RESUMEN

Hepatic veno-occlusive disease (VOD) is a frequent and severe complication of hematopoietic stem cell transplantation (HSCT) affecting 9.6-17.3 % of cases. 200 HSCT, performed between January 1995 and March 2013 in our Paediatric HSCT Centre in Trieste, were retrospectively analysed to evaluate the frequency of VOD and to identify the associated risk factors. The frequency of VOD according to the Seattle criteria was 17 %, within the range reported in literature. The mortality rate was 37.5 % (75 out of 200 transplantations) in the general population and 73.5 % (25 out of 34) in VOD patients (p < 0.05). Veno-occlusive disease significantly decreased from 38 % (1995-2000) to 8 % (2007-2013) p < 0.05. Univariate and multivariate analyses identified sepsis and pre-transplant ferritin levels above 1000 ng/ml as two significant risk factors for VOD, while the use of tacrolimus appeared to be associated with a lower VOD risk. Veno-occlusive disease still remains an important cause of transplant-related mortality even if it appears to have decreased over the last few years.

Asunto(s)

Trasplante de Células Madre Hematopoyéticas/efectos adversos , Enfermedad Veno-Oclusiva Hepática/etiología , Adolescente , Niño , Preescolar , Femenino , Enfermedad Veno-Oclusiva Hepática/diagnóstico , Enfermedad Veno-Oclusiva Hepática/tratamiento farmacológico , Enfermedad Veno-Oclusiva Hepática/epidemiología , Humanos , Lactante , Recién Nacido , Masculino , Mortalidad , Estudios Retrospectivos , Factores de Riesgo , Trasplante Homólogo , Adulto Joven

RESUMEN

Survivors of retinoblastoma (Rb) are at high risk of dying from second malignant tumour. The occurrence of second malignant neoplasm (SMN) and related mortality in a cohort of 1111 cases from the Italian Retinoblastoma Registry was analysed, considering the possible role of both genetic and iatrogenic causes. Rb patients had a greater than 10-fold excess in overall mortality compared with the general population (standardized mortality ratio (SMR) 10.73, 95% CI 9.00-12.80). Their excess risk attributable to cancers other than Rb was 14.93 95% CI 10.38-21.49). Survivors of hereditary Rb had an SMR for all causes of 16.25 (95% CI 13.20-20.00), whereas their SMR for all cancers was 25.72 (95% CI 17.38-38.07). Survivors of unilateral sporadic Rb had an SMR of 4.12 from all cancers (95% CI 1.55-10.98) and a much higher excess for overall mortality (SMR 13.34, 95% CI 10.74-16.56). As expected, survivors of hereditary Rb had higher mortality from cancers of the bone (SMR 391.90, 95% CI 203.90-753.20) and soft tissue (SMR 453.00, 95% CI 203.50-1008.40), small intestine (SMR 1375.50, 95% CI 344.00-5499.70), nasal cavity (SMR 13.71, 95% CI 1.93-97.35) and cancers of the brain and central nervous system (SMR 41.14, 95% CI 13.2-127.55).

Asunto(s)

Neoplasias Primarias Secundarias/mortalidad , Neoplasias de la Retina/patología , Retinoblastoma/patología , Estudios de Cohortes , Lateralidad Funcional , Mutación de Línea Germinal , Humanos , Italia , Sistema de Registros , Neoplasias de la Retina/genética , Retinoblastoma/genética , Análisis de Supervivencia , Sobrevivientes

RESUMEN

Hematopoietic stem cell transplantation (HSCT) represents today an important therapeutic choice for several disorders of childhood: hematologic, metabolic and neoplastic pathologies can be treated with this strategy. The aim of this paper is to resume the latest history of HSCT, paying attention to the main changes and controversies, and its efficacy as far as concerns the major indications in pediatric oncohematology.

