Primary gallbladder paraganglioma: A case report and review of literature

Abstract

Introduction: Paragangliomas are tumors arising from paraganglia of the autonomic nervous system. They are rare tumors and occurrence inside the gallbladder is exceptionally rare. Biliary paragangliomas are thought to be associated with the parasymphathetic fibers and are therefore non-functioning and benign. There are less than 10 cases reported in literature and majority are found incidentally upon cholecystectomy. There is no specific treatment for these tumors and resection is considered sufficient.

Case presentation: 63 year old female presented with recurrent bliary colic exacerbated by fatty food. She underwent imaging work up that was consistent with biliary dyskinesia. She underwent uneventful elective laparoscopic cholecystectomy and was doing well post-operatively. Pathology report was significant for chronic cholecystitis, no calculi, and a small focus of paraganglioma.

Discussion: Little is known about primary gallbladder paragangliomas. Due to the non-functioning nature of these tumors there are felt to be benign. We know the paraganglia of the gallbladder consists of both parasymphathetic and sympathetic fibers. The sympathetic paragangliomas tend to act similar to pheochromocytomas and thus have malignant potential.

Conclusion: We presented a case in which a primary gallbladder paraganglioma was identified incidentally in a patient who presented with symptomatic biliary dyskinesia. Due to the rarity of primary gallbladder paraganglioma and limited reported cases, optimal follow up remains unknown.

Keywords: Biliary; Case report; Gallbladder; Paraganglioma.

Fig. 1

(40×) Representative gallbladder with changes of chronic cholecystitis, including areas of benign-appearing mucosa that are dilated and extend into the muscularis, consistent with Rokitansky-Aschoff sinuses.

Fig. 2

(40×) Gallbladder with subserosal paraganglioma.

Fig. 3

(200×) Higher power view of the paraganglioma showing an organoid, nested island of chief cells with inconspicuous sustentacular cells and capillary network at periphery in the subserosal adipose tissue of the fundus/body of the gallbladder. There are no mitotic figures identified.

Fig. 4

(200×) Positive labeling with Synaptophysin immunohistochemistry.