Parathyroid carcinoma is a rare malignant
disease that presents as a sporadic or familial
primary hyperparathyroidism (PHP). The latter is associated with some genetic
syndromes. It occurs with equal frequency in both sexes, unlike PHP caused by
parathyroid adenoma that is more common in
women. It should be suspected in cases of severe
hypercalcemia, with high
parathyroid hormone levels and a palpable cervical mass. Given the difficulty in distinguishing between
parathyroid carcinoma and
adenoma prior to the
surgery, the
diagnosis is often made after
parathyroidectomy. The only curative
treatment is complete surgical resection with oncologic block resection of the primary
tumor to ensure free margins. Adjuvant
therapies with
chemotherapy or
radiation therapy do not modify overall or
disease-free survival.
Recurrences are common and re-operation of resectable recurrent
disease is recommended. The
palliative treatment of symptomatic
hypercalcemia is crucial in persistent or recurrent
disease after
surgery since
morbidity and
mortality are more associated with
hypercalcemia than with
tumor burden.