First reported case of esophageal paraganglioma. A review of the literature of gastrointestinal tract paraganglioma including gangliocytic paraganglioma

Paraganglioma and the variant gangliocytic paraganglioma are rare gastrointestinal tumors. We present the first reported case of an esophageal paraganglioma and a review of the literature. From this review it seems that these tumors can occur at any age and usually present with acute or chronic bleeding with or without abdominal pain. The majority of reported cases originated in the foregut, most commonly the second part of the duodenum. Macroscopically the tumor may be pedunculated, sessile or ulcerated and have been described up to 10 cm in size. There are no reported cases of gut paragangliomas shown to be producing clinically significant amounts of catecholamines. The majority of reported tumors have been benign, only 7% malignant at presentation and all with lymph node metastases. One case developed bone metastases 3 years after excision and another recurred locally. There has been no benefit seen from radiotherapy or chemotherapy to date and it is recommended that all of these tumors are widely excised together with a lymph node resection if possible.