bluuebunny/crossref_metadata_2025 huggingface dataset:
Prophylactic cranial irradiation increases the risk of testicular damage in adult males surviving all in childhood
Authors: Siimes Martti A. , Lie Sverre O. , Andersen Ole , Marky Ildiko , Rautonen Jukka , Hertz Henrik
Published: February 2007

By combining three series of Scandinavian patients, we were able to compare the late testicular sequelae in 41 adult males whose therapy had included chemotherapy alone or chemotherapy with cranial irradiation without other radiotherapy for ALL in childhood. In multivariate analysis, cranial irradiation was associated with a decrease of 5.7 (95% confidence limits 1.5–9.9) cm (P= 0.010) in height, and a decrease of 4.8 (0.3–9.2) ml (P= 0.036) in testicle size. Cyclophosphamide was associated with increases of 8.2 (−0.5–16.9) (P= 0.065) and 3.9 (0.3–7.4) U/L (P= 0.036) in serum FSH and LH concentrations, respectively. Of the 12 patients who had received both cranial irradiation and cyclophosphamide therapy, 4 (33%) had delayed pubertal development as compared with 1 (3.5%) of the other 29 patients (P= 0.008). Patients 12–16 years of age at diagnosis had larger testicles (P= 0.051) and higher testosterone concentrations (P= 0.026) than others. Neither sexual activity nor semen findings correlated with the preceding treatment. Our data indicate that prophylactic cranial irradiation may be associated with impaired growth and pubertal development in boys with ALL. © 1993 Wiley‐Liss, Inc.
Ewing's sarcoma in a child with human immunodeficiency virus (type 1) infection
Authors: Lyall E. G. Hermione , Langdale‐Brown Barbara , Eden Osborn B. , Mok Jacqueline Y. Q. , Croft Nicholas M.
Published: February 2007

A male child with vertically transmitted human immunodeficiency virus type 1 (HIV) infection developed a Ewing's sarcoma of the left fibula at 6.1 years of age. We report the antitumour chemotherapy given and the response of the tumour. Six months after tumour diagnosis the child died of probable HIV encephalopathy. This is the first reported case of Ewing's sarcoma in an HIV‐infected child. © 1993 Wiley‐Liss, Inc.
Recurrent neuroendocrine (merkel cell) carcinoma of the skin presenting as marrow failure in a man with systemic lupus erythematosus
Authors: Lentz Steven R. , Krewson Laurel , Zutter Mary M.
Published: February 2007

Neuroendocrine carcinoma of the skin, Merkel cell carcinoma, is an aggressive malignancy with a high indidence of local recurrence and metastasis. We describe a case of neuroendocrine carcinoma of the skin with unusual metastatic spread to the bone marrow in a 55‐year‐old man with systemic lupus erythematosus. Following resection of a left temporal subcutaneous nodule and complete left parotidectomy and radical lymph node dissection, the patient presented with pancytopenia. Although focal bony metastatic disease was not identified, his bone marrow was diffusely replaced by metastatic carcinoma. High‐dose chemotherapy allowed a brief remission; however, the patient subsequently died with central nervous system metastases. © 1993 Wiley‐Liss, Inc.
Introduction to the genetics of primary renal tumors in children
Authors: Knudson Alfred G.
Published: February 2007

Wilms tumor can be explained only partially by the “two hit” model that was originally developed for retinoblastoma. Heterogeneity of two kinds, operates. The first is that four other primary tumors are regularly observed in children, and the second is that Wilms tumor itself appears to represent more than one genetic entity. All five of these primary renal tumors arise from primary or secondary mesenchyme, renal blastema, or renal epithelium. Mesoblastic nephroma, and possibly clear cell sarcoma, may have some genetic affinity with Wilms tumor, but rhabdoid tumor of the kidney and renal carcinoma do not. At least three different genes seem to be important in the origin of Wilms tumor. One, WT1, whose mutations may be associated with aniridia, may follow the “two hit” model in that there are cases in which both copies of the gene are defective or lost, as expected for a tumor suppressor gene. A second gene, which is associated with Beckwith‐Wiedemann Syndrome (BWS) and which has not been cloned, appears to be imprinted in females, and may have an oncogene function. It is evidently activated by gain of a paternal allele or by loss of the inactive, but possibly trans‐sensing, maternal allele. Activation of the insulin‐like growth factor II gene may be a final common pathway for mutation in both WT1 and BWS. A third gene is unlinked to either of the other two, but its location and function are unknown. It shares with WT1 specificity for Wilms tumor, which is not true of the BWS gene. © 1993 Wiley‐Liss, Inc.
Localized dumbbell neuroblastoma: A study of 25 cases treated between 1982 and 1987 using the same protocol
Authors: Plantaz Dominique , Hartmann Olivier , Kalifa Chantal , Sainte‐Rose Christian , Lemoine Georges , Lemerle Jean
Published: February 2007

Among the 108 non‐metastatic neuroblastomas treated at the Institut Gustave Roussy between 1982 and 1987, 25 dumbbell neuroblastomas were observed. Therapeutics included: 1) an initial laminectomy in forms with neurological deficit; 2) surgical excision of the primary tumor; 3) preoperative chemotherapy in children in which the primary tumor was considered as unrespectable at diagnosis and postoperative chemotherapy in cases of incomplete resection; and 4) radiation therapy on macroscopic residual disease. Fifteen out of 25 (60%) presented a neurological deficit. A laminectomy was performed in 14 cases. Neurological recovery was good in 4 cases, partial in 4 cases, and absent in 4 cases. Two patients were worse after the procedure. The event‐free survival was 88%. This high survival rate is linked: 1) to the non‐metastatic stage; 2) to a high proportion of children under 1 year of age (18/25) (median age = 7 months); and 3) to a high proportion of thoracic location (12/25). The high incidence of macroscopically incomplete excision (13/25) did not jeopardize the prognosis. Out of the 22 survivors, there were 8 cases of major neurological sequellae (36%) and 5 cases of major orthopedic ones (26%). The coexistence of a serious functional prognosis and an excellent vital prognosis for these patients has led us to analyse the therapeutic modalities, to re‐evaluate the necessity of routine initial neurosurgical removal by laminectomy and to discuss the use of first line chemotherapy. © 1993 Wiley‐Liss, Inc.
Severe acne and hyperandrogenemia following dactinomycin
Authors: Blatt Julie , Lee Peter A.
Published: February 2007

