Recurrent primary hyperparathyroidism due to Type 1 parathyromatosis

Parathyromatosis is a rare condition consisting of multiple nodules of benign hyperfunctioning parathyroid tissue scattered throughout the neck and superior mediastinum. As a potential cause of recurrent or persistent hyperparathyroidism, parathyromatosis is a challenging condition to diagnose and treat. The optimal evaluation and management of patients with parathyromatosis is not well established. The reported case involves a patient who was initially diagnosed with primary hyperparathyroidism. The diagnosis of Type 1 parathyromatosis was made after the patient developed recurrent hyperparathyroidism with hypercalcemia and osteoporosis 17 years after the initial operation and underwent two additional operations. The majority of parathyromatosis cases are diagnosed in the setting of secondary hyperparathyroidism. Consensus regarding the preoperative diagnosis and evaluation is lacking due to the paucity of cases of this rare clinical entity. Management involves complete surgical extirpation of all identifiable rests of parathyroid tissue. Intra-operative parathyroid hormone level monitoring and frozen section examination are excellent tools that could increase the rates of initial operative success. Despite this, long-term disease remission is rare, and medical therapy, including calcimimetics and bisphosphonates, may be required for postoperative or non-operative management.