Pheochromocytoma associatedwith pregnancy: unexpected favourable outcome in patients diagnosed after delivery

JORGE L SALAZAR VEGA; DRA GLORIA LEVIN; DRA GABRIELA SANSO; ANA VIEITES; REYNALDO GOMEZ; DRA MARTA BARONTINI

JOURNAL OF HYPERTENSION

LIPPINCOTT WILLIAMS & WILKINS

Lugar: Philadelphia; Año: 2014 vol. 32 p. 1458 - 1463

0263-6352

Objective: The objective of this study is to present theclinical findings and outcome of a large cohort of pregnantwomen with pheochromocytoma (PHEO) with the aim tocontribute to the better recognition, detection andmanagement of pregnancy-related PHEO in the populationof pregnant patients with hypertension.Methods: This is a longitudinal follow-up of a singlecohort of 15 patients aged 19?40 years with PHEOassociated with pregnancy. Urinary catecholamines andvanillylmandelic acid (VMA) were analysed. Ret protooncogene,SDHB and VHL mutations were determined ingermline DNA from seven women using PCR followed bydirect sequencing.Results: During pregnancy, all women presented typicalfeatures of catecholamines excess. Nevertheless,biochemical diagnosis was performed only in four out of15 cases during pregnancy and postpartum in theremaining 11. Paroxysmal hypertension was thepredominant pattern. Urinary catecholamines and/or VMAwere increased in all patients. Tumours were adrenal in 13patients and extraadrenal in two. Mutations in the Retproto-oncogene were found in four patients, in the VHLgene in one and in the SDHB gene in one.Antihypertensive treatment resulted in effective control ofblood pressure and all women survived. In the group ofwomen diagnosed postpartum, one foetus demised.Newborns from mothers receiving adequate treatmentsurvived. One woman left the hospital after caesareansection but before PHEO surgery became pregnant againand this gestation ended with maternal-foetal dead.Conclusion: A high index of suspicion in all pregnantwomen presenting hypertension mainly paroxystic duringany gestational phase and/or a history of familial PHEO arethe keys to disclose this important diagnosis.