The etiology of dactinomycin‐associated acne has not previously been studied. An 8 1/2 year old girl with embryonal rhabdomyosarcoma of the left petrous bone was prepubertal at the time of presentation. Treatment according to a current Intergroup Rhabdomyosarcoma Study regimen consisted of vincristine, dactinomycin, and cyclophosphamide at 3 to 6 week intervals, as well as hyperfractionated radiotherapy to the tumor bed. Severe acne of the forehead developed within 10 days of starting therapy and resolved over the next 2 months. Serial measurements of serum hormones documented periodic increases in androgen levels coincident with courses of dactinomycin. These observations support a relationship between drug, dermatitis, and hormone levels. Study of other patients will be of interest to document the frequency, degree, and mechanism of hyperandrogenemia following dactinomycin. © 1993 Wiley‐Liss, Inc.
Isolated primary cutaneous aspergillosis of the labia
Authors: Raszka William V. , Shoupe Braden L. , Edwards E. Glenn
Published: February 2007

Isolated cutaneous aspergillosis is a rare complication of severe immunocompromised states. We describe a case of isolated cutaneous aspergillosis of the labia minora in an adolescent undergoing treatment for relapsed acute non‐lymphocytic leukemia. The patient was successfully treated with a regimen that consisted of amphotericin B, granulocyte‐colony stimulating factor, and surgical debridement. © 1993 Wiley‐Liss, Inc.
Critical observations on neuroblastoma treatment with 131‐I‐metaiodobenzylguanidine at diagnosis
Authors: Mastrangelo Renato , Lasorella Anna , Iavarone Antonio , Rufini Vittoria , Troncone Luigi , Danza Francesco , Riccardi Riccardo
Published: February 2007

Results of treatment with 131‐I‐Metaiodobenzylguanidine (131‐I‐MIBG) in patients with resistant neuroblastoma appear encouraging if one considers that most of the patients had far advanced, intensively pre‐treated disease. To further explore the potential role of this new drug in untreated patients, we treated 3 children with stage III neuroblastoma. All three of our cases received 131‐I‐MIBG at relatively low dose with the complete disappearance of the tumor mass in case 1, whereas in cases 2 and 3 CT scan showed a significant reduction of the tumor mass and, interestingly enough, no evidence of 131‐I‐MIBG uptake of a tracer dose in the remaining tumor. Particularly, in case 2, the persistence and subsequent progression of part of the tumor mass without 131‐I‐MIBG uptake after a therapeutic dose of 131‐I‐MIBG, which apparently destroyed the 131‐I‐MIBG‐positive cell population, clearly suggest heterogeneity at diagnosis, with a dual neuroblastoma cell population, one with 131‐I‐MIBG uptake and the other without. Besides the biological implications of the 131‐I‐MIBG uptake heterogeneity in neuroblastoma at diagnosis, our findings suggest that in stage III neuroblastoma patients even a relatively small dose of 131‐I‐MIBG administered at diagnosis is sufficient to destroy either the primary tumor completely (case 1) or the part of the tumor (case 2 and 3) which shows 131‐I‐MIBG uptake, without any significant hematologic toxicity. Furthermore, a single course of 131‐I‐MIBG at the dosage employed does not appear to jeopardize the subsequent use of chemotherapy. In conclusion, if our data are confirmed by further investigation, 131‐I‐MIBG may be included as a front line drug shortly followed by chemotherapy in future treatment strategies of advanced neuroblastoma without or with minimal bone marrow infiltration. © 1993 Wiley‐Liss, Inc.
Cytogenetic abnormalities in mesoblastic nephroma: A link to Wilms' tumour?
Authors: Roberts Paul , Lockwood Liane R. , Lewis Ian J. , Bailey Clifford C. , Batcup Gillian , Williams Jeffrey
Published: February 2007

Cytogenetic analysis of tumour material from a congenital mesoblastic nephroma is reported. Two cell lines were found, one with a normal 46,XY karyotype and the other with a hyperdiploid 51,XY karyotype, including a rearrangement of chromosome 11 at 11p15. This finding is of interest since loss of allelic heterozygosity at polymorphic 11p15 loci has been described in sporadic Wilms' tumour [1], and both cytogenetic [2] and molecular [3] changes of 11p15 are found in the Wiedemann‐Beckwith syndrome, a condition with a predisposition to embryonal tumours, particularly Wilms' tumour. Our results lead us to speculate on the implications relating to the pathogenesis of this relatively benign tumour variant with respect to the current understanding of the genetics of Wilms' tumour. © 1993 Wiley‐Liss, Inc.
Salivary gland carcinomas in children: A review of 15 cases
Authors: Taylor Roger E. , Gattamaneni H. Rao , Spooner David
Published: February 2007

