Abstract:
:Malignant ectomesenchymoma (MEM) is a rare soft tissue tumor believed to arise from a pluripotent migratory neural crest cell and composed fo both a mesenchymal element and a neuroectodermal element. The authors report the case of an 11-month-old male who presented with a local abdominal MEM and systemic metastases into lungs, liver, bones, and bone marrow. This is the first reported case of an MEM with initial bone marrow dissemination. The tumor consisted of a neuroblastoma component and a mesenchymal component with sarcomatous features. Diagnosis and therapy were complicated by the histological heterogeneity of the tumor, which also influenced the clinical appearance and course in this case. A literature search revealed 15 other evaluated cases that arose in soft tissue and had adequate clinicopathologic data. Complete surgical resection was the mainstay of treatment, and chemotherapy also appeared to be important. In all reported patients (n = 3) with initial metastases or bone marrow dissemination, as in this case, no cure could be achieved. In patients with disseminated MEM, new therapeutic approaches such as high-dose chemotherapy followed by stem cell rescue should be considered, similar to the current strategy in patients with stage VI neuroblastoma or soft tissue sarcoma.
journal_name
Pediatr Hematol Oncoljournal_title
Pediatric hematology and oncologyauthors
Müller HL,Marx A,Trusen M,Schneider P,Kühl Jdoi
10.1080/088800102753356149subject
Has Abstractpub_date
2002-01-01 00:00:00pages
9-17issue
1eissn
0888-0018issn
1521-0669journal_volume
19pub_type
杂志文章,评审abstract::Spontaneous remission/regression of cancer is defined as partial or complete disappearance of malignant disease temporarily or permanently in the absence of medical treatment. This event is named as spontaneous regression for solid tumors and spontaneous remission for leukemia. The authors report the case of a girl ag...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010801938132
更新日期:2008-04-01 00:00:00
abstract::Nesiritide is a recombinant formulation of B-type natriuretic peptide used most commonly in the treatment of adults with decompensated congestive heart failure. The physiologic effects of BNP include natriuresis, diuresis, and smooth muscle relaxation. These physiologic effects result in its beneficial therapeutic eff...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010590935248
更新日期:2005-06-01 00:00:00
abstract::To determine treatment outcome using ceftazidime-aminoglycosides in febrile neutropenic children with cancer, the authors conducted a prospective cohort study in 216 episodes. Early and complete responses to antibiotics were 108/216 (50.0%) and 133/216 (61.6%) episodes, respectively. Death, a modification of antibioti...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.1080/08880010701703636
更新日期:2007-12-01 00:00:00
abstract::The effectiveness of the different pharmacological agents and different doses of systemic corticosteroids was analyzed. A total of 1109 patients (median age 8 months; F/M: 2.3) with hemangioma, followed up in our unit for 23 years, were evaluated retrospectively. Forty-five of them received systemic corticosteroids. T...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800101750059855
更新日期:2001-01-01 00:00:00
abstract::The malignant cells of childhood acute lymphoblastic leukemia (ALL) do not form a homogenous entity but a collection of differently maturated blasts. The most immature leukemia cells may be more resistant to therapy than the bulk of more differentiated blasts. We studied 42 patients with childhood ALL treated accordin...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.3109/08880018.2013.859189
更新日期:2014-05-01 00:00:00
abstract::An 11-year-old girl with hereditary factor-VII deficiency and her family have been studied for factor VII coagulation activity (VII:C) and factor VII antigen level (VII:Ag). The proband had 11% VII:C, whereas her VII:Ag was at a level that corresponds to about 50% coagulation activity. Forty-two members of the proband...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018709141261
更新日期:1987-01-01 00:00:00
abstract::Thalassemic children are at a high risk of graft rejection in cord blood transplantation. To investigate this possible mechanism, the authors evaluated the effect of panel reactive antibody on the growth of CD34(+) cells in vitro. On semisolid medium, CD34(+) cells derived from cord blood were incubated with thalassem...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010902976098
更新日期:2009-07-01 00:00:00
