ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Endocrine Abstracts (2019) 63 P823 | DOI: 10.1530/endoabs.63.P823

Pheochromocytoma affecting Pregnancy: still searching the needle in the haystack?

Katharina Langton1, Georgiana Constantinescu2, Susan Richter1, Laura Gieldon3, Jacques Lenders4,5 & Graeme Eisenhofer1,5

1Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany; 2Department of Endocrinology, Internal Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany; 3Institute of Clinical Genetics, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany; 4Department of Internal Medicine, Radboud University Medical Centre, Nijmegen, Netherlands; 5Department of Medicine III University Hospital Carl Gustav Carus, Dresden, Germany.

Introduction: Pheochromocytoma associated with pregnancy carries a great risk for adverse fetal and maternal outcome, especially when the diagnosis is missed. Symptoms can present in a wide variety regarding intensity and duration, making it difficult for the treating midwife or obstetrician to draw correct conclusions.

Clinical Case: We present a case of a 29 years old patient whose third pregnancy was complicated by short, but frequent and intense spells of unbearable headaches and excessive vomiting up to 10 times a day. She began to experience these symptoms, accompanied by occasionally shortness of breath and a feeling of paraesthesia in her face about 6 months prior to this current pregnancy. The patient was referred to several specialists, but no abnormalities of the eyes, head and neck were found. Symptoms were treated with intravenous fluids, dimenhydrinat, metoclopramide and ranitidine. Routine antenatal care revealed no abnormalities, all routine testing including blood pressure measurements, urine and blood glucose testing remained within normal limits. Fetal growth was always satisfactory. After an especially severe episode at term the woman referred herself to the local obstetric unit. Initially her blood pressure was normal and the cardiotocogram (CTG) showed a reassuring trace, but after 2 more episodes of extreme headaches, vomiting and hypertension (blood pressure up to 256/130 mmHg) within few hours after admission and signs of fetal compromise the pregnancy was terminated by emergency caesarean section under spinal anaesthesia. A healthy male newborn was delivered. Postoperatively, extremely fluctuating blood pressure with peaks up to 245/137 mmHg (mean daytime value 163/95) combined with persisting nausea and vomiting led to the suspicion of an underlying catecholamine excess. MRI scanning revealed a tumor measuring 3.3 cm×4.3 cm×2.7 cm in the left adrenal, plasma and urinary normetanephrine were elevated to 944.4 pg/ml (normal <118.3) and 7913 nmol/24h (normal <1950 nmol/L) respectively. Histopathological examination after tumor removal confirmed a pheochromocytoma. In the context of a research protocol, which the patient gave written consent for, very high rates of fumerate were measured in the tumor tissue.The strongly suspected germline FH mutation (associated with renal cell carcinomas, leiomyomas and metastatic disease) was confirmed by genetic testing.

Discussion: This case clearly mirrors the ongoing difficulties in the timely diagnosis of a pheochromocytoma during pregnancy. In the presented case, symptoms appeared frequent, but with a short duration only. This explains why it was possible to miss the symptomatic spells completely during routine antenatal care.

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