RESUMO
Cutaneous angiosarcoma is an aggressive malignant mesenchymal vasoformative neoplasm that accounts for 1% of all soft tissue sarcomas. Using data from the National Cancer Institute's Surveillance, Epidemiology, and End Results program, we analyzed the demographics and survival of cutaneous angiosarcoma. The Surveillance, Epidemiology, and End Results program recorded 434 cases of cutaneous angiosarcoma from 1973 to 2007. The incidence was nearly the same in men (222 cases) and women (212 cases). Most patients were white (88%) with a mean age of 73 years. African Americans made up only 4% of the cases. Two hundred seventy (62%) cases were tumors of the head and neck, whereas 106 (24%) cases arose in the skin of the trunk. Grade was recorded in 194 cases (45%): 28 were grade I, 44 were grade II, 60 were grade III, and 62 were grade IV. Survival rates of cutaneous angiosarcoma correlated with age, anatomical site, and stage of disease. Patients younger than 50 years had a 10-year relative survival rate of 71.7%, whereas patients 50 years and older had a 36.8% 10-year survival rate. Tumors of the scalp and neck resulted in a 13.8% 10-year relative survival rate, whereas tumors arising in the trunk resulted in a 75.3% 10-year survival rate. Tumors localized to the skin had better prognosis (53.6% 10-year relative survival rate) than those with regional or distant stage (19.0% and 6.2%). Twenty-six percent of patients with angiosarcoma had a prior primary. Cutaneous angiosarcomas arise predominantly in the head and neck of white individuals older than 60 years.
Assuntos
Hemangiossarcoma/epidemiologia , Sarcoma/epidemiologia , Neoplasias Cutâneas/epidemiologia , Adolescente , Adulto , Negro ou Afro-Americano , Idoso , Idoso de 80 Anos ou mais , Feminino , Hemangiossarcoma/mortalidade , Hemangiossarcoma/patologia , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Vigilância da População/métodos , Prognóstico , Estudos Retrospectivos , Programa de SEER , Sarcoma/mortalidade , Sarcoma/patologia , Distribuição por Sexo , Neoplasias Cutâneas/mortalidade , Neoplasias Cutâneas/patologia , Taxa de Sobrevida , Fatores de Tempo , Resultado do Tratamento , População Branca , Adulto JovemRESUMO
CONTEXT: Neuroendocrine tumors of the ampulla of Vater constitute a heterogeneous group of neoplasms clinically and morphologically. Because they are rare, little is known about their demographics and biologic behavior. OBJECTIVE: To analyze the demographics and the 5- and 10-year relative survival rates of 139 patients with carcinoid tumors and high-grade neuroendocrine carcinomas of the ampulla. DESIGN: Using data from National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) Program from 1973 to 2006, we analyzed the demographics, morphology, and survival of patients with carcinoids and neuroendocrine carcinomas of the ampulla. RESULTS: SEER recorded 6081 cases of malignant neoplasms of the ampulla, of which 82 were carcinoid tumors and 57 were high-grade neuroendocrine carcinomas. Of these 57, 42 were neuroendocrine carcinomas, not otherwise specified; 9 were small cell carcinomas; and 6 were large cell neuroendocrine carcinomas. The incidence was higher in men than in women. Patients with carcinoid tumors were younger (mean age, 61.6 years) than those with high-grade neuroendocrine carcinomas (mean age, 67.5 years). Carcinoid tumors were smaller than high-grade neuroendocrine carcinomas. The frequency of lymph node metastasis was 28.5% for carcinoid tumors and 62% for high-grade neuroendocrine carcinomas. The 5- and 10-year relative survival rates of patients with carcinoid tumors were 82% and 71%, respectively. The 5- and 10-year relative survival rate of patients with high-grade neuroendocrine carcinomas was 15.7%. CONCLUSIONS: Carcinoids of the ampulla of Vater are relatively rare. Carcinoids and high-grade neuroendocrine carcinomas of the ampulla are biologically and clinically similar to these tumors arising in other sites. Carcinoids were smaller and metastasized less frequently than high-grade neuroendocrine carcinomas.