Abstract:
BACKGROUND:Pheochromocytomas (PCs) are neuroendocrine tumors arising from the chromaffin cells of the adrenal gland, and paragangliomas (PGLs) are their extra-adrenal counterparts arising from ganglia along the sympathetic/parasympathetic chain. Surgery is the cornerstone of treatment. A sporatic or inherited germline mutation is commonly associated. MATERIALS AND METHODS:Among over 1000 patients registered into the Tumori Rari in Età Pediatrica-rare tumors in pediatric age project-from 2000 to 2019, 50 were affected by PC/PGL. All clinical and therapeutic data were evaluated. RESULTS:Twenty-eight patients had PC and 22 had PGL. Age at diagnosis ranged between 5 and 17 years. Thirty-five patients had symptoms related to catecholamine hypersecretion; in 7 of 50 patients, diagnosis was incidental or done during assessment of a familial syndrome. In all cases, conventional imaging was effective to assess the presence of a tumor. In addition, 18 of 38 functional imaging studies were positive (61%). Forty-eight patients were eligible for surgery: a complete resection was more frequently achieved in PC than in PGL (26/28 vs 11/22). All relapses were treated with surgery alone, surgery plus medical treatment, or chemotherapy alone; one PC with metastasis at diagnosis received radiotherapy only. Forty-four patients were in the first, second, or third complete remission (10/50 recurred; 8/10 carried a germline mutation). Five of 50 patients were alive with disease. One patient died of disease. CONCLUSIONS:Surgery can be curative in most tumors but it may not be always effective in removing PGLs. Severe postsurgical sequelae may affect these patients. Genetic tests should always be considered in individuals affected, and genetic counseling should be offered to their families.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Virgone C,Andreetta M,Avanzini S,Chiaravalli S,De Pasquale D,Crocoli A,Inserra A,D'Angelo P,Alaggio R,Opocher G,Cecchetto G,Ferrari A,Bisogno G,Dall'Igna Pdoi
10.1002/pbc.28332subject
Has Abstractpub_date
2020-08-01 00:00:00pages
e28332issue
8eissn
1545-5009issn
1545-5017journal_volume
67pub_type
临床试验,杂志文章,多中心研究abstract::Myasthenia gravis is among the rare complications after allogeneic hematopoietic stem cell transplantation and is usually associated with chronic GVHD. Herein, we report a 2-year and 10 months of age female with Griscelli syndrome, who developed severe myasthenia gravis at post-transplant +22nd month and required resp...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24799
更新日期:2014-05-01 00:00:00
abstract::Little information is available on cytogenetic abnormalities and their prognostic importance in childhood mature B-cell non-Hodgkin lymphoma (B-NHL). We performed a review of 79 abnormal karyotypes in childhood B-NHL treated by a uniform protocol. Del(17p) was independently associated with significantly inferior event...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25482
更新日期:2015-07-01 00:00:00
abstract:BACKGROUND:Childhood acute lymphoblastic leukemia (ALL) survivors' increased risk for adverse health outcomes could be mitigated through consuming a balanced diet. Nonetheless, >70% of adult survivors do not meet survivorship dietary recommendations. ALL treatment may amplify risk for restricted dietary preferences (pi...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28811
更新日期:2020-12-31 00:00:00
abstract:BACKGROUND:The hallmark of sickle cell disease (SCD) is recurrent, painful vaso-occlusive episodes (VOC) and is the most common reason for hospitalization in SCD patients. Narcotics, particularly morphine, along with fluid hydration are standard treatments for painful episodes but have been associated with the developm...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20403
更新日期:2005-10-15 00:00:00
abstract::Children with primary immunodeficiency or chromosomal breakage syndromes are at increased risk of developing non-Hodgkin lymphomas; they cannot tolerate standard chemotherapy regimens. We report two children with diffuse, large, B-cell lymphoma; one had ataxia telangiectasia and one had common variable immunodeficienc...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21808
更新日期:2009-05-01 00:00:00
abstract:BACKGROUND:Childhood germ cell tumors (cGCTs), believed to arise from transformed primordial germ cells by an unknown mechanism, provide a unique model system for investigating cell signaling, pluripotency, and the microenvironment of neoplastic stem cells (NSCs) in vivo. This is the first report of proteomics of cGCTs...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23282
更新日期:2012-05-01 00:00:00
abstract::A 6-year-old female was admitted with abdominal pain and a mass in the right abdomen. Her lactose dehydrogenase level was 1,200 IU/L, and neuron specific enolase was 120 ng/ml. Computed tomography scan confirmed a large right renal mass with necrosis. A right radical nephrectomy was performed. The tumor was completely...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20831
