Hyperparathyroid crisis is a rare manifestation of parathyroid disease. We present the case of a 53-year-old gentleman with a review of the current literature. He presented in acute renal failure with epigastric pain and vomiting. His serum-corrected calcium (CCa2+) was raised at 5.2 mmol/L, in addition to a massively raised parathyroid hormone (PTH) level (3957 ng/L). Ultrasound studies of the neck revealed a 2 cm well-defined mass inferoposterior to right thyroid lobe. CT scans of the neck showed a normal mediastinum and confirmed no associated lymphadenopathy. Having undergone medical resuscitation for 9 days, a neck exploration revealed a cystic mass, which was excised. Histological investigations revealed a 9.25 g, cystic parathyroid adenoma with no features of malignancy. His PTH and CCa2+ returned to normal postoperatively. This suspicious presentation of benign disease, including a marked elevation in PTH, highlights the challenges facing the endocrine surgeon in dealing with parathyroid disease.
Primary hyperparathyroidism is a rare disease, with hyperparathyroid crisis being one of its unusual manifestations. Large rises in PTH levels in benign parathyroid disease are unusual and have been associated with more sinister diseases [
A 53-year-old gentleman presented with a 2-week history of worsening epigastric pain, vomiting, and constipation. He reported mild confusion but no loss of consciousness. There was no history of polyuria or polydipsia. He reported longstanding gastroesophageal reflux symptoms but no other abdominal history. His medical history was negative for depression and renal calculi. There was no history of carcinoma or any radiotherapy treatment of any kind. He reported no significant family history. On examination he was pale with dry mucous membranes. His chest and abdominal examination were unremarkable. There was no evidence of any bony pain and no palpable lumps in the neck.
At admission, he was tachycardic and hypotensive with an increased respiratory rate. He was in acute renal failure, with an elevated urea (19.5 mmol/L) and creatinine (272
USS neck revealing 3.69 cm × 2.11 cm lesion in right side of neck.
CT neck showing large right sided neck lesion.
The patient underwent initial conservative management with aggressive intravenous fluid resuscitation, vitamin D replacement, intravenous loop diuretic treatment, and intravenous bisphosphonate therapy. On day 9, biochemical markers had improved (Urea 5.3, Creatinine 179), and CCa2+ had fallen to 2.99 mmol/L. The patient underwent a neck exploration on day 10. During the exploration, the right inferior gland was found to be large, cystic, soft, and of a brown colour. There was a no evidence of local invasion and no lymphadenopathy. As such, a diagnosis of adenoma was made. Intraoperative PTH assay is not routinely used at our unit and as such was not performed. The right inferior parathyroid gland was excised. The right superior parathyroid gland was normal, and the contralateral neck was not explored.
Histological investigations revealed a 3.5 cm × 2 cm × 1.5 cm encapsulated parathyroid adenoma weighing 9.25 g, composed of chief and oxyphil cells with cystic change. The Ki-67 proliferative index was low (1-2%). There was no local, capsular, or vascular invasion and no other features suggestive of malignancy. Given the suspicious clinical picture, the report was further confirmed at another centre.
The patient recovered well postoperatively, with PTH levels falling to 45 ng/L within a day. Subsequent recovery included a period of hypocalcaemia with a raised Alkaline Phosphatase (ALP), which resolved with calcium replacement within a month.
Primary hyperparathyroidism is a rare disease, where the majority of patients now present asymptomatically after biochemical testing which shows a mildly raised serum CCa2+ and marginally elevated PTH level [
Preoperative and even histological differentiation between benign or malignant pathology is often difficult, and appropriate management requires judgement at the time of surgery as malignant lesions will need more radical procedures. The features suggestive of malignant lesions at the time of surgery would include adherence due to fibrosis, local infiltration, or lymph node involvement. There has been recent work into Preoperative diagnosis based on biochemical grounds with a raised CCa2+ and PTH increasing the index of suspicion for malignant disease suggesting that serum PTH levels are mildly elevated in benign disease, but carcinoma causes levels up to 10 times that of normal [
There are several published cases where benign disease presents suspiciously and PTH in particular reaches levels associated with carcinoma. These include parathyroid cysts, those presenting in developing countries, patients with oxyphil adenomas, and those presenting, as demonstrated in our case, in hyperparathyroid crisis. Parathyroid cysts are rare neck lesions [
Our case of parathyroid crisis is a further example of suspiciously elevated PTH levels, suggestive of parathyroid carcinoma. A recent study published in 2008 looking at just under 300 patients undergoing procedures for hyperparathyroidism showed 2.8% present in crisis [
Management of hyperparathyroid crisis has traditionally involved an emergency parathyroidectomy within 72 hours of presentation, which has a mortality of up to 14% [
In summary, our case involves a gentleman presenting in hyperparathyroid crisis with a massively raised serum PTH level that the authors believe to be one of the highest reported in benign disease published to date. It represents the presentation of benign cystic adenoma mimicking malignant disease. It also reinforces the importance of prompt initial medical management, Preoperative diagnostic and localising studies, and sound operative judgement, highlighting the difficulties facing the endocrine surgeon when dealing with lesions of the parathyroid gland.