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1.
Medicine (Baltimore) ; 101(3): e28539, 2022 Jan 21.
Artigo em Inglês | MEDLINE | ID: mdl-35060511

RESUMO

ABSTRACT: Primary splenic cancers represent a small number of cancer cases and studies on its clinicopathological features and outcomes are limited. Splenic lymphomas and primary splenic angiosarcoma (PSA) are the 2 most common histological types of splenic cancers. This population-based study aimed to investigate the clinical characteristics and survival outcomes of patients with splenic lymphomas or PSA.Patients diagnosed with splenic lymphomas or PSA between 2000 and 2015 were identified from the Surveillance Epidemiology and End Results database of the National Cancer Institutes. Overall survival (OS) and cancer-specific survival (CSS) rates were calculated using the Kaplan-Meier method. A Cox proportional hazard models were used to identify independent predictors of cancer-specific mortality.A total of 700 patients with splenic lymphoma and 48 patients with PSA were included in this study. The median age of patients with splenic lymphoma was 65 years and 57 years for patients with PSA. For patients with splenic lymphoma, the most prevalent histological subtypes were splenic marginal zone lymphoma and diffuse large B-cell lymphoma. A total of 52.6% of the cases had stage IV disease based on the Ann Arbor staging system. Five-year OS and CSS were 76.9% and 83.4%, respectively. Multivariate analysis revealed that independent predictors of splenic lymphoma CSS included race, stage, chemotherapy, and histological subtype. However, a much shorter OS time was seen in the PSA cohort which had a 5-year OS of 11.8%, a median OS of 10.0 months and the 5-year CSS of 12.4%. Chemotherapy was correlated with better outcomes in patients with PSA. However, the survival benefits of surgery for splenic cancer were not statistically significant in our study.The current study is the largest cohort of primary splenic cancer presented in literature based on the Surveillance Epidemiology and End Results database and our large series describe the characteristics and survival outcomes of such rare diseases which may provide reliable information for further studies and clinicians.


Assuntos
Hemangiossarcoma/mortalidade , Hemangiossarcoma/terapia , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Hemangiossarcoma/epidemiologia , Hemangiossarcoma/patologia , Humanos , Linfoma Difuso de Grandes Células B/epidemiologia , Linfoma Difuso de Grandes Células B/patologia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prognóstico , Programa de SEER , Neoplasias Esplênicas/patologia , Taxa de Sobrevida
2.
Cancer Med ; 10(20): 7060-7070, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34547191

RESUMO

BACKGROUND: Although primary splenic lymphoma (PSL) is rare, it ranks first among splenic primary malignant cancers, and the incidence of lymphoma of spleen has gradually increased in recent years. However, the efficacy of surgery for PSL has not been clinically verified by large sample data, which has affected the formulation of relevant guidelines. AIM: To assess whether surgery can enhance the prognosis PSL patients. METHODS: Extracted the data of patients with PSL from The Surveillance, Epidemiology, and End Results (SEER) database, and divided the patients into surgery and non-surgery group. Kaplan-Meier curves and log-rank tests were used to compare the overall survival (OS) and cancer-specific survival (CSS). The propensity score matching (PSM) was used to match the data, then compared the OS and CSS again. The COX proportional hazard regression model was used for univariate and multivariate analysis. Finally, we performed subgroup analysis in different Ahmann stages. RESULTS: A sum of 2207 patients with PSL were enrolled, of which 1062 (48.1%) patients received surgery, and 1145 (51.9%) patients did not undergo surgery. Overall, patients in the surgery group had better OS and CSS. After the propensity scores matching, surgery was not statistically significant in OS and CSS. In the subgroup analysis, surgery was a protective factor for the OS and CSS in Ahmann I/II. However, surgery was no statistical significance in OS and CSS in Ahmann III. In patients with Ahmann Ⅰ/Ⅱ SMZL, surgery was a protective factor for OS and CSS. In patients with Ahmann Ⅲ SMZL, surgery was also statistically significant of OS and CSS. CONCLUSIONS: Surgery can significantly improve the prognosis of patients with Ahmann Ⅰ/Ⅱ primary splenic lymphoma, but there was no survival difference in the Ahmann Ⅲ patients with or without surgery. For patients with SMZL, surgery was effective for improving OS and CSS.


Assuntos
Linfoma/cirurgia , Neoplasias Esplênicas/cirurgia , Idoso , Intervalo Livre de Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Modelos Lineares , Linfoma/mortalidade , Linfoma/patologia , Masculino , Estado Civil , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prognóstico , Pontuação de Propensão , Programa de SEER , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/patologia
3.
Clin Lymphoma Myeloma Leuk ; 21(2): 106-112.e1, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33160933

RESUMO

BACKGROUND: Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma accounting for less than 1% of non-Hodgkin lymphomas. It is generally associated with poor prognosis. PATIENTS AND METHODS: We performed a cohort study of patients with HSTCL treated at the Mayo Clinic between 1996 and 2020 exploring the clinical characteristics and therapeutic outcomes. RESULTS: Twenty-two cases of HSTCL were identified with a median (range) age at diagnosis of 45.5 (15.5-80.6) years and a male predominance (15/22, 68.2%). Clinical characteristics include massive splenomegaly in 16 patients (73%), hepatic involvement in 13 (59%), and chronic immunosuppressed state in 8 (36%). Phenotypically, lymphoma cells had gamma/delta T-cell receptor expression in 18 (82%) and alpha/beta in 4 patients. Cytogenetic abnormalities included isochromosome 7q (i7q) in 8 (62%) of 13 and trisomy 8 in 4 (44%) of 9. The median (range) follow-up of surviving patients was 33 (2.5-137) months. The median progression-free and overall survival were 9.5 months (95% CI, 1.8, 16.3) and 12.4 months (95% CI, 4.9, 18.5), respectively. Long-term survival was seen in 4 (18%) of 22 patients, with survival of 55, 74, 95, and 137 months. Moreover, 3 of 4 long-term survivors had splenectomy as part of initial treatment, and 2 of 4 long-term survivors received an allogeneic hematopoietic cell transplant (allo-HCT). CONCLUSION: Liver involvement and chronic immunosuppression were associated with shorter survival. Although splenectomy and allo-HCT have anecdotal benefit in the literature, our data do not show a statistically significant benefit of splenectomy and/or allo-HCT, likely as a result of our small sample size.