更新日期:2008-01-01 00:00:00
abstract:BACKGROUND:Pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) may rarely occur in visceral tissues, and little is known about their clinical history. The present study retrospectively analyzed a group of patients prospectively registered in Italian pediatric protocols conducted between 1979 and 2004. METHODS:I...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26490
更新日期:2017-09-01 00:00:00
abstract:BACKGROUND:Deaths during induction chemotherapy for pediatric acute lymphoblastic leukemia (ALL) account for one-tenth of ALL-associated mortality and half of ALL treatment-related mortality. We sought to ascertain patient- and hospital-level factors associated with induction mortality. PROCEDURE:We performed a retros...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24855
更新日期:2014-05-01 00:00:00
abstract:BACKGROUND:Primary hemophagocytic lymphohistiocytosis (HLH) represents a group of inherited hyperinflammatory immunodeficiencies, including familial HLH (FHL), Griscelli syndrome type 2 (GS2), and X-linked lymphoproliferative syndrome (XLP). We previously reported an annual incidence of suspected primary HLH in Sweden ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25308
更新日期:2015-02-01 00:00:00
abstract:BACKGROUND:Neuroblastoma (NB) is cytogenetically characterized by a number of non-random events. However, knowledge is limited concerning the timing of occurrence and inter-action of many of these events. METHODS:Karyotypic patterns were obtained from a study group of 49 NB tumors that had been analyzed by conventiona...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20179
更新日期:2005-02-01 00:00:00
abstract:BACKGROUND:Despite the presence of reports on correlation between major congenital defects and cancer, very few studies have investigated the frequency of minor anomalies in childhood malignancy. The aim of this study was to determine the prevalence of minor anomalies in children with hematological malignancy. PROCEDU...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22689
更新日期:2011-02-01 00:00:00
abstract::Diffuse intrinsic pontine glioma (DIPG) is characterized by a short history of brainstem symptoms and well-known magnetic resonance imaging features with a fatal outcome. However, we report three unusual cases of brainstem tumors with an initial indolent and protracted course, which subsequently developed the classica...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28102
更新日期:2020-03-01 00:00:00
abstract::Treatment recommendations for endemic Burkitt lymphoma (BL) in settings with only minimum requirements for curative treatment (PODC setting 1) are described. The reported cure rate for endemic BL is usually <50%. Facilities within setting 1 differ. Three treatment schedules are proposed based on: (1) when accurate sta...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,实务指引
doi:10.1002/pbc.24407
更新日期:2013-03-01 00:00:00
abstract::Nutrition therapy is a therapeutic approach to treating medical conditions and symptoms via diet, which can be done by oral, enteral or parenteral routes. It is desirable to include nutritional interventions as a standard of care in pediatric cancer units (PCUs) at all levels of care. The interventions are dependent o...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.28378
更新日期:2020-06-01 00:00:00
abstract:BACKGROUND:The combined inhibition of insulin-growth factor type 1 receptor (IGF-1R) and the mammalian target of rapamycin (mTOR) has shown activity in preclinical models of pediatric sarcoma and in adult sarcoma patients. We evaluated the activity of the anti-IGF-1R antibody cixutumumab with the mTOR inhibitor temsiro...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.25334
更新日期:2015-03-01 00:00:00
abstract:BACKGROUND:Coping with end-stage pediatric cancer patients and the related bereavement is a challenge for all the caregivers involved. PROCEDURE:Forty-seven cancer patients who died in 2006 were assessed as concerns the main place of care in the end stage of their disease, their symptoms, the palliative treatments rec...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22284
更新日期:2010-01-01 00:00:00
abstract::The youngest siblings may be both emotionally vulnerable and often neglected members of the family of a childhood cancer patient. The prompt identification of signs of distress in these subjects allows trained caregivers to intervene with personalized, age-appropriate, attention, and care. A narrative approach, based ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21056
更新日期:2008-03-01 00:00:00
