Pheochromocytoma: A three-decade clinical experience in a multicenter study

P Iglesias; E Santacruz; P García-Sancho; A P Marengo; F Guerrero-Pérez; H Pian; C Fajardo; C Villabona; J J Díez

doi:10.1016/j.rce.2019.12.016

Pheochromocytoma: A three-decade clinical experience in a multicenter study

Rev Clin Esp. 2021 Jan;221(1):18-25. doi: 10.1016/j.rce.2019.12.016. Epub 2020 Jul 6.

[Article in English, Spanish]

Authors

P Iglesias¹, E Santacruz², P García-Sancho³, A P Marengo³, F Guerrero-Pérez³, H Pian⁴, C Fajardo⁵, C Villabona³, J J Díez⁶

Affiliations

¹ Servicio de Endocrinología, Hospital Universitario Ramón y Cajal, Madrid, España; Servicio de Endocrinología, Hospital Universitario Puerta de Hierro, Majadahonda, Madrid, España. Electronic address: piglo65@gmail.com.
² Servicio de Endocrinología, Hospital Universitario Ramón y Cajal, Madrid, España.
³ Servicio de Endocrinología, Hospital Universitario Bellvitge, L'Hospitalet de Llobregat, Barcelona, España.
⁴ Servicio de Anatomía Patológica, Hospital Universitario Ramón y Cajal, Madrid, España.
⁵ Servicio de Endocrinología, Hospital Universitario de La Ribera, Alcira, Valencia, España.
⁶ Servicio de Endocrinología, Hospital Universitario Ramón y Cajal, Madrid, España; Servicio de Endocrinología, Hospital Universitario Puerta de Hierro, Majadahonda, Madrid, España.

PMID: 32646754
DOI: 10.1016/j.rce.2019.12.016

Abstract

Objective: To analyze the clinical and analytical features, diagnostic tests, therapies, and outcomes of pheochromocytoma (PCC).

Design and methods: A multicenter retrospective study in surgically treated patients with PCC followed in 3 Spanish tertiary referral hospitals.

Results: A total of 106 patients (61 [57.5%] women, mean age 52.3 ± 14.8 years) were evaluated. At diagnosis, PCC was symptomatic in 62% and sporadic in 83%. Patients with familial PCC were significantly younger than those with sporadic disease (40.8 ± 14.2 years vs. 54.5 ± 13.9 years, p<.001). Familial PCCs were more frequently associated with MEN2A (n=8). Levels of 24-h urinary fractionated metanephrines were positively related to tumor size. The maximum tumor diameter was 4.3cm (3-6cm); 27.7% of the patients had tumors ≥6cm. Incidental PCCs were significantly smaller than symptomatic PCCs (3.4cm [2.4-5.0cm] vs. 5.6cm [4.0-7.0cm], p<.001). Scintigraphy by ¹²³I-metaiodobenzylguanidine showed a high sensitivity (81.9%). Preoperative alpha blockade with phenoxybenzamine was used in 93.6% and doxazosin in the rest. Laparoscopic surgery was used in 2/3 of the patients, with a low conversion (1.9%) to open surgery. Perioperative complications appeared in approximately 20% of patients, mainly hypertensive crisis (9.4%). Recurrent disease appeared in 10%, and malignant PCC was uncommon (6.3%).

Conclusions: PCCs surgically treated in Spain are usually large, symptomatic, and sporadic tumors diagnosed around the sixth decade of life. Hereditary PCC is usually associated with MEN2A. The main type of surgical technique used is laparoscopic surgery, and the prevalence of metastatic PCC is low.

Keywords: Enfermedad de von Hippel-Lindau; Feocromocitoma; Multiple endocrine neoplasia; Neoplasia endocrina múltiple; Neurofibromatosis tipo 1; Neurofibromatosis type 1; Pheochromocytoma; Von Hippel-Lindau disease.