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1.
Clin. transl. oncol. (Print) ; 19(1): 76-83, ene. 2017. tab
Article in English | IBECS | ID: ibc-159121

ABSTRACT

Introduction. SIOPEN INES protocol yielded excellent 5-year survival rates for MYCN-non-amplified metastatic neuroblastoma. Patients deemed ineligible due to lack or delay of MYCN status or late registration were treated, but not included in the study. Our goal was to analyse survival at 10 years among the whole population. Materials and methods. Italian and Spanish metastatic INES patients’ data are reported. SPSS 20.0 was used for statistical analysis. Results. Among 98 infants, 27 had events and 19 died, while 79 were disease free. Five- and 10-year event-free survival (EFS) were 73 and 70 %, and overall survival (OS) was 81 and 74 %, respectively. MYCN status was significant for EFS, but not for OS in multivariate analysis. Conclusions. The survival rates of patients who complied with all the inclusion criteria for INES trials are higher compared to those that included also not registered patients. Five-year EFS and OS for INES 99.2 were 87.8 and 95.7 %, while our stage 4s population obtained 78 and 87 %. Concerning 99.3, 5-year EFS and OS were 86.7 and 95.6 %, while for stage 4 we registered 61 and 68 %. MYCN amplification had a strong impact on prognosis and therefore we consider it unacceptable that many patients were not studied for MYCN and probably inadequately treated. Ten-year survival rates were shown to decrease: EFS from 73 to 70 % and OS from 81 to 74 %, indicating a risk of late events, particularly in stage 4s. Population-based registries like European ENCCA WP 11-task 11 will possibly clarify these data (AU)


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Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Neuroblastoma/complications , Neuroblastoma/diagnosis , Neoplasm Metastasis/diagnosis , Neoplasm Metastasis/physiopathology , Eligibility Determination/standards , Prognosis , Clinical Protocols , 28599 , Survivorship/physiology , Informed Consent/standards
2.
Clin Transl Oncol ; 19(1): 76-83, 2017 Jan.
Article in English | MEDLINE | ID: mdl-27041689

ABSTRACT

INTRODUCTION: SIOPEN INES protocol yielded excellent 5-year survival rates for MYCN-non-amplified metastatic neuroblastoma. Patients deemed ineligible due to lack or delay of MYCN status or late registration were treated, but not included in the study. Our goal was to analyse survival at 10 years among the whole population. MATERIALS AND METHODS: Italian and Spanish metastatic INES patients' data are reported. SPSS 20.0 was used for statistical analysis. RESULTS: Among 98 infants, 27 had events and 19 died, while 79 were disease free. Five- and 10-year event-free survival (EFS) were 73 and 70 %, and overall survival (OS) was 81 and 74 %, respectively. MYCN status was significant for EFS, but not for OS in multivariate analysis. CONCLUSIONS: The survival rates of patients who complied with all the inclusion criteria for INES trials are higher compared to those that included also not registered patients. Five-year EFS and OS for INES 99.2 were 87.8 and 95.7 %, while our stage 4s population obtained 78 and 87 %. Concerning 99.3, 5-year EFS and OS were 86.7 and 95.6 %, while for stage 4 we registered 61 and 68 %. MYCN amplification had a strong impact on prognosis and therefore we consider it unacceptable that many patients were not studied for MYCN and probably inadequately treated. Ten-year survival rates were shown to decrease: EFS from 73 to 70 % and OS from 81 to 74 %, indicating a risk of late events, particularly in stage 4s. Population-based registries like European ENCCA WP 11-task 11 will possibly clarify these data.


Subject(s)
Biomarkers, Tumor/genetics , Clinical Trials as Topic , Gene Amplification , N-Myc Proto-Oncogene Protein/genetics , Neuroblastoma/mortality , Child , Child, Preschool , Combined Modality Therapy , Female , Follow-Up Studies , Humans , Infant , Infant, Newborn , Male , Neoplasm Staging , Neuroblastoma/genetics , Neuroblastoma/secondary , Neuroblastoma/therapy , Prognosis , Survival Rate
3.
Childs Nerv Syst ; 23(11): 1341-5, 2007 Nov.
Article in English | MEDLINE | ID: mdl-17609967

ABSTRACT

INTRODUCTION: Cerebral germinomas, the most common and least malignant intracranial germ cell tumors, usually arise in the pineal or suprasellar region and have characteristic clinical and radiological features. Germinomas more rarely occur in the thalamus, basal ganglia, and internal capsule, causing sometimes cerebral hemiatrophy and hemiparesis. More rarely, other clinical features can be fever of unknown origin, visual disturbance, and neuropsychiatric symptoms. Cerebral hemiatrophy can precede the imaging depiction of the off-midline mass. CASE: The authors present the first case of cerebral germinoma with synchronous involvement of the midline and off-midline structures, with unusual clinical and radiological presentation. DISCUSSION: The literature is reviewed, and the pathogenesis, the clinical findings, the imaging, and the therapy are discussed.


