bluuebunny/crossref_metadata_2025 huggingface dataset:
Malignant epithelial tumours in children: Incidence and aetiology
Authors: Al‐Sheyyab M. , Muir Kenneth R. , Cameron A. Hugh , Raafat Farrokh , Pincott J. R. , Parkes Sheila E. , Mann Jillian R.
Published: February 2007

The purpose of this study was to establish the incidence of carcinomas in children, changes in incidence over a 30‐year period, and to identify features of possible aetiological significance.

A total of 173 cases were identified, but after review of the histopathology, 30 patients were excluded because they were considered to have benign epithelial tumours or malignant tumours of nonepithelial origin. Seven other cases were excluded because pathology material was not available. Overall, in 28% of cases, the diagnoses were changed by pathology review. Thus, 136 children in the West Midlands Region diagnosed 1957–1986 were included, with carcinoid tumours (44) and tumours of skin (22), nasopharynx (14), salivary gland (13), adrenal cortex (13), thyroid (9), large bowel (5), other (16).

Excluding carcinoids, the age‐standardised incidence rate was 2.4 × 106 per year. Male:female ratio was 0.7:1 and 66% were aged >10 years. Incidence increased from 1.5 to 3.3 × 106 per year.

Genetic factors predisposing to carcinoma included tyrosinosis, MEN II and III, congenital adrenal hyperplasia and basal cell naevus syndrome. There was a case of Li‐Fraumeni syndrome and several other patients had relevant family histories.

Probable “environmental” causes included antenatal exposure to stilboestrol or hydroxyprogesterone hexanoate, stilboestrol given for premature menarche, neonatal hepatitis and prior radiotherapy.

The aetiology of carcinomas in children is multifactorial, both genetic and environmental factors being important. The incidence is increasing. © 1993 Wiley‐Liss, Inc.
James Ewing (1866–1943): “the chief”
Authors: Zantinga Arty R. , Coppes Max J.
Published: February 2007

“It is a growing conviction that to know cancer in man, one must study the disease most carefully in the human subject. Personally, I do not look for any startling advances or sensational discoveries, since it is much more likely that a steady reduction in the mortality from cancer will come chiefly from a large number of separate factors, of which the most significant appear to be increased control of the conditions leading to cancer, more general recognition of the preliminary stages of the disease, early diagnosis, and treatment of the established disease. From the consideration of these various functions of the modern cancer research hospital, I think that it must be evident that such an institution not only can justify its existence, but fills a very urgent need without which, progress of cancer research would be handicapped, and much relief that might early be extended to cancer victims would be unavailable. Nor is there any doubt that the function of supporting such an institution is properly exercised by the State, which support should be continuous and liberal”—James Ewing [2]. © 1993 Wiley‐Liss, Inc.
Tumor lysis syndrome after combination chemotherapy for ovarian cancer
Authors: Bilgrami Syed F. A. , Fallon Barbara G.
Published: February 2007

Tumor lysis syndrome is infrequently encountered following the treatment of solid tumors. A 47‐year‐old woman developed this syndrome after receiving combination chemotherapy for ovarian cancer. The levels of serum lactate dehydrogenase, uric acid, phosphate, and creatinine increased, whereas the serum calcium level decreased. These metabolic abnormalities were treated successfully using a combination of vigorous intravenous hydration, parenteral bicarbonate to keep urinary pH > 7, furosemide, and allopurinol. This unusual and easily treatable complication should be anticipated in patients with rapidly growing ovarian cancer undergoing aggressive treatment. © 1993 Wiley‐Liss, Inc.
Adult type (nonembryonal) soft tissue sarcomas in childhood
Authors: Skene Anthony I. , Barr Lester , Robinsond Martin , Fisher Cyril , Thomas J. Meirion
Published: February 2007

Nonembryonal soft tissue sarcomas are rare in childhood. We report our experience from a series of 28 patients treated at this centre, whose treatment was based on primary surgical resection. No patient required limb amputation. Anatomical distribution and histological subtypes differed from the adult sarcoma population. A total of 79 percent of the sarcomas were classified as high grade. Six patients (21%) developed local recurrence, and 6 patients (21%) developed distant recurrence, including 3 patients (11%) who developed both. Median follow‐up was 57 months. Actuarial 5 year survival was 82%, which compares favourably with the 5 year survival of adults with high grade soft tissue sarcomas treated at this centre (63%) and with the survival of children with embryonal sarcomas treated by chemotherapy. © 1993 Wiley‐Liss, Inc.
Autologous bone marrow transplantation for pediatric Wilms' tumor: The experience of the European bone marrow transplantation solid tumor registry
Authors: Garaventa Alberto , Hartmann Olivier , Bernard Jean‐Louis , Zucker Jean‐Marie , Pardo Nuria , Castel Vittoria , Dallorso Sandro , Adelbost Zora , Ladenstein Ruth , Chauvin Franck , Phillip Thierry
Published: February 2007