Assuntos
Transplante de Células-Tronco Hematopoéticas/estatística & dados numéricos , Neoplasias Hepáticas/mortalidade , Linfoma de Células T Periférico/mortalidade , Esplenectomia/estatística & dados numéricos , Neoplasias Esplênicas/mortalidade , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Aberrações Cromossômicas , Feminino , Seguimentos , Humanos , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/genética , Neoplasias Hepáticas/terapia , Linfoma de Células T Periférico/diagnóstico , Linfoma de Células T Periférico/genética , Linfoma de Células T Periférico/terapia , Masculino , Pessoa de Meia-Idade , Intervalo Livre de Progressão , Estudos Retrospectivos , Neoplasias Esplênicas/diagnóstico , Neoplasias Esplênicas/genética , Neoplasias Esplênicas/terapia , Transplante Homólogo , Adulto Jovem
4.
Hematology ; 24(1): 378-386, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30885066

RESUMO

OBJECTIVES: To examine the use of splenectomy, chemotherapy, and subsequent overall survival (OS) in contemporary patients with splenic lymphomas. METHODS: We analyzed records of 6450 patients with various splenic lymphomas recorded in the National Cancer Data Base (2004-2013). Survival was compared using Mantel-Byer test to account for guarantee-time bias, stratified by age, sex, comorbidities, and lymphoma stage. RESULTS: Splenectomy rate was overall 58%, and varied from 49% in splenic marginal zone (SMZL) to 77% in follicular lymphoma (FL). It significantly decreased across all histologies over time (overall from 69% in 2004, to 44% in 2013). Thirty-day mortality after splenectomy was 4%. Chemotherapy use varied from 40% in FL to 76% in diffuse large B-cell lymphoma (DLBCL), but increased significantly only for SMZL and T-cell lymphomas over time. Overall, 57% of splenectomies were performed as diagnostic procedures, which was significantly less common in academic hospitals (p < 0.0001). Following a diagnostic splenectomy, chemotherapy was not administered to 29% of patients with DLBCL, 49% with mantle cell, and 42% with T-cell lymphomas. Median OS ranged from 12.4 years for FL to 1.0 year for T-cell lymphomas. We found no association between performance of splenectomy and OS across all histologies. Patients with DLBCL who did not receive chemotherapy after a diagnostic splenectomy had significantly worse OS (p = 0.001). The association between post-splenectomy chemotherapy and OS was not observed in FL or SMZL. CONCLUSION: many splenic lymphomas may be treated without surgery, but a high proportion of diagnostic splenectomies indicates an ongoing need for less invasive diagnostic modalities.


Assuntos
Bases de Dados Factuais , Linfoma de Zona Marginal Tipo Células B , Linfoma Difuso de Grandes Células B , Esplenectomia , Neoplasias Esplênicas , Idoso , Intervalo Livre de Doença , Feminino , Humanos , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/mortalidade , Linfoma de Zona Marginal Tipo Células B/cirurgia , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/mortalidade , Linfoma Difuso de Grandes Células B/cirurgia , Masculino , Pessoa de Meia-Idade , Neoplasias Esplênicas/diagnóstico , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/cirurgia , Taxa de Sobrevida , Estados Unidos
5.
Cell Physiol Biochem ; 49(5): 1959-1969, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30235449

RESUMO

BACKGROUND/AIMS: Primary splenic angiosarcoma is an aggressive malignancy originating from endothelial cells with a particularly poor outcome despite radical therapy. Owing to its extremely low incidence, available data for splenic angiosarcoma are limited. The present study aimed to address this limitation by presenting a thorough retrospective analysis of Chinese primary splenic angiosarcoma patients over a 53-year period (1963-2016). METHODS: To determine the characteristics of Chinese primary splenic angiosarcoma and identify factors that impact the outcomes of this histology, we retrospectively retrieved reports of 110 Chinese primary splenic angiosarcoma cases published between 1963-2012. RESULTS: In total, 61 males and 49 females diagnosed with primary splenic angiosarcoma were included in the present study. The median age at diagnosis was 50 years (range 2.5-76 years). Of these patients, 25.5% had received prior radiotherapy. The rate of splenic rupture was 59.11%. The 1-year overall survival rate was 19.1% with a median overall survival time of 8.1 months. Age, gender, and radiation history showed no correlation with survival rate. However, by univariate analysis, we found that significant adverse predictors of survival were splenic rupture before surgery and large tumor size (> 5 cm), while adjuvant chemotherapy was a favorable predictor. Furthermore, multivariate analysis revealed that splenic rupture and adjuvant chemotherapy were independent adverse and favorable predictors, respectively. CONCLUSION: Our large series describes and confirms the characteristics and poor prognosis of Chinese primary splenic angiosarcoma, thus indicating a critical role for early diagnosis and surgical intervention (prior to rupture) in management, and highlights the promising potential of adjuvant chemotherapy for improving the outcome in these cases.


