Carotid body tumors in Japan: A multi-institutional study.

Abstract

e17574 Background: Carotid body tumor (CBT) is thought to be a rare disease derived from carotid body paraganglion cells. It is well known that this tumor is related to some hereditary tendencies that linked to such as SDH gene mutations. CBTs have rich vascular contents and capsules supplied by many feeding arteries. When we head and neck surgeons perform resection sugery, we sometimes encounter much blood loss or injuries of carotid arteries. There has been no study to reveal the characteristic features of CBT patients in Japan because of lack of registration and survey system for them. Methods: We attempted to analyze CBT patients all over Japan by integrating information of CBT patients from institutions which had head and neck surgeries. Results: 399 patients were referred to 112 institutes for past 20 years. Among these patients, 150 were registered to our study. There were 87 female and 63 male patients and their mean and median age were 48.0 years old and 49 years old, respectively, ranging from 8 to 78 years old. Eighteen patients had family history of paragangliomas. 15 patients had bilateral CBTs. Among 94 patients who underwent surgery to remove CBT, 23 patients had tumors classified as Shamblin type I, 59 as type II and 12 as type III. Mean operation time of the surgery and the mean amount of blood loss were calculated to compare. Angiography revealed that most frequent feeding artery of these CBTs was ascending pharyngeal artery followed by superior thyroid artery and occipital artery. Preoperative embolization of these arteries was effective to reduce the blood loss but operation time in Shamblin type I and II tumors. Conclusions: Further investigation will be needed to clarify gene mutations for hereditary paragangliomas including CBTs in Japan.