This survey includes 25 children with Wilms' tumor undergoing high‐dose chemotherapy associated with autologous bone marrow transplantation (ABMT) in the period June 1984‐December 1991 and enrolled in the European Bone Marrow Transplantation Registry for Solid Tumors. At diagnosis, 12 children presented stage IV disease, 5 stage III, 3 stage II, and 5 stage I. Before ABMT, 21 children had 1 to 4 relapses (median 1); 13 achieved a second or subsequent complete remission (CR), four stage IV children failed to respond to first line treatment and achieved either CR (3 patients), or partial remission (PR) after second line therapy. At high‐dose chemotherapy, 17 children were in CR and 8 had measurable disease. Seven different high‐dose regimens were administered, even if 20 children received melphalan mostly associated with vincristine and 8 involved field radiotherapy. Three children died early of pneumonitis; 2 developed an acute transient renal failure, 1 a chronic renal failure. Out of the 8 children with target disease at graft, 2 died of toxicity, 5 achieved CR, 1 obtained PR, and only 1 is presently alive in CCR at 39 months after ABMT. Of the 17 children grafted in CR, 8 are alive event‐free at 14‐90 months (median 34) from ABMT; 7 relapsed at 3‐23 months (median 7 months); 1 died of toxicity and 1 was lost to follow‐up in CR at 12 months. A salvage attempt with high‐dose chemotherapy in children with resistant or poor prognosis recurrent Wilms' tumor seems to be justified. An international cooperative protocol taking into account the increased risk of lung and renal toxicity is necessary. © 1994 Wiley‐Liss, Inc.
Value of postchemotherapy bioptical verification of complete clinical remission in previously incompletely resected (stage I and II pT3) malignant mesenchymal tumors in children: International society of pediatric oncology 1984 malignant mesenchymal tumors study
Authors: Godzinski Jan , Flamant Françoise , Rey Annie , Praquin Marie‐Thérèse , Martelli Hélène
Published: February 2007

Bioptical verification of complete clinical remission achieved with postoperative chemotherapy in 140 pediatric patients with nonradically resected stages I and II non‐parameningeal malignant mesenchymal tumors was evaluated. Tumor histology was rhabdomyosarcoma in 92 and nonrhabdomyosarcoma in 48 patients. All were included in the International Society of Pediatric Oncology „1984 Malignant Mesenchymal Tumors Study.”︁ Treatment given after non‐radical surgery and before the biopsy consisted of 3 to 6 IVA courses (ifosfamide, vincristine, actinomycin D). Complete clinical remission (no tumor at physical examination, X‐rays, sonography, computed tomography, scintigraphy) was bioptically confirmed in 52 rhabdomyosarcoma and 23 nonrhabdomyosarcoma cases, whereas in all the remaining patients (39 rhabdomyosarcoma and 22 nonrhabdomyosarcoma) complete clinical remission was not bioptically verified. Forty‐eight‐month local relapse rates were 51% in bioptically confirmed and 48% in nonbiopsied rhabdomyosarcoma patients. In nonrhabdomyosarcoma cases, relapse rates were 25% and 14%, respectively. In 4 cases, bioptical specimens contained tumor cells. In spite of aggressive treatment, only 1 is actually in remission. The total 48‐month relapse rate (biopsied and nonbiopsied) was significantly higher in rhabdomyosarcoma patients (50%) than in nonrhabdomyosarcoma patients (26%). The value of bioptical verification of complete clinical remission seems to be limited in our series, when known that relapse rate in bioptically confirmed complete remission cases remained high and the outcome in rare „positive biopsy patients”︁ was poor.
Right atrial thrombosis associated with central venous catheters in children with cancer
Authors: Wacker Pierre , Oberhansli Ingrid , Didier Dominique , Bugmann Philippe , Bongard Olivier , Wyss Marinette
Published: February 2007

Right atrial thrombosis (RAT) is infrequently diagnosed in children with cancer. Once RAT is documented, medical fibrinolysis or surgical thrombectomy is recommended. A RAT was documented in a child with lymphoma and was successfully lysed with recombinant tissue‐type plasminogen activator. The case is presented and therapeutic options reviewed.
Methotrexate content in squamous cell carcinoma of the head and neck after low‐dose methotrexate
Authors: Schifeling David J. , George Todd , McGuirt Fred , Capizzi Robert L. , Kamen Barton A.
Published: February 2007

Eleven patients with squamous carcinoma of the head and neck who were scheduled for surgical resection or endoscopic biopsy of tumor received 15 mg/m2 of methotrexate (MTX). Samples of tumor, normal mucosa, and plasma were obtained at surgery or endoscopy, 18‐24 hours after the last MTX dose. Tissue content and plasma concentration of MTX and folate were measured using sequential radioligand‐binding assays. Median MTX content was 50.0 pmol/g wet weight in tumor, 19.0 in normal mucosa, and <0.5 nM (pmol/ml) in plasma. Since dihydrofolate reductase (DHFR) content of human tumors has previously been shown to be less than 5 pmol enzyme/g wet weight, tumor MTX content exceeded expected DHFR content in all but one patient. These data support the concept that low doses of MTX saturate tumor DHFR and that, in this regard, dose escalation may have limited value. © 1994 Wiley‐Liss, Inc.
CD10+ cell population in the bone marrow of patients with advanced neuroblastoma
Authors: Mandel Mathilda , Rechavi Gideon , Neumann Yoram , Biniaminov Miriam , Rosenthal Esther , Toren Amos , Brok‐Simoni Frida , Ben‐Bassat Isaac , Ramot Bracha
Published: February 2007

Immunocytologic analyses of bone marrow can provide clinically useful prognostic information in neuroblastoma. While analyzing the bone marrow with a panel of monoclonal antibodies, which detect neuroblasts and other defining B‐, T‐, and myloid lineage, we identified two infants with stage IV‐S neuroblastoma whose bone marrow contained a large population of common acute lymphoblastic leukemia (ALL)‐like cells. This population expressed HLA‐DR, CD19(B1), CD10(CALLA), and occasionally CD20(B1). Since 1988, 17 additional patients with advanced neuroblastoma (IV‐S, III, and IV) were studied by us. In 10 of the 19 patients, the bone marrow revealed an expanded CD10 population (20‐70%). It appears that this group of patients has a better prognosis. Out of 9 patients who did not have an expanded CD10 population, 8 died within 9 months from diagnosis, whereas out of 10 patients with an expanded CD10 population only one died and the others are alive, 6‐30 months from diagnosis (P < 0.001). An expanded CD10 population in the bone marrow of disseminated neuroblastoma patients may therefore serve as a prognostic factor. Apart from the prognostic value of this particular population in the single patient, its presence may shed light on the interrelationship between the immune system and the neuroendocrine compartment. © 1994 Wiley‐Liss, Inc.
Pilot study of sequential combination chemotherapy in advanced and recurrent retinoblastoma
Authors: Advani S. H. , Rao S. R. , Lyer R. S. , Pai S. K. , Kurkure P. A. , Nair C. N.
Published: February 2007

