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Management of osteoporosis in patients with alkaptonuria can be challenging. This is the first case report confirming the effectiveness of teriparatide following zoledronic acid therapy in treating osteoporosis and preventing fragility fractures in a patient with alkaptonuria.
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OBJECTIVE: We report a 49-year-old woman who had minimal features of Cushing syndrome and an incidentally discovered adrenal adenoma. She was subsequently diagnosed with pituitary-dependent Cushing syndrome. METHODS: Laboratory and imaging studies including serum cortisol, plasma adrenocorticotrophic hormone (ACTH), high dose dexamethasone test, corticotropin-releasing hormone test, computed tomography (CT) scan, and magnetic resonance imaging were performed. RESULTS: A 49-year-old woman was admitted for urosepsis. An abdominal CT scan performed during the urosepsis workup showed a 2.7-cm right adrenal adenoma. She denied any abdominal striae or other symptoms. Physical examination showed normal vital signs, minimal facial fullness without central obesity, and striae. Laboratory results were as follows: 24-hour-urine cortisol 294 µg (reference 4.0-50.0), midnight serum cortisol 23.0 µg/dL (reference < 7.5), and plasma ACTH level 39 pg/mL (reference 5-27). A corticotropin-releasing hormone stimulation test showed >20% rise in serum cortisol and >35% rise in ACTH levels. A pituitary magnetic resonance image showed a 5 mm pituitary lesion. The patient underwent transsphenoidal pituitary surgery, which confirmed an ACTH-secreting lesion. Postoperatively, she required hydrocortisone replacement for the next 10 months. A follow-up adrenal CT performed 6 months later showed a decrease in the size of the adrenal adenoma (1.8 cm). CONCLUSION: This case highlights the importance of recognizing the coexistence of ACTH-dependent Cushing disease with an adrenal adenoma and partial ACTH dependency of the adrenal adenoma.
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OBJECTIVES: Renal papillary necrosis (RPN) occurring in primary hyperparathyroidism (PHPT) has not been reported. We present a 50-year-old woman who manifested RPN associated with hypercalciuria and normocalcemic PHPT. METHODS: The diagnosis of RPN was based on imaging studies (ultrasound and computed tomography [CT] scan). PHPT was diagnosed with high parathyroid hormone (PTH) and high/normal serum calcium. RESULTS: A 38-year-old woman was evaluated for hypercalcemia (serum calcium, 11.8 mg/dL; ionized calcium, 6.3 mg/dL; phosphorus, 1.8 mg/dL; intact PTH, 98 pg/mL; and 24-hour urine calcium, 543 mg). Renal ultrasound showed no nephrocalcinosis or nephrolithiasis. A parathyroid scan revealed a left parathyroid adenoma. The patient underwent parathyroidectomy, and she became normocalcemic with normal serum PTH levels postoperatively. One year later, she was diagnosed with a left-sided bronchial carcinoid tumor. Following surgery, a surveillance gallium68 positron emission tomography/CT scan performed 2 years later was negative for metastases. Twelve years later (aged 50 years), she presented for follow-up and reported no symptoms of hypercalcemia, fractures, nephrolithiasis, history of pyelonephritis, diabetes mellitus, analgesic drug use, or hypertension. Her serum calcium level was 9.1 mg/dL, PTH level was 82 pg/mL, 25-OH vitamin D level was 34 ng/mL, and 24-hour urine calcium level was 410 mg. However, renal ultrasound showed bilateral RPN that was confirmed by a CT scan. CONCLUSION: RPN may be associated with hypercalciuria and normocalcemic PHPT. Additional studies with a large number of patients are needed.
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Glucagonoma are rare neuroendocrine tumors arising in the Langerhans islets of the pancreas. We report a patient with glucagonoma differentiation into a malignant form after 13 years. A 63-year-old asymptomatic man was evaluated for an enhancing lesion at the distal pancreas. Physical examination was normal. Laboratory values were normal except for an elevated serum glucagon level (206 pg/mL, Ref. 50-150). Somatostatin and other tumor markers were normal. A PET scan confirmed abnormal uptake at the distal pancreas, correlating with a CT scan. The patient underwent laparoscopic distal pancreatectomy with resection of a 2.6-cm mass which predominantly expressed glucagon. Serum glucagon levels normalized immediately postoperatively. He remained asymptomatic for 13 years with normal blood glucose, glucagon, and chromogranin levels and normal surveillance MRI scans. Thirteen years following surgery, an elevated serum glucagon level (230 pg/mL) was observed. At this time he also remained asymptomatic. Abdominal MRI and a PET scan revealed hepatic lesions. Biopsy of the hepatic lesion confirmed metastatic glucagonoma. The patient was treated with lanreotide, which normalized the serum glucagon levels, and the tumor size remained stable for 12 months of follow-up. Complete remission without any treatment for more than 13 years confirmed the benign course of the glucagon-secreting tumor. The precipitating factors are unknown. This case highlights the importance of continuous monitoring of neuroendocrine tumors even beyond 10 years.