Asunto(s)

Enfermedades Hematológicas/terapia , Trasplante de Células Madre Hematopoyéticas , Leucemia/terapia , Niño , Humanos

Asunto(s)

Leucemia-Linfoma Linfoblástico de Células Precursoras/prevención & control , Adolescente , Niño , Preescolar , Protocolos Clínicos , Trasplante de Células Madre Hematopoyéticas , Humanos , Lactante , Cooperación Internacional , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Recurrencia , Sistema de Registros

RESUMEN

BACKGROUND/PURPOSE: The growing use of routine ultrasonography during pregnancy is leading to an increasing number of prenatally diagnosed neuroblastomas. Optimal strategy has not yet been defined for these patients, because knowledge on this particular neuroblastoma (NB) population is still limited. However, definite guidelines are needed to avoid inadequate treatment. The authors analyzed the cases of antenatally detected NB (ADNB) reported in the Italian Neuroblastoma Registry during the past 6 years to elucidate the features of this subset of NB. METHODS: The Italian Neuroblastoma Registry was reviewed for the period January 1993 to December 1998 to collect clinical, radiographic, surgical, and histopathological data on ADNB cases. NB stage was evaluated according to INSS criteria. All patients had undergone imaging (computed tomography or magnetic resonance imaging) of the primary tumor and bone marrow biopsy before surgical resection. RESULTS: Seventeen patients were identified. Primary tumour site was adrenal glands in 16 cases and retroperitoneal ganglia in 1. Stage distribution was stage I, 13 cases; stage II-A, 1 case; stage II-B, 1 case; stage IV-S, 2 cases. All cases underwent primary tumour resection. Mean age at surgery was 4 weeks. Resection of primary tumor was radical in 16 cases, partial in 1. All tumors were characterised by favourable histology according to Shimada classification. N-myc gene amplification was studied in 14 patients. N-myc amplification was detected only in a newborn with stage II-A NB, who died of massive bleeding 2 days after tumor resection. DNA index and 1p deletion were studied in 11 and 8 patients, respectively. Both diploidy and deletion of 1p were observed in a newborn who subsequently died of disease progression despite surgery, chemotherapy, and radiation therapy. Fourteen of 17 patients currently are alive and free of disease, and one with IV-S NB and short follow-up is alive with disease. CONCLUSIONS: Our data give evidence that in most cases infants with ADNB represent a subset of patients with excellent outcome. Aggressive treatment may not always be necessary. Infants with ADNB with unfavorable features should undergo early surgical excision, whereas patients with favourable features could be observed awaiting spontaneous regression of the mass, reserving delayed surgery for tumors that increase in size or do not regress.

Asunto(s)

Neoplasias de las Glándulas Suprarrenales/diagnóstico , Neuroblastoma/diagnóstico , Diagnóstico Prenatal , Neoplasias de las Glándulas Suprarrenales/congénito , Neoplasias de las Glándulas Suprarrenales/genética , Neoplasias de las Glándulas Suprarrenales/cirugía , Femenino , Estudios de Seguimiento , Eliminación de Gen , Genes myc , Humanos , Lactante , Recién Nacido , Neuroblastoma/congénito , Neuroblastoma/genética , Neuroblastoma/cirugía , Ploidias , Embarazo , Neoplasias Retroperitoneales/congénito , Neoplasias Retroperitoneales/diagnóstico , Neoplasias Retroperitoneales/genética , Neoplasias Retroperitoneales/cirugía , Estudios Retrospectivos , Ultrasonografía Prenatal

RESUMEN

Bone marrow transplantation (BMT) represents an important therapeutic choice for several kinds of disorders: hematologic, metabolic and neoplastic pathologies can be treated with this strategy. The aim of this article is to describe the main indications for allogeneic BMT in haematologic disorders of childhood and possible problems related to this procedure. We consider only hematologic aspects, paying particular attention to unusual disorders of infancy as myelodysplastic syndromes and aplastic anemia. We also consider quality of life after a BMT in patients with sickle cell anemia and thalassemia major and compare this with quality of life of patients receiving chronic periodic blood transfusions.

