Abstract
Background
A 67-year-old female patient presented to the emergency department for epigastric pain and was diagnosed with a paraganglioma on final pathology following laparoscopic cholecystectomy.
Summary
A 67-year-old female patient presented to the emergency department (ED) with complaints of epigastric pain. Initial laboratory findings were significant for mildly elevated AST, ALT, and alkaline phosphatase, with normal bilirubin. Subsequent ultrasound revealed a vascular lesion of the anterior gallbladder wall measuring 1.0 × 1.3 cm, concerning for possible gallbladder polyp versus malignancy. The patient was discharged from the ED after conservative management with instructions to follow-up outpatient in a surgery clinic but was ultimately lost to follow-up. She then returned to the ED two months later, complaining of chronic right groin pain radiating down her leg. At this time, she underwent CT abdomen/pelvis that again demonstrated the gallbladder mass, with an interval increase in size to 1.2 × 1.6 cm. The patient was admitted for MRI/MRCP, which characterized the mass as a polyp with concerns for extra-anatomic extension anteromedially. Imaging was unable to categorize the lesion as malignant or not definitively.
While gallbladder polyps represent a common etiology of benign gallbladder masses, paragangliomas of the gallbladder are a much rarer entity. Consequently, the patient underwent an uncomplicated laparoscopic cholecystectomy; final pathology revealed a 1 × 1 cm paraganglioma of the gallbladder with negative margins. Patients with asymptomatic polyps less than 1 cm in size with no risk factors for gallbladder adenocarcinoma are typically managed conservatively with serial ultrasound imaging. On the contrary, polyps greater than 1 cm in size are associated with an increased risk for malignancy, warranting management with open cholecystectomy. Meanwhile, symptomatic polyps without risk factors for adenocarcinoma can be managed with laparoscopic cholecystectomy.
Conclusion
Paragangliomas of the gallbladder represent an uncommon subset of gallbladder masses. Surveillance guidelines for gallbladder paragangliomas have yet to be defined, underscoring the importance of further research regarding this rare pathology.
Key Words
paraganglioma, gallbladder; gallbladder polyp; gallbladder cancer
Case Description
Paragangliomas are rare, extraadrenal, catecholamine-secreting neuroendocrine tumors originating from neural crest cell migration during embryogenesis. Each year, approximately only 500 to 1,000 cases of paragangliomas are diagnosed in the United States.1 These tumors can be associated with both the parasympathetic and the sympathetic nervous systems.1,2 Paragangliomas associated with the parasympathetic nervous system tend to be asymptomatic and nonfunctional, whereas those associated with the sympathetic nervous system are typically symptomatic and functional (norepinephrine-secreting). Studies have failed to demonstrate a survival difference in functional versus nonfunctional paraganglioma tumors.2 Typically, these tumors are benign; however, a small percentage may be malignant—more commonly sympathetic-associated tumors—lending to the importance of early detection and subsequent surgical resection with curative intent.1 Most commonly, paragangliomas originate in the adrenal medulla; however, there have been reported cases of their discovery in other anatomical locations, including the carotid body, mediastinum, lung, urinary bladder, the vagus nerve, retroperitoneum, and kidney as well as the hepatic duct.3‒6 In one center that reviewed 22 cases of paraganglioma of the retroperitoneum over a 40-year period at their hospital, 5 ten-year disease-free survival rates were recorded. In patients who had the tumor resected, the five- and ten-year disease-free survival rates were 75% and 45%, respectively, which dropped to 19% and 19% for unresected tumors. In this review, a predictor of survival was complete resection of the tumor.7
Paragangliomas of the gallbladder represent an even rarer subset of paraganglioma tumors, with only a limited number of published case reports. Previous case reports have demonstrated various presenting symptoms, including intermittent right upper quadrant pain, recurrent hematemesis secondary to a hemorrhagic tumor, and in patients with a tumor originating on the hepatic duct, obstructive jaundice.1‒9 Gallbladder paragangliomas can also be found in asymptomatic patients, incidentally discovered when patients undergo abdominal imaging or surgery for other reasons.10 We discuss the case of a middle-aged woman with paraganglioma of the gallbladder.
The patient is a 67-year-old female with a history of hypertension, diabetes, and hyperlipidemia. She initially presented to our hospital for epigastric pain and, on ultrasound, was found to have a vascular lesion of the anterior gallbladder wall that measured 1.0 × 1.3 cm (Figure 1). At that time, the concern was for gallbladder polyp versus malignancy. Her laboratory findings were significant for a mildly elevated ALT, AST, and alkaline phosphatase but normal bilirubin. The patient was given a referral to follow-up in the surgery clinic but never presented.
Figure 1. Transverse View of Well-Vascularized Gallbladder Lesion. Published with Permission
The patient then returned to our emergency department two months later. During the emergency department workup, she had a CAT scan of the abdomen and pelvis that again noted the gallbladder mass along the anterior wall, now measuring 1.2 × 1.6 cm (Figure 2). At that time, she was admitted for MRI/MRCP. The MRI characterized the lesion as a polyp, with bulging of the wall concerning for extraanatomic extension anteromedially, but again was unable to say whether there was an underlying malignancy definitively. The patient was optimized and underwent an uneventful laparoscopic cholecystectomy, finding grossly intact serosa without extension of the lesion into surrounding structures. The decision was made at that time to perform a simple cholecystectomy. The patient recovered without complication from the surgery and was discharged home. The final pathology returned as a 1 × 1 cm paraganglioma with negative margins.
Figure 2. Axial View of Hyper-Vascular Enhancing Gallbladder Mass during CT with IV Contrast. Published with Permission
