Parathyroid Disease Scenarios for the Practicing Clinician. Vijaya Chockalingam MD Faculty Endocrinologist Banner University Medical Center- Phoenix

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1 Parathyroid Disease Scenarios for the Practicing Clinician Vijaya Chockalingam MD Faculty Endocrinologist Banner University Medical Center- Phoenix

2 Clinical Scenario-1 73 year man (BK) with hypercalcemia on routine labs Concerned about insomnia, fatigue, memory loss, polyuria, nocturia Denied other symptoms related to hypercalcemia No family history of hypercalcemia/hyperparathyroidism No medications that could cause hypercalcemia PMHx: Hepatitis B, CAD, HTN, HLD, chronic anemia, Barrett s eshophagitis PSHx: Inguinal hernia, cataract, prostatectomy

3 Clinical Scenario-1 Physical exam: Vitals: 6 feet, 207 lbs, BMI 28, BP 140/74, HR 84/min General: Overweight gentleman Neck: No goiter, no clinically palpable nodules/neck mass CVS: Rhythm is normal, no murmurs RS: No wheezing. Chest clear to auscultation Musculoskeletal: No kyphoscoliosis Abd: Soft, non tender, no organomegaly

4 Clinical Scenario-1

5 Clinical Scenario-1 What is the diagnosis? Mild, asymptomatic PTH-mediated hypercalcemia? What is the next step in management?

6 Clinical Scenario-1 DXA scan Lumbar 1.26 gm/cm 2 T-score 0.2 Femoral 1.10 gm/cm 2 T-score 0.0

7 Clinical Scenario-1 What next? With negative Sestamibi scan, would you refer patient to surgeon?

8 What next? Clinical Scenario-1 ULTRASOUND Right paratracheal density measuring 13 x 10 mm with teardrop hypoechoic shape and vascular branching pattern compatible with parathyroid adenoma

9 Clinical Scenario-1 Indications for surgery in primary hyperparathyroidism Serum Calcium 1 mg/dl > upper limit of normal T-score less than -2.5 at lumbar spine, total hip, femoral neck or distal 1/3 radius Vertebral fracture 24 hour urine calcium > 400 mg/day and increased stone risk by biochemical stone risk analysis Presence of nephrolithiasis, nephrocalcinosis Age < 50 years of age John P. Bilezikian, Maria Luisa Brandi, Richard Eastell, Shonni J. Silverberg, Robert Udelsman, Claudio Marcocci, and John T. Potts Jr

10 Clinical Scenario-1 On Oct 13 th 2015, patient underwent focused parathyroid exploration with parathyroidectomy of right lower parathyroid adenoma.

11 Pathology Clinical Scenario-1

12 Clinical Scenario-1 6 Month Labs Jan 2016 Total calcium ( mg/dl) 9.7 Ionized calcium ( mg/dl) 5.18 Intact PTH (15-65 pg/ml) 35

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14 Clinical Scenario-2 73 year woman (MB) was diagnosed with Waldenstroms Macroglobulinemia (WM) and lymphoplasmacytic lymphoma in March 2006 Treated with plasmapheresis Progressed to non-hodgkin B-cell lymphoma IVIG Rx for neuropathy (hands, vocal cords, forehead) In remission until 2014 Relapse of WM in October 2014 Presented to us for evaluation in early 2015 for management of hypercalcemia

15 Clinical Scenario-2 Current symptoms: fatigue, constipation/diarrhea, nausea, abdominal cramps, shortness of breath Denied kidney stones, fractures Mother has osteoporosis Currently taking Vitamin D 5000 IU BID When she has a cold, she takes 30,000 IU BID for 2 days and reports that her cold gets better

16 Clinical Scenario-2 Jan 4th 2015 Total calcium ( mg/dl) 15.7 IgM ( mg/dl) 3155 IgG 266 Beta 2 microglobulin 12.1 Hemoglobin 10.2 Treated with IV fluids, calcitonin, diuretics, plasmapheresis

17 Clinical Scenario-2 Jan 9th 2015 Jan 22nd 2015 Total calcium ( mg/dl) Intact PTH (15-65 pg/ml) OH Vitamin D ( ng/ml) 96 1,25-OH Vitamin D (18-72 pg/ml) 179 Creatinine, Serum ( mg/dl) 1.85

18 Clinical Scenario-2 Diagnosis: Non PTH-mediated hypercalcemia Hypercalcemia due to vitamin D toxicity and increased 1,25 dihydroxy vitamin D

19 Clinical scenario-2 Feb 2015 Total calcium ( mg/dl) 13.1 Intact PTH (15-65 pg/ml) 9 25-OH Vitamin D ( ng/ml) 66 1,25-OH Vitamin D (18-72 pg/ml) >200 Creatinine, Serum ( mg/dl) 1.14 IgM ( mg/dl) 3000

20 Clinical Scenario-2 What next? Non PTH-mediated hypercalcemia Hypercalcemia due to continued high level of 1,25 dihydroxy vitamin D STEROIDS: Patient refused

21 Clinical Scenario-2 What next? Oncologist treated patient with IBRUTINIB Total calcium ( mg/dl) 9.6 Intact PTH (15-65 pg/ml) OH Vitamin D ( ng/ml) 50 1,25-OH Vitamin D (18-72 pg/ml) 60 April 2015 Creatinine, Serum ( mg/dl) 1.1 IgM ( mg/dl) 693

22 Summary Clinical Scenario-2 Non PTH-mediated hypercalcemia Hypercalcemia due to increased level of 1,25 dihydroxy vitamin D due to lymphoma After Rx with Ibrutinib, calcium normalized, IgM levels improved, lymphoma was better controlled

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24 Clinical Scenario-3 65 year old woman (ML) was found to have serum calcium level of 15 mg/dl in routine evaluation and referred to an endocrinologist for evaluation She was recently diagnosed with stage 1 invasive ductal breast cancer Symptoms of hypercalcemia: GERD, osteoporosis. Patient denied other symptoms of hypercalcemia. No family history of hypercalcemia/hyperparathyroidism Patient was taking hydrochlorothiazide

25 Clinical Scenario-3 PMHx: Anxiety, HTN, obesity PSHx: Cholecystectomy, TAH with BSO, resection of colon polyp Social Hx: Single, no hx of tobacco, recreational drug use. Drinks wine 5 times/week. One child.

26 Physical exam: Clinical Scenario-3 General: Obese lady Neck: No goiter, no clinically palpable nodules, neck masses CVS: Rhythm is normal, no murmurs RS: No wheezing, chest clear to auscultation Musculoskeletal: No kyphoscoliosis Abd: Soft, non tender, no organomegaly

27 Clinical Scenario-3

28 Clinical scenario-3 DXA done in 2015 Lumbar 0.84 gm/cm 2 T-score -2.9 Femoral gm/cm 2 T-score -2.0

29 Clinical scenario-3 What is the diagnosis? Severe, symptomatic PTH-mediated hypercalcemia What is the next step in management?

30 Sestamibi scan Clinical scenario-3

31 Clinical scenario-3

32 Clinical scenario-3 ULTRASOUND of thyroid/parathyroid glands

33 Clinical scenario-3 In July 2016, patient underwent focused parathyroidectomy with excision of right inferior parathyroid adenoma that was located in retroesophageal area. Date Baseline PTH (12-88 pg/ml) PTH in 10 minutes Calcium ( mg/dl)

34 Clinical scenario-3 14,700 mg adenoma (normal parathyroid weight 30 mg)

35 Thank You