Asunto(s)

Trasplante de Médula Ósea , Enfermedades Hematológicas/terapia , Trasplante de Médula Ósea/tendencias , Niño , Preescolar , Humanos

RESUMEN

BACKGROUND AND OBJECTIVES: Treatment of recurrent childhood acute lymphoblastic leukemia (ALL) has been controversial in the last decade. Conventional intensive chemotherapy (CHEMO) can cure up to 30% of children who have relapsed after ALL: similar results have been obtained with autologous bone marrow transplantation (ABMT), but allogeneic bone marrow transplantation (AlloBMT) seems to be the best therapeutic option. In this review the authors point out the contribution of current strategy in the setting of children with ALL who experience a first relapse and should be offered optimal treatment in order to obtain the best disease-free survival (DFS). The principal objective of this issue is to reach a possible consensus on the more controversial points regarding factors considered strong predictors of the outcome of the relapsed patients, second-line chemotherapy, optimal timing and type of transplantation. EVIDENCE AND INFORMATION SOURCE: Data published in the literature over the last decade concerning early and late relapse in childhood ALL suggest that improvements in cure rates may be achieved by intensification of the relapse treatment both with chemotherapy and with different types of transplantation. An accurate search for Medline data has been performed in order to understand the risk-benefit ratio of aggressive therapy adopted in this setting. STATE OF ART: Modern first-line treatment protocols for childhood ALL have contributed to curing an ever larger number of patients but this strategy could be responsible for creating a more resistant leukemic clone at the time of systemic or extramedullary relapse. This hypothesis emerges from a number of single or multicenter experiences; thus clinical and biological features in relapsed patients are being studied carefully in order to understand which risk-directed second-line therapy should be best adopted. The BFM group classified ALL relapses as "very early", "early", or "late" according to the time from diagnosis to first relapse (i.e. < 18, > 18 and < 30 or more than 30 months) and has shown that about 2/3 of the small fraction of children with late extramedullary relapses or late non T-marrow relapses or early combined non T-relapses can be rescued by chemotherapy; in contrast ALL early relapses or T-immunophenotype ALL relapses can be rescue only by AlloBMT. Since 1990 the AIEOP group adopted BFM-like first-line treatment and experienced similar situations for relapsed patients so that, even in absence of a real common relapse protocol, they went in the same direction as the BFM group as far as hematopoietic stem cell transplantation (HSCT) procedures and decision were concerned. A recent AIEOP study on the destiny of 192 consecutive patients with ALL in 2nd complete remission and not having an HLA suitable sibling donor is presented in this issue. The value of different HSCT procedures is addressed and the protection against a new relapse seems to be real, although, of course, the risk-benefit ratio should always be evaluated. PERSPECTIVES: Very few prospective studies on the treatment of childhood ALL relapse have been set up in the last decade because of many difficulties regarding common second-line therapies, some reluctance versus HSCT in consideration of the transplant-related mortality and the so-called late effects. Additional modifications of allogeneic family and unrelated donor HSCT strategies and the promising results both of cord HSCT and auto-grafting methods including in vitro purging or post-transplant immunotherapy, are making transplantation procedures for ALL relapsed patients more appropriate and increasing confidence in their use. The possibility of performing common prospective international studies should be encouraged over the next years in order to elucidate an area of great research as is that of the treatment of childhood ALL relapse.