Eight patients with advanced/recurrent retinoblastomas were treated with sequential combination chemotherapy incorporating cyclophosphamide, cisplatin, adriamycin, and etoposide. All patients achieved complete clinical response (CR) at the end of the first 75 day cycle. Three patients developed recurrence of which 2 patients had recurrence in the central nervous system and 1 patient had local recurrence. Median time to treatment failure was 30 weeks. Two patients succumbed to chemotherapy related neutropenic sepsis. One patient is alive and disease free for 72 weeks from start of treatment. This combination chemotherapy shows promise in patients with advanced/recurrent retinoblastoma and merits further Study. © 1994 Wiley‐Liss, Inc.
Management of pineal non‐germinoma germ cell tumor with residual teratoma and normal alpha‐fetoprotein
Authors: Nashold James R. B. , Oakes W. Jerry , Friedman Henry S. , Halperin Edward C. , Soo Mary , Hockenberger Beverly , Fuller Gregory N. , Tien Robert
Published: February 2007

A 16‐year‐old white male presented with multiple abnormal extraocular movements secondary to an enhancing pineal tumor. Subtotal resection of the lesion revealed a mixed malignant germ cell tumor. The pre‐operative serum alpha‐fetoprotein (AFP) was markedly elevated at 155 IU/L. The patient subsequently received radiotherapy and adjuvant chemotherapy consisting of cisplatin rotating monthly with vincristine and cyclo‐phosphamide, with dramatic tumor regression and return of AFP to normal. Eighteen months later the persistence of a substantial tumor mass despite a normal AFP raised concern for residual active tumor. Histological examination of the resected lesion revealed benign teratoma and fibrous tissue. Repeat surgical intervention may contribute to the management of mixed malignant germ cell tumors, which demonstrate a persistent mass following an initial response to treatment. © 1994 Wiley‐Liss, Inc.
Childhood anaplastic large cell lymphoma Ki‐1/CD30: Clinicopathologic features of 19 cases
Authors: Rubie Hervé , Gladieff Laurence , Robert Alain , Gaubert Isabelle , Huguet Françoise , Rochaix Philippe , Pein François , Michel Gérard , Hoerni Bernard , Sommelet Danièle , Delsol Georges
Published: February 2007

The authors report their cumulative experience of 19 children with what was previously called malignant histiocytosis (MH) but is now considered a true lymphoma and termed anaplastic large cell lymphoma (ALCL). The median age at diagnosis was 10 years and 6 months (range 2 y, 11 m, to 15 y). There were 13 males and 6 females. Most cases presented with fever, wasting and enlarged, often tender, lymph nodes. Other features were: fleeting cutaneous rashes in 7 cases; spontaneous regression of lymph nodes and skin lesions were seen in 5 patients. Bone marrow involvement was present in 3 cases, pulmonary infiltrate in 5, kidneys in 2, and central nervous system in none. The morphology of lymph node involvement was consistent with so‐called MH, a description originally applied to sinusoïdal infiltration by large „histiocytic”︁ cells. The coexpression of lymphoid activation antigens Ki‐1/CD 30 (18/19), epithelial membrane antigen EMA (18/19) and interleukin‐2 receptor/CD 25 (10/10) was the unifying immunopathologic feature of the neoplasm. Lineage antigens were not identifiable in 8/19 instances (null phenotype), while 10/19 expressed a T‐cell phenotype. None of the tumors expressed histiocytic markers. After variable, but intensive, combination chemotherapy, 15 children out of 18 evaluable achieved complete remission (CR). Among all patients, thirteen are still alive in CR (ten in first CR) with a median follow‐up of 5 years. This evaluation in the pediatric age group reinforces that so‐called MH is a lymphoid neoplasm, a conceptual change that could lead to improved understanding and therapy.© 1994 Wiley‐Liss, Inc.
Randomized study for the treatment of adult advanced Hodgkin's disease: Epirubicin, vinblastine, bleomycin, and dacarbazine (EVBD) versus mitoxantrone, vinblastine, bleomycin, and dacarbazine (MVBD)
Authors: Avilés Agustín , Guzmán Renaldo , Talavera Alejandra , García Edna L. , Díaz‐Maqueo José C.
Published: February 2007

Seventy patients with previously untreated advanced Hodgkin's disease and without bulky disease were entered in a prospective randomized clinical trial comparing epirubicin in combination with vinblastine, bleomycin, and dacarbazine (EVBD) with a regimen containing mitoxantrone, vinblastine, bleomycin, and dacarbazine (MVBD). Both groups were comparable for the variables of age, sex, stage, and presence of B symptoms and histology. Thirty‐one (88%) of EVBD‐treated patients achieved a pathologically documented complete remission (CR) compared to the 24 cases (68%) of the MVBD‐treated group. After a median follow‐up of 36 months, duration of CR is better in the EVBD‐treated patients with an actuarial 5‐year duration of CR of 80%, statistically different to the MVBD group: 53% (P < 0.01). Both regimens ahowed the same gastrointestinal toxicity, but the patients treated with the MVBD regimen shown most and severe hematological and cardiac toxicities. Also, biochemical alterations in hepatic test were observed in these patients. The alternative use of epirubicin in combination chemotherapy appears to be as effective in advanced Hodgkin's disease without bulky disease, with reduced clinical toxicity. Mitoxantrone containing regimen was not found to have an equivalent efficacy and clinical toxicity was most frequent and severe. We feld that mitoxantrone could be consider a second‐line drug in the treatment of advanced Hodgkin's disease. © 1994 Wiley‐Liss, Inc.
False positive results in a neuroblastoma screening programme
Authors: Bell Susan , Parker Louise , Craft Alan W. , Dale Gordon , McGill Andrew C. , Seviour John , Cole Michael , Smith Julian
Published: February 2007

