Department of General Surgery, Division of Endocrine Surgery, Brigham and Women's Hospital, Boston, Massachusetts 02115, USA.
Endocr Pract. 2012 Jul-Aug;18(4):e81-4. doi: 10.4158/EP11149.CR.
To describe a patient presenting with hemorrhagic shock attributable to bleeding pheochromocytomas and the sequential management strategy used for treating this patient.
We summarize the clinical presentation, diagnostic work-up, surgical management, and pathologic features of our patient and review the pertinent literature.
A 38-year-old man with multiple endocrine neoplasia type 2A and bilateral pheochromocytomas presented initially with nearly fatal retroperitoneal and intraperitoneal hemorrhage rather than the characteristic hypertensive paroxysms. After lifesaving operative intervention and a 5-month period of rehabilitation and convalescence, the patient underwent bilateral retroperitoneoscopic adrenalectomy as definitive treatment. Thus, the abdomen that had been operated on multiple times because of hemorrhage was left undisturbed, and the patient had a successful recovery.
Near-fatal intraperitoneal hemorrhage is a very rare initial manifestation of pheochromocytoma. Our current patient with bilateral pheochromocytomas presented in this dramatic manner. This case shows that a sequential management strategy of damage-control surgical treatment followed by future resection of the tumors after appropriate a-adrenergic blockade is a safe and effective therapeutic option.
描述一位因出血性嗜铬细胞瘤导致出血性休克的患者，并介绍针对该患者的连续治疗策略。
我们总结了患者的临床表现、诊断检查、手术治疗和病理特征，并回顾了相关文献。
一位 38 岁男性患有多发性内分泌肿瘤 2A 和双侧嗜铬细胞瘤，最初表现为几乎致命的腹膜后和腹腔内出血，而非典型的高血压发作。经过抢救性手术干预和 5 个月的康复和疗养后，患者接受了双侧后腹腔镜肾上腺切除术作为确定性治疗。因此，曾经因出血而多次手术的腹部得以保留，患者成功康复。
致命性腹腔内出血是嗜铬细胞瘤非常罕见的初始表现。我们当前的双侧嗜铬细胞瘤患者以这种戏剧性的方式表现出来。该病例表明，损伤控制外科治疗的序贯管理策略，随后在适当的α-肾上腺素能阻滞剂治疗后进行肿瘤切除，是一种安全有效的治疗选择。
