Complete laparoscopic excision of a giant retroperitoneal paraganglioma

摘要

Paragangliomas (or 'extra-adrenal phaeochromocytomas') are rare tumours arising from neural crest cells. They occur most commonly in the abdomen along the paraspinal sympathetic ganglion. The malignancy rate can be up to 35% and surgical resection is the recommended treatment. While laparoscopic excision of phaeochromocytomas is now well established, the overall number of cases of paragangliomas reported is much smaller owing to their rarity (even more so for giant paragangliomas of >= 8cm) and controversy remains over the completeness of excision. Furthermore, the risk of malignancy and recurrence rate are higher in paragangliomas, with incomplete excision giving rise to recurrent endocrinopathy and carcinomatosis. We report the case of a 26-year-old woman who underwent laparoscopic excision of an 8.2cm functioning paraganglioma. Complete resection of the tumour was achieved and the patient's symptoms resolved. Twelve months later, she remains well with no evidence of recurrence.