Aorto-iliac paraganglioma: Case report and literature review

Abstract

Introduction: Paraganglioma and pheochromocytoma are uncommon conditions that affect around 1.5-9 patients per million. The most frequent symptoms are headache, hypertension and diaphoresis; however, palpitations or tachycardia could be present. Malignancy is not frequent, and when is suspected, positron emission tomography (PET) should be performed. Surgery it's the gold standard treatment, with acceptable rates of morbidity and mortality.

Presentation of the case: A 33-year-old woman presented to private practice with long-standing symptoms consisting of asthenia, adynamia, and sensation of palpable masses in the neck. Due to her medical history and imaging findings, urine metanephrines were obtained, showing high values of adrenaline 6.69 (μg/24 h), noradrenaline 130.09 (μg/24 h), dopamine 262.59 (μg/24 h). PET was performed to identify hyperfunctioning masses in other locations, finding bilateral carotid hypermetabolic masses and a nodular lesion anterior to the aortoiliac bifurcation, probably malignant. Laparoscopic retroperitoneal tumor resection was performed by a laparoscopic and metabolic surgeon, with intraoperative findings of a vascularized mass (30 × 25 mm) closely related to the left aortoiliac bifurcation and peritoneal fluid.

Discussion: Paragangliomas are rare tumors that frequently produce catecholamines with varied symptoms. Diagnosis requires patient history, laboratory studies including 24-hour urine-metanephrines and plasma metanephrine levels. Imaging such as CT, MRI and PET scan are necessary. Perioperative management needs to be performed and surgery is the basis of the treatment in patients with localized disease. Metastatic disease has a 50% mortality at 5 years and requires a different approach.

Conclusion: Paraganglioma is a rare and complex entity that requires a multidisciplinary approach.

Keywords: Case report; Diagnosis; Laparoscopy; Paraganglioma; Surgery.

Fig. 1

Axial Computed Tomography Angiography of the neck vessels showing findings of bilateral vagal paragangliomas with small involvement of the carotid body, the largest on the right side (see red asterisks in A and B).

Fig. 2

PET/CT with maximum grayscale projection, demonstrating bilateral vagal paragangliomas and a nodular lesion anterior to the aortoiliac bifurcation (see A). 18F-FDG-PET/CT revealing strongly FDG-avid masses in bilateral neck and in the vicinity of the left aortoiliac bifurcation (see B). PET/CT fusion image showing abnormal increase of tumor metabolism (see C).

Fig. 3

Vascularized mass anterior to the left aortoiliac bifurcation (see A). Intraoperative photos depicting the dissection of the mass (see B and C). Surgical specimen obtained (see D).