abstract:BACKGROUND:Epoetin alfa (EPO, PROCRIT) pharmacokinetics and pharmacodynamics were evaluated in children with malignant solid tumors receiving chemotherapy. PROCEDURE:Children initially received IV EPO 600 IU/kg (max dose 40,000 IU) or placebo once weekly for 16 weeks. Dose was increased to 900 IU/kg (max dose 60,000 I...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,随机对照试验
doi:10.1002/pbc.20685
更新日期:2006-10-15 00:00:00
abstract:BACKGROUND:Although respiratory syncytial virus (RSV) is the leading cause of pediatric lower respiratory tract infections, the burden of RSV in children with sickle cell disease (SCD) is unknown. METHODS:We conducted a retrospective, nested, case-control study of children with SCD <18 years who had respiratory viral ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28759
更新日期:2021-01-01 00:00:00
abstract:BACKGROUND:Self-concept was compared between adult survivors of childhood acute lymphoblastic leukemia (ALL) and sibling controls. Adult survivor subgroups at greatest risk for negative self-concept were identified. PROCEDURE:Survivors (n = 578) aged > or =18 years, treated before age 20 years on Children's Cancer Gro...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.10434
更新日期:2004-03-01 00:00:00
abstract:BACKGROUND:The Kids ITP Tools (KIT) is a disease-specific measure of health-related quality of life for children with immune thrombocytopenia (ITP). To facilitate use in international trials it has been cross-culturally adapted for France, Germany, the United Kingdom and Uruguay. This study assessed the validity and re...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24257
更新日期:2013-01-01 00:00:00
abstract:BACKGROUND:In low- and middle-income countries, therapeutic options for advanced, refractory, or relapsing malignancies are limited due to local constraints such as cost of drugs, distance from oncology centers, and lack of availability of new anticancer drugs. Metronomics, which combines metronomic chemotherapy (MC) a...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28508
更新日期:2020-09-01 00:00:00
abstract:BACKGROUND:The objective of this report was to estimate long-term outcome and prognostic factors in children and adolescents with osteosarcoma. A large group of osteosarcoma patients were analyzed at our national oncology center. PROCEDURE:To evaluate the efficacy of surgery and multiagent chemotherapy for treating os...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23172
更新日期:2011-09-01 00:00:00
abstract::Both hepatoblastoma and hypoplastic kidneys are rare in children. A review of all patients with hepatoblastoma treated at our institution between 1993 and 2011 revealed three cases of hepatoblastoma occurring in children with hypoplastic kidneys and significantly impaired renal function. Two patients were treated with...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24936
更新日期:2014-08-01 00:00:00
abstract:BACKGROUND:Burnout is a work-related syndrome consisting of emotional exhaustion, depersonalization, and diminished feelings of personal accomplishment. Physicians who care for patients with life-threatening illnesses are at high risk for developing burnout. This survey evaluates the prevalence of burnout among pediatr...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23121
更新日期:2011-12-15 00:00:00
abstract:BACKGROUND:Morphological evaluation of early response to chemotherapy and measurement of minimal residual disease by flow cytometry or PCR are being used for evaluation of prognosis and treatment stratification in children with acute lymphoblastic leukaemia (ALL). PROCEDURE:In a series of 14 consecutive bone marrow in...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23081
更新日期:2011-08-01 00:00:00
abstract:BACKGROUND:Many pediatric oncology patients undergoing hematopoietic stem cell transplantation (HSCT) require nutritional support (NS) because of their inability to consume adequate caloric intake enough calories orally. Although NS can be provided either enteraly (EN) or parenteraly (PN), EN is the preferred method of...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25473
更新日期:2015-08-01 00:00:00
abstract:INTRODUCTION:The rate of bacterial infections in children with sickle cell disease (SCD) has decreased in recent years, mainly due to penicillin prophylaxis and vaccination. OBJECTIVES:To determine the rate of severe bacterial infection (SBI) in a cohort of children with SCD and to describe low-risk factors for confir...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27667
更新日期:2019-06-01 00:00:00
abstract::In this retrospective study of patients with overt orbital retinoblastoma, we evaluated minimally disseminated disease (MDD) in bone marrow and cerebrospinal fluid (CSF) using CRX and/or GD2 synthase as markers. Ten patients were evaluated-five (50%) at diagnosis and five upon relapse. MDD was detected in four cases (...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27662
更新日期:2019-06-01 00:00:00