A HEART BREAKING DIAGNOSIS

Abstract

Objective: To report a case of a rare paraganglioma causing blood pressure variability and life-threatening events. Design and method: Authorized consultation of the patient's medical records. Results: A 20-year-old female with a medical history of migraines presented to the emergency department with a 2-day history of painless gross hematuria and dysuria. She denied fever, vomits and gastrointestinal symptoms. During physical examination the patient was pale and sweaty, with tachycardia of 154bpm, temperature of 37.9 °C and blood pressure of 110/65mmHg. Her abdominal exam was notable for moderate right flank and costovertebral tenderness. Blood tests revealed moderate leucocytosis and CRP of 92 mg/L. Reno-vesical radiography showed severe right ureterohydronephrosis and subsequent CT-scan revealed a bulky heterogeneous ovarian mass. Further evaluation revealed normal values of tumour markers. Due to persistent ureterohydronephrosis and an unidentified ovarian mass, an exploratory laparotomy was performed. Surgery came to an early stop as the patient suffered severe hemodynamic instability. The hypothesis of a retroperitoneal neuroendocrine was then confirmed by a five-fold increase in plasma free metanephrines and a positive 123-MIBG scintigraphy. When asked, the patient reported several episodes of lipothymia and two episodes of syncope. She also noted an increased frequency of headache and palpitations episodes. ABPM recording revealed a nondipper profile, with several non-sustained peaks of elevated blood pressure values as well as three severe hypotension records. Alpha-blockage was initiated, followed by beta-blockage. An ECG and echocardiography were performed and revealed no signs of chronic hypertension. The mass was excised by laparotomy and the diagnosis of paraganglioma was confirmed. In postoperative follow-up the patient's blood pressure and catecholamine levels had normalized. Conclusions: Retroperitoneal malignant paragangliomas are rare neuroendocrine tumours. As with pheochromocytomas, tumour production of vasoactive catecholamines lead to symptoms of catecholamine excess of which hypertension is the most common feature. In this case, several hypotension episodes were also present, a common finding with dopamine-producing paragangliomas, which are quite rare and tend to present late with mass effect and malignant spread. Surgical resection is the mainstay of treatment but blood pressure fluctuates dramatically intra- and post-operatively, increasing the risk of cardiovascular complications.