Department of Internal Medicine, Stanford University Medical Center, Stanford, California 94305, USA.
Pain Pract. 2012 Apr;12(4):286-9. doi: 10.1111/j.1533-2500.2011.00499.x. Epub 2011 Sep 2.
Among adrenal incidentalomas, pheochromocytomas are rare. Malignant pheochromocytoma is even less common, and it typically presents with classic hormonal symptoms, such as palpitations, labile blood pressures, and headaches. Bony metastasis usually occurs late in disease, but we report an unusual case of incapacitating bony pain as the initial presentation of malignant pheochromocytoma. Our patient is a 70-year-old woman with neurofibromatosis type 1 and a history of primary hyperparathyroidism, who tested negative for the ret mutation. She came to medical attention with chest pain and palpitations and was incidentally found to have an adrenal mass. Serum and urine testing was consistent with pheochromocytoma. Her blood pressure was easily controlled as she awaited elective adrenalectomy; however, she quickly developed severe, diffuse bony pain. She represented with hypercalcemia, spontaneous fractures, and incapacitating pain that required such high doses of pain medications that she had to be intubated. Further imaging and bone marrow biopsy confirmed metastatic neuroendocrine tumor. She received one round of chemotherapy with no change in her bony pain, which was her primary complaint. Unfortunately, her treatment options were limited by the heavy sedation required for comfort, and in the end, it was her bony pain rather than hormonal symptoms that made her disease untreatable.
在肾上腺偶发瘤中，嗜铬细胞瘤较为罕见。恶性嗜铬细胞瘤则更为罕见，其典型表现为心悸、血压波动和头痛等典型的激素症状。骨转移通常发生在疾病晚期，但我们报告了一例罕见的以骨痛为首发表现的恶性嗜铬细胞瘤病例。
我们的患者是一位 70 岁的女性，患有 1 型神经纤维瘤病和原发性甲状旁腺功能亢进症病史，RET 突变检测阴性。她因胸痛和心悸就诊，偶然发现肾上腺肿块。血清和尿液检测符合嗜铬细胞瘤的特征。她的血压很容易控制，等待择期肾上腺切除术；然而，她很快出现严重、弥漫性骨痛。她出现高钙血症、自发性骨折和使她无法行动的剧烈疼痛，需要使用高剂量的止痛药，以致需要插管。进一步的影像学和骨髓活检证实为转移性神经内分泌肿瘤。她接受了一轮化疗，但骨痛没有变化，这是她的主要诉求。不幸的是，由于需要大量镇静剂来缓解她的痛苦，她的治疗选择受到限制，最终使她无法治疗的是她的骨痛，而不是激素症状。
