Abstract
Parathyroid carcinoma is a rare cause of primary hyperparathyroidism. In most cases it is encountered as a sporadic tumor.
Nevertheless, some familial syndromes carry higher risk of this malignancy. Several candidate genes are suspected to be causative in tumorigenesis in falimial and sporadic tumors.
Clinical picture, laboratory findings and macroscopic appearance during operation can in part differ from benign forms of primary hyperparathyroidism and lead to suspicion of parathyroid carcinoma. Comprehensive resection of tumor is the first line treatment in primary disease and even in many cases of recurrent process.
Adjuvant radiation therapy has been proven to significantly lower the local recurrence rate. Relapse of the disease usually brings about more difficult diagnostic approach and worsens the prognosis.
Hypercalcaemia is the cause of death in most of cases, not tumor progression itself. Growing knowledge about tumor-supresor genes and oncogenes can in future make the testing of germline mutations in kindred of patients with parathyroid carcinoma possible.