Twenty thousand, eight hundred and twenty‐nine babies were screened for neuroblastoma at 6 months of age by measuring homovanillic (HVA) and vanillylmandelic (VMA) acid in urine and ratioing these to creatinine. Using a „cut off”︁ of the mean + 3 SD, 10 were found to be positive. Two were found on evaluation to have neuroblastoma and in the remaining 8 the raised levels of HVA and/or VMA returned to normal. Only one of the 8 false positive babies was absolutely normal, most having a chronic disorder or illness. Utilising new centiles which relate HVA and VMA to creatinine, only 3 of the 8 would have remained positive, a false positive rate of 0.01%. The false negative rate would have remained unchanged.
Malignant histiocytosis and large cell anaplastic (Ki‐1) lymphoma in childhood: Guidelines for differential diagnosis—report of the histiocyte society
Authors: Bucsky Peter , Favara Blaise , Feller Alfred C. , Nezelof Christian , Radzun Heinz‐Jochen , Schlegelberger Brigitte , Janka‐Schaub Gritta
Published: February 2007

A workshop of the Histiocyte Society was recently held, in order to discuss the problems and confusion of malignant histiocytosis (MH) and large cell anaplastic (Ki‐1) lymphoma (LCAL). The aims of this workshop were to clarify the terminology for malignant histiocytic disorders and LCAL and to produce reliable criteria for diagnosis of both MH and LCAL. This article summarises the conclusions reached. © 1994 Wiley‐Liss, inc.
Bone scintigraphy in nonsurgically treated Ewing's sarcoma at diagnosis and follow‐up: Prognostic information of the primary tumor site
Authors: Hugenholtz Elise A. L. , Piers Do A. , Kamps Willem A. , Cohen Ian J. , Lerman Chedva , Lubin Ernesto , Zaizov Rina
Published: February 2007

In order to detect skeletal metastases in patients with Ewing's sarcoma, bone scanning is commonly used. However, little information is available about the scintigraphic aspects of the primary Ewing's sarcoma during non‐surgical treatment and follow‐up. We studied retrospectively the significance of bone scintigraphic findings at the primary tumor site of 58 patients with a Ewing's sarcoma. These patients had chemotherapy and radiotherapy. At presentation 53/58 patients showed an increased tracer uptake at the primary tumor site while 5 patients with a pelvic or sacral bone localization had a normal scan. Bone scans made during treatment and more than 2 years thereafter in the 32 eligible patients demonstrated three patterns. In 16 patients the hot spot disappeared and no local tumor recurrence was encountered. In the other 16 patients the high uptake at the primary tumor site either persisted or diminished first to a normal uptake after a median period of 18 months (range 12‐36 months) and returned again to a high uptake within 6‐12 months. In these patients local Ewing's sarcoma was still present in 13, while in the other 3 cases a benign disorder (fracture, ectopic bone formation) was the underlying cause. These findings suggest that in non‐surgically treated Ewing's sarcoma persisting increased tracer uptake or its recurrence is highly suspicious for the presence of Ewing's sarcoma, while bone scans becoming negative and remaining so for more than 12 months suggest the absence of local tumor. © 1994 Wiley‐Liss, Inc.
Cytosine arabinoside and mitoxantrone treatment of relapsed or refractory childhood leukemia: Initial response and relationship to multidrug resistance gene 1
Authors: Wells Robert J. , Odom Lorrie F. , Gold Stuart H. , Feusner James , Krill Carl E. , Waldron Peter , Moulton Thomas A. , Knoppell Eva , White Michael L , Cairo Mitchell S.
Published: February 2007

The objective of this study was to determine the response rate and toxicity of high‐dose cytosine arabinoside (AC) and mitoxantrone (M) in relapsed or refractory childhood acute myeloid leukemia (AML) and acute lymphocytic leukemia (ALL) and to correlate response with the expression of the multidrug resistance gene 1 (mdr1). Twenty‐nine patients were treated with AC 1.0 g/m2 infused over 2 h every 12 h for eight doses (days 1‐4) and M 12 mg/m2 infused over 1 h (days 3‐6). Mdr1 expression was determined by a polymerase chain reaction (per) assay. Ten of 15 patients (67%) with AML obtained a complete remission (CR) of 3 to 30+ months duration. Eight of 14 (57%) ALL patients obtained a CR of 1 to 23+ months duration. The major toxicities were hematopoietic and infectious. Seventy‐nine per cent of patients developed a documented infection during induction. Mdr1 did not correlate with a lower induction rate. This AC/M regimen is active in childhood AML and ALL. © 1994 Wiley‐Liss, Inc.
Pituitary adenoma and unexpected sudden infant death: A case report
Authors: Matsuura Hiroyuki , Kitazawa Yoshiaki , Tanaka Munehumi , Morooka Keiichi
Published: February 2007

Pituitary adenomas can cause sudden death in adults, but they are quite rare during infancy. Herein we present an unusual case of sudden infant death associated with a pituitary adenoma. The clinical presentations were „quietness”︁ complained of by the parents and probable gigantism with accelerated growth rate during the first 2 months of life. The female infants had been in good health until one day when, at the age of 3 months, she was found immobile in bed. Microscopic examination showed that the cells of the entire pituitary gland were replaced by bizarre undifferentiated tumor cells. There were no invasive lesions in the surrounding brain tissue. It was also found that the cortex of the adrenal glands was atrophic. No metastatic lesions were observed in any organ. While the mechanism leading our infant to death is both controversial and unknown, we speculated that insufficient secretion of steroid hormones might have caused cardiovascular collapse. © 1994 Wiley‐Liss, Inc.
Malignant lymphoma with primary cardiac manifestations: A case report
Authors: Stein Moshe , Zyssman Isaac , Kantor Angelo , Spencer Dave , Lewis Dorothy , Bezwoda Werner
Published: February 2007

