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. 2010 Jan;2(1):12-6.
doi: 10.4103/0974-7796.62919.

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

Jin Wen  1 , Han-Zhong Li, Zhi Gang Ji, Quan Zhong Mao, Bing Bing Shi, Wei Gang Yan
Affiliations

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

Jin Wen et al. Urol Ann. 2010 Jan.

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.

Keywords: Extra-adrenal paraganglioma; diagnosis; retroperitoneal tumor; treatment.

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Conflict of interest statement

Conflict of Interest: None.

Figures

Figure 1a
Figure 1a
MSCT shows a round or similarly round retroperitoneal tumor with inner central hemorrhage and necrosis. Tumors show obvious intensification after injection of constrast medium. a) Multiple extra-adrenal retroperitoneal paragangliomas. Coronal contrastenhanced reformatted CT scan shows two lobulated soft-tissueattenuation masses (arrows) above the aortic bifurcation
Figure 1b
Figure 1b
MSCT shows a round or similarly round retroperitoneal tumor with inner central hemorrhage and necrosis. Tumors show obvious intensification after injection of constrast medium. b) Coronal contrast-enhanced reformatted CT scan shows one lobulated soft-tissue-attenuation masses (arrows) near the right renal hilum
Figure 2a
Figure 2a
Tissue sample acquisition; a) The speciman of paraganglioma after operation showed integrated amicula and abundant blood supply
Figure 2b
Figure 2b
Tissue sample acquisition; b)There was a large area remote hemorrhage and necrosis inside of tumor
Figure 3
Figure 3
Under light microscope, the tumor cells were same in size with distinct boundary. Tumor was an alveolar-like structure with many vascular septa. Mitotic structures and nuclear atypia are infrequent
See this image and copyright information in PMC

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