Salivary gland carcinomas are rare in childhood. We have reviewed the case records of 15 children aged 3–14 years (median 11) identified from children's tumour registries. Primary sites were parotid: 11, submandibular: 3, and base of tongue: 1. The range of histologies was similar to that occurring in adults. Six were treated by complete excision, with one given post‐operative radiotherapy (RT). All six remain disease‐free at 2 months to 21 years after completion of treatment. Five were treated by partial or sub‐total excision. Four were given post‐operative RT, of whom 3 are disease‐free at 3 years, 6 months ‐ 18 years and 1 lost to follow‐up (LTFA). One not given RT developed a local recurrence at 11 months and was given RT and LTFA. Four patients had a biopsy only. Three were treated by RT. One is disease‐free at 8 years, one died of metastatic disease at 6 months, and one developed a local recurrence at 11 years and has remained disease‐free following salvage surgery. One patient with advanced disease not suitable for RT died 3 months after diagnosis. Complete excision is the treatment of choice. Following sub‐total or incomplete excision post‐operative RT can prevent recurrence. Careful RT planning is necessary to minimise late effects. © 1993 Wiley‐Liss, Inc.
Congenital anomalies and genetic syndromes in 173 cases of medulloblastoma
Authors: Evans Gareth , Burnell Liza , Campbell Richard , Gattamaneni H. Rao , Birch Jillian
Published: February 2007

One hundred seventy‐three consecutive cases of medulloblastoma recorded in the Manchester Children's Tumour Registry from 1954 to 1989 were studied. After review of case notes, X‐rays, and health surveys the clinical outcome and incidence of congenital anomaly was determined. A previously unreported association with Rubinstein Taybi syndrome was found. Evidence of a genetic syndrome or congenital anomaly was found in 6.4%. These figures provide further evidence of the higher‐than‐expected incidence of congenital abnormalities. © 1993 Wiley‐Liss, Inc.
Association of malignancy and langerhans' cell histiocytosis: Report of three cases
Authors: De Camargo Beatriz , Alves Antonio Correa , Gorender Ethel F. , Bianchi Alois
Published: February 2007

Among 44 children with Langerhans' cell histiocytosis (LCH) seen at the Pediatric Department of the A.C. Camargo Hospital, São Paulo, Brazil, three developed malignancy, two before and one after the diagnosis of LCH. Malignancy could be attributed to treatment in one of the three children. Whether the cancer in the other two children represents a chance association of the two processes or is treatment‐related, is unknown.
Langerhans' cell histiocytosis presenting with the superior vena cava syndrome: A case report
Authors: Mogul Mark , Hartman Gary , Donaldson Sarah , Gelb Arnold , Link Michael , Amylon Michael , Glader Bertil
Published: February 2007

A case of Langerhans' cell histiocytosis confined to the mediastinum and presenting with de novo superior cava syndrome is reported. The causes of superior vena cava syndrome in childhood are discussed as is the importance of obtaining pathologic diagnosis prior to initiating therapy. © 1993 Wiley‐Liss, Inc.
Carcinoma of the choroid plexus: A pediatric experience
Authors: Pierga Jean‐Yves , Kalifa Chantal , Terrier‐Lacombe Marie‐Jose , Habrand Jean‐Louts , Lemerle Jean
Published: February 2007

Carcinoma of the choroid plexus (CCP) is a rare tumor of the central nervous system which occurs more frequently in patients under 3 years of age. We report 10 cases of CCP in children. There were 5 males and 5 females, aged 5 to 84 months at diagnosis (median 32 months). Eight CCP were located in the lateral ventricle and 2 in the fourth ventricle. All patients underwent initial surgery, with complete resection in 5 cases. Postoperative treatment included radiotherapy alone in 4 patients, chemotherapy alone in 4, both in 1 patient, and no further treatment in one patient. Six patients remain in first continuous complete remission with a follow‐up of 12 to 156 months (mean 34 months). Five of these six patients had a complete surgical resection of their tumor and 4 of them received chemotherapy alone. A comparison with the series in the literature suggests that total resection of the tumors is the major prognostic factor for survival. Recourse to additional treatments to prevent local relapses and CNS dissemination seems necessary. In our opinion, radiotherapy should be avoided as far as possible because of its deleterious effect in very young children. As some of the long term survivors did not receive radiation treatment, we conclude that CCP can be treated post operatively using chemotherapy protocols designed for infants with malignant brain tumors. © 1993 Wiley‐Liss, Inc.
Chemotherapy in the management of infantile fibrosarcoma
Authors: Kynaston J. Anne , Malcolm Archie J. , Craft Alan W. , Davies Stella M. , Jones Patricia H. Morris , King Derek J. , Mitchell Christopher D. , Oakhill Anthony , Stiller Charles A.
Published: February 2007

Infantile fibrosarcoma (IF) has traditionally been treated with surgery, which may have considerable morbidity. Chemotherapy has been suggested in order to reduce the need for extensive surgery. Nine children with histologically confirmed IF who received chemotherapy are described. Six children were treated with chemotherapy initially, two following conservative surgery, and one following recurrence after surgery. All received vincristine (V) and actinomycin D (A), and six received additional drugs including ifosfamide (I), cyclophosphamide (C), adriamycin (Ad), etoposide (E), and cis‐platinum (CDDP). Objective responses were achieved in eight: three responded completely (CR), two responded partially (PR), which allowed conservative surgery, one had stable disease, one had an initial PR, but subsequently had tumour recurrence 1 month after cessation of treatment, necessitating further surgery and chemotherapy, and one had an initial PR but died following local and regional metastases. One child had no response to chemotherapy but is alive with stable residual disease. Thus, five of nine children achieved a CR—three with chemotherapy alone. With the inclusion of chemotherapy as part of their treatment, five children, for whom curative surgery may have resulted in amputation, remain alive with limbs intact. Chemotherapy including V and A should be given to infants with fibrosarcoma in whom curative surgery would be mutilating. © 1993 Wiley‐Liss, Inc.
N‐myc oncogene and urinary catecholamines in children with neuroblastoma
Authors: Barontini Marta , Gutiérrez Marina I. , Levin Gloria , Mur Nora , Diez Blanca
Published: February 2007

