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2.
Clin Oncol (R Coll Radiol) ; 31(7): 471-478, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31000431

RESUMO

AIMS: To investigate the protocol efficacy and prognostic factors for paediatric hepatoblastoma in a multidisciplinary model in our centre. MATERIALS AND METHODS: Consecutive hepatoblastoma patients (<18 years old) treated at Shanghai Children's Medical Center in China from August 2011 to October 2017 were analysed retrospectively for clinical features, chemotherapy courses, surgical treatment and outcomes. RESULTS: One hundred and four cases of paediatric hepatoblastoma (64 males, 40 females; median age at diagnosis 1.64 years) had a median follow-up of 30.68 months (range 8.3-73.3 months). First complete remission was achieved in 95 cases, 85 of which achieved continuous complete remission. Another three cases were lost to follow-up after a median of 24.73 months in complete remission. Seven cases relapsed later, with two achieving a second complete remission and four deaths. Nine cases did not achieve complete remission and five of them died. In general, the 5-year overall survival rate and 5-year event-free survival (EFS) rate were 86.3 ± 5.0% and 81.8 ± 4.3%, respectively. Thirty-two cases were classified as standard risk and 72 as high risk with 5-year EFS of 96.8 ± 3.2% and 75.7 ± 5.7% (P = 0.029) and 5-year overall survival of 100% and 80.5 ± 7.0%, respectively. The mean platelet count (P = 0.0036), lactate dehydrogenase (P = 0.0443) and ferritin level (P = 0.0006) at diagnosis were much higher in the high-risk group than in the standard-risk group. Univariate analysis showed that patients <5 years of age (P = 0.018), with higher α-fetoprotein (AFP) level (>100 ng/ml, P = 0.008), without metastases at diagnosis (P = 0.001) and postoperative AFP recovery after no more than three chemocycles (P = 0.014) had better overall survival. In addition, the above factors, except metastases at diagnosis and risk group, were associated with prognosis in the multivariate analysis. CONCLUSIONS: The result of this protocol had similar overall survival and EFS rates compared with those in developed countries. Normal postoperative AFP levels after three chemocycles has prognostic value.

4.
J Intellect Disabil Res ; 63(8): 936-946, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30775821

RESUMO

BACKGROUND: While accelerated ageing is recognised among individuals with Down syndrome (DS), the trajectory of their bone health across adulthood remains poorly understood. METHODS: This study aimed to determine the age-related loss of bone mineral density (BMD) of the lumbar spine in 128 adults with DS aged 18 to 54 years compared with 723 counterparts without DS. RESULTS: Men and women with DS had lower level of BMD than counterparts without DS across age groups. Magnitude of decrement in BMD as reflected in the z-scores was similar between younger and older men with DS. Older women with DS, on the contrary, showed greater decrement in older ages especially in their fourth decade of life. Osteopenia and osteoporosis as defined using age-specific and gender-specific T-scores affected greater number of men with DS (38% and 25%) than women (17% and 17%) aged 40-49 years. CONCLUSIONS: Findings supported adults with DS, especially men, to have early bone mineral testing.