Assuntos
Hemangiossarcoma/diagnóstico , Neoplasias Esplênicas/diagnóstico , Adolescente , Adulto , Idoso , Quimioterapia Adjuvante , Criança , Pré-Escolar , China , Bases de Dados Factuais , Feminino , Hemangiossarcoma/tratamento farmacológico , Hemangiossarcoma/mortalidade , Hemangiossarcoma/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Induzidas por Radiação/diagnóstico , Neoplasias Induzidas por Radiação/patologia , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Neoplasias Esplênicas/tratamento farmacológico , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/patologia , Taxa de Sobrevida , Adulto Jovem
6.
Pathol Res Pract ; 214(12): 1952-1958, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30087035

RESUMO

BACKGROUND: To study the effects of splenectomy on treatment and diagnosis of tumours of lymphoid tissue of the spleen. METHODS: Fifty-three cases were reviewed from Peking University People's Hospital from 2002 to 2017. According to WHO classification of tumours of haematopoietic and lymphoid tissues (2008) and classification updated (2016), the cases were studied by microscopy, immunohistochemistry and in situ hybridization, combined with the bone marrow biopsy and clinical examination. RESULTS: In 53 cases, the male to female ratio was 3.4:1, the mean age was 55.4 years old, the median survival time was 17.0 months, and all patients present with variable degree of splenomegaly. The elevated percentage of lymphocyte in peripheral blood can be seen in 22 cases, and elevated LDH level in 24 cases. Abnormal blood counts can be seen in 26 cases before operation, and 22 cases remission to normal level partly or completely after operation. The clinical symptoms included abdominal pain or distension, fatigue, fever, and weight loss, etc. Seventeen cases present with lymphoadenopathy of abdomen or other sites. Fourteen cases were stage I or II, whereas 6 were stage III, 28 were stage IV. Forty-three cases were splenic B-cell marginal zone lymphoma (SMZL)(48.8%,21/43), DLBCL(23.3%,10/43), splenic diffuse red pulp small B-cell lymphoma (SDRPSBL)(11.6%,5/43), mantle cell lymphoma (MCL)(9.3%,4/43), follicular lymphoma (FL)(4.7%,2/43), composite lymphoma (CL, DLBCL and classical Hodgkin lymphoma)(2.3%,1/43) in turn, and the remaining 10 cases were chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL/SLL) (n = 4), hairy cell leukaemia (HCL) (n = 1), hepatosplenic T-cell lymphoma (HSTL) (n = 5). The survival period of SMZL and DLBCL was 25.7, 18.6 months, respectively. Thirteen cases were dead (27.1%, 13/48). The chemotherapy protocol included Hyper-CVAD A/B with/without R (Rituximab), COP, CHOP with/without R etc. The prognosis of those with elevated LDH level, high clinical staging, B symptom, and older than 60 year old was obviously worse, and the prognosis of DLBCL was worse than that of SMZL. CONCLUSIONS: Most splenic lymphoid tumors present with splenomegaly and abnormal blood counts, and complete or part remission of blood counts can be seen after splenectomy, and splenectomy is also helpful for pathological diagnosis. The most common pathological types are SMZL and DLBCL. The definite diagnosis can be made by combining with clinical features, histopathology, immunophenotype, genetics, bone marrow biopsy and laboratory examination.


Assuntos
Tecido Linfoide/patologia , Linfoma de Zona Marginal Tipo Células B/patologia , Linfoma Folicular/patologia , Linfoma Difuso de Grandes Células B/patologia , Neoplasias Esplênicas/patologia , Adulto , Idoso , Feminino , Humanos , Tecido Linfoide/cirurgia , Linfoma de Zona Marginal Tipo Células B/mortalidade , Linfoma de Zona Marginal Tipo Células B/cirurgia , Linfoma Folicular/mortalidade , Linfoma Folicular/cirurgia , Linfoma Difuso de Grandes Células B/mortalidade , Linfoma Difuso de Grandes Células B/cirurgia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Esplenectomia , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/cirurgia , Taxa de Sobrevida , Resultado do Tratamento , Adulto Jovem
7.
Best Pract Res Clin Haematol ; 31(1): 65-72, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29452668

RESUMO

BACKGROUND: SMZL is a relatively rare low grade B-cell lymphoma, characterized usually by an indolent clinical behavior. Since there is no prospective randomized trials to establish the best treatment approach, decision on therapeutic management should be based on the available retrospective series. Based on these data, rituximab and splenectomy appear to be the most effective. Splenectomy represented the standard treatment modality until early 2000s. More than 90% of the patients present quick amelioration of splenomegaly related symptoms along with improvement of cytopenias related to hypersplenism. The median progression free survival was 8.25 years in the largest series of patients published so far, while the median 5- and 10- year OS were 84% and 67%, respectively. Responses to splenectomy are not complete since extrasplenic disease persists. Patients with heavy bone marrow infiltration, lymphadenopathy or other disease localization besides the spleen are not good candidates for splenectomy. Furthermore splenectomy is a major surgical procedure accompanied by acute perioperative complications as well as late toxicities mainly due to infections. For that reasons splenectomy is not appropriate for elderly patients or patients with comorbidities with a high surgical risk. On the other hand rituximab monotherapy displays high efficacy with minimal toxicity. Several published series have shown an ORR more than 90%, with high CR rates (∼50%). The 10-year PFS and OS were 63% and 85%, respectively in a series of 104 SMZL patients. The role of rituximab maintenance has been investigated by only one group. Based on these data, maintenance with rituximab further improved the quality of responses by increasing significantly the CR rates (from 42% at the end of induction to 71% at the end of maintenance treatment), as well as the duration of responses: 7-year PFS was 75% for those patients who received maintenance vs 39% for those who did not (p < 0.0004). However no difference in OS has been noticed between the two groups, so far. Summarizing the above data, it is obvious that Rituximab monotherapy is associated with high response rates, long response duration and favorable safety profile, rendering it as the treatment of choice in SMZL.