This paper reports the analysis of N‐myc amplification, urinary vanillylmandelic acid (VMA), and norepinephrine (NE) excretion and survival in 22 children with neuroblastoma and 7 with ganglio‐neuroblastoma. Five patients had N‐myc amplification (from 30 to more than 200 copies), all of whom had advanced‐stage disease. The urinary excretion of VMA was normal in all of them, only one showed increased NE excretion. All patients with stage C‐D disease and one copy of N‐myc had increased VMA urinary excretion and increased (9/14) or normal (5/14) NE urinary excretion. All patients with stage A or B disease had one copy of N‐myc. Half of them showed increased VMA urinary levels, while only 3/10 showed increased NE urinary values.

Comparison of cumulative survival curves in relation to N‐myc amplification and to VMA and NE urinary excretion showed a clear parallelism. Amplification of N‐myc corresponded to normal VMA and NE urinary excretion, and was associated with the worst prognosis (P < 0.01), while increased VMA and/or NE excretion was found in patients with only one copy of N‐myc. © 1993 Wiley‐Liss, Inc.
Clear cell sarcoma of the kidney in a 4‐month‐old infant: A case report
Authors: Newbould Melanie J. , Kelsey Anna M.
Published: February 2007

A case of clear cell sarcoma of the kidney is described in a 4‐month‐old male infant. The tumor had a typical histological pattern, consisting of sheets of tumor cells possessing “empty” nuclei set within a delicate capillary network. There was extensive necrosis and tumor cell cytoplasm contained vimentin. This tumor occurs only rarely in infants less than six months of age. The different renal neoplasms of childhood have diverse prognoses and demand specific therapy so that accurate diagnosis is mandatory. The present case illustrates that clear cell sarcoma, a highly malignant neoplasm, must be considered in the differential diagnosis of a renal mass occurring in this age group. © 1993 Wiley‐Liss, Inc.
Avascular femoral head necrosis in pediatric cancer patients
Authors: Hanif Iftikhar , Mahmoud Hazem , Pui Ching‐Hon
Published: February 2007

Avascular femoral head necrosis was identified in 15 patients treated at a major pediatric oncology center from 1974 to 1991. The predominant underlying diagnosis was acute leukemia (lymphoblastic, n = 6; non‐lymphoblastic, n = 2); two patients had chronic myeloid leukemia, two Hodgkin's disease, and three other solid tumors. Patients ranged from 7 to 27 years of age at diagnosis of this complication, with a median interval of 25 months (range, 0–11 years) from primary diagnosis. Both steroids and radiation therapy appear implicated in the pathogenesis of avascular necrosis: nine patients had received high cumulative doses of prednisone (3.4–14 g/m2), four had received 35–64.8 Gy local irradiation involving the femoral head, and one underwent total body irradiation (12 Gy). Of the 11 surviving patients, six are asymptomatic and fully active. Two patients have joint pain that is responsive to conservative measures. Severe pain and joint deterioration necessitated arthroplasty in two cases, and a third child is undergoing orthopedic evaluation because of worsening symptoms. Thus, like adult cancer patients, children who receive high doses of steroids or local irradiation involving femoral heads are at risk for avascular necrosis. In patients with chronic myeloid leukemia, the complication may be disease related. Although outcomes in our series are better than those reported in adults, the long period of risk for these children and young adults precludes definitive conclusions. © 1993 Wiley‐Liss, Inc.
Hemihypertrophy, bilateral Wilms' tumor, and clear‐cell adenocarcinoma of the uterine cervix in a young girl
Authors: Stalens Jean‐Philippe , Maton Pierre , Gosseye Serge , Clapuyt Philippe , Ninane Jacques
Published: February 2007

We report the case of a young girl who had hemihypertrophy and developed Wilms' tumor in both kidneys and clear‐cell adenocarcinoma of the uterine cervix with no maternal history of stilbestrol ingestion during pregnancy. Karyotype on the second Wilms' tumor showed monosomy 22. The link between hemihypertrophy and Wilms' tumor is well known, but their association with clear‐cell carcinoma of the cervix has not been previously described. © 1993 Wiley‐Liss, Inc.
Hypertrophic osteoarthropathy in childhood malignancy
Authors: Staalman Christoffel Rudolf , Umans Ulco
Published: February 2007

Hypertrophic osteoarthropathy (HOA), well known in adults, is rarely encountered in children. The clinical features include clubbing of the fingers and toes, arthritis, and a sometimes painful ossifying periostitis of the tubular bones.

Apart from a hereditary form (primary HOA), most of the cases encountered in children are secondary and associated with conditions such as chronic suppurative lung processes (e.g., cystic fibrosis), congenital heart disease, biliary atresia, and polyposis coll. The association with malignant disorders, which is relatively common in adults, is very rare in children. In 1986 the authors published a case report of a patient with carcinoma of the nasopharynx who developed HOA. Another similar patient has been encountered. In both, the appearance of HOA was associated with a very poor prognosis.

A meticulous research of the literature from 1890 to 1990 revealed only 24 children (19 boys, 5 girls) under the age of 18, with malignancy and associated HOA. Among them were 10 patients with a carcinoma of the nasopharynx, 8 with osteosarcoma, 3 with Hodgkin's lymphoma, 1 with a periosteal sarcoma, 1 with mesothelioma of the pleura, and 1 with carcinoma of the thymus. In five patients with HOA, there were no abnormalities of the lungs, mediastinum, or pleura, and none developed during the course of the disease.