5.
Zhonghua Xue Ye Xue Za Zhi ; 39(9): 717-723, 2018 Sep 14.
Artigo em Chinês | MEDLINE | ID: mdl-30369180

RESUMO

Objective: To analyze the clinical characteristics and long-term outcomes with multicenter study for acute lymphoblastic leukemia (ALL) in children over 10 years old and adolescents. Method: Newly diagnosed ALL patients aged from 10 to 18 years old in three hospitals were included in the study from May 1(st) 2005 to April 30(th) 2015. They were received ALL-2005/2009 protocol following up to December 31(st) 2016. The clinical characteristics, outcomes and the prognostic analysis were evaluated between the two protocols. Results: Totally, 237 patients were involved in the study, 76 cases for ALL-2005 and 161 cases for ALL-2009 protocol. Complete remission (CR) after induction therapy was 94.5%. 64 (28.6%) patients relapsed with a median time of 14.5 months and 70 (29.5%) patients passed away during the following time. In long-term follow-up, the 5-year event-free survival (EFS) and 5-year overall survival (OS) of ALL patients were (63.1±3.3)% and (68.4±3.2)%. The 7-year EFS and OS were (61.0±3.5)% and (67.6±3.3)%.The 5-year EFS of intermediate risk group in ALL-2005 and ALL-2009 protocol were (73.6±6.1)% and (71.7±4.3)% with no difference (χ(2)=0.064, P=0.801). The 5-year EFS of high risk group in two protocols were (27.6±9.6)% and (33.9±9.3)%, showing no significant difference (χ(2)=0.296, P=0.586). Five years relapsed rate of two protocols were (33.8±5.7)% and (32.6±4.1)% with no difference (χ(2)=0.055, P=0.815). The mortalities were 36.8% and 29.8% separately (χ(2)=2.869, P=0.090). Univariate analysis indicated that age, male, risk, BCR/ABL translocation/t(9;22) and resistant to induction were risk prognostic factors in long-term survival (χ(2)=4.764, 4.796, 46.410, 9.560, 25.450; P=0.029, 0.029, <0.001, 0.049, <0.001). Cox multivariate analysis showed male, risk and resistant to induction were independent risk prognostic factors (RR=1.790, 2.727, 2.719; P=0.021, 0.000, 0.012). Conclusion: Protocol ALL-2009 enhanced the chemotherapy intensity in intermediate risk group with no benefit of survival. BCR-ABL fusion or t(9;22) translocation was still the risk factor of prognosis. TKI inhibitor used in these patients could improve survival. EFS rate was increased a little and death rate was decreased in ALL-2009 protocol with no significant lower relapsed rate comparing with ALL-2005 protocol.


Assuntos
Leucemia-Linfoma Linfoblástico de Células Precursoras , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica , Criança , Intervalo Livre de Doença , Humanos , Masculino , Prognóstico , Indução de Remissão , Fatores de Risco , Translocação Genética
7.
Zhonghua Er Ke Za Zhi ; 56(7): 511-517, 2018 Jul 02.
Artigo em Chinês | MEDLINE | ID: mdl-29996184

RESUMO

Objective: To evaluate the long-term efficacy and prognostic factors of childhood acute lymphoblastic leukemia (ALL) enrolled in Shanghai Children's Medical Center-Acute Lymphoblastic Leukemia-2005(SCMC-ALL-2005) multicenter study. Methods: Between May 2005 and December 2014, 1 497 newly diagnosed ALL patients were enrolled and treated in 5 hospitals of SCMC-ALL-2005 study group, using risk-stratified SCMC-ALL-2005 protocol. Risk group classification and treatment intensity were based on clinical features, genetic abnormalities, early response to treatment and levels of minimal residual disease (MRD). Kaplan-Meier method was used to generate overall survival (OS) and event-free survival(EFS) curves. Cox proportional hazards models were used for multivariate analyses. Results: The patients were followed up to December 31, 2016, the median follow-up time was 69 months (24-141 months). The 5-year and 10-year OS rates were (80.0±1.0)% and (76.0±2.0)%. The 5-year and 10-year EFS rates were (69.0±1.0)% and (66.0±2.0)%. The 5-year and 10-year relapse rates were (23.0±1.0)% and (25.0±2.0)%. The 5-year OS and EFS for low risk (LR), intermediate risk (IR) and high risk (HR) were (91.1±1.4)% and (83.3±1.8)%, (79.2±1.5)% and (68.9±1.7)%, (52.9±4.4)% and (30.0±3.8)%, respectively. MRD negative status (<0.01%) on day 55 was seen in 792 patients (82.8%) and positive MRD on day 55 was associated with poor prognosis (OR=1.9, 95%CI: 1.3-2.7, P=0.001). Twenty-four HR patients received allogeneic hematopoietic stem cell transplantation and 17(70.8%) of them were alive and in remission. A total of 164 severe adverse events occurred, 46 of them died, treatment-related mortality was 3.1%. Conclusions: In this large sample research, the overall outcome for multi-center SCMC-ALL-2005 study was favorable. This helps to promote the standardized treatment of childhood ALL to the whole country. MRD results on day 55 of induction therapy have important prognostic and therapeutic implications.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica , Transplante de Células-Tronco Hematopoéticas , Leucemia-Linfoma Linfoblástico de Células Precursoras , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , China , Intervalo Livre de Doença , Humanos , Neoplasia Residual , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Prognóstico , Indução de Remissão , Resultado do Tratamento
8.
Appl Radiat Isot ; 132: 212-221, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29239757

