RESUMO
BACKGROUND: Retroperitoneal sarcomas (RPS) are rare, histologically heterogeneous, and anatomically complex tumors. National Comprehensive Cancer Network guidelines recommend evaluation and management by multidisciplinary teams with experience in sarcoma. Our aim was to determine an appropriate hospital volume threshold for the treatment of RPS. PATIENTS AND METHODS: Patients undergoing resection of RPS were identified from the National Cancer Data Base (1998-2012). Multivariable modeling with restricted cubic splines was employed to examine the association between hospital volume and survival and identify possible hospital volume threshold. RESULTS: The study included 5,340 patients who underwent surgery at 909 different hospitals. Median annual volume was two cases per year. After adjustment, hospital volume was associated with improved survival (p=0.01), without cutoff. The cohort was then grouped into: Low-volume (≤5 cases/year), intermediate-volume (6-10 cases/year), and high-volume (>10 cases/year). The majority of patients were treated in low-volume hospitals (86%), compared to 9% in intermediate- and 5% in high-volume centers; 44% of patients were treated in hospitals that performed one case per year. Compared to low-volume, high-volume hospitals more often had patients with high-grade and larger tumors. Adjusted 90-day mortality was significantly lower in high- vs. low-volume hospitals (odds ratio(OR)=0.25, p=0.02). With adjustment, treatment in high- vs. low-volume hospitals was associated with lower odds of margin positivity (OR=0.58, p=0.001), and improved overall survival (hazard ratio(HR)=0.61, p=0.002). CONCLUSION: Treatment of RPS in high-volume centers is associated with significant reduction in short-term mortality and improved long-term survival. Hospital volume may be a surrogate for the infrastructure and support necessary for the optimal management of these complex malignancies.
Assuntos
Hospitais com Alto Volume de Atendimentos , Hospitais com Baixo Volume de Atendimentos , Doenças Raras/mortalidade , Neoplasias Retroperitoneais/mortalidade , Sarcoma/mortalidade , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Raras/cirurgia , Neoplasias Retroperitoneais/cirurgia , Sarcoma/cirurgiaRESUMO
Thyroid carcinoma on struma ovarii (TCSO) is a rare ovarian tumour, derivate from monodermic teratomas. It represents about 0.01% of overall ovarian tumours and 5 to 10% of struma ovarii. The diagnosis is histologic and retrospective after pelvic surgery; radiographic imaging being unspecific. Because of its rarity, the treatment of TCSO is not consensual and should be validated in multidisciplinary team involved in rare ovarian carcinoma. The first treatment is a surgical removal, with a laparoscopic approach. A fertility-conservative surgery is recommended for young women. If the tumour is unresectable and/or with metastatic spread, an adjuvant iodine 131 treatment might be proposed after thyroidectomy. Recurrence of TCSO should be taken care of as a thyroid carcinoma with tyrosine kinase inhibitor in case of progressive distant relapse, refractory to iodine 131 treatment. If the recurrence is localised, a complete surgery is the preferred option. There is no gold standard for the follow up.
Assuntos
Neoplasias Ovarianas/patologia , Doenças Raras/patologia , Estruma Ovariano/patologia , Neoplasias da Glândula Tireoide/patologia , Adulto , Feminino , Preservação da Fertilidade , Humanos , Achados Incidentais , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Neoplasias Ovarianas/mortalidade , Neoplasias Ovarianas/cirurgia , Doenças Raras/mortalidade , Doenças Raras/cirurgia , Estruma Ovariano/mortalidade , Estruma Ovariano/cirurgia , Teratoma/mortalidade , Teratoma/patologia , Teratoma/cirurgia , Neoplasias da Glândula Tireoide/mortalidade , Neoplasias da Glândula Tireoide/cirurgia , TireoidectomiaRESUMO
Traditional serrated adenoma (TSA) is a type of serrated polyp of the colorectum and is thought to be a precancerous lesion. There are three types of serrated polyps, namely, hyperplastic polyps, sessile serrated adenomas/polyps, and TSAs. TSA is the least common of the three types and accounts for about 5% of serrated polyps. Here we report a pediatric case of TSA that was successfully resected by endoscopic submucosal dissection (ESD). This rare case report describes a pediatric patient with no family history of colonic polyp who was admitted to our hospital with hematochezia. On colonoscopy, we found a polypoid lesion measuring 10 mm in diameter in the lower rectum. We selected ESD as a surgical option for en bloc resection, and histopathological examination revealed TSA. The findings in this case suggest that TSA with precancerous potential can occur in children, and that ESD is useful for treating this lesion.
