Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-Ku, Tokyo, 162-8655, Japan.
Department of Diabetes, Endocrinology and Metabolism, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-Ku, Tokyo, 162-8655, Japan.
Clin J Gastroenterol. 2023 Jun;16(3):457-463. doi: 10.1007/s12328-023-01784-z. Epub 2023 Mar 21.
In malignant pheochromocytoma, the survival benefit of metastasectomy remains unclear. However, excessive catecholamines secreted from pheochromocytomas can cause cardiovascular and cerebrovascular complications. Debulking metastasectomy can be performed to reduce excess catecholamine secretion when curative resection is impossible. We present a case of metastatic pheochromocytoma to the liver, wherein a significant reduction in catecholamine secretion was achieved by repeat debulking hepatectomy. A 62-year-old woman who had undergone left adrenalectomy for primary pheochromocytoma 10 years prior to our surgical management, had multiple liver metastases of pheochromocytoma. Curative hepatectomy was infeasible because of insufficient remnant liver volume; thus, debulking hepatectomy was conducted. Preoperatively, increased doses of alpha-blockers and catecholamine synthesis inhibitors were administered. Nevertheless, substantial fluctuations in blood pressure and massive hemorrhage were observed intraoperatively. Eight months after the initial hepatectomy, repeat hepatectomy for the remnant lesions was performed due to the worsening of catecholamine levels and catecholamine-related symptoms. The patient survived, with serum catecholamines remaining within the normal range after repeat hepatectomy. Repeat debulking hepatectomy for metastatic pheochromocytoma to the liver is a feasible treatment strategy to effectively decrease catecholamine secretion and alleviate the symptoms thereof. However, special attention should be paid to perioperative catecholamine management and intraoperative surgical techniques.
在恶性嗜铬细胞瘤中，转移灶切除术的生存获益仍不明确。然而，嗜铬细胞瘤分泌的过量儿茶酚胺会引起心血管和脑血管并发症。当无法进行治愈性切除时，可以进行减瘤性转移灶切除术以减少过量儿茶酚胺的分泌。我们报告了一例肝转移的嗜铬细胞瘤病例，通过重复肝切除术实现了儿茶酚胺分泌的显著减少。一位 62 岁女性，在我们进行手术治疗前 10 年因原发性嗜铬细胞瘤行左侧肾上腺切除术，患有多发嗜铬细胞瘤肝转移。由于剩余肝体积不足，无法进行治愈性肝切除术，因此进行了减瘤性肝切除术。术前给予了增加剂量的α受体阻滞剂和儿茶酚胺合成抑制剂。然而，术中仍观察到血压显著波动和大量出血。首次肝切除术后 8 个月，由于儿茶酚胺水平和儿茶酚胺相关症状恶化，进行了残余病灶的重复肝切除术。患者存活，重复肝切除术后血清儿茶酚胺保持在正常范围内。对于肝转移的嗜铬细胞瘤，重复减瘤性肝切除术是一种可行的治疗策略，可以有效减少儿茶酚胺的分泌并缓解其症状。然而，应特别注意围手术期儿茶酚胺管理和术中手术技术。
