巨大的胸膜肿瘤和严重的低血糖:doge - potter综合征在先前健康的女性。

Nádia Junqueira, João Caldeira, Ricardo Ferreira, Filipe Costa, Joana Silva, Teresa Monteiro, Ângelo Nobre
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引用次数: 0

摘要

doge - potter综合征是一种罕见的副肿瘤综合征,由低血糖和胸膜孤立性纤维性肿瘤组成。这些肿瘤占所有胸膜肿瘤的不到5%,只能通过手术治愈。在这篇文章中,我们报告一个病人表现为严重的低血糖,作为唯一的症状,肿块占据了整个左半胸。病例介绍:54岁女性,严重低血糖,胸片显示左半胸几乎全混浊,计算机断层扫描显示左半胸肿块。手术切除30cm × 18cm × 11cm的肿块,重3195g。术后过程顺利,低血糖立即消失。免疫组化诊断为胸膜孤立性纤维性肿瘤。结论:胸膜孤立性纤维性肿瘤非常罕见。不到5%的患者伴有低血糖,表现为多吉-波特综合征。放疗和化疗反应率低,完全手术切除是唯一能治愈的方法。本病例报告一例罕见的胸膜巨大孤立性纤维性肿瘤伴严重低血糖,经手术成功治疗。术后对患者的长期随访对于发现任何可能的复发是必要的。
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Giant Pleural Tumor And Severe Hypoglycemia: Doege-Potter Syndrome In A Previously Healthy Female.

AIntroduction: Doege-Potter's syndrome is a rare paraneoplastic syndrome, consisting in hypoglycemia and solitary fibrous tumor of the pleura. These tumors represent < 5% of all pleural tumours and can only be cured by surgery. In this article, we report a case of a patient presenting with severe hypoglycemia, as the only symptom, and a mass occupying the entire left hemithorax. Case presentation: A54 year old female with severe hypoglycemia, a chest radiography with almost total opacification of the left hemithorax and a computed tomography scan with a mass in the left hemithorax. Surgery was performed and a mass with 30cm × 18cm × 11cm weighing 3195g was resected. The postoperative course was uneventful with immediate resolution of the hypoglycemia. The immunohistochemistry diagnosis was solitary fibrous tumor of the pleura. Conclusions: Solitary fibrous tumor of the pleura are very rare. Less than 5% are associated with hypoglycemia, taking the form of Doege-Potter Syndrome. Radiation therapy and chemotherapy have shown low response rate and complete surgical resection is the only procedure that offers cure. This case reports describes a rare giant solitary fibrous tumor of the pleura with severe hypoglycemia, successfully treated by surgery. Long-term follow-up of the patient after the surgery is necessary for detection of any possible recurrence.

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