ABSTRACT
Parathyroid carcinoma [PTC] is an extremely rare malignancy that typically presents with severe primary hyperparathyroidism [PHPT]. More often the surgeon is confronted with the diagnosis after simple parathyroidectomy for presumed benign parathyroid adenoma. This results in unacceptably high recurrence rate ranging from 1/3-2/3 of patients. Faced with the rarity of the disease and lack of reliable preoperative diagnostic test; a high-index of preoperative and intraoperative suspicion of malignancy is warranted in patients with severe hyperparathyroidism as en-block-resection of the gland along with surrounding adherent tissue during the initial exploration offers the only potential cure of the disease. Herein, we present a 46-year-old lady who presented with severe form of PHPT, eventually proved to be caused by PTC. We will also review the literature related to this extremely rare tumor, highlighting the difficulties encountered in its management