Many authors mention the predictive value of HOA, especially in association with malignant tumors. In contrast to suppurative processes in the lungs, in those with neo‐plastic disease involving the chest, HOA may precede pulmonary symptoms by 1–18 months. A striking feature of HOA in these instances is the reversibility of the complaints after successful treatment of the disorder of the chest, both in benign and malignant conditions.

The present case is the second reported by the authors and the first description of a girl with carcinoma of the nasopharynx developing HOA. © 1993 Wiley‐Liss, Inc.
Ifosfamide‐induced subclinical nephrotoxicity and its potentiation by cisplatinum
Authors: Rossi Rainer , Danzebrink Sigrid , Hillebrand Dirk , Linnenbürger Katrin , Ullrich Kurt
Published: February 2007

Renal function was assessed in 72 children and adolescents 3.5 to 123 months after completion of chemotherapy employing ifosfamide (n = 39) or ifosfamide plus cisplatinum (n = 33). No patient had preexisting renal parenchymal disease. Whereas reduction in glomerular filtration rate was present in six of 69 patients (8.7%), impairment of tubular transport for phosphate, glucose, and amino acids was more frequent: 32.8% of the patients showed reduction in phosphate reabsorption, and glucose and amino acid reabsorption was lowered in 16.4% and 55.0%, respectively. Elevated sodium excretion was found only occasionally, and there was no evidence of renal tubular acidosis. Proximal tubular damage is related to ifosfamide chemotherapy, but correlation between ifosfamide dose and phosphate reabsorption was not linear. The most severe depletion of phosphate reabsorption was seen in patients treated with both ifosfamide and cisplatinum. On reexamination of phosphate reabsorption after a median interval of 8 months, the majority of patients with initially reduced values showed further deterioration of this function. © 1994 Wiley‐Liss, Inc.
Cervical epidural analgesia for a cancer child at home
Authors: Veyckemans Francis , Scholtes Jean‐Louis , Inane Jacques N , Catheter Cervical Epidural
Published: February 2007

A cervical epidural catheter was placed in a 32‐month‐old girl suffering from painful bone metastases of a relapsing neuroblastoma in the left forearm. This therapeutic approach was chosen because oral morphine had failed to provide analgesia while producing intolerable side effects. The epidural injection of a mixture of morphine and bupivacaine provided complete analgesia with no side effects. This enabled the parents to take care of their child at home until the end, 35 days later. © 1994 Wiley‐Liss, Inc.
Total body hyperthermia in combination with etoposide and melphalan in a child with acute myelomonocytic leukemia
Authors: Wessalowski Rüdiger , Willnow Ulrich , Jürgens Herbert , Dehnen Herman , Göbel Ulrich
Published: February 2007

In vitro and clinical studies have shown antineoplastic effects of hyperthermia alone and in combination with other treatment modalities. Synergistic cytotoxic effects of chemotherapy and hyperthermia have been demonstrated on leukemic cell clones in vitro. It seems that hyperthermia is effective in overcoming chemotherapy resistance. Several groups treated solid tumors by using total body hyperthermia (TBHT). However, only a few studies have been reported investigating the clinical effects of TBHT in myeloproliferative disorders. We report the case of a 7‐year‐old boy with myelomonocytic leukemia treated with TBHT (2 hours, 42°) combined with etoposide (600 mg/m2), melphalan (30 mg/m2) and hyperglycemia (200‐300 mg/dl). Within 24 hours after TBHT, the leukemic cells decreased after TBHT from 53,000/μl to zero. Skin leukemic infiltrates, resistant to conventional treatment, also responded well. Although our patient relapsed 34 days after TBHT, these results indicate that TBHT in combination with cytotoxic treatment may be a useful treatment modality in refractory leukemia.
Gastric lymphoma in an 11‐year‐old boy: A case report
Authors: Ashorn Per , Lähde Pirkko‐Liisa , Ruuska Tarja , Mäkipernaa Anne
Published: February 2007

A child with prolonged abdominal symptoms was shown to have a concomitant gastric non‐Hodgkin lymphoma and gastritis caused by Helicobacter pylori infection. After a course of antibiotics and 6 months of chemotherapy, the patient is disease free.

The relationship of the microbial infection and tumor development is discussed. The case supports a hypothesis that local H. pylori infection may be a predisposing factor for gastric lymphoma. © 1994 Wiley‐Liss, Inc.
Hepatocellular carcinoma in the very elderly: To treat or not to treat?
Authors: Arbaje Yamil M. , Carbone Paul P.
Published: February 2007

The authors briefly review the age distribution of HCC and the treatments used. We concluded that therapy should not be arbitrarily withheld based solely on chronological age. Older cancer patients deserve the right to be treated if they so wish. © 1994 Wiley‐Liss, Inc.
Factors that influence the further survival of patients who survive for five years after the diagnosis of cancer in childhood or adolescence
Authors: Green Daniel M. , Zevon Michael A. , Reese Peter A. , Lowrie Geoffrey S. , Michalek Arthur M.
Published: February 2007

To evaluate the further survival, and to identify disease and treatment factors which influence the further survival, of five‐year survivors of cancer diagnosed during childhood or adolescence, we reviewed the courses of 591 previously untreated patients who were less than 20 years of age at diagnosis and survived for five years after diagnosis. Fifty‐three of 143 patients who experienced disease recurrence during the first five years after diagnosis died during the period of observation, compared to 18 of 448 patients who did not experience disease recurrence during the first five years after diagnosis. The sex‐specific standardized mortality ratios for the group of patients who never relapsed or relapsed more than five years after diagnosis were not significantly different from those of the New York State population. Cox proportional hazards modelling of the subgroup of patients who relapsed during the first five years after diagnosis demonstrated that disease which was treated surgically, a diagnosis of Hodgkin's disease or acute lymphoblastic leukemia, and older age at diagnosis were significantly associated with further survival in this group, whereas similar modelling of the patients who did not experience disease recurrence during the first five years after diagnosis failed to identify any variables which were associated with continued survival. The results of this study suggest that childhood and adolescent cancer patients who survive for five years without disease recurrence have a survival rate similar to that of the general population. Continued follow‐up of this cohort is required to determine if the present findings can still be demonstrated as the majority of the cohort ages beyond 35 years of age. © 1994 Wiley‐Liss, Inc.
Intraabdominal desmoplastic small‐cell tumor with divergent differentiation: Clinicopathological findings and DNA ploidy
Authors: Schmidt Dietmar , Köster Eckhard , Harms Dieter
Published: February 2007

