A decade of clinical experience with extra-adrenal paragangliomas of retroperitoneum: Report of 67 cases and a literature review

• Main Authors: Wen Jin, Li Han-Zhong, Ji Zhi, Mao Quan, Shi Bing, Yan Wei
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2010-01-01
• Series: Urology Annals
• Subjects: Extra-adrenal paraganglioma; retroperitoneal tumor; diagnosis; treatment
• Online Access: http://www.urologyannals.com/article.asp?issn=0974-7796;year=2010;volume=2;issue=1;spage=12;epage=16;aulast=Wen

<b>Object:</b> The purpose was to highlight the diagnosis and treatment of extra-adrenal para-gangliomas, which often causes catecholamine hypersecretion and hypertension. <b> Methods:</b> 67 cases of extra-adrenal paraganglioma of retroperitoneum proven pathologically from 1999 to 2009 were reviewed and studied after operation. Endocrine secretion examinations, B-US, CT, MRI, 131-MIBG, octreotide and hands microcirculation inspection were used to diagnose the disease. <b> Results:</b> All patients underwent successful surgical resection of the tumors, which proved to be paragangliomas. They were from 3 cm to 25 cm in size. Almost all of them were diffusely positive for cgA, syn, NSE and s-100 by immunohitochemical staining. There were nine cases assayed malignant paraganglioma by the follow-up. <b>Conclusions:</b> 131-MIBG and octreotide have high sensitivity and accuracy in diagosing extra-adrenal paraganglioma. Surgical treatment should be carried out on the basis of correct drug preparation of a-receptor blocker, such as prazosin and phenoxybenzamine. Complete surgical excision is the treatment of choice for extra-adrenal paragangliomas as well as recurrent or metastatic disease, which could be resected laparoscopically. Intimate lifelong follow-up is necessary and important.