This case report describes a 29‐year‐old black patient presenting with right heart failure secondary to massive lymphomatous cardiac involvement. Additional sites of involvement included mesenteric lymph nodes and the small bowel. Malignant lymphoma with primary cardiac manifestations is very rare and follows an aggressive course. The importance of early diagnosis and commencement of immediate therapy is emphasized. © 1994 Wiley‐Liss, Inc.
Second primary neoplasms in a population‐based series of patients diagnosed with renal tumours in childhood
Authors: Hartley Ann L. , Birch Jillian M. , Blair Valerie , Jones Patricia Morris , Gattamaneni H. Rao , Kelsey Anna M.
Published: February 2007

Eight second malignant tumours developed in a population‐based series of 218 patients diagnosed with renal tumours in childhood: renal cell carcinoma of the contralateral kidney, hepatocellular carcinoma, Hodgkin's disease, and 4 basal cell and 1 squamous cell carcinomas of skin. Excess risk of developing a second malignancy (excluding skin carcinomas but including a registrable spinal neurofibroma) was 14.7 (95% Cl 4.0‐37.7, P = 0.0003) for Wilms' tumour patients. Cumulative incidence of second malignant neoplasms (excluding skin carcinoma) was zero at 10 years, 5.0% at 20 years, and 10.2% at 30 years. The most common second neoplasms seen were benign osseous/chondromatous tumours and 4 of the 7 Wilms' tumour patients with malignant tumours had previous or synchronous tumours of this kind. Development of bony exostoses may be a marker for those patients at particularly high risk of subsequent malignancy. © 1994 wiley‐Liss, Inc.
Immunosuppression: Preliminary results of alternative maintenance therapy for familial hemophagocytic lymphohistiocytosis (FHL)
Authors: Loechelt Brett J. , Egeler Maarten , Filipovich Alexandra H. , Jyonouchi Harumi , Shapiro Ralph S.
Published: February 2007

Hemophagocytic lymphohistiocytosis (HLH) describes a group of disorders with similar clinical features that are associated with a very high mortality rate. Patients with HLH, and particularly the infantile form referred to as familial hemophagocytic lymphohistiocytosis (FHL), are often treated with multiple courses of epipodophyllotoxins, such as etoposide, for prolonged periods of time. Because of the concern regarding the risk of epipodophyllotoxin‐induced acute myelogenous leukemia (AML) we have explored the use of immunosuppression as maintenance therapy for patients with FHL while they await the only known definitive treatment, i.e., bone marrow transplantation (BMT). We report 2 infants with FHL who had significant central nervous system involvement at diagnosis. Both were initially treated with etoposide, methotrexate, and glucocorticosteroids. Once clinical improvement was achieved these patients were successfully maintained in clinical remission of FHL on daily cyclosporine A (CSA) and glucocorticosteroids along with intermittent intrathecal methotrexate for 5 months until appropriate unrelated donors could be identified for BMT.
Mitoxantrone, 5‐fu, and leucovorin in breast cancer
Authors: Swain Sandra M. , Honig Susan Flamm
Published: February 2007

Eighteen patients (17 evaluable for response) with metastatic breast cancer were treated with mitoxantrone 10 mg/m2 day 1, followed by 5‐fluorouracil 375 mg/m2 day 1‐5, and high‐dose leucovorin 500 mg/m2 day 1‐5 given every 28 days. There was one complete and four partial responses for an objective response rate of 30% and 41% stable disease. The major toxicities were hematologic and gastrointestinal. This regimen has activity in metastatic breast cancer comparable to conventional and/or standard chemotherapy regimens for advanced disease. © 1994 Wiley‐Liss, Inc.
Radiation therapy for alveolar soft‐part sarcoma
Authors: Sherman N. , Vavilala M. , Pollock R. , Romsdahl M. , Jaffe N.
Published: February 2007

A clinical experience with radiotherapy in 18 patients with alveolar soft‐part sarcoma is presented. Adjuvant radiotherapy was associated with prolonged local control in six of six patients without metastatic disease at diagnosis; later one patient relapsed systematically. Meaningful palliation was achieved in all patients with extra‐skeletal (and possibly skeletal) metastatic disease. Radiation therapy may be beneficial for patients with alveolar soft‐part sarcoma by enhancing local control achieved with limited surgery, by retarding progression of metastatic deposits, and by providing meaningful palliation. © 1994 wiiey‐Liss, inc.
Peripheral T‐cell lymphoma in childhood: A report of five cases in Taiwan
Authors: Lin Kai‐Hsin , Su Ih‐Jen , Chen Rong‐Long , Lin Dong‐Tsamn , Tien Hwei‐Fang , Chen Bo‐Wen , Lin Kuo‐Sin
Published: February 2007