RESUMO

A white neutron beam line using back-streaming neutrons from the spallation target is under construction at China Spallation Neutron Source (CSNS). Different spectrometers, to be installed in the so-called Back-n beam line for nuclear data measurements, are also being developed in phases. The physical design of the beam line is carried out with the help of a complicated collimation system and a sophisticated neutron dump, taking the overview of the neutron beam characteristics into account. This includes energy spectrum, flux and time structure, the optimizations of neutron beam spots and in-hall background. The wide neutron energy range of 1eV-100MeV is excellent for supporting different applications, especially nuclear data measurements. At Endstation#2, which is about 80m away from the target, the main properties of the beam line include neutron flux of 106n/cm2/s, time resolution of a few per mille over nearly the entire energy range, and in-hall background of about 0.01/cm2/s for both neutron and gamma. With its first commission in late 2017, Back-n will not only be the first high-performance white neutron source in China, but also one of the best white neutron sources in the world.

11.
Zhonghua Er Ke Za Zhi ; 55(10): 721-723, 2017 Oct 02.
Artigo em Chinês | MEDLINE | ID: mdl-29050106
13.
Zhonghua Er Ke Za Zhi ; 55(10): 743-747, 2017 Oct 02.
Artigo em Chinês | MEDLINE | ID: mdl-29050111

RESUMO

Objective: To investigate the long-term efficacy and prognostic factors of pediatric relapsed Wilms tumor (WT) after retreatment. Method: Sixteen children in Shanghai Children's Medical Center with relapsed Wilms tumor were enrolled consecutively in this study between April 2006 and June 2016. All patients were diagnosed according to pathology, imaging and medical and surgical oncologist's assistance. Relapse treatment included surgical excision, chemotherapy and selective radiation therapy. The clinical features, long-term outcomes and prognostic factors of patients were analyzed retrospectively.Survival data were analyzed by Kaplan-Meier.Log-Rank analysis was used for univariate analysis. Result: One case was excluded because of giving up the therapy even though no disease progress was identified. A total of 15 cases (5 males and 10 females) were included in this study. The median age at diagnosis was 3.8 years (range 0.5-9.1 years). The tumor staging at diagnosis included one case of stageⅠ, 7 cases of stageⅡand 7 cases of stage Ⅲ. Among cases of stage Ⅲ, 6 cases had radiation therapy history. The pathology of all patients' recurrent tumor was favorable histology (FH). The median follow-up time was 34.6 months (range 12.5-132.7 months) until March 21, 2017. The time from initial diagnosis to relapse was 7.9 months (range 3.1-17.9 months). Four cases experienced local recurrence, 9 cases relapsed with metastases (6 cases in lungs, 2 in livers, 1 in mediastinum) and 2 cases relapsed in both local site and with metastases. Except to 2 cases received irregular retreatment, 13 cases received regimen I (doxorubicin, vincristine, epoposide and cyclophosphamide for 25 weeks) as relapsed chemotherapy. Five cases received autologous bone marrow transplantation (ABMT). Until the last follow-up, 8 cases achieved continuous complete remission (range 6.7-104.3 months), 3 cases had relapse again or progressing and 4 cases died. The estimated 5-year overall survival (OS) rate and event free survival (EFS) rate were (70±15)% and (52±15)%. According to whether received ABMT or not, the 5-year EFS rate were 51% and 53%. According to whether relapsed within 6 months after diagnosis or not, the 5-year EFS rate were 38% and 56% respectively. Conclusion: The 5-year EFS rate of pediatric relapsed FH WT have reached above 50% by multi-disciplinary treatment in our experience and we encourage patients and doctors to receive retreatment.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Tumor de Wilms/tratamento farmacológico , Transplante de Medula Óssea , Criança , Pré-Escolar , China , Ciclofosfamida , Intervalo Livre de Doença , Doxorrubicina , Feminino , Seguimentos , Humanos , Lactente , Masculino , Recidiva Local de Neoplasia , Estadiamento de Neoplasias , Indução de Remissão , Retratamento , Estudos Retrospectivos , Taxa de Sobrevida , Vincristina
14.
Zhonghua Er Ke Za Zhi ; 55(10): 754-759, 2017 Oct 02.
Artigo em Chinês | MEDLINE | ID: mdl-29050113