Assuntos
Linfoma de Zona Marginal Tipo Células B/terapia , Rituximab/uso terapêutico , Esplenectomia , Neoplasias Esplênicas/terapia , Humanos , Linfoma de Zona Marginal Tipo Células B/metabolismo , Linfoma de Zona Marginal Tipo Células B/mortalidade , Linfoma de Zona Marginal Tipo Células B/patologia , Neoplasias Esplênicas/metabolismo , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/patologia , Esplenomegalia/metabolismo , Esplenomegalia/mortalidade , Esplenomegalia/patologia , Esplenomegalia/terapia
8.
J Small Anim Pract ; 59(2): 85-91, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29210452

RESUMO

OBJECTIVES: To investigate thalidomide as an adjuvant treatment for canine haemangiosarcoma. MATERIALS AND METHODS: Fifteen dogs with splenic haemangiosarcoma, initially treated by splenectomy, were included. Following recovery from surgery, all dogs received thalidomide continuously until their death. Tumour stage was established using CT scans of the chest and abdomen immediately before starting thalidomide treatment and again three months later. Cause of death was confirmed by post mortem examination. RESULTS: The median survival time of dogs receiving thalidomide was 172 days (95% confidence interval: 93 to 250 days). Five dogs (33% of the population receiving thalidomide) survived more than 1 year (range 458 to 660 days) after surgery. Dogs with stage 2 disease that received thalidomide also had a longer survival time than dogs with stage 3 disease (median survival time 303 versus 40 days). Of 15 dogs, 13 died from metastatic haemangiosarcoma. CLINICAL SIGNIFICANCE: Treatment using thalidomide may improve survival of dogs with splenic haemangiosarcoma and should be considered a possible adjuvant therapy.


Assuntos
Inibidores da Angiogênese/uso terapêutico , Doenças do Cão/tratamento farmacológico , Hemangiossarcoma/veterinária , Neoplasias Esplênicas/veterinária , Talidomida/uso terapêutico , Inibidores da Angiogênese/administração & dosagem , Animais , Quimioterapia Adjuvante/veterinária , Doenças do Cão/mortalidade , Cães , Feminino , Hemangiossarcoma/tratamento farmacológico , Hemangiossarcoma/mortalidade , Masculino , Neoplasias Esplênicas/tratamento farmacológico , Neoplasias Esplênicas/mortalidade , Talidomida/administração & dosagem
9.
Hematology Am Soc Hematol Educ Program ; 2017(1): 371-378, 2017 12 08.
Artigo em Inglês | MEDLINE | ID: mdl-29222281

RESUMO

Splenic marginal zone lymphoma (SMZL) and nodal marginal zone lymphoma (NMZL) are rare indolent chronic B-cell lymphomas. Prognosis is typically good with median survival around 10-15 years. Management is generally based on the presence of symptoms or high tumor burden. There are no standard treatments for these 2 entities, and therapeutic strategies are rapidly evolving. Clinical developments for these 2 entities are oriented by genomic studies, with largely overlapping mutational profiles involving the NOTCH, B-cell receptor (BcR) and nuclear factor κB (NF-κB) signaling, chromatin remodeling, and the cytoskeleton. Although new therapeutic options based on targeting signaling pathways and overcoming resistance are increasingly available, few specific prospective studies are performed for these rare subtypes, limiting the conclusions that can be drawn. Novel drugs targeting B-cell signaling have shown promise, including ibrutinib and copanlisib. The second-generation oral immunomodalator (IMiD) lenalidomide showed impressive results when combined with rituximab. Other potential solutions include targeting the NF-κB, JAK/STAT, BCL2, NOTCH, and Toll-like receptor signaling pathways; however, studies in these 2 MZL entities are yet to prove a definitive benefit. Molecular profiling is now a cornerstone of diagnostic, prognostic, and therapeutic strategies to offer patient- and disease-specific solutions. The development of a wider range of effective targeted therapies and prognostic biomarkers is keenly awaited and is expected to strongly affect the natural history of SMZL and NMZL.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma de Zona Marginal Tipo Células B , Neoplasias Esplênicas , Adenina/análogos & derivados , Intervalo Livre de Doença , Humanos , Lenalidomida , Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Linfoma de Zona Marginal Tipo Células B/genética , Linfoma de Zona Marginal Tipo Células B/metabolismo , Linfoma de Zona Marginal Tipo Células B/mortalidade , Mutação , Proteínas de Neoplasias/genética , Proteínas de Neoplasias/metabolismo , Piperidinas , Pirazóis/uso terapêutico , Pirimidinas/uso terapêutico , Quinazolinas/uso terapêutico , Rituximab/uso terapêutico , Transdução de Sinais/efeitos dos fármacos , Transdução de Sinais/genética , Neoplasias Esplênicas/tratamento farmacológico , Neoplasias Esplênicas/genética , Neoplasias Esplênicas/metabolismo , Neoplasias Esplênicas/mortalidade , Taxa de Sobrevida , Talidomida/análogos & derivados , Talidomida/uso terapêutico
10.
Ann Hematol ; 96(12): 2063-2070, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28975390

RESUMO

Follicular lymphoma (FL) is a low-grade lymphoma that is usually characterized by generalized lymphadenopathy. Extranodal invasion by FL generally involves the bone marrow, skin, and duodenum; splenic infiltration often occurs in the advanced stages. However, primary splenic FL is very rare. Hence, few studies have been performed on splenic FL, and its clinicopathological features have not been established. This study aimed to investigate the clinicopathological features of primary splenic FL, as compared to nodal FL. We analyzed 17 patients diagnosed with primary splenic FL and 153 control patients with systemic FL. Hepatitis C virus (HCV)-positive status was significantly more common in patients with splenic FL than in the control patients (p = 0.02). Ann Arbor stage III or IV (p = 0.0003) and high-risk FLIPI (Follicular Lymphoma International Prognostic Index) (p = 0.03) were significantly less common in patients with splenic FL than in the control patients; however, the overall and progression-free survival curves were not significantly different between the groups. Among the 17 patients with splenic FL, the progression-free survival was significantly worse in patients who underwent splenectomy without receiving postoperative chemotherapy than in those who did (p = 0.03). These results suggest that primary splenic FL should be considered different from systemic FL; accordingly, its management should also be conducted differently.