We encountered five children with peripheral T‐cell lymphoma (PTL) at National Taiwan University Hospital (NTUH) from 1985–1989. The patients were four boys and one girl, aged between 5 and 13 years. The duration of prediagnostic symptoms varied from 1 month to 5 years. All had pyrexia and lymphadenopathy; one had a prolonged history of granulomatosis with repeated infection. Four had hepatosplenomegaly. One patient presented with diffuse pulmonary infiltration and impending respiratory failure. All patients were negative for human T‐cell leukemia virus (HTLV)‐I antibody, and positive for HBsAg. Four patients who had EBV‐viral capsid antigen (VCA) IgG and who were IgM tested were positive for EBV‐VCA IgG, but only two had evidence of active EBV infection. Tumor cell markers were examined and showed the following phenotypes: all patients were CD2, CD3, and CD7 positive but CD19 and CD20 negative; three patients were CD4 positive and CD8 negative; the other two patients were CD4 negative and CD8 positive. Four patients died 2–7 months after diagnosis. The remaining patient received allogeneic bone marrow transplantation and has survived free of disease for more than 22 months after transplant. Our five cases reconfirm the high frequency of diagnostic delay, the heterogenous immunophenotypes, high mortality, and poor responsiveness to conventional therapy for PTL. Bone marrow transplantation in the early stage might be a possible cure of this disease. ©1994 Wiley‐Liss, inc.
Juvenile xanthogranuloma and acute leukemia: A case report
Authors: Rotte Joan J. , de Vaan Gérard A. M. , Koopman Roland J. J.
Published: February 2007

Juvenile xanthogranuloma (JXG) is a rare benign disease of the skin. It is seen in combination with juvenile chronic myelo‐monocytic leukemia (JCML) and/or neurofibromatosis type 1 (NF1). The association with acute lymphoblastic leukemia is hardly mentioned in the literature. A case report of this rare combination is described and a review of the literature is given. © 1994Wiley‐Liss, Inc.
Epidural metastasis in Wilms' tumor: A case report and review of the literature
Authors: Darendeliler Emin , Ayan Inci , Kebudi Rejin , Aǧaoǧlu Leyla , Bilge Nijad , Kinay Münir
Published: February 2007

Spinal canal involvement is not a common pattern of metastasis in Wilms' tumor. Although early detection and treatment can achieve improvement of neurological deficit, mortality remains high. We present a 5‐year‐old girl who had an epidural metastasis while she was receiving chemotherapy for stage IV Wilms' tumor. Within 2 months following laminectomy, total removal of tumor, radiotherapy, and adjuvant chemotherapy some of the neurological signs improved. © 1994 Wiley‐Liss, Inc.
Treatment strategy for disseminated langerhans cell histiocytosis
Authors: Gadner Helmut , Heitger Andreas , Grois Nicole , Gatterer‐Menz Irmgard , Ladisch Stephan
Published: February 2007

Treatment of Langerhans cell histiocytosis (LCH) remains problematic. To test the hypothesis that rapid initiation and longterm continuation of chemotherapy can improve survival and reduce recurrence and late consequences of disseminated LCH, we have completed a prospective clinical trial (DAL HX‐83). One hundred six newly diagnosed patients were stratified into three risk groups (A: multifocal bone disease [n = 28]; B: soft tissue involvement without organ dysfunction [n = 57]; C: organ dysfunction [n = 21]). All patients received an identical initial 6‐week treatment (etoposide [VP‐16], prednisone, and vinblastine), and continuation treatment for 1 year, slightly adapted according to stratification at diagnosis. It included oral 6‐mercaptopurine and eight pulses of vinblastine and prednisone for all patients, plus VP‐16 in group B and VP‐16 and methotrexate in group C. Eighty‐nine percent and 91% of patients in groups A and B and 67% of the most severely affected group C, achieved complete resolution of disease. The speed of resolution was rapid (median 4 months) and independent of disease severity. The frequency of recurrence after initial resolution was low (12%, 23%, and 42% in groups A, B, and C); overall fully 77% of patients have remained free of recurrence. Permanent consequences developed after diagnosis in 20% of the patients. Diabetes insipidus after initiation of treatment occurred in only 10% of patients. Mortality (9%) was limited to patients of groups B (two patients) and C (eight patients). Finally, among the 106 patients treated by DAL HX‐83 none have developed a malignancy (median follow‐up 6 years, 9 months). The shorter duration of active disease, low rate of recurrence and permanent consequences, and improved survival among patients with poor prognosis support the strategy of rapid initiation of a predefined prolonged treatment upon the diagnosis of disseminated LCH. © 1994 Wiley‐Liss, Inc.
Hypertrophic pulmonary osteoarthropathy in a child with thymic carcinoma: An Unusual presentation in childhood
Authors: Ilhan Inci , Kutluk Tezer , Göǧüş Safiye , Besim Aytekin , Büyükpamukçu Münevver
Published: February 2007

We report here a case of intrathoracic undifferentiated carcinoma in the thymus with hypertrophic pulmonary osteoarthropathy in a 13‐year‐old girl. The association of the two conditions is very rare. © 1994 Wiley‐Liss, Inc.
Late recurrent metastasis in Wilms' tumour
Authors: Gallego‐Melcón Soledad , de Toledo José Sánchez , Doste Divina , López Diana , Moraga Fernando , Rodríguez Carlos , Vidal Teresa , Bellmunt Joaquim
Published: February 2007

Recurrence of Wilms' tumour after 5 years of disease‐free survival is rare. We present the case of a 26‐year‐old man who had been diagnosed of Wilms' tumour at the age of 6 years treated by surgery, chemotherapy, and radiotherapy and who remained disease free for 8 years, after which lung metastases were detected. Second complete remission was attained with surgery and chemotherapy and 20 years after initial diagnosis he again presented with lung metastases, similarly achieving complete remission with surgery and secondline chemotherapy.

