背景:嗜铬细胞瘤是一种罕见的儿茶酚胺分泌型肿瘤,具有术后复发的可能性,需要长时间的随访。此病例突出了治疗复发性嗜铬细胞瘤的诊断和治疗挑战。
方法:一位25岁的女性,十年前有左嗜铬细胞瘤的病史,右侧肾上腺肿块.尽管控制了高血压,尿液中的间肾上腺素升高提示复发.影像显示右侧肾上腺肿物及可疑左侧主动脉旁淋巴结,确认左肾上腺床和右肾上腺复发性恶性嗜铬细胞瘤的诊断,转移到主动脉旁淋巴结。患者接受了右肾上腺切除术和细胞减灭术(CRS),以切除左侧肾上腺床复发和左主动脉旁淋巴结清扫术的形式,术中放射治疗(IORT),和腹腔热化疗(HIPEC)。在六年后的随访中,患者没有复发。
结论:该病例说明了嗜铬细胞瘤患者持续监测的重要性,即使是那些风险低的人。这种情况下的复发,尽管最初的肿瘤体积较小,没有遗传倾向,强调嗜铬细胞瘤的不可预测的性质。CRS的成功管理,IORT,HIPEC强调需要个性化和多方面的治疗方法。
结论:嗜铬细胞瘤患者,包括那些最初被认为是低风险的,由于复发的风险,需要长期监测。CRS的利用,IORT,在这种情况下,HIPEC在有效管理复发性和转移性恶性疾病方面至关重要,展示了全面的意义,在这种复杂情况下的多学科治疗策略。
BACKGROUND: Pheochromocytomas are rare catecholamine-secreting tumors with a high potential for recurrence post-surgery, necessitating prolonged follow-up. This
case highlights the diagnostic and therapeutic challenges in managing recurrent pheochromocytoma.
METHODS: A 25-year-old female, with a history of left pheochromocytoma treated with adrenalectomy a decade earlier, presented with a right adrenal mass. Despite controlled hypertension, elevated urine metanephrines suggested recurrence. Imaging showed a right adrenal mass and suspicious left paraaortic lymph nodes, confirming the diagnosis of recurrent malignant pheochromocytoma in the left adrenal bed and right adrenal gland, with metastasis to the paraaortic lymph nodes. The patient underwent right adrenalectomy coupled with cytoreductive surgery (CRS) in the form of excision of left-sided adrenal bed recurrence and left paraaortic lymph node dissection, intraoperative radiation therapy (IORT), and hyperthermic intraperitoneal chemotherapy (HIPEC). On follow-up six years later, the patient remains free from recurrence.
CONCLUSIONS: This
case illustrates the importance of continued surveillance in pheochromocytoma patients, even those with a low-risk profile. The recurrence in this
case, despite a smaller initial tumor size and no genetic predispositions, underscores the unpredictable nature of pheochromocytomas. The successful management with CRS, IORT, and HIPEC emphasizes the need for a personalized and multifaceted treatment approach.
CONCLUSIONS: Pheochromocytoma patients, including those initially considered low risk, require long-term monitoring due to the risk of recurrence. The utilization of CRS, IORT, and HIPEC in this
case was pivotal in managing the recurrent and metastatic malignant disease effectively, demonstrating the significance of a comprehensive, multidisciplinary treatment strategy in such complex cases.