A 64yo lady with Carney’s triad presented to the emergency department with abdominal pain. Her pain was severe and lasted several hours, but resolved without intervention. Her past medical history includes left lower lobectomy and gastrectomy for pulmonary chondroma and gastric stromal tumours respectively.
Clinical examination revealed hypertension of 174/94. Her abdomen was soft and non-tender with no palpable masses. The right lobe of the thyroid was mildly enlarged with no other masses palpable in the neck.
Abdominal CT demonstrated a known left adrenal lesion (24 x 13mm) and new left infra-renal mass (16 x 15mm). A screen for secondary causes of hypertension (aldosterone:renin ratio, plasma metanephrines and normetanephrines, and 24 hour urinary free cortisol, catecholamines, metanephrines, and normetanephrines) was negative. A 68Ga-Dotatate PET scan revealed avidity within the infra-renal mass, the right lobe of the thyroid, a lesion in the left neck at the level of C4, but not within the adrenal lesion. This is consistent with a left infra-renal paraganglioma with a head and neck paraganglioma. A fine needle aspirate biopsy of the thyroid lesion was non diagnostic (Bethesda category I). Serum calcitonin <0.6pmol/L (<4.0) and CEA 1.2ug/L (<5.1) were normal.
She will undergo surgery to resect her abdominal paraganglioma due to its malignant potential, followed by a right hemithyroidectomy. Her head and neck glomus vagale paraganglioma will be monitored.
Carney’s triad is a rare multiple endocrine neoplasia syndrome associated with gastric stromal tumour, pulmonary chondromas, paragangliomas, adrenal cortical adenomas and oesophageal leiomyomas1. This case presents a management dilemma for PET Dotatate avid lesions within the abdomen, thyroid and neck. Gastric stromal tumours are malignant, whereas other manifestations are mostly benign1. The condition is not heritable, rather it occurs due to epigenetic hypermethylation of the promoter of SDHC2. Affected patients require lifelong monitoring for development of metachronous tumours.