosis. The patient was treated by the BEP protocol (bleomycin, etoposide, and cisplatin), six cycles every three weeks. Partial response was attained after the first and complete response after the fourth cycle. The patient, who is being followed up, has been in remission for five months. © 1993 Wiley‐Liss, Inc.
Neurofibromatosis type I and malignancy: Review of 32 pediatric cases treated at a single institution
Authors: Shearer Patricia , Parham David , Kovnar Edward , Kun Larry , Rao Bhaskar , Lobe Thom , Pratt Charles
Published: February 2007

Thirty‐two cases of neurofibromatosis Type I (NF1) were identified among 6,678 pediatric cancer patients treated at St. Jude Children's Research Hospital over a 29‐year period. A total of 35 malignant neoplasms have been diagnosed in these patients. Two of three patients with second malignant neoplasms had colon cancer as the primary or second tumor. Of particular interest are two cases in which both NF1 and malignant peripheral nerve sheath tumors were present in multiple successive generations: a patient with colon cancer and non‐Hodgkin lymphoma who has a constitutional abnormality of the p53 gene, and a patient with acute lymphoblastic leukemia with the Philadelphia chromosome and other cytogenetic abnormalities, including the t(8;14). Outcome of patients in the largest subgroup, that of malignant peripheral nerve sheath tumors, was favorable only for those patients having resectable extremity lesions. In contrast, all patients with central nervous system tumors are surviving. These cases reflect the molecular and cytogenetic abnormalities that can be present in NF1 and the variety of tumors that may result in these patients. © 1994 Wiley‐Liss, Inc.
Therapy of disseminated malignant melanoma with recombinant α 2b‐interferon and piroxicam: Clinical results with a report of an unusual response‐associated feature (Vitiligo) and unusual toxicity (diffuse pulmonary interstitial fibrosis)
Authors: Harris Jules , Bines Steven , Das Gupta Tapas
Published: February 2007

Fifteen patients with disseminated malignant melanoma were treated with recombinant a 2b‐interferon (20 mU/m2 intravenously 5 days per week for 4 weeks and then 10 mU/m2 subcutaneously tiw) and piroxicam (10 mg a day orally for 10 days prior to beginning interferon and daily thereafter). Two complete responses of soft tissue disease and stabilization of disease in two other patients were obtained. One complete response was associated with the development of vitiligo. One patient who had entered complete remission was removed from the study because of diffuse pulmonary interstitial fibrosis, believed due to therapy. The combination of α 2b‐interferon and piroxicam offers no clinical advantage over the use of α 2b‐interferon alone. © 1994 Wiley‐Liss, Inc.
Treatment of relapsed or refractory acute leukemia in childhood with bisantrene combined with high dose aracytine
Authors: Leblanc Thierry , Deméocq François , Leverger Guy , Baruchel André , Lemerle Sophie , Vannier Jean Pierre , Nelken Brigitte , Guillot Thierry , Schaison Gérard
Published: February 2007

Bisantrene is an anthracene derivative which has demonstrated activity in acute myeloblastic leukemia (AML) and in lymphoma. The present study was designed to assess the reinduction rate and toxicity of bisantrene (250 mg/m2/d × 5) associated with aracytine (1000 mg/m2 twice a day × 5) in refractory and relapsed acute childhood leukemia. Patients who relapsed after bone marrow transplantation were eligible. Twenty‐six children were included. Diagnoses were as follows: 13 AML, 9 acute lymphoblastic leukemia (ALL), and 4 undifferentiated leukemia (AUL). All patients had been very highly pretreated, especially with anthracyclines, and most of them were of poor prognosis. The overall response rate was 46% with a 95% confidence interval ranging from 27‐65%. According to diagnosis, complete remission (CR) rates are: AML: 5/13, ALL: 5/9, and AUL: 2/4. Four children died, three from infection and one from acute lysis syndrome. The major toxicity was infection with grade 3 and 4 episodes occurring in 42% of patients. No significant cardiac toxicity was noted. Hepatic and renal toxicity were limited and transient. Bisantrene in association with aracytine is effective in both AML and ALL of childhood. Bisantrene should be evaluated with a five‐day schedule in other pediatric malignancies. In children with acute leukemia previously treated with high dose aracytine, new combination regimen is warranted. © 1994 Wiley‐Liss, inc.
Arteriovenous malformation mimicking recurrent medulloblastoma
Authors: Moghrabi Albert , Friedman Henry S. , McLendon Roger , Pa‐C Beverly Hockenberger , Tien Robert D. , Halperin Edward C. , Oakes W. Jerry
Published: February 2007

Magnetic resonance imaging (MRI) is becoming the method of choice for evaluation of central nervous system tumors. However, the sensitivity of this modality raises concern that new lesions in patients previously diagnosed with a brain tumor may not necessarily represent recurrent disease. We report a patient previously treated with surgery, radiotherapy, and chemotherapy for a medulloblastoma who developed a new lesion in the floor of the fourth ventricle. Histologic review following excision revealed an arteriovenous malformation. © 1994 Wiley‐Liss, inc.