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  • • Associated with profound hypercalcemia

Epidemiology

  • • Found in 0.5-1% of patients with hyperparathyroidism

    • Cancer is palpable in 50% of the patients

    • 5% of parathyroid cancers are nonfunctional

    • Patients with parathyroid cancer are on average younger at the time of diagnosis than patients with benign hyperparathyroidism

    • Equal distribution among men and women

Symptoms and Signs

  • • Palpable neck mass

    • Hoarseness

    • Fatigue, depression, nausea, vomiting, dehydration, polydipsia, polyuria

    • Pathologic fractures

    • Nephrolithiasis (70% of patients)

    • Nephrocalcinosis

    • Severe renal dysfunction (20-50% of patients)

    • Pancreatitis and peptic ulceration (10-15% of patients)

Laboratory Findings

  • • Profound hypercalcemia

    • Elevated intact parathyroid hormone (PTH)

  • • Parathyroid carcinoma is suspected at operation if parathyroid is hard, whitish, has an irregular capsule, or is invasive

    • Rarely diagnosed preoperatively

  • • Physical exam

    • PTH and calcium levels

    • Chest and neck CT scan

  • • Anecdotal evidence for role of radiation therapy in local control of disease

Surgery

  • • Surgical resection is mainstay of treatment

    • En bloc resection should be carried out where possible (including ipsilateral thyroid lobe and central compartment lymph nodes)

    • Resection of local recurrent or metastatic disease is recommended if possible

Indications

  • • All parathyroid carcinomas should be resected

Radiation

  • • Anecdotal evidence for role of radiation therapy in local control of disease

Medications

  • • Palliative drug: IV bisphosphonate to maintain eucalcemia

Treatment Monitoring

  • • Physical exam

    • Serum calcium and intact PTH levels

Prognosis

  • • Indolent malignancy, with local and distant metastases occurring over many years

    • 5-year survival rates range from 40% to 69%

References

Shattuck TM et al: Somatic and germ-line mutations of the HRPT2 gene in sporadic parathyroid carcinoma. N Engl J Med 2003;349:1722.  [PubMed: 14585940]

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