Parathyromatosis: a rare cause of recurrent, debilitating hypercalcemia with limited treatment options

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SAT 199-223-Disorders of Bone & Calcium Homeostasis: Case Reports
Clinical
Saturday, June 15, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SAT-209
Alina Elperin*1, Richard Prinz1, Janardan Khandekar2, Igor Jovanovic2 and Romy Jill Block3
1Northshore University Health System, 2NorthShore University Health System, 3Northshore University Healthsyst, Highland Park, IL
Background: Recurrent hyperparathyroidism after parathyroidectomy is defined as recurrent hypercalcemia > 6 months after initial surgery. Parathyromatosis is a difficult to diagnose and treat cause of recurrent hyperparathyroidism. It is defined as multiple rests of hypercellular, hyperfunctioning parathyroid tissue. While parathyromatosis is well documented in patients with renal disease, little is known about parathyromatosis in primary hyperparathyroidism. We present a case series of three patients with persistent hypercalcemia due to parathyromatosis.

Case 1: A 35yo female had persistent hypercalcemia after 4 parathyroidectomies. Her labs were: Ca-11.3mg/dL (normal 8.5-10.3 mg/dL), PTH-251pg/mL (normal 12-88 pg/mL). Sestamibi showed abnormal uptake and CT scan of the chest showed multiple nodules in the mediastinum. She underwent 3 more surgeries to remove this abnormal tissue. Pathology each time confirmed parathyromatosis. Despite 7 surgeries, the patient is hypercalcemic (12-13 mg/dL) and requires Cinacalcet and periodic IV Zoledronic Acid. She is currently undergoing familial genetic testing.

Case 2: A 41yo female had persistent hypercalcemia. Her labs were Ca-11.3 mg/dL, PTH-61.2pg/mL. Sestamibi showed single gland uptake. She underwent a parathyroidectomy and pathology showed multi-gland hyperplasia.  Postoperatively, she remained hypercalcemic.  Repeat Sestamibi was normal but a neck CT showed a lesion in the mediastinum. She underwent her second parathyroidectomy with pathology consistent with parathyromatosis.  She remained hypercalcemic: Ca-10.5 mg/dL, PTH-100pg/mL and she began medical management with Cinacalcet.

Case 3: A 47yo female had recurrent hypercalcemia despite 4 parathyroidectomies. At presentation, her labs were:  Ca-16.5mg/dL, PTH-317pg/mL. Sestamibi showed abnormal uptake in the thyroid and neck CT revealed 2 nodules in the mediastinum. She had her 5th neck exploration. Pathology was consistent with parathyromatosis. She remained normocalcemic for a year then her hypercalcemia recurred:  Ca-13.3 mg/dL, PTH-65pg/ml. She was given IV Zoledronic Acid and started on Cinacalcet.

Conclusion: We present a case series of 3 patients with recurrent or persistent primary hyperparathyroidism from parathyromatosis. These patients have debilitating symptoms despite multiple operations.  Medical therapy to control symptoms of hypercalcemia in parathyromatosis is limited and minimally effective.

Nothing to Disclose: AE, RP, JK, IJ, RJB

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