Five cases of intraabdominal small‐cell tumor with divergent differentiation are reported. All patients were of male sex. They were 10, 15, 20, 21, and 30 years of age at time of diagnosis, respectively. By light microscopy, the tumors consisted of small cells arranged in groups, nests, and clusters separated by a collagen‐rich desmoplastic stroma. Immunohistochemical studies revealed the coexpression of mesenchymal, epithelial, and neural markers. Notably, all tumors coexpressed vimentin, cytokeratin, and desmin, the latter in a remarkable paranuclear dot‐like fashion. In contrast to other authors, we did not find chromogranin. DNA image cytometry on four cases demonstrated two diploid and two aneuploid (hyperdiploid) cases. No correlation was found between ploidy and prognosis. One patient died from disease, another died from venoocclusive disease after bone marrow transplantation, and the remaining patients are alive, but have progressive intraabdominal disease. Thus, our findings support the poor prognosis in this type of tumor. © 1994 Wiley‐Liss, Inc.
Evaluation of six tumor markers in patients with carcinoma of unknown primary
Authors: Pavlidis Nicholas , Kalef‐Ezra John , Briassoulis Evangelos , Skarlos Dimosthenis , Kosmidis Paris , Saferiadis Kostas , Bairaktari Eleni , Bafaloukos Dimitrios , Maravegias Athanassios , Theoharis Dimitrios , Fountzilas George
Published: February 2007

We have retrospectively evaluated six serum tumor markers in 85 patients with carcinoma of unknown primary. The serum levels of carcinoembryonic antigen (CEA), CA 19‐9, CA 15‐3, CA 125, β‐chorionic gonadotropin (β‐HCG) and α‐fetoprotein (AFP) were related with the histological pattern (undifferentiated carcinoma or adenocarcinoma), the number and the site of metastases, as well as the response to chemotherapy and the patients' survival. More than 40% of the patients had increased serum levels of all six tumor markers, except of AFP which was found to be increased in only 17% of them. Increased levels of CA 19‐9 were related to metastatic adenocarcinoma, whereas CA 19‐9 and CA 15‐3 had a relationship with more advanced disease. Patients with liver involvement had higher mean levels of CEA and CA 19‐9 as compared to those with nodal disease. None of these markers was found to have a predictive value for response to chemotherapy or survival.

Although the present study has a retrospective nature, it allows us to conclude that patients with CUP have a nonspecific over‐expression of the above serum tumor markers and that routine use of these markers does not offer any diagnostic or prognostic assistance. © 1994 wiley‐Liss, lnc.
Toxocariasis simulating hepatic recurrence in a patient with Wilms' tumor
Authors: Almeida Maria T. , Ribeiro Raul C. , Kauffman William M. , Maluf Paulo T. , Brito José Luiz B. C. , Cristofani Lilian M. , Jacob Cristina A. , Odone‐Filho Vicente
Published: February 2007

We report the case of a 3‐year‐old girl with stage I Wilms' tumor of favorable histology. During the course of chemotherapy 5 months post‐diagnosis, an abdominal ultra‐sonogram revealed hypoechoic areas consistent with hepatic tumor recurrence. A liver biopsy performed to rule out recurrence of the malignancy was suggestive of toxocariasis and the diagnosis was confirmed by serologic testing. Although the patient had few classic signs of visceral larva migrans, her eosinophilia and family social history should have suggested this possibility. This case demonstrates that hepatic toxocariasis should be considered in evaluating hepatic hypoechoic lesions in a child, even when features typical of the disease are absent. © 1994 Wiley‐Liss, Inc.
Radiotherapy in the SIOP (international society of pediatric oncology) nephroblastoma studies: A review
Authors: Jereb Berta , Burgers J. Marion V. , Tournade Marie‐France , Lemerle Jean , Bey Pierre , Delemarre Jan , Habrand Jean‐Louis , Voúte P. A.
Published: February 2007

For decades radiation has generally been accepted as a valuable supplement to surgery in the treatment of Wilms' tumor; unfortunately, it may produce undesirable late effects. It turned out, however, that when treatment is adjusted to known variables, the risk for late sequelae can be diminished in some groups of children.

