Medina Gaviria V, Rodríguez Romero L, Molina Ramírez I
Servicio de Cirugía Pediátrica. Universidad Nacional de Colombia-Fundación Hospital Pediátrico de la Misericordia (HOMI). Colombia.
Cir Pediatr. 2019 Apr 22;32(2):109-112.
Pheochromocytoma is an infrequent neuroendocrine tumor, originated from neural crest cells. 10% of them are extra adrenal, located at sympathetic nodules and are known as paragangliomas. The most common place is the Zuckerkandl organ, 10 to 26% are malign and has a mortality around de 26% of the cases.
We present two cases of teenagers with Zuckerkandl's organ paraganglioma, who debut with high blood pressure, diaphoresis and dyspnea. Complete tumor resection was performed in both cases, during which they presented hipertensive crisis and, after vascular pedicle ligation, hypotension.
Zuckerkandl's organ paraganglioma is a rare pathology in pediatric population, therefore represents an important diagnostic and therapeutic challenge. Surgery differs from other tumors because of catecholamine secretion that produces hemodynamic changes and demands prompt and accurate management from surgeon and anesthetist.
嗜铬细胞瘤是一种罕见的神经内分泌肿瘤,起源于神经嵴细胞。其中10%为肾上腺外肿瘤,位于交感神经节,被称为副神经节瘤。最常见的部位是祖克坎德尔器官,10%至26%为恶性,病例死亡率约为26%。
我们呈现两例患有祖克坎德尔器官副神经节瘤的青少年病例,他们以高血压、多汗和呼吸困难为首发症状。两例均进行了肿瘤完整切除,术中出现高血压危象,在血管蒂结扎后出现低血压。
祖克坎德尔器官副神经节瘤在儿科人群中是一种罕见的病理情况,因此是一项重要的诊断和治疗挑战。由于儿茶酚胺分泌会引起血流动力学变化,该手术与其他肿瘤手术不同,需要外科医生和麻醉师迅速而准确的处理。