ESA-SRB-AOTA 2019

Severe hyperparathyroidism – primary hyperparathyroidism vs parathyroid carcinoma (#612)

Jaime Lin 1 2
  1. Woodlands Health Campus, Singapore
  2. Endocrinology, Tan Tock Seng Hospital, Singapore

Background

 

The most common presentation of Primary hyperparathyroidism (PHPT) is asymptomatic hypercalcaemia. Parathyroid hormone (PTH) levels are generally mildly elevated or inappropriately normal given the hypercalcaemia. Parathyroid carcinoma, in contrast presents with severe hypercalcaemia and PTH elevation between 3 and 10 times of the normal ranges1.  Metabolic bone and renal complications are more common. 

We describe 2 cases of profound hyperparathyroidism (HPT), suspicious for parathyroid carcinoma with varying clinical and biochemical complications.

 

Case 1

K.K, a 55-year-old female referred for elevated Alkaline Phosphatase (ALP) of 627U/L (RR:40-120U/L)+, with corrected calcium (cCa) of 2.72mmol/L (RR:2.15–2.50mmol/L)*, hypophosphatemia of 0.6mmol/L (RR:0.8-1.4mmol/L)~, markedly raised intact PTH (iPTH) of 182pmol/L (RR:0.8-6.8pmol/L)^ and Vitamin D of 9ug/L (RR:20-50ug/L)#. Bone mineral densitometry (BMD) revealed severe osteoporosis.   Thyroid ultrasound (TUS) and MRI neck showed a left parathyroid mass of 3.6x0.6x1.4cm with features suspicious for oesophageal wall involvement.

Hypercalcaemia remained stable with oral hydration. K.K underwent parathyroidectomy 2 months later. 

 

Case 2

J.S, a 40-year-old female with 4-year history of recurrent renal calculi, was referred for severe hypercalcaemia of 3.34mmol/Land  iPTH of 150pmol/L^, phosphate of 0.5mmol/L~, and vitamin D of 17ug/L#. ALP was 125U/L+. TUS showed a 4.8cm well-circumscribed parathyroid nodule. BMD revealed osteopenia.

 

Hypercalcaemia was managed inpatient with intensive intravenous fluids, Calcitonin, Bisphosphonate and Cinacalcet.   A transient improvement was achieved with a nadir cCa of 2.7mmol/L*, which subsequently rise within days to a peak of 3.22mmol/L*. Inpatient parathyroidectomy was performed. 

 

In both cases, no hypocalcaemia or hungry bone syndrome was evident post operatively.  Histologies were consistent with parathyroid adenoma.

 

Conclusion

  • PHPT can be associated with severely raised iPTH greater than 100pmol/L.
  • Despite severely raised iPTH, hypercalcaemia can be mild or resistant.
  • Patients with metabolic bone disease and/or recurrent renal calculi should be screened for HPT.
  • Parathyroid Carcinoma, should be suspected in patients with high PTH levels.