SIOP clinical trials have been based on children with unilateral tumors of standard histology and free of metastasis at diagnosis. The first two clinical trials, SIOP‐1 (started in 1971) and SIOP‐2 (started in 1974), established the beneficial effect (such as less ruptures, lower stage) of preoperative radiation and actinomycin D (AMD) in SIOP‐2, with all children having radiation therapy either pre‐operatively, postoperatively, or both. In the SIOP‐5 trial (started in 1977), preoperative radiation therapy and AMD were compared with preoperative chemotherapy resulting in only 50% of children having radiation. The result permitted disuse of preoperative radiation in the SIOP‐6 trial (started in 1980), where only one‐third of the patients received postoperative radiation therapy. At present, in the SIOP‐9 trial (started in 1987), fewer than 20% of children are having radiotherapy. The survival rates meanwhile have been increasing steadily from 64% in SIOP‐1 to 84% in SIOP‐6 for stages I, II, and III combined. © 1994 Wiley‐Liss, Inc.
Combination trial of subcutaneous recombinant α2b interferon and oral cyclophosphamide in follicular low‐grade non‐Hodgkin's lymphoma
Authors: Ozer Howard , Anderson James R. , Peterson Bruce A. , Budman Daniel R. , Cooper M. Robert , Kennedy B. J. , Silver Richard T. , Henderson Edward S. , Duggan David B. , Barcos Maurine , Bloomfield Clara D. , Gottlieb Arlan J.
Published: February 2007

The follicular non‐Hodgkin's lymphomas (NHL) have been among those tumors demonstrated to show frequent responses to alpha interferon in phase I and II clinical trials. In addition, there are data suggesting that alpha interferon demonstrates synergistic antitumor activity with alkylating agents in animal models for a number of tumors. Based on these data, Cancer and Leukemia Group B (CALGB) undertook a phase II pilot study of the combination of interferon rlFNα2b (2 × 106 IU/m2 s.c. tiw) and cyclophosphamide (100 mg/m2 per day orally) with the ultimate purpose of examining this combination as long‐term therapy of follicular lymphoma in comparison to oral cyclophosphamide alone. One hundred five advanced stage III or IV eligible patients with pathologically diagnosed International Working Formulation B or C histology were entered on CALGB 8553 to determine toxicity and response rates to the combination. Both previously chemotherapy‐treated patients (32) and patients without prior chemotherapy (73) were entered on study. For patients without prior chemotherapy the overall response rate to the combination regimen was 86% with 58% of chemotherapy‐treated patients achieving complete response. Chemotherapy‐treated patients had a total response rate of 62% with only 25% complete responders. Complete responses in patients without prior chemotherapy were positively correlated with absence of B symptoms, and good performance status and negatively correlated with the histological subtype of follicular mixed small‐cleaved and large cell histology (IWF C); only performance status was significantly correlated with response in patients who had previously had chemotherapy. Survival at 5 years is estimated to be 63% for those without chemotherapy and 39% for those previously treated with chemotherapy patients. The maximum toxicities experienced during therapy with the combination regimen of cyclophosphamide and interferon alpha were primarily related to myelosuppression. Sixty‐seven percent of patients without prior chemotherapy and 65% of patients receiving prior chemotherapy experienced severe leukopenia while severe thrombocytopenia and anemia occurred in 6‐31% of these patients. Non‐myelosuppressive toxicities were less frequently seen. These response rates are similar to those achieved in a previous CALGB trial with oral cyclophosphamide as a single agent, although severe myelotoxicity was increased to approximately 60% of patients from less than 10% with the single‐agent therapy. The combination of alpha interferon and cyclophosphamide administered in this fashion is safe when peripheral counts are carefully monitored. Randomized studies of this regimen in comparison to oral cyclophosphamide are currently in progress. © 1994 Wiley‐Liss, Inc.
Catecholamine metabolites in ganglioneuroma
Authors: Lucas Kenneth , Gula Mary J. , Knisely A. S. , Virgi Mohamed A. , Wollman Michael , Blatt Julie
Published: February 2007

With rare exception, ganglioneuroma (GN) is a benign lesion which presents as a localized mass without metastatic potential and which is chemotherapy resistant. Thus, its distinction from neuroblastoma (NB) may be important. The diagnosis of GN implies the absence of neuroblastic elements. Incomplete resection prevents complete microscopic examination and raises the possibility that focal NB was not sampled. In an attempt to determine what features other than histology distinguish these two entities, we reviewed the charts of 25 patients with GN with regard to patient age and sex, tumor location and size, and urine catecholamine metabolite levels. One patient with GN (5%) and gross total resection had elevated quantitative vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels (2.4 × upper limit of normal for age), and two others had positive spot analyses for VMA. An additional patient with a large mass, multiple biopsies of which documented GN, also had greatly elevated (approximately 5 × normal) VMA and HVA levels. However, a subsequent attempt at resection disclosed several gross foci of NB. Even excluding this patient, there was a trend for elevated values in GN patients to correlate with tumor size (P = .07 and .14 for VMA and HVA, respectively). The incidence of elevated values appears to increase as a function of tumor size, and small tumors are not likely to result in positive urinary measurements. We conclude that while elevations of VMA and HVA are consistent with a well‐documented diagnosis of GN, extreme elevations (>3 × nl) should prompt careful serial evaluation for occult NB.© 1994 Wiley‐Liss, Inc.
Phase II study of carboplatin as a single drug for relapsed Wilms' tumor: Experience of the Brazilian Wilms' tumor study group
Authors: De Camargo Beatriz , Melaragno Renato , Silva Nagsla Saba E , Mendoncla Nubia , Alvares Maria Nunes , Morinaka Elizabeth , Marques Armando , Cusato Maria P.
Published: February 2007

A total of 15 patients with relapsed Wilms' tumor were treated with carboplatin as a single agent. There were six females and nine males, aged between 2 and 9 years (me‐dian:5). The treatment consisted of carboplatin given I.V. as a single agent at a dose of 550 mg/m2 over 1 hour every 3 weeks. There were four (26%) complete responses, four (26%) partial responses, one stable disease, and six with progressive disease. In all patients a total of 56 courses were given and the toxicities found were leukopenia and thrombocytopenia. The overall response rate was encouraging and the toxicity was acceptable and reversible. © 1994 Wiley‐Liss, Inc.
Esthesioneuroblastoma presenting as an orbital mass in a young child
Authors: Bobele Gary B. , Sexauer Charles , Barnes Patrick A. , Krous Henry F. , Bodensteiner John B.
Published: February 2007

