Soo Chun Ian, Ng Boon Hau, Tan Eng Liang, Abdul Hamid Faisal
Respiratory Unit, Medical Department, Hospital Canselor Tuanku Muhriz, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia.
Department of Internal Medicine, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia.
SAGE Open Med Case Rep. 2016 May 30;4:2050313X16650323. doi: 10.1177/2050313X16650323. eCollection 2016.
Pulmonary epithelioid hemangioendothelioma is an uncommon lung malignancy of endothelial origin. Besides demonstrating unpredictable presentation features and prognosis, the paucity of established treatment guidelines remains a challenge in managing these patients. We present two patients. The first patient presented with chronic productive cough over 1-year duration. He was initially diagnosed and showed partial response to treatment for cardiac failure. A persistent right upper zone consolidation on chest radiograph prompted further investigations which revealed the diagnosis of pulmonary epithelioid hemangioendothelioma. The second patient presented with right-sided hemiparesis for 1-month duration. Initial computer tomography scan of the brain showed findings of distant metastatic foci. Subsequent investigations revealed pulmonary epithelioid hemangioendothelioma as the primary lesion. Both patients succumbed without any treatment due to rapid progression of the disease. We believe that pulmonary epithelioid hemangioendothelioma is undoubtedly rarely reported in south-east Asia region. In these two case reports, the patients were diagnosed in west and east Malaysia, respectively, in the same year (2015). Both cases highlight the increasing prevalence of pulmonary epithelioid hemangioendothelioma. We postulate that this could possibly be secondary to the advancement in diagnostic capabilities and improved healthcare facilities available in this region. Late presentation of pulmonary epithelioid hemangioendothelioma generally results in grave prognosis. Further investigations are required to elucidate the nature of progression and therapeutic options for patients with pulmonary epithelioid hemangioendothelioma.
肺上皮样血管内皮瘤是一种罕见的起源于内皮细胞的肺部恶性肿瘤。除了表现出不可预测的临床表现特征和预后外,缺乏既定的治疗指南仍然是管理这些患者的一项挑战。我们报告两例患者。首例患者有超过1年的慢性咳痰症状。他最初被诊断为心力衰竭并接受治疗,治疗后有部分缓解。胸部X线片上右上肺野持续存在的实变影促使进一步检查,最终确诊为肺上皮样血管内皮瘤。第二例患者有右侧偏瘫症状,持续1个月。最初的脑部计算机断层扫描显示有远处转移灶。随后的检查发现肺上皮样血管内皮瘤是原发性病变。由于疾病迅速进展,两名患者均未接受任何治疗而死亡。我们认为,肺上皮样血管内皮瘤在东南亚地区无疑很少见报道。在这两篇病例报告中,两名患者分别于同一年(2015年)在马来西亚西部和东部被确诊。这两例病例均凸显了肺上皮样血管内皮瘤发病率的上升。我们推测,这可能继发于该地区诊断能力的提高和医疗设施的改善。肺上皮样血管内皮瘤的晚期表现通常导致预后不良。需要进一步研究以阐明肺上皮样血管内皮瘤患者的病情进展本质和治疗选择。