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  • • Associated with profound hypercalcemia

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Epidemiology

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  • • 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

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Symptoms and Signs

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  • • 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)

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Laboratory Findings

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  • • Profound hypercalcemia

    • Elevated intact parathyroid hormone (PTH)

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

    • Rarely diagnosed preoperatively

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  • • Physical exam

    • PTH and calcium levels

    • Chest and neck CT scan

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

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Surgery

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  • • 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

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Indications

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  • • All parathyroid carcinomas should be resected

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Radiation

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

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Medications

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  • • Palliative drug: IV bisphosphonate to maintain eucalcemia

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Treatment Monitoring

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  • • Physical exam

    • Serum calcium and intact PTH levels

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Prognosis

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

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

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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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