Assuntos
Hepacivirus , Hepatite C , Linfoma Folicular , Neoplasias Esplênicas , Idoso , Idoso de 80 Anos ou mais , Intervalo Livre de Doença , Feminino , Hepatite C/sangue , Hepatite C/mortalidade , Hepatite C/terapia , Humanos , Linfoma Folicular/sangue , Linfoma Folicular/mortalidade , Linfoma Folicular/terapia , Linfoma Folicular/virologia , Masculino , Pessoa de Meia-Idade , Esplenectomia , Neoplasias Esplênicas/sangue , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/terapia , Neoplasias Esplênicas/virologia , Taxa de Sobrevida
11.
Hematology ; 22(7): 405-411, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28105889

RESUMO

OBJECTIVES AND METHODS: Splenic marginal zone lymphoma (SMZL) is a rare non-Hodgkin lymphoma. We sought to identify prognostic factors and define outcomes in a cohort of 64 patients with SMZL who were treated at two large academic medical centers in North America in the rituximab era. RESULTS: Over a median follow-up of 37.8 (range 6-167.1) months, Kaplan-Meier estimate of median OS was 156.3 months and median PFS was 52.9 months. On univariate analysis, baseline hemoglobin <12 g/dl was associated with inferior OS (p = 0.045). High-risk FLIPI score was associated with inferior PFS when compared with intermediate/low risk (p = 0.05) and marginally significant with regard to OS (p = 0.056). Splenectomy was not predictive of OS or PFS (p = 0.563 and 0.937, respectively). Transformation to diffuse large B-cell lymphoma occurred in four (6.3%) patients during the observation period. OS was comparable to contemporaneous cohorts of patients with extranodal and nodal marginal lymphomas and FLIPI score was highly predictive for inferior PFS and OS when all three cohorts were analyzed together. CONCLUSION: Outcomes of SMZL, in our series, were excellent, with a median OS of >13 years. Low hemoglobin and high-risk FLIPI were associated with inferior outcomes.


Assuntos
Antineoplásicos/uso terapêutico , Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Linfoma de Zona Marginal Tipo Células B/mortalidade , Rituximab/uso terapêutico , Neoplasias Esplênicas/tratamento farmacológico , Neoplasias Esplênicas/mortalidade , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores , Estudos de Coortes , Terapia Combinada , Feminino , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Masculino , Pessoa de Meia-Idade , Metástase Neoplásica , Estadiamento de Neoplasias , Razão de Chances , Prognóstico , Neoplasias Esplênicas/diagnóstico , Resultado do Tratamento
12.
Mol Cell ; 64(6): 1074-1087, 2016 12 15.
Artigo em Inglês | MEDLINE | ID: mdl-27889452

RESUMO

Repetitive genomic regions include tandem sequence repeats and interspersed repeats, such as endogenous retroviruses and LINE-1 elements. Repressive heterochromatin domains silence expression of these sequences through mechanisms that remain poorly understood. Here, we present evidence that the retinoblastoma protein (pRB) utilizes a cell-cycle-independent interaction with E2F1 to recruit enhancer of zeste homolog 2 (EZH2) to diverse repeat sequences. These include simple repeats, satellites, LINEs, and endogenous retroviruses as well as transposon fragments. We generated a mutant mouse strain carrying an F832A mutation in Rb1 that is defective for recruitment to repetitive sequences. Loss of pRB-EZH2 complexes from repeats disperses H3K27me3 from these genomic locations and permits repeat expression. Consistent with maintenance of H3K27me3 at the Hox clusters, these mice are developmentally normal. However, susceptibility to lymphoma suggests that pRB-EZH2 recruitment to repetitive elements may be cancer relevant.


Assuntos
Fator de Transcrição E2F1/genética , Proteína Potenciadora do Homólogo 2 de Zeste/genética , Inativação Gênica , Linfoma/genética , Sequências Repetitivas de Ácido Nucleico , Proteína do Retinoblastoma/genética , Animais , Carcinoma Hepatocelular/genética , Carcinoma Hepatocelular/metabolismo , Carcinoma Hepatocelular/mortalidade , Carcinoma Hepatocelular/patologia , Fator de Transcrição E2F1/metabolismo , Proteína Potenciadora do Homólogo 2 de Zeste/metabolismo , Fibroblastos/citologia , Fibroblastos/metabolismo , Predisposição Genética para Doença , Histonas/genética , Histonas/metabolismo , Neoplasias Hepáticas/genética , Neoplasias Hepáticas/metabolismo , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/patologia , Linfoma/metabolismo , Linfoma/mortalidade , Linfoma/patologia , Mesentério/metabolismo , Mesentério/patologia , Camundongos , Mutação , Cultura Primária de Células , Ligação Proteica , Proteína do Retinoblastoma/metabolismo , Neoplasias Esplênicas/genética , Neoplasias Esplênicas/metabolismo , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/patologia , Análise de Sobrevida
13.
Leuk Res ; 44: 53-60, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-27030961