The clinical and biological aspects of late metastasis in this neoplasm are discussed. © 1994 Wiley‐Liss, Inc.
Failure of granulocyte‐macrophage colony‐stimulating factor to reduce febrile neutropenia in children with recurrent solid tumors treated with ifosfamide, carboplatin, and etoposide chemotherapy
Authors: Marina Neyssa M. , Shema Sarah J. , Bowman Laura C. , Rodman John , Douglass Edwin C. , Furman Wayne L. , Pappo Alberto , Santana Victor M. , Hudson Melissa , Meyer William H. , Pratt Charles B.
Published: February 2007

Ifosfamide, carboplatin, and etoposide (ICE) chemotherapy has promising activity against various solid tumors but produces significant myelotoxicity that might be ameliorated by hematopoietic growth factors. Twelve patients with relapsed solid tumors were treated with ICE chemotherapy. Carboplatin was given on day 1 at a targeted area under the concentration‐time curve (AUC) of 8 mg/mL × min (adjusted for each patient's glomerular filtration rate), followed by ifosfamide 2 g/m2 and etoposide 100 mg/m2 on days 2 through 4. Granulocyte‐macrophage colony‐stimulating factor (GM‐CSF), 1,000 μg/m2/day, was started 24 hours after each course and given for 17 days or until the absolute neutrophil count (ANC) reached 10 × 109/L. Myelotoxicity and responses in these patients were compared to those of eight patients who received the same therapy without GM‐CSF. Patients received a median of three courses (range, 1–8). All 20 patients developed grade 4 neutropenia and grade 3 or 4 thrombocytopenia. The median duration of neutropenia was significantly shorter in patients who received GM‐CSF (16.75 vs. 10 days, P = 0.005). However, the two groups did not differ in the proportion of courses associated with hospitalization for febrile neutropenia, the duration of hospitalization, or the median duration of thrombocytopenia. There were two complete, four partial, and three objective responses in the 12 patients treated with ICE plus GM‐CSF, and two partial and three objective responses in the 8 patients treated with ICE only. GM‐CSF did not reduce the occurrence of febrile neutropenia or the duration of thrombocytopenia associated with ICE chemotherapy. Studies of other hematopoietic growth factors in conjunction with this promising combination are merited. © 1994 Wiley‐Liss, Inc.
Secondary breast cancer in patients presenting with osteosarcoma: Possible involvement of germline p53 mutations
Authors: Russo Carolyn L. , McIntyre James , Goorin Allen M. , Link Michael P. , Gebhardt Mark C. , Friend Stephen H.
Published: February 2007

Second malignancies following treatment for osteosarcoma are unusual. Breast cancer occurring in patients with osteosarcoma has been reported following therapeutic chest irradiation. We now report three cases of breast cancer occurring in young women who were successfully treated for osteosarcoma. These women had not received therapeutic chest irradiation and in two of the three women there was no family history of breast cancer. Peripheral blood was available for study from one case. Of import, this case demonstrated a germline mutation in exon 7 of the tumor suppressor gene, p53. The mutation was detected by constant denaturing gradient gel electrophoresis and confirmed by DNA sequencing. In this particular patient, inactivation of the p53 gene may be involved in the development of both the first and second malignancy. © 1994 Wiley‐Liss, Inc.
Primary intraspinal soft‐tissue sarcoma in childhood: Report of two cases with a review of the literature
Authors: Raney R. Beverly , Ater Joann L. , Herman‐Liu Alisa , Leeds Norman E. , Cleary Karen R. , Womer Richard B. , Rorke Lucy M.
Published: February 2007

Two young children who presented with lower spinal cord dysfunction manifested by bilateral leg weakness and urinary retention were diagnosed with intraspinal soft‐tissue sarcoma. Neither patient had a significant extradural mass. Both tumors had histochemical features of rhabdomyosarcoma. Temporary responses were noted after combination chemotherapy either with vincristine, actinomycin D, and cyclophosphamide or with ifosfamide/mesna and etoposide. However, both patients developed uncontrollable cerebrospinal fluid (CSF) dissemination of tumor and died within 6 months of diagnosis, despite intrathecal chemotherapy and irradiation for one and very high‐dose intravenous methotrexate (33 g/m2) for the other. This rare tumor can respond to parenteral antisarcoma chemotherapy, but better strategies are needed to prevent CSF spread and ultimate demise. Early institution of intrathecal cytostatic agents may retard or prevent CSF dissemination and prolong survival. © 1994 Wiley‐Liss, Inc.
Benign hematologic neoplasm associated with mediastinal mature teratoma in a patient with Klinefelter's syndrome: A case report
Authors: Zon Robin , Orazi Attilio , Neiman Richard S. , Nichols Craig R.
Published: February 2007

An 18‐year‐old male with Klinefelter's syndrome presented with a mature teratoma of the mediastinum. He was treated with de‐bulking surgery and brief cisplatin‐based chemotherapy. Ten years later, he presented with paraplegia and was found to have an isolated epidural mass. The mass was composed of mature histiocytoid cells reminiscent of those seen in malignant histio‐cytosis. There was no evidence of medullary dissemination and there was no evidence of recurrence of the germ cell tumor. He received local radiation therapy and a single course of leukemic induction. He remains well 4 years later. This case represents a localized variant of the diffuse histiocytic malignancies associated with malignant mediastinal germ cell tumors. © 1994 Wiley‐Liss, Inc.
Malignant peripheral primitive neuroectodermal tumor (PNET) of the kidney
Authors: Mor Yoram , Nass Dvora , Raviv Gil , Neumann Yoram , Nativ Ofer , Goldwasser Benad
Published: February 2007

We describe a 61‐year‐old patient with primitive neuroectodermal tumor (PNET) arising from the kidney. Despite intensive treatment including surgery, combination chemotherapy and radiotherapy, rapid progression of the tumor was encountered and the patient died within six months with widespread disease. This appears to be the first recorded case of PNET of the kidney. © 1994 Wiley‐Liss, Inc.
Ependymoblastoma in an HIV‐positive hemophilic girl
Authors: Mandel Mathilda , Toren Amos , Hadani Moshe , Engelberg Isaac , Martinowitz Uri , Rechavi Gideon
Published: February 2007

A case of an HIV‐positive hemophilic girl with an ependymoblastoma is presented. The unusual association between the HIV‐related condition and her brain tumor is discussed, speculating a viral mechanism involved in the induction of neoplasia. © 1994 Wiley‐Liss, Inc.
Monocyte/macrophage system and malignancies
Authors: Schmidt Dietmar
Published: February 2007