Subject(s)
Brain Neoplasms/pathology , Cerebrum/pathology , Germinoma/pathology , Pituitary Neoplasms/pathology , Adolescent , Adult , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Atrophy , Brain Neoplasms/complications , Brain Neoplasms/drug therapy , Brain Neoplasms/surgery , Child , Functional Laterality , Germinoma/complications , Germinoma/drug therapy , Germinoma/surgery , Humans , Male , Paresis/etiology , Pituitary Neoplasms/complications , Pituitary Neoplasms/drug therapy , Pituitary Neoplasms/surgery , Thalamic Diseases/drug therapy , Thalamic Diseases/pathology , Treatment Outcome
4.
J Pediatr Endocrinol Metab ; 19 Suppl 1: 399-405, 2006 Apr.
Article in English | MEDLINE | ID: mdl-16700317

ABSTRACT

Aggressive surgery of craniopharyngioma can cause severe, life-long hypothalamic and pituitary dysfunctions and possibly further impair visual function; conventional radiation therapy (RT) can affect intellectual functioning and cause secondary tumours. Because of the severe morbidity associated with aggressive surgery, many authors nowadays recommend a less radical approach followed by RT. This combined approach allows achieving 70-83% 10-year local control rates which are comparable to that achieved with aggressive surgery. The main morbidity of this conservative combined approach is represented by pituitary dysfunction secondary to RT, however, sparing severe hypothalamic disturbances. The interval between treatment and onset of the disorder is much longer than in the case of aggressive surgery and this can have a beneficial impact on quality of life, especially in children. This alternative therapeutic approach has become more appealing now that modern RT techniques allow safer delivery of the RT, particularly in childhood.


Subject(s)
Craniopharyngioma/radiotherapy , Craniopharyngioma/surgery , Pituitary Neoplasms/radiotherapy , Pituitary Neoplasms/surgery , Child , Combined Modality Therapy , Craniopharyngioma/complications , Humans , Neurosurgical Procedures/adverse effects , Pituitary Neoplasms/complications , Radiotherapy/adverse effects , Treatment Outcome
5.
J Clin Oncol ; 19(1): 183-90, 2001 Jan 01.
Article in English | MEDLINE | ID: mdl-11134211

ABSTRACT

PURPOSE: To report on the treatment of patients with newly diagnosed neuroblastoma presenting with spinal cord compression (SCC). PATIENTS AND METHODS: Of 1,462 children with neuroblastoma registered between 1979 and 1998, 76 (5.2%) presented with signs/symptoms of SCC, including motor deficit in 75 patients (mild in 43, moderate in 22, severe [ie, paraplegia] in 10), pain in 47, sphincteric deficit in 30, and sensory loss in 11. Treatment of SCC consisted of radiotherapy in 11 patients, laminectomy in 32, and chemotherapy in 33. Laminectomy was more frequently performed in cases with favorable disease stages and in those with severe motor deficit, whereas chemotherapy was preferred in patients with advanced disease. RESULTS: Thirty-three patients achieved full neurologic recovery, 14 improved, 22 remained stable, and eight worsened, including three who become paraplegic. None of the 10 patients with grade 3 motor deficit, eight of whom were treated by laminectomy, recovered or improved. In the other 66 patients, the neurologic response to treatment was comparable for the three therapeutic modalities. All 11 patients treated by radiotherapy and 26 of 32 patients treated by laminectomy, but only two of 33 treated by chemotherapy, received additional therapy for SCC. Fifty-four of 76 patients are alive at time of the analysis, with follow-up of 4 to 209 months (median, 139 months). Twenty-six (44%) of 54 survivors have late sequelae, mainly scoliosis and sphincteric deficit. CONCLUSION: Radiotherapy, laminectomy, and chemotherapy showed comparable ability to relieve or improve SCC. However, patients treated with chemotherapy usually did not require additional therapy, whereas patients treated either with radiotherapy or laminectomy commonly did. No patient presenting with (or developing) severe motor deficit recovered or improved. Sequelae were documented in 44% of surviving patients.