RESUMO

Splenic marginal zone lymphoma (SMZL) is an indolent lymphoma in which watch and wait (W&W) approach as well as splenectomy and chemo-immunotherapy are usually recommended. The role of the different approaches in relation to risk factors was evaluated. One hundred patients with SMZL were retrospectively studied. Median age was 65 years. HCV positivity was 3.1%. The 10-year overall-survival was 95.1% (CI: 90-100%). Sixty-two asymptomatic, low tumour burden patients were submitted to W&W. A low-risk group not requiring treatment was identified. Patients requiring treatment received splenectomy (36), chemotherapy-alone (27) and rituximab ± chemotherapy (16). In multivariate analysis, negative predictors for starting treatment were female-sex, splenomegaly, ECOG ≥ 1. Patients with low IIL-Score had a better 5-year TFT (24%). The median TFT of the W&W cohort was 58.5 months; at 10 years, 17% of patients were still on W&W. Splenectomy and rituximab ± chemotherapy showed similar results, while chemotherapy alone proved inferior. This real-life single-centre study of SMZL confirmed its very good prognosis with a survival likelihood overlapping that of general population. The prognostic role of IIL-Score was confirmed. The W&W approach allowed a median PFS longer than in follicular lymphoma. Finally, our data confirm the inferiority of chemotherapy compared to splenectomy and rituximab±chemotherapy.


Assuntos
Antineoplásicos/uso terapêutico , Linfoma de Zona Marginal Tipo Células B/terapia , Rituximab/uso terapêutico , Esplenectomia/mortalidade , Neoplasias Esplênicas/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Terapia Combinada , Feminino , Seguimentos , Humanos , Linfoma de Zona Marginal Tipo Células B/mortalidade , Linfoma de Zona Marginal Tipo Células B/patologia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prognóstico , Estudos Retrospectivos , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/patologia , Taxa de Sobrevida
14.
Hum Pathol ; 50: 109-17, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26997444

RESUMO

Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell lymphoma commonly associated with cytopenias. The pathogenesis of cytopenias in patients with HSTCL is not well defined, although the presence of dyspoietic hematopoietic cells and the common association with trisomy 8 raise the possibility of an associated myelodysplastic syndrome (MDS). In 25 bone marrow specimens involved by HSTCL, we systematically assessed for morphologic features of dyspoiesis and correlated the findings with peripheral cytopenia(s), cytogenetic findings, and detection of chromosome 8 by fluorescence in situ hybridization. The median patient age was 33 years. One patient had a history of MDS diagnosed 1 year prior to the diagnosis of HSTCL. Thirteen (54%) patients had anemia less than 100 g/L, 10 (53%) of 19 had neutropenia less than 1.8 × 10(9)/L, and 15 (60%) had thrombocytopenia less than 100 × 10(9)/L. Dyspoietic features were identified in 1 to 3 hematopoietic cell lineages in 20 (80%) of 25 patients. Cytogenetic analysis identified trisomy 8 in 7 cases. Patients with trisomy 8 had a lower platelet count, but trisomy 8 was not associated with cytopenias, dyspoietic features, or cytogenetic abnormalities. Combined morphologic and fluorescence in situ hybridization analysis showed that trisomy 8 was restricted to the lymphoma cells, except in the 1 patient with a history of MDS. In conclusion, dyspoietic changes are common in the bone marrow of patients with HSTCL. These changes are not associated with cytopenias or chromosomal abnormalities, suggesting that dyspoiesis in patients with HSTCL is not a manifestation of a MDS.


Assuntos
Medula Óssea/patologia , Neoplasias Hepáticas/complicações , Linfoma de Células T/complicações , Síndromes Mielodisplásicas/complicações , Neutropenia/etiologia , Neoplasias Esplênicas/complicações , Trombocitopenia/etiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/genética , Exame de Medula Óssea , Criança , Pré-Escolar , Cromossomos Humanos Par 8/genética , Feminino , Predisposição Genética para Doença , Humanos , Hibridização in Situ Fluorescente , Estimativa de Kaplan-Meier , Neoplasias Hepáticas/genética , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/terapia , Contagem de Linfócitos , Linfoma de Células T/genética , Linfoma de Células T/mortalidade , Linfoma de Células T/patologia , Linfoma de Células T/terapia , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/genética , Síndromes Mielodisplásicas/patologia , Neutropenia/sangue , Neutropenia/genética , Neutropenia/mortalidade , Neutropenia/patologia , Neutropenia/terapia , Fenótipo , Contagem de Plaquetas , Prognóstico , Fatores de Risco , Neoplasias Esplênicas/genética , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/patologia , Neoplasias Esplênicas/terapia , Trombocitopenia/sangue , Trombocitopenia/genética , Trombocitopenia/mortalidade , Trombocitopenia/patologia , Trombocitopenia/terapia , Fatores de Tempo , Trissomia/genética , Adulto Jovem
15.
Am J Surg Pathol ; 40(5): 676-88, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-26872013

RESUMO

Hepatosplenic T-cell lymphoma (HSTCL) is a rare type of lymphoma. Patients have a poor prognosis, and there is no standard of care. We evaluated 28 HSTCL patients to determine factors that may be associated with outcome. There were 19 men and 9 women with a median age of 32.5 years. Most patients had massive splenomegaly, and bone marrow showed sinusoidal involvement by lymphoma. The HSTCL cells expressed γδ T-cell receptor (TCR) in 20 (74%), αß TCR in 5 (19%), and neither in 2 (7%) patients (1 case not assessed). Conventional cytogenetics and/or fluorescence in situ hybridization analysis in 24 patients at diagnosis showed isochromosome 7q (i7q) in 10 (42%) and trisomy 8 in 8 (33%) patients. Median overall survival (OS) and event-free survival (EFS) were each 28.3 months. Serum bilirubin level ≥1.5 mg/dL, αß TCR expression, and trisomy 8 each correlated significantly with shorter OS and EFS. Patients with HSTCL received a variety of chemotherapy regimens with no regimen better than any other. However, patients who underwent stem cell transplant showed longer survival (OS: hazard ratio 0.3, P=0.09; EFS: hazard ratio 0.2, P=0.034). In conclusion, although HSTCL patients have a poor prognosis overall, the data presented support the novel suggestions that HSTCL patients can be stratified into 2 prognostic groups, with an elevated serum bilirubin level, αß TCR expression, and trisomy 8 identifying a poorer prognostic group. In addition, the outcomes of this patient cohort suggest that stem cell transplantation has value for the treatment of patients with HSTCL.


