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1.
Ann Surg Oncol ; 30(6): 3681-3689, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-36800129

RESUMO

BACKGROUND: Outcomes of unplanned excisions of extremity soft tissue sarcomas (STSE) range from poor to even superior compared with planned excisions in developed countries. However, little is known regarding outcomes in low-to-middle-income countries. This study aimed to determine whether definitively treated STSE patients with a previous unplanned excision have poorer oncologic outcomes compared with those with planned excisions. PATIENTS AND METHODS: Using the database of a single sarcoma practice, we reviewed 148 patients with STSE managed with definitive surgery-78 with previous unplanned excisions (UE) and 70 with planned excisions (PE). RESULTS: Median follow-up was 4.4 years. UE patients had more surgeries overall and plastic reconstructions (P < 0.001). On multivariate analysis, overall survival (OS), local recurrence-free survival (LRFS), and distant metastasis-free survival (DMFS) were not worse among UE patients compared with PE patients. Negative predictors for LRFS were high tumor grade (P = 0.031) and an R1 surgical margin (P < 0.001). High grade (P <0.001), local recurrence (P = 0.001), and planned excisions (P = 0.009) predicted poorer DMFS, while age over 65 years (P = 0.011) and distant metastasis predicted poorer OS (P < 0.001). CONCLUSIONS: We recommend systematic re-excision for patients with unplanned excisions. Our study shows that STSE patients with UE, when subjected to re-excision with appropriate surgical margins, can achieve oncologic results similar to those for PE patients. However, there is an associated increased number of surgeries and plastic reconstruction for UE patients. This underscores the need, especially in a resource-limited setting, for education and collaborative policies to raise awareness about STSE among patients and physicians.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Humanos , Idoso , Resultado do Tratamento , Estudos Retrospectivos , Extremidades/cirurgia , Extremidades/patologia , Reoperação , Sarcoma/patologia , Neoplasias de Tecidos Moles/patologia , Margens de Excisão , Recidiva Local de Neoplasia/cirurgia , Recidiva Local de Neoplasia/patologia
2.
JAMA Oncol ; 6(5): 685-695, 2020 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-32105305

RESUMO

Importance: Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale. Objectives: To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis. Design, Setting, and Participants: A total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017. Main Outcomes and Measures: Age at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. Results: The cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low- and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI, 12.94-24.80], and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI, 4.30-7.68]). Conclusions and Relevance: This study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs.


Assuntos
Retinoblastoma/economia , Retinoblastoma/epidemiologia , Pré-Escolar , Feminino , Humanos , Lactente , Masculino
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