RESUMO

Objective: To assess the clinical features and long-term outcomes of neuroblastoma (NB) in children less than 18 months of age, so as to provide evidence for further improvement of treatment. Method: Clinical data(sex, age, stage, risk group, treatment response, follow-up, etc.) of 155 NB patients under age of 18 months from June 2000 to December 2015 in Shanghai Children's Medical Center were analyzed retrospectively. The clinical features were summarized and the long-term follow-up results were evaluated. The overall survival (OS) and event-free survival (EFS) were analyzed by using Kaplan-Meier method. Factors including age, stage, risk group, bone marrow and bone metastasis, N-MYC status and dehydrogenase(LDH) level were analyzed by Log-Rank test. Result: Totally 155 eligible patients (96 males, 59 females) were included. The median age of disease onset was 7 months (11 days to 18 months). There were 31 cases of stage 1, 19 cases of stage 2, 45 cases of stage 3, 38 cases of stage 4 and 21 cases of stage 4S. The median follow-up time was 36 months (range 4 to 189 months), the 3-year and 5-year EFS rate were 89.6% and 85.2% respectively and the 3-year and 5-year OS rate were 96.2% and 94.1%, respectively. A total of 15 recurrent or progressed cases were observed. The median time to first recurrence was 11 months (range 3 to 39 months), 6 cases eventually died. Second malignancy occurred in one patient. The patients who had relapsed disease within 12 months from initial diagnosis have much lower 3-year OS rate than those in whom the disease recurred 12 months later (25.7% vs. 83.3%, P=0.020). Although the number of chemotherapy courses in median-high risk group reduced from 8.6 courses to 7.5 courses after the revision in 2008, the survival rate showed no significant difference between before and after (5-year EFS 74.4% vs. 84.3%, 5-year OS 89.0% vs. 92.9%, both P>0.05). In patients with stage 1 and stage 2, the 3-year EFS of 34 cases with surgery alone and 16 cases accepted chemotherapy were both 100%. Age at diagnosis, stage, risk group, MYCN status, LDH level, bone marrow involvement and bone infiltration had significant impacts on prognosis(all P<0.05). Conclusion: Satisfactory outcomes could be achieved in neuroblastoma in children aged within 18 months; the prognosis was better in children at age less than 12 months compared with 12-18 months. MYCN amplification, LDH more than 5 times upper limit of normal range, bone marrow and bone infiltration were associated with worse prognosis.Excellent survival rates could be achieved in children with stage 1 and 2 disease within 18 month's old accepted surgery alone, chemotherapy or radiotherapy could be avoided in these patients so as to reduce long-term adverse reactions.