Assuntos
Neoplasias Hepáticas , Linfoma de Células T , Neoplasias Esplênicas , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores Tumorais/análise , Biomarcadores Tumorais/genética , Medula Óssea/patologia , Exame de Medula Óssea , Criança , Pré-Escolar , Cromossomos Humanos Par 7 , Cromossomos Humanos Par 8/genética , Intervalo Livre de Doença , Feminino , Predisposição Genética para Doença , Hepatomegalia/patologia , Humanos , Imuno-Histoquímica , Hibridização in Situ Fluorescente , Isocromossomos , Estimativa de Kaplan-Meier , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/genética , Neoplasias Hepáticas/imunologia , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/patologia , Linfoma de Células T/tratamento farmacológico , Linfoma de Células T/genética , Linfoma de Células T/imunologia , Linfoma de Células T/mortalidade , Linfoma de Células T/patologia , Masculino , Pessoa de Meia-Idade , Fenótipo , Modelos de Riscos Proporcionais , Receptores de Antígenos de Linfócitos T alfa-beta/genética , Receptores de Antígenos de Linfócitos T alfa-beta/imunologia , Receptores de Antígenos de Linfócitos T gama-delta/genética , Receptores de Antígenos de Linfócitos T gama-delta/imunologia , Estudos Retrospectivos , Fatores de Risco , Neoplasias Esplênicas/tratamento farmacológico , Neoplasias Esplênicas/genética , Neoplasias Esplênicas/imunologia , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/patologia , Esplenomegalia/patologia , Terapêutica , Fatores de Tempo
16.
Klin Khir ; (10): 9-11, 2016 Oct.
Artigo em Inglês, Ucraniano | MEDLINE | ID: mdl-30479104

RESUMO

Immediate and late results of the combined gastrectomy (CG) performance in 719 patients, suffering locally­spread gastric cancer (LSGC), were analyzed. Additional resection of adjacent оrgans was performed in 165 observations. In early postoperative period complications in 116 (16.1%) patients have had occurred, including surgical complications ­ in 77.7%, and nonsurgical ­ in 22.3%. Lethality in 30 postoperative days have constituted 11.1%. Тhe patients' postoperative life time was at average (22.9 ± 1.67) mo, mediana­ 9.3 mo; indices of 3­year and 5­year survival ­ (18.9 ± 1.72) and (12.9 ± 1.51)%,accordingly. Essential difference in favor of subtotal distal gastric resection was established, basing on comparison data between this procedure and CG. The data obtained witnessed the expediency of combined operative interventions, what have had widened possibilities of the patients' radical treatment for LSGC.


Assuntos
Neoplasias Esofágicas/cirurgia , Gastrectomia/métodos , Neoplasias Intestinais/cirurgia , Neoplasias Hepáticas/cirurgia , Neoplasias Pancreáticas/cirurgia , Neoplasias Esplênicas/cirurgia , Neoplasias Gástricas/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Empiema Pleural/diagnóstico , Empiema Pleural/etiologia , Empiema Pleural/mortalidade , Empiema Pleural/patologia , Neoplasias Esofágicas/mortalidade , Neoplasias Esofágicas/secundário , Feminino , Gastrectomia/efeitos adversos , Humanos , Neoplasias Intestinais/mortalidade , Neoplasias Intestinais/secundário , Obstrução Intestinal/diagnóstico , Obstrução Intestinal/etiologia , Obstrução Intestinal/mortalidade , Obstrução Intestinal/patologia , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/secundário , Masculino , Pessoa de Meia-Idade , Neoplasias Pancreáticas/mortalidade , Neoplasias Pancreáticas/secundário , Peritonite/diagnóstico , Peritonite/etiologia , Peritonite/mortalidade , Peritonite/patologia , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/patologia , Estudos Retrospectivos , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/secundário , Estômago/patologia , Estômago/cirurgia , Neoplasias Gástricas/mortalidade , Neoplasias Gástricas/patologia , Análise de Sobrevida , Fatores de Tempo , Resultado do Tratamento
18.
Klin Khir ; (7): 5-8, 2015 Jul.
Artigo em Ucraniano | MEDLINE | ID: mdl-26591208

RESUMO

Radical surgery for tumors of the left anatomical and surgical segment of the pancreas proved for distal resection in various versions, central resection and enucleation of tumors. The causes of early postoperative complications and mortality in 129 patients aged from 14 to 81 years, operated on for neoplastic lesions of the left anatomical segment of the pancreas in the period from 2009 to 2014 were analysed. The influence of various factors of risk of complications and mortality were studied in particular, extended resection, for tumor invasion of adjacent organs, and adjacent vessels.