Assuntos
Adenoma/cirurgia , Neoplasias do Colo/cirurgia , Pólipos do Colo/cirurgia , Hemorragia Gastrointestinal/cirurgia , Lesões Pré-Cancerosas/cirurgia , Doenças Raras/cirurgia , Adenoma/complicações , Adenoma/diagnóstico por imagem , Adenoma/patologia , Criança , Neoplasias do Colo/complicações , Neoplasias do Colo/diagnóstico por imagem , Neoplasias do Colo/patologia , Pólipos do Colo/complicações , Pólipos do Colo/diagnóstico por imagem , Pólipos do Colo/patologia , Colonoscopia , Ressecção Endoscópica de Mucosa , Hemorragia Gastrointestinal/diagnóstico por imagem , Hemorragia Gastrointestinal/etiologia , Humanos , Masculino , Lesões Pré-Cancerosas/diagnóstico por imagem , Lesões Pré-Cancerosas/patologia , Doenças Raras/complicações , Doenças Raras/diagnóstico por imagem , Doenças Raras/patologia , Reto/diagnóstico por imagem , Reto/patologia , Reto/cirurgiaAssuntos
Anestesia Geral/métodos , Anestesia Local/métodos , Malformações Arteriovenosas/cirurgia , Síndrome do Hamartoma Múltiplo/cirurgia , Dor Pós-Operatória/tratamento farmacológico , Doenças Raras/cirurgia , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Analgesia Epidural/métodos , Anestesia Geral/instrumentação , Anestésicos Locais/administração & dosagem , Determinação da Pressão Arterial/métodos , Criança , Ecocardiografia , Endoscopia do Sistema Digestório/efeitos adversos , Síndrome do Hamartoma Múltiplo/diagnóstico por imagem , Humanos , Máscaras Laríngeas , Laringoscópios , Laringoscopia/instrumentação , Laringoscopia/métodos , Imageamento por Ressonância Magnética , Masculino , Monitorização Intraoperatória/métodos , Dor Pós-Operatória/etiologia , Doenças Raras/diagnóstico por imagem , Coxa da Perna/irrigação sanguínea , Coxa da Perna/cirurgiaRESUMO
The etiology and outcomes of acute liver failure (ALF) have changed since the definition of this disease entity in the 1970s. In particular, the role of emergency liver transplantation has evolved over time, with the development of prognostic scoring systems to facilitate listing of appropriate patients, and a better understanding of transplant benefit in patients with ALF. This review examines the changing etiology of ALF, transplant benefit, outcomes following transplantation, and future alternatives to emergency liver transplantation in this devastating condition.
Assuntos
Falência Hepática Aguda/cirurgia , Transplante de Fígado/tendências , Doenças Raras/cirurgia , Acetaminofen/efeitos adversos , Acetilcisteína/uso terapêutico , Analgésicos não Narcóticos/efeitos adversos , Doença Hepática Induzida por Substâncias e Drogas/tratamento farmacológico , Contraindicações , Sequestradores de Radicais Livres/uso terapêutico , Humanos , Falência Hepática Aguda/tratamento farmacológico , Falência Hepática Aguda/etiologia , Falência Hepática Aguda/mortalidade , Prognóstico , Qualidade de Vida , Doenças Raras/tratamento farmacológico , Doenças Raras/etiologia , Taxa de Sobrevida , Resultado do Tratamento , Listas de Espera/mortalidadeRESUMO
A paquidermoperiostose primária é uma doença rara, caracterizada por aposição excessiva do periósteo do crânio, coexistindo com espessamento da epiderme e derme (paquidermia), provocando deformidades grosseiras. Devido à diversidade de estruturas acometidas, há várias opções cirúrgicas e métodos complementares que são utilizados no tratamento das alterações faciais desses pacientes. Esse trabalho apresenta o lifting subperiosteal como uma opção de tratamento estético para a face de pacientes portadores dessa síndrome, através do relato de dois casos operados no Hospital das Clínicas da Universidade Federal de Minas Gerais.
Primary pachydermoperiostosis is a rare disease characterized by excessive skull affixing of the periosteum, coexisting with thickening of the epidermis and dermis (pachydermia), thereby causing gross deformities. Owing to the variety of affected structures, there are several surgical options and complementary methods that are used in the treatment of facial alterations in these patients. This report describes the use of subperiosteal detachment as an aesthetic treatment option for the faces of two patients with primary pachydermoperiostosis, operated at the Hospital das Clínicas of the Federal University of Minas Gerais.