Assuntos
Recidiva Local de Neoplasia , Estadiamento de Neoplasias , Neuroblastoma , Medula Óssea , China , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Neuroblastoma/patologia , Neuroblastoma/terapia , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida
15.
Zhonghua Er Ke Za Zhi ; 55(5): 364-368, 2017 May 04.
Artigo em Chinês | MEDLINE | ID: mdl-28482388

RESUMO

Objective: To investigate the efficacy and the prognostic factors in pediatric hepatoblastoma according to the standard diagnostic and therapeutic regimen. Method: Eighty-four consecutive patients were enrolled in this study between June 2000 and June 2015. Diagnosis and staging was decided by the multi-disciplinary team including oncologists, surgeons, pathologists and sub-specialized radiologists refering to protocol of Children's Oncology Group(COG) and International Society of Pediatric Oncology Liver Tumor Study Group (SIOPEL) in a case observational study. Univariate analysis was tested by the log-rank and multivariate analysis by COX regression. All consecutive cases were divided into low risk group and high risk group according to grouping criteria. Complete remission was defined as both imaging negative and α fetoprotein (AFP) normalization. Retrospective analysis was performed in clinical features, long-term outcomes and prognostic factors. Result: Ten patients were excluded because of giving up after less than or equal to three cycles of treatment. A total of 74 cases were included in this study; 45 males and 29 females. The median age at diagnosis was 1.7 years(range 0.2-14.8 years). Untill August 30, 2016, the median follow-up time was 24.2 months (range 4.1-135.3 months); 59 cases achieved complete remission.The estimated five years overall survival (OS) and event free survival(EFS) were 90%(68/74)and 72%(58/74). AFP could be normalized after 5 circles of treatment or 2 circles of postoperation.In univariate analysis , the five years OS and EFS in low risk group were both 100%(18/18), and those in high risk group were 88%(50/56)and 68%(40/56), respectively. The five years OS rates were 75%(15/19) and 95%(53/55) in patients with or without distant metastasis (P=0.016). After 3 cycles of chemotherapy post tumor resection, we divided these patients into 2 groups according to AFP recover or not, the five years OS were 100%(43/43)and 81%(22/26), respectively (P=0.011). Conclusion: The result of this protocol is reasonable when comparing with other worldwide research. Except for staging, metastasis, pathological subtypes, postoperative AFP recover or not is a prognostic factor after 3 cycles of chemotherapy.


Assuntos
Hepatoblastoma/terapia , Neoplasias Hepáticas/terapia , Adolescente , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Lactente , Masculino , Análise Multivariada , Indução de Remissão , Estudos Retrospectivos , Taxa de Sobrevida , alfa-Fetoproteínas
16.
Eur Rev Med Pharmacol Sci ; 21(7): 1532-1540, 2017 04.
Artigo em Inglês | MEDLINE | ID: mdl-28429353

RESUMO

OBJECTIVE: The aim of this study was to investigate the roles of the Nrf2/HO-1 pathway in the responses to the oxidative stress created by ischemia-reperfusion brain injury in rats. MATERIALS AND METHODS: 54 healthy, adult, male SD rats were included in the study. Eighteen (18) rats were placed in the sham group. The ischemia-reperfusion model was created in the other 36 rats, among which 18 received injections of Nrf2 agonist before the surgery. The suture method was used to create artery occlusions in the right brain of the rats; and reperfusion was done after 90-minute ischemia (MCAO); while no suture was inserted in the sham group. At 3, 6, 12, 24, 48 and 72 hours after the modeling, their neurological functions were evaluated. Also, at different time points, rats were decapitated, and their fresh brain tissues were used to detect the infarct volume percentages by TTC staining and the brain water contents by the dry-wet weight method. The SOD contents in the brain tissue were measured by Xanthine oxidase assay. RT-PCR was used to detect the mRNA expression of HO-1 in the brain tissues, and western blot method was used to detect the expression level of HO-1 and Nrf-2. RESULTS: The rats in the sham group had no obvious neurological defects; while those in the MCAO group showed significant neurological defects at all time points. The MCAO group had higher neurological evaluation scores than the sham group. TTC staining showed that infarct in the MCAO group kept increasing over time and peaked at 24h. Measurements of SOD found that the sham group had the highest SOD among the three groups, and showed no significant fluctuation over time. The MCAO group had much lower SOD activities than the sham group at all the time points. The higher the level of HO-1mRNA and protein expression in the brain tissue of rats in each group, the higher the degree of brain injury, but the lower the level of Nrf2 protein expression and the lower degree of brain injury. Nrf2 agonist markedly improved all these indicators in the rats which underwent the MCAO surgery. CONCLUSIONS: The expression of HO-1 after ischemia-reperfusion brain injury may contribute to the increased infarct volume. Activation of Nrf2 could improve the prognosis of ischemia-reperfusion brain injury.


