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

Abstract

Object:The purpose was to highlight the diagnosis and treatment of extra-adrenal para-gangliomas, which often causes catecholamine hypersecretion and hypertension.Methods: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.Results: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.Conclusions: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 α-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.

Imaging of Pheochromocytoma and Paraganglioma

“…As mentioned earlier, the 10% estimation for familial or genetic predisposition is an underestimate. Moreover, the rate of metastasis for trunk paragangliomas is much higher than that for adrenal pheochromocytomas, reaching up to 60% in some case series (12,13). As for the location, paragangliomas, also referred to as extra-adrenal pheochromocytomas (EAPs), constitute at least 15% of PPGLs in adults and 30% in children (14).…”

Pheochromocytoma and paraganglioma: nimaging characteristics

“…Although the rule of 10s is a helpful approximation, current research has stressed the increased importance of genetic predisposition in the development of pheochromocytomas and paragangliomas. Furthermore, the rate of metastases varies widely depending on the tumor origin [ 3 ] . Only 2–10% of pheochromocytomas metastasize compared with 20–70% of extraadrenal paragangliomas [ 3 ] .…”

“…Furthermore, the rate of metastases varies widely depending on the tumor origin [ 3 ] . Only 2–10% of pheochromocytomas metastasize compared with 20–70% of extraadrenal paragangliomas [ 3 ] .…”

Resection of a Catecholamine-Elaborating Retroperitoneal Paraganglioma Invading the Inferior Vena Cava

“…All patients with periaortic or pericaval masses who were screened for catecholamine excess were discovered to have hyperfunctioning tumors, 95% resulting in hypertension [ 1 ]. A 2010 review of 67 retroperitoneal paraganglioma cases found similarly that 57% were periaortic (without further specification) and 82% were hyperfunctional [ 2 ]. Our case supports the data of periaortic paragangliomas typically being hyperfunctional.…”