Diagnostic and surgical challenges of a giant pheochromocytoma in a resource limited setting—A case report

Abstract

INTRODUCTION: Pheochromocytomas are catecholamine producing tumours which arise from chromaffin cells within the adrenal medulla. Patients with these tumours commonly present with a triad of headache, palpitations and hypertension. CASE PRESENTATION: We present a case of a 37-year-old male patient who presented with dull left sided abdominal pain and discomfort for 6 weeks. A preoperative Computed tomography (CT) scan showed a huge left suprarenal tumour but urinary vanillylmandelic acid (VMA) were negative. The patient underwent an open surgical resection via an extraperitoneal approach without untoward intraoperative and postoperative events. Histopathological evaluation of the specimen showed a pheochromocytoma with a PASS score of 9. The successful management of the patient highlights the good results of team work despite the limitations of preoperative diagnosis. DISCUSSION: Giant pheochromocytomas by definition are tumours more than 7 cm in size and are rare. They rarely secrete catecholamines and commonly present with vague abdominal symptoms. A computerized tomogram helps suggest the diagnosis whilst the biochemical workup for pheochromocytoma may be diagnostic. If the tumours are biochemically active, preoperative alpha-blockade is necessary and care must be taken at operation in handling the tumour. The surgical and anaesthetic team must be prepared to manage hypertensive crisis should it occur. CONCLUSION: This case brings to the attention of clinicians the need to have a high index of suspicion of a giant pheochromocytoma in a patient presenting with vague abdominal symptoms whose CT scan shows a large retroperitoneal tumour, even in the absence of clinical symptoms and negative or absent biochemical workup.