1. Li, Henry Winston Li, Kingbherly Lichauco, Rafael Lim, Imee Loren Lim, Jason Morven Lim, John Harold

2.  20 years old, female  Chief Complaint: RECURRENT LUMBAR PAINS

3.  Vital Signs BP:120/70RR: 20/min PR: 70/min  Neck 2 x 2cm firm palpable mass within the right lobe of the thyroid which moves with deglutition; no other palpable masses  Chest (normal)  Abdomen Flat, normoactive bowel sounds, liver is not enlarged, no splenomegaly, (+) CVA tenderness

4.  Urinalysis: (+) red blood cells and crystals  IVP: (+) bilateral kidney stone

5.  20 year old female  Recurrent lumbar pains  Bilateral kidneys stones  RBC and crystals in urine  2 x2 cm palpable mass within the right lobe of the thyroid with no other palpable mass  Costovertebral angle tenderness  No hepatomegaly, no splenomegaly

6. a. Serum tumor markers b. Screen for pheochromocytoma c. Screen for hyperparathyroidism

7.  Calcitonin: ◦ produced by C-cells, an antihypercalcemic hormone which inhibits osteoclast-mediated bone resorption; ◦ minimal role in calcium regulation ◦ >10 pg/mL = diagnostic of MTC  CEA ◦ Not specific for MTC ◦ Also seen in colon CA and metastasis to the liver

8. Fragment of granular and amyloid material

9.  Procedures detect distant metastases especially if there is a very high level of calcitonin  Imaging studies requested only if there is suspected invasion

10.  24h urine cathecholamines and metanephrines  Treated preoperatively

11. Actual Results Normal values Serum calcium 20 mg/dL 8.5- 10.5 mg/dL ↑ Ionized calcium 8 mg/dL 4.4- 5.2 mg/dL ↑ PTH levels70 mg/dL 50 mg/dL ↑

12.  Determination of serum calcium levels, ionize calcium and parathyroid hormone level  24 hour urinary calcium  to differnetiate from BFHH  X-ray of spine and abdomen  Fine needle biopsy of the mass in the right lobe of the thyroid

14.  Salivary glands  Thyroid glands  Palpable mass

15.  Sestamibi: small protein which is labeled with the radio-pharmaceutical technetium-99  Radioactive agent is injected into the veins of a patient with parathyroid disease  Radionuclide is concentrated in thyroid and parathyroid tissue but usually washes out of normal thyroid tissue in under an hour. It persists in abnormal parathyroid tissue.

16.  After 1-2 hours, radioactivity in suspected parathyroid adenoma should persist.

17.  Not used to confirm diagnosis of PHPT  Used to identify the location of the offending gland  > 80% sensitivity for parathyroid adenoma  Generally complemented with neck ultrasound which has 77% sensitivity

18.  Medullary thyroid carcinoma with concurrent primary hyperparathyroidism  BASIS: MTC- 2 x2 cm palpable mass within the right lobe, FNAC examination revealed granular amyloid material; PHPT- bilateral urolithiasis, elevated PTH and calcium assay

19.  5% of thyroid malignancies and arise from the parafollicular or C cells of the thyroid  Forms: Sporadic (80%) hereditary (20%)- autosomal dominant inheritance, mutation of RET proto-oncogene

20.  Increased parathyroid proliferation and PTH secretion independent of calcium levels  Affects females more than male  Sporadic type more common  Etiology -Parathyroid adenoma (80%) -Multiple adenoma or hyperplasia (15- 20%) -Parathyroid CA (1%)

21.  Manage the symptomatic disease (Medullary thyroid cancer and primary hyperparathyroidism)

22.  Total thyroidectomy -treatment of choice due to high incidence of multicentricity -bilateral central neck node dissection should be routinely performed due to frequent involvement of the central compartment nodes -patients with tumors larger than 1.5 cm should undergo ipsilateral prophylactic modified radical neck dissection, because greater than 60% of these patients have nodal metastases

23.  Calcitonin and CEA 2-3 months post-op  If calcitonin >100, evaluate for residual neck disease or +/- distant metastasis  MEN IIA and MEN IIB: annual screen for pheochromocytoma

24.  10-year survival rate is approximately 80%  decreases to 45% in patients with lymph node involvement.  worst (35% at 10 years) in patients with MEN2B

25.  PARATHYOIDECTOMY Indications - Markedly increased serum calcium - Episode of life threatening hypercalcemia episode - Reduced creatinine clearance - Kidney stones - Markedly elevated 24 hr urinary Ca excretion - Substantially decreased bone mass - Age: < 50 years old

26.  In patients who have hypercalcemia at the time of thyroidectomy, only obviously enlarged parathyroid glands should be removed.  The other parathyroid glands should be preserved

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