Introduction: Malignant pheocromocytomas are rare. Metastatic disease may be present at the time of diagnosis or may only be evident after. Prognosis is poor. Currently there is no effective cure.
Case report: We describe the case of a 66-year-old woman with a history of pheocromocytoma submitted to left adrenalectomy in 1992. In the past 10 years, she complained of episodes of dorsal back pain associated with hypertensive peaks and tachycardia. In 4/2010, a dorsal MRI was performed revealing D10 pathologic fracture with moderate spinal compression, compatible with bone metastasis. MIBG-SPECT/CT: increased uptake in D10. Guided biopsy: the lesion was immunoreactive for neuroendocrine markers. Octreoscan was negative. She was admitted at the Endocrinology Department in 8/2010. Analytically: chromogranin-A 112 ng/ml (1998), urinary metanephrines 219 μg/24 h (25312), VMA 2.45 mg/24 h (<15), 5-HIAA 5.64 mg/24 h (26). She started fenoxibenzamine titrated until 10 mg 2id. In 9/2010, she was submitted to radiotherapy of the lesion (total dose 30 Gy). No adverse effects were reported; back pain was not substantially reduced. Spinal CT after showed lesion stability. No neurological deficit. Analytically chromogranin-A 7.8 nmol/l (<6); normal urinary metanephrines. MIBG-SPECT/CT (2/2011): D10 metastasis maintained with similar degree of activity. In 3/2011, she was submitted to 200 mCi of 131I-MIBG, with no complications. The following MIBG-SPECT/CT showed similar degree of activity in D10 and focus of increased uptake in segment VI of the liver. In 6/2011, she was submitted to D10 kyphoplasty. Abdominal CT showed no image suggestive of secondary lesions. In last evaluation, under fenoxibenzamine 10 mg 2id, the patient had minimal back pain with no limitations in her daily activities. Analytically chromogranin-A 14 nmol/l and normal urinary metanephrines. Genetic study was negative.
Conclusion: This case illustrates the importance of treatment individualization. A combination of radiotherapy, MIBG and kyphoplasty was performed in order to decrease pain and to stabilize vertebral secondary disease. Metastasis diagnosed after 18 years reinforce the need of an accurate and prolonged follow-up.
27 Apr - 01 May 2013
European Society of Endocrinology