Giant parathyroid adenoma and challenges with preoperative differentiation from malignancy

Giant parathyroid adenomas are rare and underreported benign tumours of parathyroid gland. Preoperative differentiation between giant parathyroid adenoma (GPA) and parathyroid carcinoma can be challenging, as they both may present as a large parathyroid tumour with hyperparathyroidism. Very few GPAs had been described in the literature, with only 60 cases including our present case. We report a case of GPA and the preoperative diagnostic challenges we faced in differential diagnosis of a potential parathyroid malignancy. As a parathyroid malignancy could not be excluded preoperatively the parathyroid tumour was excised en bloc with surrounding thyroid lobe. The histology was contributory to exclude parathyroid malignancy confirming a benign GPA.

Hyperparathyroidism in pregnancy

Hyperparathyroidism is a rare disease during pregnancy, which has increased risks, including miscarriage and fetal growth restriction. However, the diagnosis of hyperparathyroidism is frequently not recognised or delayed as symptoms are non-specific and calcium is not routinely measured. With a thorough medical history and clinical suspicion, early diagnosis and treatment can reduce the risk of some pregnancy complications. A 35-year-old woman presented at 13/40 with hyperemesis gravidarum. She had elevated serum calcium and a parathyroid lesion on ultrasound. She underwent a parathyroidectomy with rapid normalisation of her calcium. Histopathology confirmed a parafibromin-deficient parathyroid tumour, suggestive of hyperparathyroidism jaw tumour syndrome. At 30/40, she presented with pre-eclampsia (hypertension, hyper-reflexia, proteinuria and intrauterine growth restriction) and had a caesarean section at 30+1/40, delivering a male infant, 897 g (fifth percentile). She had a prior 12-month history of chronic constipation and nephrolithiasis but was not investigated further despite elevated calcium (2.82 mmol/L).

Ultrasonography alone can reliably locate parathyroid tumours and facilitates minimally invasive parathyroidectomy

Introduction Minimally invasive parathyroidectomy (MIP) is performed via a short incision (≤3cm). Previous studies have employed multiple imaging modalities including ultrasonography, sestamibi imaging and/or intraoperative parathyroid hormone assay. We present our eight-year experience of MIP using ultrasonography alone. Methods One hundred parathyroidectomies performed by a single surgeon between April 2004 and December 2012 were identified in a prospectively maintained database. All patients underwent ultrasonography including preoperative marking of the lesion by a single radiologist. No other localising diagnostic tests were performed. Results Of the 100 patients (69% female) who underwent parathyroidectomy, 93 had MIP. The median age of all cases was 58 years (range: 19–90 years). All patients exhibited an elevated parathyroid hormone level (median: 19pmol) in the presence of hypercalcaemia (median: 2.86mmol/l, range: 2.54–3.94mmol/l). Conventional surgery was indicated in seven patients owing to the need for concurrent thyroidectomy. The median operative time was 30 minutes (range: 10–130 minutes). Ultrasonography localised parathyroid tumour position correctly in 98% of patients who underwent MIP, and in 97% across both MIP and non-MIP groups. Postoperative complications requiring treatment included pancreatitis and symptomatic hypocalcaemia. Follow-up review at 6–8 weeks demonstrated that 86% of open cases (6/7) and 94% of MIP cases (87/93) were rendered normocalcaemic. Conclusions Our study is the first to demonstrate that the sole use of ultrasonography including preoperative marking can localise parathyroid tumours correctly in 98% of cases suitable for MIP.

Tumour nuclear oestrogen receptor beta 1 correlates inversely with parathyroid tumour weight

Primary hyperparathyroidism (PHPT) is a common endocrinopathy, frequently caused by a parathyroid adenoma, rarely by a parathyroid carcinoma that lacks effective oncological treatment. As the majority of cases are present in postmenopausal women, oestrogen signalling has been implicated in the tumourigenesis. Oestrogen receptor beta 1 (ERB1) and ERB2 have been recently identified in parathyroid adenomas, the former inducing genes coupled to tumour apoptosis. We applied immunohistochemistry and slide digitalisation to quantify nuclear ERB1 and ERB2 in 172 parathyroid adenomas, atypical adenomas and carcinomas, and ten normal parathyroid glands. All the normal parathyroid glands expressed ERB1 and ERB2. The majority of tumours expressed ERB1 (70.6%) at varying intensities, and ERB2 (96.5%) at strong intensities. Parathyroid carcinomas expressed ERB1 in three out of six cases and ERB2 in five out of six cases. The intensity of tumour nuclear ERB1 staining significantly correlated inversely with tumour weight (P=0.011), and patients whose tumours were classified as ERB1-negative had significantly greater tumour weight as well as higher serum calcium (P=0.002) and parathyroid hormone levels (P=0.003). Additionally, tumour nuclear ERB1 was not expressed differentially with respect to sex or age of the patient. Levels of tumour nuclear ERB2 did not correlate with clinical characteristics. In conclusion, decreased ERB1 immunoreactivity is associated with increased tumour weight in parathyroid adenomas. Given the previously reported correlation with tumour-suppressive signalling, selective oestrogen receptor modulation (SERMs) may play a role in the treatment of parathyroid carcinomas. Future studies of SERMs and oestrogen treatment in PHPT should consider tumour weight as a potential factor in pharmacological responsiveness.

In the labyrinth of calcium metabolism

The authors present the case of a 27-year-old male patient. In 2010, he suffered from a bone fracture of the pelvis. As imaging techniques showed multiple osseal lytic lesions, diagnostic investigations were performed for multiple myeloma. Later, a mass lesion measuring 37 mm in size was removed from the left side of his mandible. Histology revealed a giant-cell tumour of the bone and oncologic therapy was considered. However, before this planned treatment a PET-CT was performed, which showed numerous distinct lesions with enhanced glucose metabolism in the skeleton as well as in soft tissue behind the right lobe of the thyroid. Hence, the patient was referred to endocrinologists. On the basis of severe hypercalcemia (serum calcium 3.66 mmol/l) and high serum parathyroid hormone level (162.5 pmol/l) the diagnosis of a right sided parathyroid tumour was established. After surgical excision of the parathyroid tumour, high levels of serum calcium and parathyroid hormone returned to normal. Histology failed to show malignancy and the patient recovered soon. This case report may shed some light on the importance of serum calcium measurements and the differential diagnostic significance of primary hyperparathyroidism. Orv. Hetil., 2013, 154, 351–355.