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.

Diagnostic value of four-dimensional computed tomography and four-dimensional magnetic resonance imaging in primary hyperparathyroidism when first-line imaging was inadequate.

INTRODUCTION: Accurate localisation of the abnormal hyperfunctioning gland with preoperative imaging has a critical role in parathyroid surgery to obtain a successful outcome. This study aimed to evaluate the diagnostic performance of second-line imaging and their contribution to the treatment success in primary hyperparathyroidism when the first-line methods were negative or discordant. METHODS: Among the patients who underwent parathyroidectomy due to primary hyperparathyroidism, 33 who underwent four-dimensional computed tomography and/or four-dimensional magnetic resonance imaging because of negative or discordant first-line imaging results were included. Persistent and recurrent cases were excluded. RESULTS: The majority of the patients were female (84.8%) and the mean age was 59.2 years. Seventeen patients had four-dimensional computed tomography and 25 had four-dimensional magnetic resonance imaging, respectively. Four-dimensional computed tomography and four-dimensional magnetic resonance imaging localised the culprit gland successfully in 52.9% and 84%, respectively. Twenty-five patients in whom single adenoma was detected underwent focused parathyroidectomy. The culprit gland was solitary in 32 cases and one patient had double adenoma. Normocalcaemia was achieved in all cases. Among the 29 patients who completed their postoperative sixth month success rate was 100%. CONCLUSION: Four-dimensional magnetic resonance imaging had high accuracy with fast dynamic imaging in detecting parathyroid adenomas. When the first-line imaging methods were negative or inconclusive, four-dimensional magnetic resonance imaging should be considered primarily since it is cost effective in Turkey and emits no radiation.

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.

Intraoperative nerve monitoring during thyroidectomy: evaluation of signal loss, prognostic value and surgical strategy.

INTRODUCTION: Intraoperative neural monitoring of the recurrent laryngeal nerve has been widely used to avoid nerve injury during thyroidectomy. We discuss the results of the change in surgical strategy after unilateral signal loss surgeries using intermittent intraoperative neural monitoring in a high-volume referral centre. MATERIALS AND METHODS: Details of consecutive patients who underwent thyroidectomy with intermittent intraoperative neural monitoring between January 2014 and December 2017 were prospectively recorded and retrospectively reviewed. Loss of signal was defined as recurrent laryngeal nerve amplitude level lower than 100 µV during surgery. The rate of loss of signal and change in surgical strategy during the operation were evaluated. RESULTS: Loss of signal was detected in 25 (5.4%) of 456 patients for whom intermittent intraoperative neural monitoring was performed. Four patients had anatomic nerve disruption and surgery was completed by an experienced endocrine surgeon making use of intraoperative neural monitoring with continuous vagal stimulation. Staged thyroidectomy was performed on 16 patients with unilateral loss of signal in whom the nerves were intact visually. Postoperative vocal cord paralysis was encountered in 18 of 21 (85.7%) patients with loss of signal, and 16 of 18 (88.8%) were improved during the follow-up period. Patients' voices were subjectively normal to the surgeon postoperatively in 9 of 21 (42.8%) patients who were found to have loss of signal with intact nerves. CONCLUSIONS: Intraoperative neural monitoring can be used safely in thyroid surgery to avoid recurrent laryngeal nerve injury. It enables the surgeon to diagnose recurrent laryngeal nerve injury intraoperatively to estimate the postoperative nerve function and to modify the surgical strategy to avoid bilateral vocal cord paralysis.