Subject(s)
Neuroblastoma/pathology , Neuroblastoma/therapy , Spinal Cord Compression/therapy , Spinal Cord Neoplasms/pathology , Spinal Cord Neoplasms/therapy , Adolescent , Child , Child, Preschool , Combined Modality Therapy , Female , Humans , Infant , Infant, Newborn , Laminectomy , Male , Neoplasm Invasiveness , Neuroblastoma/mortality , Spinal Cord Compression/drug therapy , Spinal Cord Compression/radiotherapy , Spinal Cord Neoplasms/mortality , Survival Rate , Treatment Outcome
6.
J Clin Oncol ; 18(22): 3829-36, 2000 Nov 15.
Article in English | MEDLINE | ID: mdl-11078496

ABSTRACT

PURPOSE: To improve autologous leukapheresis strategies in high-risk neuroblastoma (NB) patients with extensive bone marrow involvement at diagnosis. PATIENTS AND METHODS: Anti-G(D2) immunocytochemistry (sensitivity, 1 in 10(5) to 10(6) leukocytes) was used to evaluate blood and bone marrow disease at diagnosis and during the recovery phase of the first six chemotherapy cycles in 57 patients with stage 4 NB and bone marrow disease at diagnosis. A total of 42 leukapheresis samples from the same patients were evaluated with immunocytology, and in 24 of these patients, an anti-G(D2) immunomagnetic enrichment step was used to enhance tumor-cell detection. RESULTS: Tumor cytoreduction was much faster in blood compared with bone marrow (3.2 logs after the first cycle and 2.1 logs after the first two cycles, respectively). Bone marrow disease was often detectable throughout induction, with a trend to plateau after the fourth cycle. By direct anti-G(D2) immunocytology, a positive leukapheresis sample was obtained in 7% of patients after either the fifth or sixth cycle; when NB cell immunomagnetic enrichment was applied, 25% of patients had a positive leukapheresis sample (sensitivity, 1 in 10(7) to 10(8) leukocytes). CONCLUSION: Standard chemotherapy seems to deliver most of its in vivo purging effect within the first four cycles. In patients with overt marrow disease at diagnosis, postponing hematopoietic stem-cell collection beyond this point may not be justified. Tumor-cell clearance in blood seems to be quite rapid, and earlier collections via peripheral-blood leukapheresis might be feasible. Immunomagnetically enhanced NB cell detection can be highly sensitive and can indicate whether ex vivo purging should be considered.


Subject(s)
Bone Marrow Neoplasms/pathology , Immunomagnetic Separation/methods , Leukapheresis/methods , Neoplastic Cells, Circulating/pathology , Neuroblastoma/pathology , Adolescent , Antibodies, Monoclonal , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Bone Marrow Neoplasms/immunology , Bone Marrow Neoplasms/secondary , Bone Marrow Purging/methods , Child , Child, Preschool , Gangliosides/immunology , Hematopoietic Stem Cell Transplantation , Humans , Infant , Neoplastic Cells, Circulating/immunology , Neuroblastoma/blood , Neuroblastoma/therapy
7.
Pediatr Med Chir ; 20(3): 175-8, 1998.
Article in Italian | MEDLINE | ID: mdl-9744008

ABSTRACT

A highly sensitive and specific methodology to detect neuroblastoma cells in the bone marrow and peripheral blood of children with neuroblastoma is of critical importance for proper staging and treatment of these patients. In addition, patients with bone marrow infiltration at diagnosis need to undergo regular investigation to measure the effectiveness of chemotherapy (so called "in vivo" purging). Finally, the evaluation of autologous stem cells taken from bone marrow or peripheral blood is necessary to rule out or minimise the possibility of reinfusing tumor cells to the patient following myeloablative therapy. The authors provide a "state of the art" data on this complicated issue and give their preliminary results of their own experience, mainly concerning the immunocytological methods.


Subject(s)
Bone Marrow Neoplasms/pathology , Bone Marrow Neoplasms/secondary , Neoplastic Cells, Circulating/pathology , Nervous System Neoplasms/pathology , Neuroblastoma/pathology , Neuroblastoma/secondary , Biomarkers, Tumor/metabolism , Bone Marrow/metabolism , Bone Marrow/pathology , Bone Marrow Neoplasms/metabolism , Bone Marrow Neoplasms/therapy , Bone Marrow Purging , Bone Marrow Transplantation , Child , Humans , Immunohistochemistry , Neoplasm, Residual , Neoplastic Cells, Circulating/metabolism , Nervous System Neoplasms/metabolism , Nervous System Neoplasms/therapy , Neuroblastoma/metabolism , Neuroblastoma/therapy , Prognosis
8.
J Hematother ; 7(4): 361-6, 1998 Aug.
Article in English | MEDLINE | ID: mdl-9735867

