Case:A 59-year-old female, with no history of endocrine tumours or familial cancer syndromes, had a laparoscopic partial left adrenalectomy for a left adrenal nodule concerning for lung squamous cell carcinoma oligometastasis. The surgery was complicated by acute pulmonary oedema and severe cardiomyopathy, which required stabilisation with an intra-aortic balloon pump. The Intensivist and Cardiologist diagnosed catecholamine surge as the most likely cause. Histology from the surgery demonstrated a ganglioneuroma.
20 years later, the patient was referred to our Endocrinology Clinic for a 2.6 cm left adrenal nodule. Clinically, the patient was hypertensive and reported headaches, sweating, and palpitations; biochemically, plasma normetanephrine 2970 (<1560) and metadrenaline 1280 (<447) were elevated. The adrenal lesion was avid on F-DOPA PET. As such, suspicions were raised for a phaeochromocytoma. The patient subsequently underwent an uncomplicated left re-do retroperitoneoscopic completion adrenalectomy, with histology confirming a low-risk phaeochromocytoma.
The two adrenal specimens, from the first stage partial adrenalectomy and second stage completion adrenalectomy, were reviewed at our MDT meeting. The histologies were unequivocally different, with no phaeochromocytoma tissue evident on the first specimen, and no residual ganglioneuroma evident on the second specimen. We postulate that, based on her complications during the first surgery, she must have had a phaeochromocytoma present – in addition to the ganglioneuroma – which was left behind and has grown over the intervening years.
Discussion:Composite phaeochromocytomas are rare neuroendocrine tumours, comprising phaeochromocytoma and neurogenic components1. There are 110 published cases2. They may present heterogeneously like ordinary phaeochromocytomas and are ideally managed surgically with a multidisciplinary approach3. Our case required curative surgical resection 20 years after the initial surgery, which appears to be the first case of its kind. Clinicians should maintain a high index of suspicion for this condition, as more research is vital to fully understand its behaviour and outcomes.