Challenging case of perioperative management of paraganglioma
B. Zilaitiene*a (Prof), A. Maksimova-Cesnavicieneb (Dr), A. Dauksac (Prof), A. Macasd (Prof), R. Knispelise (Dr), G. Barauskasc (Prof)
a Department of Endocrinology and Institute of Endocrinology, Lithuanian University of Health Sciences, Kaunas, LITHUANIA ; b Department of Oncology, Lithuanian University of Health Sciences, Kaunas, LITHUANIA ; c Department of Surgery, Lithuanian University of Health Sciences, Kaunas, LITHUANIA ; d Anaesthesiology Department, Lithuanian University of Health Sciences, Kaunas, LITHUANIA ; e Department of Endocrinology, Lithuanian University of Health Sciences, Kaunas, LITHUANIA
Introduction. Pheochromocytomas and paragangliomas are very rare neuroendocrine tumors with an incidence of less than 10 cases per 1 million people. Because of the produced catecholamines, these tumors present with a variety of symptoms, usually imitate other diseases and, if untreated, lead to high cardiovascular morbidity and mortality.
Observations. We present a case report of a 73-year-old woman who presented to a general practitioner's office with an unidentified cause of leukocytosis. She has a known history of type 2 diabetes and arterial hypertension. After further investigation, the unknown origin tumor with the diameter of almost 6 cm was identified in the right suprarenal retroperitoneum. To verify the tumor, a core needle biopsy was performed. A pathologic diagnosis of paraganglioma was obtained. Laboratory tests showed high levels of plasma free Metanephrines (metanephrine and normetanephrine levels were fifteen and five times higher than ULN, respectively), Chromogranin A (eightfold higher than ULN). The patient was hospitalized for preoperative preparation. After 2 weeks of pretreatment with doxazosin (8 mg/d) and additional beta-blocker bisoprolol (10 mg/d), target blood pressure was reached and laparoscopic extirpation of tumor was attempted. However, because of an uncontrolled hypertensive crisis during the surgery, resection of the tumor was not completed, and the patient was transferred to the intensive care unit. A multidisciplinary team decided to prolong preoperative preparation to 3 more weeks, with more aggressive titration of doxazosin till 32mg and metoprolol 200mg daily. After another round of preparation, the second surgery via laparotomy was successfully performed. After removal of paraganglioma, the patient's blood sugar levels and blood pressure became normal, and the patient was successfully discharged from the hospital.
Discussion. Perioperative management of PPGLs might be challenging and these patients should be treated by multidisciplinary teams at centers with appropriate expertise to ensure favorable outcome.
The author has declared no conflict of interest.