abstract::In patients undergoing bone marrow transplantation cryptococcosis is rarely encountered. We report a fatal case of Cryptococcus meningitis in a 12-year-old girl with acute lymphoblastic leukemia (ALL) in second remission who had a transplant from a human leukocyte antigen (HLA)-identical unrelated bone marrow donor. T...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019709028778
更新日期:1997-09-01 00:00:00
abstract::Euthyroid sick syndrome is related to profound changes in thyroid metabolism induced by nonthyroidal diseases. To determine whether children with newly diagnosed Hodgkin disease might present thyroid abnormalities and to establish their predictive value, the authors performed regular thyroid function testing. Seven ch...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010151114886
更新日期:2001-04-01 00:00:00
abstract::Osteoporosis is common in patients with thalassemia major. A 16-year-old patient with thalassemia major was referred for evaluation of osteoporosis. The results of dual-energy X-ray absorptiometry in a patient with thalassemia major are presented. The patient underwent measurements of the spine in both AP and lateral ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010500278707
更新日期:2005-12-01 00:00:00
abstract::Since the mid-1960s intrathecal chemotherapy (methotrexate [MTX], cytarabine [Ara-C], or both, plus hydrocortisone) has constituted the standard approach to prophylaxis and treatment of central nervous system (CNS) leukemia and lymphoma. Intrathecal chemotherapy-related neurotoxicity has been described in a variable p...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019509029588
更新日期:1995-07-01 00:00:00
abstract::Bloodstream infections (BSI) represent one of the most serious complications in patients in the hematology-oncology unit. In this study, the prevalence, distribution, drug sensitivity profiles, and clinical outcome of BSI were analyzed in pediatric patients with hematological malignancies. Patients admitted to the ped...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2019.1667462
更新日期:2019-11-01 00:00:00
abstract::Beta-thalassemia major is a severe, transfusion-dependent anemia that also causes infertility due to iron deposition to endocrine organs after overtransfusion. Very few pregnancies have been reported among such patients after modern therapies. In this study, 32 women with thalassemia who were admitted to Ali Asghar Ch...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,多中心研究
doi:10.1080/08880010500313306
更新日期:2006-01-01 00:00:00
abstract:OBJECTIVE:Physiologic uptake of 18F-fluorodeoxyglucose (FDG) in brown adipose tissue (adipose tissue) of cancer patients may confound interpretation of positron emission tomography (PET) scans. Uptake in adipose tissue occurs in up to half of pediatric oncology patients undergoing PET scans, and is especially common in...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.1080/08880018.2017.1338806
更新日期:2017-04-01 00:00:00
abstract::A 7-year-old boy with prolonged and marked leukopenia diagnosed at 6 months of age is described. The polymorphonuclear cells presented no hypersegmented nuclei or concentrated nuclear chromatin, although vacuolated myeloid cells appeared in bone marrow smears. Neutrophils reversed in response to administration of G-CS...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800100276532
更新日期:2000-03-01 00:00:00
abstract::Endocrine system dysfunctions are the significant complications of excessive iron overload in beta thalassemia patients. The aim of this study was to evaluate the long-term effect of chelation with deferasirox on endocrine complications. The study group consisted of children with beta thalassemia who had been evaluate...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2020.1734124
更新日期:2020-09-01 00:00:00
abstract::Between 1973 and 1991, 10 patients with locally advanced [stages III and IV] nasopharyngeal carcinoma were treated at the Northern Israel Oncology Center. All patients were treated with wide-field irradiation to the primary tumor, including the base of skull, neck, and supraclavicular region. After 1984, 6 patients al...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019509029568
更新日期:1995-05-01 00:00:00
abstract::The authors report the results of 58 children with ALL in 2CR after related (n = 31) or unrelated (n = 27) AHSCT. Characteristics at diagnosis and initial and after relapse antileukemic treatment were similar in the related donor (RD) and the unrelated donor (UD) groups. Conditioning consisted of TBI/CY +/- VP-16 for ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010802016557
更新日期:2008-06-01 00:00:00
abstract::Neuroblastoma presenting with obstructive jaundice is a rare event. Management of this condition includes surgery, chemotherapy, radiotherapy, temporary cholecystostomy tube, endoscopic retrograde cholangiopancreatography (ERCP), and internal biliary drainage (IBD). We herein describe our experience with one infant af...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2014.981901