ABSTRACT

High-sensitivity immunocytochemistry was used to evaluate the relative frequency of neuroblastoma cells in bone marrow and peripheral blood in patients with neuroblastoma (NB). A total of 51 concomitant paired blood and marrow samples (102 total) from 35 patients with NB (age 4 months-31 years; stage 29 stage IV, 4 stage III, 2 stage IVS; 14 at diagnosis, 18 in relapse, 12 during treatment, and 7 off-therapy) were analyzed. Cytospins containing up to 10(6) cells each were prepared using the mononuclear cell (MNC) fraction. For immunocytologic staining, a primary mouse monoclonal anti-GD2 antibody (3F8), a secondary antimouse biotinylated antibody, and a streptavidin-alkaline phosphatase complex were used. A minimum of two cytospins containing a mean of 1.4 x 10(6) total MNCs was analyzed in addition to a negative and a positive control. No circulating tumor cells were detected when the concomitant marrow samples were negative or had <10 positive cells per 106 MNC (23 of 51 samples). Of the 18 marrow samples positive at 10-10,000 cells per 106 MNC, 6 had detectable NB cells in the corresponding blood sample, whereas for marrow samples with >10,000 NB cells per 10(6) MNC (1%), the concomitant blood sample was positive for 9 of the 10. When both marrow and blood samples were positive (15 BM-PB pairs), NB cell frequency was significantly lower in blood, with a mean difference of 2.14 logs (median 2.22, range -0.16-4.8, standard error 0.38). In patients with NB, circulating tumor cell frequencies seem to be substantially lower than in concomitant marrow samples, with a mean difference of >2 logs.


Subject(s)
Bone Marrow/pathology , Brain Neoplasms/pathology , Immunohistochemistry/methods , Neuroblastoma/pathology , Adolescent , Adult , Animals , Brain Neoplasms/blood , Brain Neoplasms/diagnosis , Child , Child, Preschool , Humans , Infant , Mice , Neoplasm, Residual , Neuroblastoma/blood , Neuroblastoma/diagnosis , Recurrence , Sensitivity and Specificity
9.
Pediatr Med Chir ; 14(4): 455-7, 1992.
Article in Italian | MEDLINE | ID: mdl-1461788

ABSTRACT

An unusual case of abdominal neuroblastoma, whose extension involved the psoas muscle, leading to an ultrasonographic and Magnetic Resonance imaging simulating an haematoma, is described. Histology disclosed the malignant nature of the muscle mass. The Authors discuss the value of the new imaging techniques and in particular of Magnetic Resonance in the diagnostic work-up of paediatric malignancies.


Subject(s)
Abdominal Neoplasms/diagnosis , Neuroblastoma/diagnosis , Psoas Muscles , 3-Iodobenzylguanidine , Abdominal Neoplasms/diagnostic imaging , Child , Contrast Media , Diagnosis, Differential , Female , Humans , Iodine Radioisotopes , Iodobenzenes , Magnetic Resonance Imaging , Neuroblastoma/diagnostic imaging , Radionuclide Imaging
10.
J Neural Transm Gen Sect ; 84(3): 183-93, 1991.
Article in English | MEDLINE | ID: mdl-1679335

ABSTRACT

d-Amphetamine (d-AMP) is a potent releaser of dopamine (DA), and its central nervous system stimulant action is mediated primarily through its effect on the substantia nigra and ventral tegmental area dopaminergic neurons (nuclei A9 and A10, respectively). The purpose of the present experiment was to use electrophysiological techniques to examine dendritic release of DA in the in vitro slice preparation, and determine whether: (1) d-AMP inhibits the firing rates of both A9 and A10 cells; (2) the d-AMP-induced inhibition is mediated via the dendritic release of DA; and (3) there is spontaneous dendritic release of DA. Superfusion with d-AMP (2-100 microM) produced identical inhibitory dose-response curves for A9 and A10 cells, and a dose of 6.25 microM caused more than 50% inhibition in the cell firing rates. The d-AMP-induced inhibition was attenuated by blocking DA synthesis. Either D2 receptor blockade (sulpiride, 1 microM), or DA synthesis inhibition (alpha-methylparatyrosine, 50 microM) resulted in a marked increase in the firing rates of dopaminergic cells. These data suggest that d-AMP comparably releases DA from both A9 and A10 cell dendrites, that it releases newly-synthesized DA to inhibit cell firing, and that DA is tonically released to regulate cell firing rates via interactions with inhibitory D2 autoreceptors.


Subject(s)
Dendrites/drug effects , Dextroamphetamine/pharmacology , Dopamine Agents/pharmacology , Dopamine/metabolism , Substantia Nigra/drug effects , Tegmentum Mesencephali/drug effects , Action Potentials , Animals , Dendrites/metabolism , Electrophysiology , Male , Methyltyrosines/pharmacology , Mice , Mice, Inbred BALB C , Mice, Inbred CBA , Receptors, Dopamine/drug effects , Secretory Rate/drug effects , Substantia Nigra/metabolism , Sulpiride/pharmacology , Synaptic Transmission/drug effects , Tegmentum Mesencephali/metabolism , alpha-Methyltyrosine
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