Assuntos
Fator 2 Relacionado a NF-E2 , Estresse Oxidativo/efeitos dos fármacos , Animais , Lesões Encefálicas/tratamento farmacológico , Isquemia Encefálica/tratamento farmacológico , Masculino , Fármacos Neuroprotetores/farmacologia , Ratos , Ratos Sprague-Dawley , Traumatismo por Reperfusão/tratamento farmacológico
17.
Zhonghua Nei Ke Za Zhi ; 56(2): 127-129, 2017 Feb 01.
Artigo em Chinês | MEDLINE | ID: mdl-28162183

RESUMO

The aim of the study was to explore the effect and its clinical relevance of short-term intensive insulin treatment on plasma concentrations of lipoprotein-associated phospholipase A(2) (Lp-PLA(2)) and secretory phospholipase A(2) (sPLA(2)) in newly diagnosed type 2 diabetes mellitus (T2DM). Ninety newly diagnosed T2DM patients were recruited and received continuous subcutaneous insulin infusion (CSII) for about 2 weeks. After CSII, sPLA(2) levels [173.78 (80.95, 278.09) µg/L] were significantly decreased compared with the levels before [219.33 (130.03, 337.30) µg/L], P<0.01, while no statistic significant changes could be viewed in Lp-PLA(2) levels. Correlation analysis showed that the changes of Lp-PLA(2) and sPLA(2) were both positively correlated with the changes of homeostasis model assessment of insulin resistance(HOMA-IR)after CSII (r=0.537, 0.493 respectively, all P<0.05). The Lp-PLA(2) and sPLA(2) level reduction after CSII might help to protect the patients from diabetic macroangiopathy. Trial registration Chinese Clinical Trial Registry, ChiCTR-TRC-10001618.


Assuntos
1-Alquil-2-acetilglicerofosfocolina Esterase/sangue , 1-Alquil-2-acetilglicerofosfocolina Esterase/efeitos dos fármacos , Diabetes Mellitus Tipo 2/tratamento farmacológico , Hipoglicemiantes/uso terapêutico , 1-Alquil-2-acetilglicerofosfocolina Esterase/metabolismo , Adulto , Glicemia , China , Diabetes Mellitus Tipo 2/sangue , Diabetes Mellitus Tipo 2/diagnóstico , Quimioterapia Combinada , Feminino , Humanos , Hipoglicemiantes/farmacologia , Insulina/administração & dosagem , Insulina/farmacologia , Resistência à Insulina , Masculino , Pessoa de Meia-Idade
19.
Leukemia ; 31(5): 1079-1086, 2017 05.
Artigo em Inglês | MEDLINE | ID: mdl-27807368

RESUMO

The origin of cancers is associated with etiology as well as therapeutics. Several studies reveal that malignancies in children can originate in utero. However, a diagnostic approach to distinguish between cancers initiated pre- or postnatally is absent. Here we identified a transcriptional factor FEV (fifth Ewing variant) that was expressed in fetal hematopoietic cells and became silent after birth. We characterized that FEV was essential for the self-renewal of hematopoietic stem cells (HSCs). We next found that FEV was expressed in most infant leukemia samples, but seldom in adult samples, in accord with the known prenatal origins of the former. We further determined the majority of pediatric acute lymphoid leukemia (ALL) and acute myeloid leukemia (AML) were FEV positive. Moreover, FEV knockdown markedly impaired the leukemia-propagating ability of leukemic stem cells. We therefore identified FEV is unique to fetal HSCs and stably expressed in leukemic cells of prenatal origin. It may also provide a tractable therapeutic target.


