Nizami Imran, Mohammed Shamayel, Abouzied Mohei El Din
Dr. Imran Yaqoob Nizami, Organ Transplant Center, MBC 96, King Faisal Specialist Hospital and Research Centre, PO Box 3354 Riyadh 11211, Saudi Arabia, T: 966-11-4647272 loc 76164, F: 966-11-4647272 loc 76167,
Ann Saudi Med. 2014 Sep-Oct;34(5):447-9. doi: 10.5144/0256-4947.2014.447.
We describe a case of pulmonary epitheloid hemangioendothelioma (PEH) in a 13 years old girl, the aggressive nature of the tumor in this particular case and the PET CT findings. PEH are rare tumors of vascular origin, first described by Dial and Liebow in 1975. This is an uncommon pulmonary neoplasm, 4 times more common in young women. This tumor can affect multiple organs (lung, liver, bones and soft tissue, skin, heart, central nervous system). However lung and liver represent 2 main locations. Clinical manifestations are variable; typically patients are asymptomatic, and PEH is detected on routine chest radiographs as bilateral small (1 cm or less) nodules in the lungs Diagnosis usually requires a surgical lung biopsy. The prognosis is very unpredictable, with life expectancy ranging from 1 to 15 years. The tumor is usually considered as low to intermediate grade sarcoma. There is no single effective treatment however spontaneous remissions and aggressive behavior has been described.
我们描述了一名13岁女孩患肺上皮样血管内皮瘤（PEH）的病例、该特殊病例中肿瘤的侵袭性本质以及PET CT检查结果。PEH是一种罕见的血管源性肿瘤，于1975年由戴尔（Dial）和利博（Liebow）首次描述。这是一种不常见的肺肿瘤，在年轻女性中更为常见，是男性的4倍。这种肿瘤可累及多个器官（肺、肝、骨和软组织、皮肤、心脏、中枢神经系统）。然而，肺和肝是两个主要受累部位。临床表现多样；典型情况下患者无症状，PEH在常规胸部X线检查中表现为肺部双侧小（1厘米或更小）结节。诊断通常需要进行肺手术活检。预后非常难以预测，预期寿命为1至15年。该肿瘤通常被视为低级别至中级别肉瘤。目前尚无单一有效的治疗方法，不过已有自发缓解和侵袭性行为的相关描述。