Asunto(s)

Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Trasplante de Médula Ósea , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Niño , Preescolar , Terapia Combinada , Humanos , Leucemia-Linfoma Linfoblástico de Células Precursoras/patología , Recurrencia , Trasplante Autólogo , Trasplante Homólogo , Resultado del Tratamiento

RESUMEN

BACKGROUND AND OBJECTIVE: Since 1988 the AIEOP has used BFM-based chemotherapy for childhood ALL. Current organization and results and role of cranial irradiation in the AIEOP-ALL 91 study are reported. DESIGN AND METHODS: From 1991 to 1995, 1194 children (< 15 years) with non-B ALL, were enrolled and assigned to the standard risk [SR: age > 1 year, non-T-ALL, BFM risk factor (RF) < 0.8], intermediate risk (IR: RF > or = 0.8 but < 1.7, or with RF < 0.8 and age < 1 year, or T-ALL), or high risk [HR: RF > or = 1.7, or t(9;22), or t(4;11) or prednisone poor response or late response or CNS involvement] groups. All patients received initially protocol Ia. Thereafter SR patients received HD-MTX 2 g/m2, a modified protocol II, and continuation therapy with triple intrathecal chemotherapy (TIT); IR patients received protocol Ib, HD-MTX 5 g/m2, protocol II and continuation therapy with TIT; HR patients received 9 polychemotherapy blocks, cranial irradiation and continuation therapy. Duration of treatment was 24 months. A randomized study was conducted to evaluate the impact of high-dose asparaginase in non high risk patients: the results of this study cannot be disclosed yet. RESULTS: One thousand one hundred and fifty-two (96.5%) patients achieved CR. Overall EFS (SE) at 5-years was 71.0% (1.4), with a survival of 80.3% (1.3). Relapse occurred in 262 children (21.9%), either in the marrow (n = 192 isolated and 32 with other sites, 18.7%), in the CNS (n = 18, 1.5%), or elsewhere (n = 20, 1.7%). 5-year EFS (SE) was 83.3% (2.4) in SR, 74.7% (1.8) in IR, and 39.7% (3.5) in HR groups, respectively. INTERPRETATION AND CONCLUSIONS: Overall cure rate was higher than in the previous AIEOP-ALL 88 study. Treatment intensification with polychemotherapy blocks did not improve results in HR. Cranial irradiation can be safely omitted in over 80% of children treated with BFM based chemotherapy.

Asunto(s)

Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Adolescente , Asparaginasa/administración & dosificación , Trasplante de Médula Ósea , Niño , Preescolar , Aberraciones Cromosómicas , Terapia Combinada , Irradiación Craneana , Ciclofosfamida/administración & dosificación , Citarabina/administración & dosificación , Daunorrubicina/administración & dosificación , Dexametasona/administración & dosificación , Supervivencia sin Enfermedad , Doxorrubicina/administración & dosificación , Femenino , Humanos , Lactante , Italia/epidemiología , Leucovorina/administración & dosificación , Masculino , Mercaptopurina/administración & dosificación , Metotrexato/administración & dosificación , Leucemia-Linfoma Linfoblástico de Células Precursoras/clasificación , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidad , Leucemia-Linfoma Linfoblástico de Células Precursoras/radioterapia , Prednisona/administración & dosificación , Pronóstico , Inducción de Remisión , Riesgo , Tioguanina/administración & dosificación , Resultado del Tratamiento , Vincristina/administración & dosificación

RESUMEN

We conducted a population-based cohort study in the province of Trieste, Italy, to assess whether the first-degree relatives of children with malignancies had an increased risk of cancer compared with the general population. We examined cancers occurring in all first-degree relatives of children who experienced malignancies under the age of 15 years between 1971 and 1993 (probands). A cohort of the 394 relatives of the 125 probands contributed 7,939 person-years of observation. Among the relatives as a whole, we found a statistically significant increased risk of developing all malignancies except non-melanoma skin carcinoma (21 observed relatives with cancer and 12.46 expected, for a standardized incidence ratio [SIR] of 1.69), of developing breast cancer (SIR = 3.09) and of developing haemolymphatic system neoplasms (SIR = 4.03). This was mainly due to the excess cancer risk in the relatives of probands with intracranial tumours, who showed a significant 3.1-fold risk for developing all cancers but non-melanoma skin tumours. Our findings and the previously reported steep rise in the incidence of childhood brain tumours in our area may imply that not only genetic factors but also shared environmental agents might be involved in the observed aggregation of cancer in the families of probands with intracranial tumours.

