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Endocrine Abstracts (2018) 56 P152 | DOI: 10.1530/endoabs.56.P152

1Service of Endocrinology and Nutrition, University Healthcare Complex of Salamanca, Salamanca, Spain; 2Service of Genetics, University Healthcare Complex of Salamanca, Salamanca, Spain; 3Service of Otolaryngology, University Healthcare Complex of Salamanca, Salamanca, Spain.


Introduction: Tumors derived from the paraganglionic system are rare. 90% of them are located on the adrenal gland, and the remaining 10% are extra-adrenal. Within this last group, 85% are located in the abdomen, 12% in the thorax, and 3% in the head and neck region (HNPG). Its proximity to important structures represents a great difficulty for resection. This makes it necessary to refer these patients to reference centers with multidisciplinary teams and specific surgical training.

Objectives: To determine whether there is a correlation between the genetic mutation of the head and neck paragangliomas, their location and the presence of metastasis.

Methods: Retrospective study. Inclusion criteria: patients with head and neck paragangliomas (HNPG) treated in our hospital between the years 2000 and 2016. In total, 97 patients were included, 65 women (66%) and 32 men (33%), with an age range of 14–84 years (mean age: 49 years). All of them were surgically removed. The SDHB, C and D genes were studied.

Results: Ninety-eight percent of the tumors were nonfunctional (normal metanephrines in urine after 24 h). Out of the 97 patients, 24 (23%) showed genetic mutations. Within this group, 9 patients had the SDHB mutation (41%); 8 patients had the SDHC mutation (37%); and 5 patients had the SDHD mutation (20%). There was no correlation between the genetic mutation and the location of the tumor. In the patients with SDHB mutation, 11% had a metastasis on diagnosis, compared with 3% in the group with sporadic tumors. Vagal HNPG represented 11% (10 patients) and had a higher malignacy rate: 15% of the cases.

Conclusions: In the HNPC in our series, the most frequently found mutation was SDHB, which was not associated with the location and which showed shorter survival in cases with metastasis.

Volume 56

20th European Congress of Endocrinology

Barcelona, Spain
19 May 2018 - 22 May 2018

European Society of Endocrinology 

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