Carotid body tumor (CBT) is a rare disease derived from carotid body paraganglion cells. We organized a study group, the Japan Carotid Body Tumor Research Group (JCBTRG), and initiated a survey of the patients with CBT in Japan.
The study design was a multi-institutional retrospective review of medical records. Research protocols were assessed and accepted by the institutional research boards of individual institutions. A total of 316 responses were sent back to the research bureau and 150 patients were registered in our study.
From 1995 to 2015, 399 patients with CBT were referred to 112 institutions. In summary, 194 patients underwent surgery and 205 patients were under follow-up without surgery. In registered 150 cases, 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. Seventeen patients had family history of paragangliomas. 15 patients had bilateral CBTs. Among 93 patients who underwent surgery to remove CBT, 23 patients had tumors classified as Shamblin I, 58 as Shamblin II and 12 as Shamblin 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 the ascending pharyngeal artery followed by the superior thyroid artery and the occipital artery. Preoperative embolization of these arteries was effective to reduce the blood loss but operation time in Shamblin I and II tumors.
Further investigation would be needed to reveal detailed gene mutations for hereditary carotid body tumors in Japan.