Session: MON 37-82-Pheochromocytoma & Paraganglioma
Poster Board MON-79
A 47-year-old Caucasian male with history of uncontrolled hypertension presented with severe headache, flushing, palpitations, sweating and chest discomfort associated with diffuse abdominal pain, polyuria and polydipsia for 6 weeks. Family history was negative for endocrine malignancies. Vitals signs were temp 38.2 °C, BP: 146/77 mmHg. An abdominal CT showed a 8.0 x 8.4 cm heterogenic right adrenal mass. His plasma free normetanephrine 2686 pg/ml (<148 pg/ml), plasma total metanephrine 2757pg/ml (<205 pg/ml) and 24 hr urine normetanephrine 3713 umol/mol CRT (0-247 umol/mol CRT) confirmed the diagnosis of pheochromocytoma. Patient also had hypercalcemia with calcium 11.9 mg/dl (8.7-10.5 mg/dl), PTH-rp 7.0 pmo/L (< 2.0 pmol/L), calcitonin 8.9 pg/ml (0.0-7.5 pg/ml), undetectable PTH and 1,25 vitamin D of 68 pg/ml (15-75 pg/ml). The patient was prepared preoperatively with phenoxybenzamine and IV fluids. His hypercalcemia was normalized with hydration. He successfully underwent laparoscopic right adrenalectomy. After surgery plasma free metanephrine, plasma free normetanephrine, and plasma total metanephrine became normal. Calcium levels decreased to 7.2 mg/dl (8.7-10.5 mg/dl), PHT-rp decreased to 0.2 mg/dl (8.7-10.5 mg/dl) and PTH significantly increased to 80 pg/ml (10–65 pg/ml). The pathology reported a large mass of 10 centimeters compatible with pheochromocytoma. Immunohistochemical stains were positive for chromogranin, S100 and synaptophysin. PTH-rp staining was not performed.
Discussion: Benign pheochromocytoma with hypercalcemia secondary to PTH-rp is very unusual. This case summarizes the clinical and biochemical findings of a pheochromocytoma co-secreting both metanephrine and PTH-rp. Although PTHrp was not measured in the tumor, the post operative changes of calcium, PTH and PTHrp strongly suggested hypercalcemia secondary to the tumor production of PTHrp.
Nothing to Disclose: DAC, NNT, SLD, BAP
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