Background: Pheochromocytoma is a rare neuroendocrine tumor from the adrenal medulla’s chromaffin cells that secrete catecholamines. The mainstay of treatment is surgery. Although rare, it has a recurrence rate of 6.5-16.5% even after adequate surgical removal with a notable increase in prevalence among genetic syndromes, extra-adrenal tumors, larger tumor size, and younger age of diagnosis. Case: A 23-year-old Filipino male with no known familial disease presented with episodic headache, palpitations, diaphoresis, and resistant hypertension. Two masses in the left adrenal gland, with the larger one measuring 6.0cm x 5.0cm x 3.0cm, were surgically removed. Histologic examination revealed pheochromocytoma. Post-operatively, there was the normalization of urinary metanephrines and the resolution of the signs and symptoms. He was lost to follow-up and returned five years later with an abdominal ultrasound demonstrating a right adrenal mass. He had no accompanying signs and symptoms; the physical examination was unremarkable. On workup, biochemical testing revealed two 24-hour urinary metanephrine levels were markedly elevated, 14.49 and 19.97 (NV:0-1mg/24 hours). Calcitonin: 644 (NV:0-18pg/ml) and carcinoembryonic antigen (CEA): 23.52 (NV:<5ng/ml) were also elevated. Mild hypercalcemia was noted with elevated intact PTH 101.74 (NV:0-65pg/ml). Parathyroid scintigraphy was normal. The neck ultrasound showed multiple bilateral thyroid nodules, with the largest measuring 1.2cmx0.9cm (TIRADS IV). The abdominal CT scan showed two well-defined, homogenous right adrenal masses, with the largest measuring 6.3cm x 5.6cm x 7.4cm. He underwent right adrenalectomy, and histopathology showed pheochromocytoma with Pheochromocytoma of the Adrenal Scale Score (PASS) of 3. One month later, he underwent total thyroidectomy with neck dissection and inferior parathyroid gland resection. Histopathology confirmed medullary thyroid cancer with nodal metastasis and parathyroid adenoma. The patient recovered well without complications. Biochemical tests normalized eight weeks post-surgery. The presence of synchronous recurrent pheochromocytoma, medullary thyroid cancer (MTC), and primary hyperparathyroidism is consistent with multiple endocrine neoplasia 2A (MEN2A) syndrome. Conclusion: Lifelong follow-up is essential in patients treated for pheochromocytoma despite the complete removal of the adrenal masses due to recurrence risk. Recurrence should likewise raise the suspicion of MEN2, a genetic syndrome. In addition, the pheochromocytoma has malignant characteristics, and the MTC has metastases to the cervical lymph nodes. Early detection and prompt intervention are essential for the treatment of the disease.
Early screening of osteoporosis decreases fracture risk. Several identified clinical risk factors led to the development of screening tools to estimate osteoporosis risk. Bone Mineral Densitometry (BMD) as a diagnostic tool for screening is not practical because of high cost and poor availability. The extensively studied osteoporosis screening tools are: Simple Calculated Osteoporosis Risk Estimation (SCORE), the Osteoporosis Risk Assessment Instrument (ORAI), the Age Bulk One or Never Estrogen (ABONE), body weight (WEIGHT), and the Osteoporosis Risk Index (OSIRIS). These tools were developed and validated in Caucasians. Validation of these tools for specific populations is necessary because of the observed variations in BMD across geographic and ethnic groups. To date, the utility of these screening tools in the Philippines is unknown. We conducted a cross-sectional analysis of all patients who underwent BMD screening for osteoporosis in a tertiary hospital from January 2015 to September 2020. The study participants were postmenopausal Filipino women aged 45 to 65 years. The subjects had no history of osteopenia, osteoporosis, hip or spine fractures, use of osteoporosis medications, renal insufficiency, bilateral oophorectomy, hysterectomy, or early menopause. We identified demographic and clinical risk factors. These risk factors were used to calculate the risk score of five osteoporosis risk assessment tools: ORAI, ABONE, WEIGHT, OSTA, and ORISIS. Using the DEXA T-score as an external criterion, the sensitivity, specificity, positive and negative predictive values, positive and negative likelihood ratios, and diagnostic accuracy for each tool were calculated. Included were 1869 subjects with a mean age of 57.9 + 4.3 years old. Osteoporosis, with a T-score of <-2.5 at the lumbar or femoral neck area, was seen in 665 (35.58%). Risk factors such as weight, height, BMI, menopausal years, history of previous fractures, and intake of oral calcium supplements correlated significantly with a higher risk (OR=1.025, 95%CI: 0.974–1.079; OR=1.059, 95%CI: 0.84–1.338; OR=1.063, 95%CI: 0.817–1.383; OR=1.74, 95%CI: 1.198–2.528; and OR=1.088, 95%CI: 0.869–1.319), of having osteoporosis in the said population. ORAI and WEIGHT have the highest probability of identifying patients with a sensitivity of 88.42% and 91.28%, and accuracy of 85.71% and 87.98%. Both performed equally in screening for osteoporosis in this setting. However, ABONE, OSTA, and ORISIS underestimated the number of high-risk osteoporosis patients, because of their low sensitivity and diagnostic accuracy. Both ORAI and WEIGHT are simple and easy to calculate and can serve as an initial screening tool to identify Filipino postmenopausal women who are at high risk for osteoporosis. A prospective study with a correlation of fracture occurrences may provide evidence for the value of these tools as a screening test.
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