Asunto(s)

Leucemia/epidemiología , Leucemia/genética , Neoplasias/epidemiología , Neoplasias/genética , Adolescente , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Lactante , Recién Nacido , Italia/epidemiología , Leucemia/etiología , Masculino , Neoplasias/etiología , Neurofibromatosis 1/etiología , Neurofibromatosis 1/genética , Núcleo Familiar , Linaje , Factores de Riesgo , Xerodermia Pigmentosa/genética

Asunto(s)

Disqueratosis Congénita/diagnóstico , Humanos , Masculino , Enfermedades de la Uña/patología , Pterigion/patología

RESUMEN

The study objectives were: 1) to analyse the incidence and death rates from cancer among children aged 0-14 years resident in the north-eastern Italian province of Trieste between 1972-1993, using data from the population-based Trieste Cancer Registry; 2) to evaluate the local diagnostic facilities by analysing the accuracy of histological diagnoses, the causes of delay in the diagnosis, and the interval between onset of symptoms and diagnosis of cancer; 3) to calculate the proportion of patients treated following the most effective therapy protocols known at the time of the tumour detection, and to compute the actuarial five-year survival rates since diagnosis. We recorded 123 new cases of cancer (93% microscopically verified) corresponding to a rate, age-standardized to the world population, of 161.9 (standard error [SE] = 15.1) per million child-years. The most common diagnostic group was that of primary brain tumours: 40 cases, rate = 51.0 (SE = 8.4). In 102 cases the diagnosis was made at hospitals in the province of Trieste, with a median time of seven days (25th-75th percentile = 1-16) between admission and diagnosis. In 37 cases the length of the interval between the advancing of the diagnostic hypothesis of cancer and the microscopic diagnosis ranged from eight to 57 days: 20% of the interval was spent in the identification of the lesion, 50% elapsed between the identification and the biopsy, and 30% was spent in performing the microscopic diagnosis. Out of 123 cases, 30 were partly treated or completely treated at centres not located in our province, i.e., at seven different Italian hospitals (14 cases), nine European hospitals (15 cases), and at one North-American centre (one case). The 40 children with brain tumours were spread among 12 institutions. The five-year survival rate increased from 52.4% (SE = 6.3) for the 63 children with cancers diagnosed in 1972-1981 to 62.5% (SE = 7.0) for the 48 with malignancies detected in 1982-1990.

Asunto(s)

Neoplasias/epidemiología , Calidad de la Atención de Salud/estadística & datos numéricos , Adolescente , Niño , Preescolar , Femenino , Humanos , Incidencia , Lactante , Recién Nacido , Italia/epidemiología , Masculino , Mortalidad/tendencias , Neoplasias/diagnóstico , Neoplasias/terapia , Sistema de Registros/estadística & datos numéricos

