RESUMO
Epithelioid hemangioendothelioma (EHE) is a rare indolent vascular tumor which occurs at liver, lung, bone, and so on. However, the etiology of EHE is evasive.These patients were enrolled at the First Affiliated Hospital of Xi'an Jiaotong University from January 2011 to December 2015. Retrospective analysis is done by demographic data of clinical manifestations, laboratory parameters, CT imaging, histological, and immunohistochemical features in 9 cases in Shaanxi.Of the patients, 8 were females (88.9%) and 1 were males (11.1%). The age ranged from 34 to 71 years (mean 49 years; median 49 years). Anatomical sites of primary lesions were as follows: liver (nâ=â6, 66.7%), upper extremities (nâ=â1, 11.1%), sublingual gland (nâ=â1, 11.1%), and spine (nâ=â1, 11.1%). Metastatic disease was diagnosed in 5 cases (55.6%) with occurrence in lung (nâ=â4, 44.4%), bone (nâ=â2, 22.2%), upper extremities (nâ=â1, 11.1%), pleura (nâ=â1, 11.1%), and spleen (nâ=â1, 11.1%). Tumor size ranged from 0.5 to 6.8âcm (mean 3âcm). The most tumors were composed of highly cellular areas with small and prominent nucleoli in vesicular nuclei, and ERG (100%) was the most frequently positive in these cases, followed by CD31 (88.9%) and CD34 (77.8%) via histology and immunohistochemistry techniques.EHE is a very rare in Shaanxi. It is significant to find its clinical, radiological, and pathological characters, helping for EHE early diagnosis and treatment, reducing misdiagnosis and improving life quality.
Assuntos
Hemangioendotelioma Epitelioide/diagnóstico , Hemangioendotelioma Epitelioide/terapia , Adulto , Idoso , China , Feminino , Seguimentos , Mãos , Hemangioendotelioma Epitelioide/patologia , Hemangioendotelioma Epitelioide/fisiopatologia , Humanos , Imuno-Histoquímica , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/fisiopatologia , Neoplasias Hepáticas/terapia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Neoplasias da Coluna Vertebral/diagnóstico , Neoplasias da Coluna Vertebral/patologia , Neoplasias da Coluna Vertebral/fisiopatologia , Neoplasias da Coluna Vertebral/terapia , Neoplasias da Glândula Sublingual/diagnóstico , Neoplasias da Glândula Sublingual/patologia , Neoplasias da Glândula Sublingual/fisiopatologia , Neoplasias da Glândula Sublingual/terapia , Vértebras Torácicas , Tomografia Computadorizada por Raios X , Carga TumoralRESUMO
A rare case of acinic cell carcinoma of the sublingual gland accompanied by bone formation is reported. The patient is a 79-year-old male who was referred to Yokohama Minami Kyosai Hospital with sublingual swelling. A tumor mass, 20 x 10 mm in diameter, was detected on the right side of the floor of the mouth. Computed tomography (CT) revealed a mass lesion with calcification in the sublingual gland. The patient underwent total sialadenectomy of the sublingual gland with conservation of the lingual nerve. Histologically, the lesion showed amylase-positive atypical cells with thyroid gland-like arrangement, and mature bone tissue in the stroma. Based on these findings, the tumor was diagnosed as acinic cell carcinoma accompanied by bone formation. Postoperative recovery was uneventful, and two years after surgery, there are no signs of distant metastases or recurrence.