Neoplasias of the monocyte/macrophage system are rare. They include malignant histocytosis, sarcomas of dendritic cells, and possibly a malignant form of Langerhans cell histiocytosis. Characterization of all these neoplasms can be easily done along the lines which have been developed for the derivation of the normal cellular counterparts. The possibility of a malignant transformation of the cells of the monocyte/macrophage system is suggested by the fact that all members of this system can undergo mitotic division. The most controversial among the different entities is malignant histiocytosis, which has proved to be anaplastic large cell lymphoma (ALCL) with Ki1 expression in most cases. The few cases of true malignant histocytosis may or may not express the Ki1 antigen. Sarcomas of dendritic cells have been related mainly to interdigitating or follicular dendritic cells. Prognosis of follicular dendritic cell sarcoma is probably more favorable than that of interdigitating cell sarcoma. Besides these more frequent subtypes, a sarcoma of the sinus lining cells can be separated which represents a low‐grade malignant neoplasm. All cases encountered so far were associated with a protracted clinical course, albeit multiple recurrences. A distinctly more aggressive behavior is encountered in those cases which have been identified as a malignant form of Langerhans cell histiocytosis. However, the issue of this particular form of histiocytic disorder is still unsettled. © 1994 Wiley‐Liss, Inc.
Osteosarcoma of the trunk treated by multimodal therapy: Experience of the cooperative osteosarcoma study group (COSS)
Authors: Bielack Stefan S. , Wulff Beate , Delling Günther , Göbel Ulrich , Kotz Rainer , Ritter Jörg , Winkler Kurt
Published: February 2007

The case histories of all patients with osteosarcoma of the trunk entered into the consecutive studies COSS 80 through COSS 86 of the Cooperative German/Austrian Osteosarcoma Study Group (COSS) were analyzed in order to evaluate their clinical characteristics and the impact of modern neoadjuvant therapy on prognosis. They were compared to those of all patients with extremity osteosarcoma treated according to the same protocols. While tumors of the trunk comprised only 32 (4.8%) of 665 primary classical osteosarcomas, secondary osteosarcomas were much more likely to be located in bones of the axial skeleton (6 of 18, 33%). Patients with primary osteosarcoma of the axial skeleton were older (mean: 20.8 vs. 15.2 years,P< 0.01) and were more likely to present with metastases at diagnosis (34% vs 12%,P< 0.001) than those with primary extremity osteosarcoma.

In contrast to extremity tumors, local surgical treatment failure was very common in osteosarcomas of the trunk. Complete tumor removal was achieved in less than half of all evaluable cases. The prognosis of eight patients with localized primary axial osteosarcoma and effective surgical local control was not inferior to that of 483 equally evaluable patients with extremity tumors.

In conclusion, while secondary systemic spread of axial osteosarcoma may be avoided in patients treated with multiagent chemotherapy, successful treatment is often barred by primary metastatic disease and inability to control the local tumor site. © 1995 Wiley‐Liss, Inc.
Primary thyroid carcinoma in children: A retrospective study of 20 patients
Authors: Massimino Maura , Gasparini Marco , Ballerini Emanuela , Del Bo Romualdo
Published: February 2007

A total of 20 children (median age 11 years) were treated for primary thyroid carcinoma from 1976 to 1990. Papillary adenocarcinoma was diagnosed in 19 and follicular in one case. Nineteen of 20 patients were considered amenable to surgery, which consisted of total thyroidectomy in 14 and partial thyroidectomy in 5. Only one patient with extensive perithyroid soft tissue infiltration was treated with external beam radiotherapy. Monolateral or bilateral cervical nodal dissection was performed in eight and six children, respectively; in nine cases without clinical evidence of metastatic nodes. Pathological examination showed that tumor extent was greater than that clinically assessed: Multiple tumor foci within the thyroid were assessed in 8/19, unilateral positive nodes in 8, and bilateral in 6, and soft tissue infiltration in 7. Subsequently 10 patients received thyroid‐stimulating hormone (TSH) suppressive hormonotherapy. Relapses occurred in 7/20 at 2–48 months (median 18) from primary treatment: Four in cervical nodes, two in cervical nodes and lungs, and one in lungs. These seven patients were salvaged with node dissection and radioiodine therapy for lung metastases. All the 20 children are alive and disease‐free after a median follow‐up of longer than 10 years. The incidence of relapse was greater in the group of patients not given TSH‐suppressive hormonotherapy. Total thyroidectomy produced permanent hypoparathyroidism in 5/14 (36%). Thyroid carcinoma in children of this series frequently presented with multiple tumor foci within the thyroid and cervical node metastases. Prognosis was favourable even after relapse and was not related to the extent of surgical treatment. Limited surgery and suppressive hormonotherapy may be adequate therapy for thyroid carcinoma in children. © 1995 Wiley‐Liss, Inc.
Induction toxicity in childhood acute lymphoblastic leukemia: A comparison of two schedules of daunorubicin administration
Authors: Shaw Peter J. , Eden Tim , Paradisis Mary
Published: February 2007

The haematological toxicity of the induction phase of chemotherapy for acute lymphoblastic leukaemia (ALL) was compared in two cohorts of patients. The principal difference between these two cohorts was the mode of administration of the anthracycline, daunorubicin (DNR). Both groups received four‐drug induction chemotherapy, which produced a high remission rate. Those receiving DNR on days 1 and 2 experienced a profound but shorter period of neutropenia and more severe thrombocytopenia than those who received the DNR weekly. The pattern of hospitalisation and support facilities in the individual unit may determine which regimen is to be preferred. These observations are relevant for the newly diagnosed patient in whom an anthracycline is retained in the induction therapy for ALL. © 1995 Wiley‐Liss, Inc.