RESUMEN

Wilms tumor, although rare, is the most frequent malignant renal tumor in children. With approximately 70 new cases diagnosed annually in Italy, it is important to collect these rare cases and to treat them in a uniform way. At the dawn of this century virtually all children with Wilms tumors died: today 95% of them survive; the treatment is carefully tailored to well defined risk factors (histology-stage-molecular markers) to minimize short and long-term toxicities. The object of this review is to describe the pathway to the progress. The most important step was to recognize that a single institution could not collect enough patients to answer complex questions, whereas this was possible for super-groups like the National Wilms Tumor Study Group (NWTSG) in the USA, the multinational SIOP study group in Europe, the United Kingdom group, the Italian group (CNR-AIEOP) and more recently the Brazilian one. Wilms tumor is the paradigm for the multimodal treatment of a pediatric malignant solid tumor, the development in surgical technique, the recognition of the sensitivity of Wilms tumor to irradiation and the availability of several chemotherapeutic agents led to a dramatic change in the prognosis for most patients. Much progress has been made in the study of histopatology of childhood renal tumors: the patients must be stratified into two groups, the favourable and the unfavourable histology, further subdivided into anaplasic tumors (focal or diffuse), clear cell tumors and rhabdoid tumors. The past few years have provided a breakthrough in understanding some of the genetic factors involved in Wilms' tumor: moreover, possible chromosomal prognostic factors have been identified: loss of heterozygosity of 16q markers and 1p markers. Today the results of the treatment of Wilms tumors are very good. In the NWTS-3 the four year relapse-free survival rate in stage 1 with favourable and with anaplastic histology was 92%, in stage 2 with favourable histology 88%, in stage 3 with favourable histology 79%, in stage 4 and in stage 2, 3 and 4 with unfavourable histology 71%. The Italian group has obtained less impressive results in the ¿80, but similar results in the first stage with the ¿92 protocol. There is a debate about the immediate nephrectomy preferred by NWTS and the preoperative strategies adopted by SIOP group. Successful treatment may be associated with many late effect: in patients cured of Wilms tumor the risk of congestive heart failure has been less than 1.7%, the risk of a second tumor less than 1%. The must important late effect remains the relapse of the disease: the risk is about 14-20%.

Asunto(s)

Neoplasias Renales/terapia , Tumor de Wilms/terapia , Niño , Predicción , Humanos , Neoplasias Renales/genética , Neoplasias Renales/patología , Pronóstico , Tumor de Wilms/genética , Tumor de Wilms/patología

Asunto(s)

Colágeno/sangre , Cirrosis Hepática/tratamiento farmacológico , Metotrexato/efectos adversos , Procolágeno-Prolina Dioxigenasa/sangre , Biomarcadores/sangre , Humanos , Sensibilidad y Especificidad

RESUMEN

PURPOSE: To define factors that influence outcome in children with localized but unresectable neuroblastoma by retrospective investigation of response to therapy and outcome in 21 Italian institutions. PATIENTS AND METHODS: Of 145 assessable children diagnosed between 1979 and 1990, 77 were treated between 1979 and 1984 with three consecutive standard-dose (SD) protocols, and 68 between 1985 and 1990 with a high-dose (HD) protocol. All protocols included chemotherapy, followed by resection of primary tumor if feasible. If at least partial resection was achieved, consolidation therapy followed, except that from 1985 onward, patients considered disease-free following surgery received no further treatment. RESULTS: Ninety-four of 145 patients (65%) achieved a complete response (CR) or partial response (PR) with chemotherapy and 75 (52%) subsequently underwent complete resection of the primary tumor. Eighty-one patients are alive (73 without disease, eight with disease), 63 have died, and one is lost to follow-up. The 5-year overall survival (OS) rate is 55% and progression-free survival (PFS) rate 50%. Both OS and PFS correlated with response to chemotherapy, removal of primary tumor, HD therapy, and serum lactate dehydrogenase (LDH) levels. Infants (< 1 year), independent of primary tumor site, and children aged 1 to 15 years with a nonabdominal primary tumor, did better compared with children aged 1 to 15 years with an abdominal primary tumor (PFS, 72% and 64% v 30%; P < .001 and < .01, respectively). Outcome of this last group improved with the HD protocol (PFS, 40% v 23%; P = .01). CONCLUSION: In children with unresectable neuroblastoma, risk categories can be defined by age and primary tumor site. HD chemotherapy should be investigated for the poor-risk category age 1 to 15 years with an abdominal primary tumor.

