Challenges and pitfalls in the management of parathyroid carcinoma: 17-year follow-up of a case and review of the literature

Abstract

A 29-year-old man presented to his primary care physician with nausea, severe weight loss and muscle weakness. He had a hard, fixed neck swelling. He was severely hypercalcaemic with 10-fold increased parathyroid hormone (PTH) concentrations. A diagnosis of primary hyperparathyroidism was established and the patient was referred for parathyroidectomy. At neck exploration, an enlarged parathyroid gland with invasive growth into the thyroid gland was found and removed, lymph nodes were cleared and hemithyroidectomy was performed. A suspected diagnosis of parathyroid carcinoma was confirmed histologically. Serum calcium and PTH levels normalised post-operatively, but hyperparathyroidism recurred within 3 years of surgery. Over the following 17 years, control of hypercalcaemia represented the most difficult challenge despite variable success achieved with repeated surgical interventions, embolisations, radiofrequency ablation of metastases and treatment with calcimimetics, bisphosphonates and haemodialysis using low-dialysate calcium. In this paper, we report the challenges and pitfalls we encountered in the management of our patient over nearly two decades of follow-up and review recent literature on the topic.

Fig. 1

Disease course of our patient spanning over 17 years

Fig. 2

Radiological (a pre-RF ablation, b after RF ablation) and biochemical changes after RF ablation of the lung metastasis, demonstrating a severe drop in serum PTH concentrations, which persisted for almost a year

Fig. 3

Radiological and biochemical changes following lobectomy of the lung metastasis, which resulted in only partial improvement in serum calcium and PTH concentrations

Fig. 4

CT scan of the thorax demonstrating a large (4 ×3 × 2 cm) subcarinal mediastinal lymph node metastasis (white arrow) (a, b), which was successfully excised using a transpericardial approach (c)