ECE2023 Poster Presentations Endocrine-related Cancer (62 abstracts)
Multidisciplinary Management of Paragangliomas in a Reference Unit Hospital in Spain
Maria Isabel Del Olmo Garcia 1 , Stephan Prado 2 , Rosana Palasi 3 , Angel Segura 4 , Ignacio Llópez 5 , Maria Ana Estornell 6 , Alvaro Torres 7 , Neus García Albors 1 , Carlos Zac Romero 8 , Monica Ballesta 9 , Francisca Moreno 10 , Julia Balaguer 11 , Jorge Cortés Saez 12 , Maria Jose Villar 1 , Antonia Perez-Lázaro 1 , Pilar Bello 2 & Juan Francisco Merino Torres 1
1University Hospital La Fe, Endocrinology and Nutrition, Valencia, Spain; 2University Hospital La Fe, Nuclear Medicine, Valencia, Spain; 3University Hospital La Fe, Endocrine surgery, Valencia, Spain; 4University Hospital La Fe, Medical Oncology, Valencia, Spain; 5University Hospital La Fe, Otorhinolaryngology, Valencia, Spain; 6University Hospital La Fe, Radiotherapy, Valencia, Spain; 7University Hospital La Fe, Vascular surgery, Valencia, Spain; 8University Hospital La Fe, Anatomopathology, Valencia, Spain; 9University Hospital La Fe, Radiology, Valencia, Spain; 10University Hospital La Fe, Pediatric Endocrinology, Valencia, Spain; 11University Hospital La Fe, Pediatric Oncology, Valencia, Spain; 12University Hospital La Fe, Pediatric Surgery, Valencia, Spain
Introduction: Paragangliomas and pheochromocytomas (PGGs) are infrequent tumors of the autonomic nervous system that represent a diagnostic and therapeutic challenge. Management and follow-up of PGGs requires specialized knowledge and solid experience, which, given the rarity of these diseases, is available only in highly specialized centers. University Hospital La Fe (Spain) was designated a reference unit on Neuroendocrine Tumors by local sanitary authorities in 2018. The aim of this study is to describe multidisciplinary management of PGGs in our center from Nov 2021-Nov 2022.
Material and Methods: Descriptive and unicentric study that includes 173 patients with PGG evaluated at Hospital Universitari i Politècnic La Fe in Valencia, Spain.
Results: 173 patients have been evaluated (27 pediatric age) during the period november 2021-2022, 27 of them in a first evaluation. Mean adult age at visit 45.6 years (SD). 66% (n=114) of the totality of patients were known germline mutation carriers: 28% MEN 2A, 22% SDHD, 27% SDHB, 3.5% SDHA, 1.7% SDHC, 9.7% VHL, 2.6% FH, 3.5% MEN 2B, MAX 0,5% and 1.7% NF1. 43 (24.8%) were mutation carriers non-affected and 14 (8%) had metastatic disease. During this year all of the patients have been followed in the Endocrinology department (adult or pediatrics), 48%(n=83) of the patients have required follow-up from more than one speciality. Patients have required evaluation during this year by the following specialists: Oncology 14 (8%), Endocrine Surgery 10 (7.5%), Pediatric Surgery 2 (1.1%), Head-neck surgeon 44 (25.4%), Radiotherapy 16 (9.2%). A new diagnosis of PGG tumor was made in 26 patients. All patients have performed urine catecholamine and metanephrine analyses, and 17% were functioning tumors. Imaging tools have included: 27 total body MRI, 30 head-neck MRI, 18 abdominal MRI, 60 PET/CT 68Ga-DOTATOC, 11 123-I-MIBG, 3 PET/CT 18F-DOPA, 56 PET/CT 18F-FDG. Surgical procedures during this period included: 10 adrenalectomies, 8 head and neck surgeries, 1 complex peritoneal surgery of PGG implants. 9 patients required presurgical medication with alpha and beta blockade. Nuclear medicine treatment included: 5 I131-MIBG and 3 Lu-DOTA-TATE. Other treatments include: 4 radiotherapy, 2 chemotherapy and 10 others.
Conclusions: All patients with PGG must be followed by the Endocrinology department following current clinical guidelines. Patients with PGG often require evaluation by more than one specialist and require multidisciplinary management in highly specialized centers which offer surgical, medical and imaging technique expertise.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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