Asunto(s)

Neuroblastoma/terapia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Análisis Multivariante , Estadificación de Neoplasias , Neuroblastoma/tratamiento farmacológico , Neuroblastoma/patología , Neuroblastoma/cirugía , Inducción de Remisión , Análisis de Supervivencia , Resultado del Tratamiento

RESUMEN

Avascular necrosis of bone (AVNB) is reported in two children after allogeneic bone marrow transplantation. Preparation therapy for transplantation included cyclophosphamide and total body irradiation. Corticosteroids, cyclosporine A, and methotrexate were used for graft-versus-host-disease prophylaxis. The possible role of combination therapy in development of AVNB is discussed, but a direct relationship with single agents was not found. However, an early diagnosis is important to institute conservative treatment and prevent irreversible damage to affected joints. Magnetic resonance imaging was found to be more sensitive than plain radiography in early detection of AVNB.

Asunto(s)

Trasplante de Médula Ósea/efectos adversos , Leucemia/complicaciones , Osteonecrosis/etiología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Terapia Combinada , Humanos , Leucemia/terapia , Imagen por Resonancia Magnética , Masculino , Osteonecrosis/diagnóstico , Irradiación Corporal Total

RESUMEN

Since there is little information regarding the possible prognostic significance of tumor rupture in localized neuroblastoma, we have analyzed the clinical courses of 163 children registered from 1979-1990 in 12 italian pediatric oncology Centers participating in the Neuroblastoma Cooperative Group of the A.I.E.O.P. (Italian Association for Paediatric Haematology-Oncology). Ten instances (6%) of tumor rupture were described. Ruptures occurred preoperatively in one child, during the operation in 9; among these 9, two were provoked by the surgeon to allow radical tumor excision, 7 were accidental. Of these 10 children, 7 relapsed at 3-25 months (median, 8 months) from diagnosis. Relapses were local in 5 children (2 of the 5 died), disseminated in one (who died), local + disseminated in one (presently alive with disease). Two local relapses were followed by bony or haematologic spread at 4 and 8 months, respectively. Of the 7 children who relapsed, 2 are alive in complete remission at 29, 100 months, respectively; two are alive with disease at 3 and 65 months, 3 died at 8, 15 and 24 months, respectively. We conclude that rupture of a localized neuroblastoma is a factor predisposing to relapse and may compromise the chance of cure. The surgeon should be aware of the risks connected with this complication and make any effort to avoid it.

Asunto(s)

Neuroblastoma/mortalidad , Neuroblastoma/fisiopatología , Neoplasias Abdominales/fisiopatología , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Recurrencia Local de Neoplasia , Pronóstico , Rotura Espontánea , Neoplasias Torácicas/fisiopatología

RESUMEN

CD30/Ki-1 antigen expression in 243 cases of malignant lymphomas was examined using Ber-H2 monoclonal antibody. Among them 20 cases were categorized as Ki-1 anaplastic large cell lymphoma. In two of these cases histiocyte-associated markers were also expressed. In these cases histopathologic and extensive in situ immunophenotypic analyses were used with genotypic studies in the determination of cell lineage. A sinusoid histologic pattern of involvement with partial lymph node infiltration by pleomorphic neoplastic cells was noticed in the nodes from both patients. Solid areas of node replacement resembling metastatic carcinoma were seen in Patient 1. Immunohistologically, tumor cells of both cases were positive for CD30, CD25, CD71, LN3 (HLA-DR), EMA, CD45, CD74, vimentin, alpha-1-antichymotrypsin, and CD68. Patient 1 was also CD45RO+, CD43+, whereas Patient 2 was positive for alpha-1-antitrypsin and CD4 tumor cells. Genotypic studies revealed that TCR beta and TCR gamma chain genes were clonally rearranged in Patient 1, whereas no rearrangements were detected in Patient 2. This study supports the view that some Ki-1 anaplastic large cell lymphomas may express multiple histiocyte-associated antigens and confirms that this group of neoplasms have immunophenotypic heterogeneity. The results of genotypic analyses used with immunophenotyping does not exclude that the tumor cells in these cases may be of true histiocytic origin despite the Ki-1-positive phenotype.