A 3‐year‐old boy presented with uniocular proptosis and ophthalmoplegia. Investigation revealed a mass involving the right orbit with extension into the left orbit and paranasal sinuses, and intracranial extension involving both frontal lobes. Biopsy of an enlarged cervical node and the intranasal mass revealed esthesioneuroblastoma. This tumor has been reported rarely in a child this age, and only 12 case reports document patients under 10 years of age. The presentation as an orbital mass is previously unreported and must now be considered in the differential diagnosis of proptosis in childhood. The usual clinical, radiological, and pathological features of olfactory esthesioneuroblastoma are reviewed. © 1994 Wiley‐Liss, Inc.
Primary testicular and paratesticular tumors of childhood
Authors: Coppes Max J. , Rackley Raymond , Kay Robert
Published: February 2007

Testicular and paratesticular neoplasms are uncommon tumors of childhood. Consequently, the experience gained with regard to their optimal management is limited in any given children's cancer centre. Here we review the classification, diagnosis, and staging of testicular and paratesticular neoplasms and subsequently discuss the more frequently occurring ones: germ cell tumors, gonadal stromal tumors, gonadoblastoma, tumors of the supporting tissue, lymphomas and leukemias, tumor‐like lesions, secondary tumors, and tumors of the adnexa. © 1994 Wiley‐Liss, Inc.
Hyperthermic isolated limb perfusion with Cis‐diamminedichloro‐platinum. II. An experimental study in dogs with a balloon‐occlusion technique for repeated high‐dose treatment
Authors: Wessalowski R. , Wilhelm M. , Torsello S. , Sager M. , Güttler J. , Jürgens H. , Göbel U.
Published: February 2007

Isolated organ perfusion is attractive for regional high‐dose chemotherapy because of its advantage to reduce whole body toxicity. Intraoperative hyperthermic isolated perfusion procedures involving a heart‐lung machine have been developed, but repeated treatments carry a high risk of vessel and tissue damage. Therefore, a study of isolated hyperthermic limb perfusion in four dogs was conducted using a balloon‐occlusion technique including a hyperthermia unit, two low‐flow rotary pumps, a bubble oxygenator, and two polyurethane balloon catheters. After 15 min infusion of cisplatinum the concentrations of serum platinum (Pt) in the isolated limb and in the whole body were measured by atomic absorption Spectroscopy. Regional exposure to Pt was more than 10‐fold higher than systemic exposure. After 60 min isolated limb perfusion, the area under the curve (AUC) of Pt concentrations in the isolated limb showed values between 767.4 and 1055.6 μg/l × 60 min, whereas in the whole body values between 59.8 and 75.9 μg/l × 60 min were obtained. Repeated isolated limb perfusions with the balloon‐occlusion technique were performed in three dogs without systemic side effects. This model of regional chemotherapy may be useful for preoperative chemotherapy in malignant tumors of the limbs. © 1994 Wiley‐Liss, Inc.
Kaposi's sarcoma of the bone marrow presenting with fever of unknown origin
Authors: Levin M. , Hertzberg L.
Published: February 2007

Kaposi's sarcoma (KS) has become more common in the United States with the spread of the Acquired Imunodeficiency Syndrome (AIDS) epidemic. The epidemic form associated with AIDS involves primarily skin and visceral organs. Bone marrow involvement is rare. We present a case of Kaposi's sarcoma that was diagnosed upon bone marrow biopsy, without skin or visceral involvement, that presented with fever of unknown origin which responded to indomethacin and anti‐KS chemotherapy. Kaposi's sarcoma of the bone marrow should be considered in the differential of febrile illness of unknown origin in patients with AIDS. © 1994 Wiley‐Liss, Inc.
Successful treatment of acute lymphoblastic leukemia in a child with cystic fibrosis
Authors: Gururangan Sridharan , Horner Michelle , Rodman John H. , Marina Neyssa M.
Published: February 2007

A 3 1/2 year old girl with cystic fibrosis who underwent successful treatment for acute lymphoblastic leukemia remains in complete remission 36 months after diagnosis. We also report high clearance rates of three antineoplastic agents in this patient. Drug doses were adjusted to achieve optimal systemic exposure. © 1994 Wiley‐Liss, Inc.
Childhood malignancies in the south‐west region of England, 1976–1985
Authors: Foreman Nicholas K. , Thorne Robert , Berry P. Jeremy , Oakhill Anthony , Mott Martin G.
Published: February 2007

The incidence and pattern of childhood cancer in the South‐West Region of the United Kingdom was studied in the period 1976–1985 to see if there was any unevenness in distribution. This region consists of five counties with considerable variation in radon exposure and urbanisation. Cases were identified from multiple sources and the basis of the diagnosis reviewed. Internationally accepted diagnostic categories were used. Six hundred seventy‐eight cases were found, giving an age standardised incidence rate of 114.2 per million childhood years. The incidence of different cancers by sex was strikingly similar to the Manchester Children's Tumour Registry, from a noncontiguous region, with three exceptions. In the South‐West, there was a higher rate of soft tissue sarcoma in boys (P = 0.01) and lower rates of sympathetic nervous system and germ‐cell tumours in girls (P = 0.01 and P = 0.04, respectively). There was no significant variation in cancer incidence among the five counties or with time. The incidence rate in counties of high radon exposure was not higher than with low radon exposure. The overall incidence in counties with urban districts was not significantly increased compared to those without, although there was a nonsignificant trend towards a higher incidence of leukaemia (P = 0.08). The incidence and type of childhood cancer are similar to those in a noncontiguous region. Geographical and temporal incidence are remarkably stable. Local environmental variation may not play a major role in the causality of childhood cancer. © 1994 Wiley‐Liss, Inc.