Assuntos
Adenocarcinoma/cirurgia , Neoplasias Hepáticas/cirurgia , Neoplasias Pancreáticas/cirurgia , Complicações Pós-Operatórias , Neoplasias Esplênicas/cirurgia , Neoplasias Vasculares/cirurgia , Adenocarcinoma/irrigação sanguínea , Adenocarcinoma/mortalidade , Adenocarcinoma/secundário , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Neoplasias Hepáticas/irrigação sanguínea , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/secundário , Masculino , Pessoa de Meia-Idade , Pâncreas/irrigação sanguínea , Pâncreas/patologia , Pâncreas/cirurgia , Pancreatectomia/métodos , Neoplasias Pancreáticas/irrigação sanguínea , Neoplasias Pancreáticas/mortalidade , Neoplasias Pancreáticas/patologia , Período Pós-Operatório , Estudos Retrospectivos , Fatores de Risco , Neoplasias Esplênicas/irrigação sanguínea , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/secundário , Análise de Sobrevida , Neoplasias Vasculares/irrigação sanguínea , Neoplasias Vasculares/mortalidade , Neoplasias Vasculares/secundário
19.
Anticancer Res ; 35(10): 5649-54, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26408738

RESUMO

Ovarian cancer represents an aggressive gynecological malignancy with a high capacity for dissemination. Once the tumor cells go beyond the pelvic area, upper abdominal involvement, including hepatic, diaphragmatic or even splenic, is frequently seen. The aim of the present study was to determine the impact on survival of parenchymatous versus peritoneal splenic metastases versus splenic hilum lymph node involvement at the time of primary cytoreduction for advanced-stage epithelial ovarian cancer. Sixty-six patients with a mean age of 54.12 years (range=25-80 years) were submitted to splenectomy in the context of primary cytoreduction at the Dan Setlacec Center of Gastrointestinal Disease and Liver Transplantation, Fundeni Clinical Institute, between January 2002 and May 2014. Although complete macroscopic resection was attempted in all cases, an R0 resection was achieved only in 57 out of the 66 cases. Histopathological studies confirmed the presence of serous subtype in 61 cases, while in the other five cases, the mucinous subtype was found. When studying the specimens of splenectomy, capsular invasion was found in 35 cases (53%), parenchymatous involvement was present in 19 (28.7%), and hilar involvement was present in 12 (18.1%). The overall morbidity rate was 30%, while the 30-day postoperative mortality rate was 7%. The median overall survival for cases with peritoneal seeding was 58.4 months, while that for patients with parenchymatous involvement was 24.5 months (p=0.0126); patients diagnosed with hilar involvement had a median overall survival of 40.6 months (p=0.362). In conclusion, the presence of parenchymatous splenic metastases at primary cytoreduction for advanced-stage ovarian cancer is associated with significantly poorer survival when compared to hilar or peritoneal seeding.


Assuntos
Adenocarcinoma Mucinoso/patologia , Cistadenocarcinoma Seroso/patologia , Procedimentos Cirúrgicos de Citorredução/efeitos adversos , Neoplasias Ovarianas/patologia , Neoplasias Peritoneais/secundário , Complicações Pós-Operatórias , Neoplasias Esplênicas/secundário , Adenocarcinoma Mucinoso/mortalidade , Adenocarcinoma Mucinoso/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Cistadenocarcinoma Seroso/mortalidade , Cistadenocarcinoma Seroso/cirurgia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Inoculação de Neoplasia , Estadiamento de Neoplasias , Neoplasias Ovarianas/mortalidade , Neoplasias Ovarianas/cirurgia , Neoplasias Peritoneais/irrigação sanguínea , Neoplasias Peritoneais/mortalidade , Neoplasias Peritoneais/cirurgia , Prognóstico , Estudos Retrospectivos , Esplenectomia , Neoplasias Esplênicas/irrigação sanguínea , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/cirurgia , Taxa de Sobrevida
20.
J Am Vet Med Assoc ; 247(4): 393-403, 2015 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-26225611

RESUMO

OBJECTIVE: To determine survival time for dogs with splenic hemangiosarcoma treated with splenectomy alone, identify potential prognostic factors, and evaluate the efficacy of adjuvant chemotherapy. DESIGN: Retrospective case series. ANIMALS: 208 dogs. PROCEDURES: Medical records were reviewed, long-term follow-up information was obtained, and survival data were analyzed statistically. RESULTS: 154 dogs were treated with surgery alone, and 54 were treated with surgery and chemotherapy. Twenty-eight dogs received conventional chemotherapy, 13 received cyclophosphamide-based metronomic chemotherapy, and 13 received both conventional and metronomic chemotherapy. Median survival time of dogs treated with splenectomy alone was 1.6 months. Clinical stage was the only prognostic factor significantly associated with survival time. When the entire follow-up period was considered, there was no significant difference in survival time between dogs treated with surgery alone and dogs treated with surgery and chemotherapy. However, during the first 4 months of follow-up, after adjusting for the effects of clinical stage, survival time was significantly prolonged among dogs receiving any type of chemotherapy (hazard ratio, 0.6) and among dogs receiving both conventional and metronomic chemotherapy (hazard ratio, 0.4). CONCLUSIONS AND CLINICAL RELEVANCE: Clinical stage was strongly associated with prognosis for dogs with splenic hemangiosarcoma. Chemotherapy was effective in prolonging survival time during the early portion of the follow-up period. Combinations of doxorubicin-based conventional protocols and cyclophosphamide-based metronomic protocols appeared to be more effective than either type of chemotherapy alone, but prolongations in survival time resulting from current protocols were modest.


Assuntos
Doenças do Cão/mortalidade , Hemangiossarcoma/veterinária , Neoplasias Esplênicas/veterinária , Animais , Quimioterapia Adjuvante/veterinária , Terapia Combinada , Doenças do Cão/tratamento farmacológico , Doenças do Cão/cirurgia , Cães , Feminino , Hemangiossarcoma/mortalidade , Masculino , Massachusetts , Estudos Retrospectivos , Esplenectomia/veterinária , Neoplasias Esplênicas/mortalidade , Análise de Sobrevida , Resultado do Tratamento
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