Giant parathyroid tumours in primary hyperparathyroidism: a systematic review.

Purpose: Giant parathyroid adenoma (GPA) can present with severe biochemical derangement similar to the clinical presentation of parathyroid carcinoma (PC). This study aims to present the current evidence on surgical management of GPAs in primary hyperparathyroidism.

Methods: A systematic review of the literature on GPAs was conducted following the PRISMA guidelines. Data on clinical, biochemical, preoperative diagnostic, and surgical methods were analysed.

Results: Sixty-one eligible studies were included reporting on 65 GPAs in eutopic, ectopic mediastinal, and intrathyroidal locations (61.5%, 30.8%, and 7.7%, respectively). A palpable neck mass was present in 58% of GPAs. A total of 90% of patients had symptoms including fatigue, skeletal pain, pathological fracture, nausea, and abdominal pain. Ninety percent of patients had significant hypercalcaemia (mean 3.51 mmol/L; range: 2.59-5.74 mmol/L) and hyperparathyroidism with PTH levels on average 14 times above the upper limit of the normal reference. There was no correlation between the reported GPA size and PTH nor between GPA weight and PTH (p = 0.892 and p = 0.363, respectively). Twenty-four percent had a concurrent thyroidectomy for suspicious features, intrathyroidal location of GPA, or large goitre. Immunohistochemistry such as Ki-67, parafibromin, and galectin-3 was used in 18.5% of cases with equivocal histology. Ninety-five percent of GPAs were benign with 5% reported as atypical adenomas.

Conclusion: The reported data on GPAs are sparse and heterogeneous. In GPAs with suspicious features for malignancy, en bloc resection with concurrent thyroidectomy may be considered. In the presence of equivocal histological features, ancillary immunohistochemistry is advocated to differentiate GPAs from atypical adenomas and PCs.