更新日期:2015-04-01 00:00:00
abstract::Primary immunodeficiency disorders (PID) are under-reported from the developing world. We present data regarding diagnosis and outcome from a hospital-based registry in India. Forty-seven patients fulfilled diagnostic criteria. Majority were males. Subgroups were disorders of immune dysregulation-29%, B&T-cell abnorma...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2013.805346
更新日期:2014-04-01 00:00:00
abstract::A single institution's experience of three patients with nephroblastoma extending via the inferior vena cava to the right heart is described. The case reports and the pertinent literature illustrate that preoperative failure to detect the intracardial extension increases the risk of right outflow obstruction and pulmo...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880019109028807
更新日期:1991-10-01 00:00:00
abstract::Medulloblastoma (MB) is a cancer of the cerebellum and the most common primary pediatric malignancy of the central nervous system. Classified as a primitive neural ectoderm tumor; it is thought to arise from granule cell precursors in the cerebellum. The standard of care consists of surgery, chemotherapy and age-depen...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880018.2012.698372
更新日期:2012-09-01 00:00:00
abstract::The purpose of this study was to evaluate the feasibility and tumor response of 3 cycles of sequential high-dose chemotherapy (HDCT) in advanced pediatric solid tumor patients. Medical records of 11 children who underwent 2 consequent courses of reduced conditioning HDCT followed by final HDCT with autologous HSC infu...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/14992020903352226
更新日期:2010-02-01 00:00:00
abstract::An episode of transient encephalopathy after the first course of intravenous high-dose methotrexate (HD-MTX; 1000 mg/m2) was observed in a 4-year-old girl with acute lymphoblastic leukemia. The neurological abnormalities took place 5 days after HD-MTX therapy. She experienced complex partial seizure and left hemipares...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019209018331
更新日期:1992-04-01 00:00:00
abstract::Autologous blood-derived stem cells were used for stem-cell rescue in a 5-year-old boy with chemotherapy-resistant B-Non-Hodgkin's lymphoma (B-NHL) involving bone marrow. The high dose chemoradiotherapy was carried out 5 months after initial diagnosis during partial remission. The preparative regimen consisted of 12 G...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019009033383
更新日期:1990-01-01 00:00:00
abstract::Endothelial protein C receptor (EPCR) is primarily localized on the endothelial cells of large blood vessels and is very low or absent in the microvascular endothelium of most tissues. EPCR augments the thrombin/thrombomodulin-dependent activation of protein C by 5- to 20-fold. EPCR appears to be physiologically signi...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010701198811
更新日期:2007-04-01 00:00:00
abstract::There are limited studies that have focused on the causes for hospitalization as an indicator of morbidity in patients with β-thalassemia major (BTM). A cross-sectional study was conducted to determine the main causes for hospitalization and death in hospitalized BTM patients in a referral hospital in Shiraz, southern...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2010.503336
更新日期:2011-03-01 00:00:00
abstract::The hematologic phenotype was characterized in heterozygotes for three of the most common beta-thalassemia mutations in the Greek population. The study included 17 carriers of beta++ IVS1-n6 (T-->C), 21 carriers of beta+ IVS1-n110 (G-->A), and 17 carriers of beta 0 CD39 (C-->T). The 55 beta-thalassemia heterozygotes w...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019409141689
更新日期:1994-09-01 00:00:00
abstract::The authors describe the relation between clamping time and blood volume collected, and two enrichment systems of CD34+ stem cells from umbilical cord blood, to determine an excellent recovery with high proliferate ability and bone marrow reconstitution. After an obstetrician-based cord blood collection, the purificat...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010252899406
更新日期:2002-06-01 00:00:00
abstract::A patient with well-differentiated monoblastic leukemia (ANLL FAB-M5b) is described in whom acute leukemia was diagnosed 25 months after having completed postoperative adjuvant chemotherapy for osteogenic sarcoma of the femur. All analyzed metaphases showed 48xy, dup 1(q12), +3, +9. ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018809031250
更新日期:1988-01-01 00:00:00