Diagnosis and surgical treatment of retroperitoneal paraganglioma: A single-institution experience of 34 cases

Abstract

The present study aimed at identifying the clinical, radiological and pathological characteristics of retroperitoneal paragangliomas, and determining the association between the tumor features and the prognosis of patients following surgery. A total of 34 patients with retroperitoneal paragangliomas, who underwent resection between November 1999 and December 2015, were included in the present retrospective study. The patients' demographics, clinical symptoms and signs, tumor functional status, surgical procedure, intraoperative results, tumor pathology, radiological results, and postoperative survival time were recorded and analyzed. Of the 34 patients, the most common type of presenting symptom was abdominal mass (46%), followed by hypertension (39%) and abdominal pain (32%). Functional tumors occurred in 20 patients (59%). Computed tomography (CT) and magnetic resonance imaging revealed soft-tissue masses, with marked enhancement in the arterial phase, indicative of retroperitoneal paragangliomas. The preoperative CT diagnostic accuracy rate between 2010 and 2015 was markedly improved, compared with that between 1999 and 2009. The tumors were primarily located close to the renal arteries and veins surrounding the abdominal aorta and inferior vena cava. With the exception of one malignant paraganglioma, the majority of paragangliomas were positive for chromogranin A, S-100 protein, vimentin and heat-shock protein 90, and exhibited decreased expression of Ki-67 antigen and insulin-like growth factor 2. All tumors were completely removed by surgery. Distant metastasis, but not tumor size, functional status and local invasion, was markedly associated with survival. The preoperative diagnostic accuracy rate of retroperitoneal paragangliomas may be improved by focusing on the predilection sites and CT characteristics. In addition, immunohistochemical markers were useful to determine tumor malignancy. Complete surgical resection was appropriate for all patients and postoperative survival time was identified to be associated with tumor metastasis.

Surgical treatment of abdominal paragangliomas

“…When growing expansively, they may appear between the inferior vena cava and aorta, bilaterally below the renal vessels, and at the anterior aspect of the aortic wall between the inferior mesenteric artery and aortic bifurcation (organ of Zuckerkandl) [4]. Disease presentation in our study reflected this tendency.…”

“…The remaining cases are usually found by chance due to imaging of other abdominal organs (incidentaloma). More infrequently, clinical indications such as abdominal pain or palpable abdominal mass [4,5] lead to specific diagnostic imaging and discovery of paraganglioma. In the presented material paroxysmal hypertension was found in 46.4% of patients.…”

Practical approach to primary retroperitoneal masses in adults

“…In some cases, there is catecholamine hypersecretion ( 2 , 3 , 33 ) . Approximately 40% of retroperitoneal paragangliomas are malignant, usually leading to necrosis and distant metastases ( 27 , 33 , 37 ) . Functional imaging studies are useful for localization of the tumor and of distant metastases, the methods including MIBG scintigraphy, 18 F-FDG PET/CT (Figure 4), and 68 Ga-DOTATATE PET/CT, the last being superior for lesion detection ( 38 , 39 ) .…”

“…Functional imaging studies are useful for localization of the tumor and of distant metastases, the methods including MIBG scintigraphy, 18 F-FDG PET/CT (Figure 4), and 68 Ga-DOTATATE PET/CT, the last being superior for lesion detection ( 38 , 39 ) . Surgical resection is the treatment of choice, even in cases with metastasis, because it is associated with improved survival ( 3 , 37 ) .…”

Postoperative acute kidney failure and incision skin necrosis caused by a giant retroperitoneal paraganglioma

“…Most produce neuropeptides (e.g., catecholamines), grow rapidly, and progress with distant metastasis. [ 1 , 2 , 3 ] Surgical excision is currently the only curative treatment as retroperitoneal paragangliomas appear to be resistant to radiochemotherapy. [ 2 , 3 ] At the 3-month follow-up visit, the proteinuria had resolved and the serum albumin was nearly normal.…”