Assuntos
Proteínas de Ligação a DNA/análise , Doenças Fetais/diagnóstico , Células-Tronco Hematopoéticas/metabolismo , Leucemia/etiologia , Proteínas Nucleares/análise , Animais , Células Cultivadas , Proteínas de Ligação a DNA/biossíntese , Feminino , Doenças Fetais/metabolismo , Expressão Gênica , Xenoenxertos , Humanos , Leucemia/diagnóstico , Leucemia/metabolismo , Leucemia Mieloide Aguda , Camundongos , Proteínas Nucleares/biossíntese , Leucemia-Linfoma Linfoblástico de Células Precursoras , Gravidez , Fatores de Transcrição
20.
Zhonghua Er Ke Za Zhi ; 54(12): 917-922, 2016 Dec 02.
Artigo em Chinês | MEDLINE | ID: mdl-27938592

RESUMO

Objective: To analyze the clinical characteristics and prognosis of rare soft tissue sarcomas. Method: Clinical data of 51 patients with rare soft tissue sarcomas including fibrosarcoma, synovial sarcoma, extrarenal rhabdoid tumor, alveolar soft part sarcoma, desmoplastic small round cell tumor and undifferentiated sarcoma in children and adolescents, diagnosed at Shanghai Children's Medical Center from June 1998 to December 2013, were retrospectively analyzed. All types were treated with the same strategy and chemotherapy regimens. Their clinical features, treatment and prognosis were discussed. Result: Seventeen patients with fibrosarcoma, 10 with synovial sarcoma, 9 with extrarenal rhabdoid tumor, 6 with alveolar soft part sarcoma, 3 with desmoplastic small round cell tumor and 6 with undifferentiated sarcoma were included. The mean age at initial diagnosis was 5 years(range from 1 month to 13.5 years). The most common primary site of tumors was limbs, followed by the thoracic and abdominal cavity, accounting for 41% and 24% respectively. Twelve cases presented distant tissue or organ involvement in which bone metastases occupied the first place. Seven cases(accounting for 14%)were at stage Ⅰ, 13 cases were at stage Ⅱ(accounting for 25%), 19 cases were at stage Ⅲ(accounting for 37%) and 12 cases were at stage Ⅳ(accounting for 24%). The median follow-up period was 36 months(range from 1 month to 123 months). Forty-four patients achieved complete remission and 3 patients achieved partial remission after initial treatment, the overall response rate was 92%. Subsequent follow-up showed 29 patients remained relapse-free while 13 patients had relapsed disease. Overall survival and event-free survival at 2 years were 88% and 57%.Postoperative surgical staging was the main prognostic factors. Patients with stage Ⅲ+ Ⅳ had poorer results than those with Ⅰ+ Ⅱ (χ2=4.909, P=0.027). Conclusion: These 6 types of soft tissue sarcomas are rare in children and adolescents. The tumor can occur anywhere in the body but commonly presents in the extremities. Complete resection of tumor remains the most important modality of treatment and is directly related to prognosis. Neoadjuvant chemotherapy helps improve the resection rate of some unresectable tumors at diagnosis. Radiation therapy is primarily adopted for focal tumor control.


Assuntos
Neoplasias Ósseas/secundário , Sarcoma/patologia , Sarcoma/terapia , Neoplasias de Tecidos Moles/patologia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/patologia , Neoplasias Ósseas/terapia , Criança , China/epidemiologia , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Extremidade Inferior , Masculino , Recidiva Local de Neoplasia , Estadiamento de Neoplasias , Período Pós-Operatório , Prognóstico , Indução de Remissão , Estudos Retrospectivos , Neoplasias de Tecidos Moles/terapia , Taxa de Sobrevida
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