Asunto(s)

Antígenos de Diferenciación/análisis , Antígenos de Neoplasias/análisis , Histiocitos/inmunología , Linfoma de Células B Grandes Difuso/inmunología , Adolescente , Niño , ADN de Neoplasias/genética , Antígenos HLA-DR/inmunología , Antígenos de Histocompatibilidad/análisis , Humanos , Inmunofenotipificación , Antígeno Ki-1 , Antígenos Comunes de Leucocito , Linfoma de Células B Grandes Difuso/genética , Linfoma de Células B Grandes Difuso/patología , Masculino , Glicoproteínas de Membrana/análisis , Mucina-1 , Vimentina/análisis , alfa 1-Antiquimotripsina/inmunología , alfa 1-Antitripsina/inmunología

RESUMEN

Thirty asymptomatic patients with acute lymphoblastic leukemia who had received prophylactic cranial irradiation (16 pts had 2400 cGy, 14 pts 1800 cGy) and intrathecal methotrexate were studied by computed tomography of the brain 60 to 148 months after initiation of prophylaxis. Three of 30 (10%) patients presented abnormal findings: widening of frontal subarachnoid space (1 patient), little area of decreased attenuation coefficient (1 patient), and intracerebral calcifications (1 patient Tomography abnormalities could be detected either in patients treated with 2400 cGy and in those treated with 1800 cGy. None of our patients showed central nervous system dysfunctions on physical examination. The results of our study suggest that tomography findings have a poor clinical significance.

Asunto(s)

Neoplasias Encefálicas/prevención & control , Encéfalo/diagnóstico por imagen , Leucemia-Linfoma Linfoblástico de Células Precursoras/prevención & control , Traumatismos por Radiación/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Metotrexato/uso terapéutico , Leucemia-Linfoma Linfoblástico de Células Precursoras/radioterapia

RESUMEN

The clinical features and the treatment of undifferentiated (embryonal) sarcoma of the liver in 8 patients younger than 19 years old were analyzed. All these cases were registered in the retrospective multicentric study on childhood malignant tumors of the liver, conducted between 1983 and 1985 by the Italian Association of Pediatric Hematology Oncology. The age of the patients ranged from 94 to 190 months (median = 113.5 months); all children were males. An abdominal mass was the common presenting features. Abnormalities in hemogram and common liver tests were rarely reported. Angiography revealed various degrees of vascularization in these tumors. Two patients achieved a surgical complete remission (CR) at diagnosis; one patient achieved surgical CR after primary chemotherapy with vincristine, adriamycin, cyclophosphamide and 5-fluorouracil, which reduced the tumor volume and permitted surgical resection. Two of these patients are still in CR at 14 and 60 months after diagnosis; the third patient died of liver failure without evidence of recurrence 6 months after diagnosis. All of the other patients, who never achieved CR, died of disease. One was lost to follow-up, and one surgical death occurred. Reports of childhood undifferentiated sarcoma are reviewed.

Asunto(s)

Neoplasias Hepáticas/diagnóstico , Mesenquimoma/diagnóstico , Niño , Preescolar , Humanos , Lactante , Italia , Neoplasias Hepáticas/terapia , Masculino , Mesenquimoma/terapia , Estudios Retrospectivos

RESUMEN

Neural tumors, Wilms' tumor, rhabdomyosarcoma and several types of leukemia have been previously described in association with neurofibromatosis (NF). In a nation-wide collection of cases in Italy, 15 children (0-14 years of age) with NF and cancer or leukemia were identified; 13 of them had been diagnosed with cancer between 1976-83. The expected number of children with cancer and NF in 1976-83 was 4.48. The distribution of tumor types was different from that found in the general population, with a higher proportion of tumors of neural crest origin as well as soft tissue sarcomas. In 7/15 the family history was positive for NF; in 5/7 the individuals affected included the mother and/or a maternal relative.

Asunto(s)

Neoplasias Primarias Múltiples/diagnóstico , Neoplasias/diagnóstico , Neurofibromatosis 1/diagnóstico , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Italia , Masculino