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DOI: 10.1055/s-0034-1372132
A case of primary hyperparathyroidism associated with Cushing's syndrome due to an adrenal cortical adenoma
A 78 year old male patient (Pt) with Epilepsy and monoclonal gammopathy progressing to Plasmocytoma (Stage I to Durie and Salmon, without osteolysis) was referred to our department with a history of hypercalcaemia and impaired fasting glucose.
We conducted an ultrasound examination (US) of the parathyroid gland (PTG), which revealed a hypoechoic 14, 5 × 7 mm structure between the PTG and the mandible. The PTG scintigraphy scan (MIBI) was suggestive of an ectopic submandibular parathyroid tissue and a PTG adenoma (AD) caudally to the left THR lobe. The 24h urinaryfree-cortisol excretion was 3 x higher than the upper normal limit. Plasma adrenocorticotropic hormone (ACTH) level was suppressed (< 5 pg/ml). The low-dose dexamethasone suppression test (DST) failed to suppress Pt's cortisol levels. The high-dose DST showed suppression greater than 50% compared to baseline. In the Corticotropin-releasing hormone (CRH) test, the plasma ACTH level remained suppressed. The pituitary-magnetic resonance imaging (MRI) could not verify any AD. The abdominal MRI verified a low density lesion in the right adrenal gland (34 × 33 × 27 mm). Plasma aldosterone and free-metanephrine level were in the normal range. We also detected by thyroid (THR) US multiple THR nodules (a suspicious one posteriorly to the left THR lobe). Scintigraphy confirmed this nodule of reduced tracer uptake. The Pt underwent THR and PTG subtotal resection and a right laparoscopic adrenalectomy. Histology confirmed the cortisol excreting adrenocortical adenoma (ACA) and the PTH excreting PTG AD. The THR tissue was highly regressive but not malignant. Genetic analysis showed a heterozygotic mutation in Exon 3, Codon 791 of the RET-Protooncogen.
Conclusion: Our findings suggest that the Pt's Cushing Syndrome resulted from an ACA in association with primary hyperparathyroidism most likely representing another variant of multiple endocrine neoplasia type 2.