Severe hypercalcaemia of primary hyperparathyroidism: Could giant adenoma be the real culprit rather than carcinoma?

INTRODUCTION: Hypercalcaemic crisis is a rare manifestation of hyperparathyroidism and occurs in 1.6-6% of patients with primary hyperparathyroidism (pHPT). Although such high serum calcium levels (>14mg/dl) are attributed to malignancy, it is also associated with benign disease of the parathyroid glands. The aim of this study was to evaluate the clinical features and treatment modalities of patients with severe hypercalcaemia who underwent surgery for pHPT. METHODS: The medical records of 537 patients who underwent parathyroidectomy in our department for pHPT between 2005 and 2019 were reviewed retrospectively. Twenty-four (4.4%) of the patients were described as having severe hypercalcaemia. RESULTS: Among 24 patients, 71% were female and the mean age was 55.7 years (range: 40-71 years). The mean serum calcium level at time of diagnosis was 15.9mg/dl (range: 14-22.7mg/dl). According to postoperative pathology reports, solitary adenoma, parathyroid cancer and parathyromatosis were diagnosed with the rates of 87.5%, 8.3% and 4.1% respectively. The mean weight of the solitary parathyroid lesions was 14.9g (standard deviation: 8.9g, range: 4-38g). The mean longest diameter was 2.87cm (standard deviation: 1.4cm, range: 1-5.5cm). Serum calcium levels were within the normal range on the first postoperative day in 75% of the cases. CONCLUSIONS: Severe hypercalcaemia is a rare but urgent condition of pHPT and requires prompt management. Accelerated surgery after adequate medical treatment should be performed. It is important to emphasise that giant adenoma, which is a benign disease, may be a more common cause of severe hypercalcaemia than carcinoma, unlike previously thought.

Spindle epithelial tumour with thymus-like differentiation: report of two cases.

Spindle epithelial tumour with thymus-like differentiation (SETTLE) is a very rare neck malignancy arising from thymic tissue or branchial pouch, mostly seen in the early decades of life. SETTLE is thought to be a low-grade neoplasm but distant metastases have been reported. Fewer than 50 cases have been reported in the PubMed database. We report on the four-year follow-up for a 13-year-old boy, with no signs of recurrence or distant metastasis. In addition, a 45-year-old woman was admitted to hospital with neck mass. Fine-needle aspiration biopsy was compatible with medullary thyroid carcinoma but serum levels of calcitonin were normal. Total thyroidectomy and central compartment lymph node dissection was planned with an initial diagnosis of medullary thyroid carcinoma. At surgery, frozen section analysis converted our diagnosis to SETTLE and the intraoperative strategy was changed. SETTLE should be considered in a patient with normal serum calcitonin levels in the presence of suspicious medullary thyroid carcinoma at fine-needle aspiration biopsy. Frozen section analysis may help to manage the intraoperative strategy. This report describes the first case of SETTLE successfully diagnosed by frozen section analysis.

Parathyromatosis: a very rare cause of recurrent primary hyperparathyroidism - case report and review of the literature.

Parathyromatosis is a rare entity and usually appears as a consequence of the seeding on previous parathyroid surgery which was applied for the secondary hyperparathyroidism. A 63-year-old woman presented with a history of subtotal thyroidectomy 20 years ago and parathyroidectomy due to primary hyperparathyroidism (PHPT) four years ago. Imaging methods revealed multiple parathyromatosis foci on subcutaneous tissue of the neck. En-bloc resection was performed and pathological examination confirmed the diagnosis of parathyromatosis. After an uneventful 10 months, biochemical and radiological tests revealed recurrence on bilateral thyroid lodges. En-bloc resection was performed. The patient has remained well for 24 months after the second operation and has been followed-up with normal parathormone and serum calcium values. To the best of our knowledge, this report describes the twenty-first case of parathyromatosis in PHPT setting in the literature. It should be kept in mind that parathyromatosis may recur at different sites in the neck even in patients with PHPT.