Zhang Yue, Zhou Bo
Department of Endocrinology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China.
Department of Geriatrics, Xijing Hospital, Fourth Military Medical University, Xi'an, Shaanxi, 710032, China.
Discov Oncol. 2024 Dec 30;15(1):846. doi: 10.1007/s12672-024-01730-6.
Although giant cystic pheochromocytoma and paraganglioma (PPGL) are uncommon, they can be life-threatening when it occurs. Unfortunately, prior case reports have shown that giant cystic PPGLs are highly susceptible to diagnostic errors. Therefore, this study aimed to explore giant cystic PPGLs by comparing them with non-cystic PPGLs, defining the clinical features of the affected patients, and analyzing the characteristics of misdiagnosis and mistreatment associated with PPGLs. The goal is to provide insights for the timely and accurate diagnosis and treatment of giant cystic PPGLs.
A total of 170 cases of pheochromocytoma and paraganglioma (PPGL) diagnosed at the First Affiliated Hospital of Chongqing Medical University from April 2011 to April 2020 were confirmed through clinical evaluation, measurement of catecholamine metabolites, imaging studies, or surgical pathology. The patients were classified into two groups based on the maximum tumor diameter and the presence of cystic components. The clinical characteristics of patients in the giant cystic group, including timely diagnosis and instances of misdiagnosis, are summarized. Additionally, the differences in demographics, clinical manifestations, laboratory examinations, imaging features, treatment approaches, and tumor characteristics between the two groups were analyzed.
Among the 17 patients in the giant cystic group, eight (47.1%) patients were misdiagnosed. The misdiagnosis and mistreatment characteristics of PPGLs were as follows: female (50%); median age, 51 years; normal blood pressure; greater mass effect at onset; larger tumors; median time to diagnosis extension, 180 days; and misdiagnosed disease, which was mainly tumors of adjacent organs, the digestive system, the cardiovascular system, the urinary system, the nervous system, or the endocrine system. The giant cystic group had greater mass effects than did the non-cystic group (P < 0.05). The proportion of patients with the triad of pheochromocytoma (heart palpitations, sweating and headache) and hypertension was lower; however, this difference was not statistically significant. The blood MN (metanephrine) level was greater, and the hemoglobin and blood low-density lipoprotein cholesterol (LDL-cholesterol) levels were lower (P < 0.05). The proportions of patients with tumor calcification and hemorrhagic necrosis were significantly greater (P < 0.05). There was more intraoperative bleeding, more patients who underwent blood transfusions, and a longer postoperative hospital stay; however, the difference was not statistically significant.
Giant cystic pheochromocytomas and paragangliomas (PPGLs) are rare, and their clinical manifestations are atypical. Additionally, imaging findings can be challenging to identify due to the presence of large space-occupying adjacent organs. As a result, these giant cystic lesions of unknown origin are often misdiagnosed. Early biochemical examination, in conjunction with imaging characteristics, is essential to accurately determine the nature of the tumor and reduce the rate of misdiagnosis.
尽管巨大囊性嗜铬细胞瘤和副神经节瘤（PPGL）并不常见，但一旦发生可能危及生命。不幸的是，既往病例报告显示巨大囊性PPGL极易出现诊断错误。因此，本研究旨在通过将巨大囊性PPGL与非囊性PPGL进行比较，明确受累患者的临床特征，并分析与PPGL相关的误诊和误治特点，以探索巨大囊性PPGL。目的是为巨大囊性PPGL的及时准确诊断和治疗提供见解。
对2011年4月至2020年4月在重庆医科大学附属第一医院确诊的170例嗜铬细胞瘤和副神经节瘤（PPGL）患者，通过临床评估、儿茶酚胺代谢产物测定、影像学检查或手术病理进行确诊。根据肿瘤最大直径和是否存在囊性成分将患者分为两组。总结巨大囊性组患者的临床特征，包括及时诊断和误诊情况。此外，分析两组在人口统计学、临床表现、实验室检查、影像学特征、治疗方法和肿瘤特征方面的差异。
巨大囊性组的17例患者中，8例（47.1%）被误诊。PPGL的误诊和误治特征如下：女性（50%）；中位年龄51岁；血压正常；起病时占位效应更大；肿瘤更大；诊断延迟的中位时间为180天；误诊疾病主要为相邻器官、消化系统、心血管系统、泌尿系统、神经系统或内分泌系统的肿瘤。巨大囊性组的占位效应比非囊性组更大（P<0.05）。嗜铬细胞瘤三联征（心悸、出汗和头痛）和高血压患者的比例较低；然而，这种差异无统计学意义。血MN（甲氧基肾上腺素）水平更高，血红蛋白和血低密度脂蛋白胆固醇（LDL-胆固醇）水平更低（P<0.05）。肿瘤钙化和出血坏死患者的比例显著更高（P<0.05）。术中出血更多，接受输血的患者更多，术后住院时间更长；然而，差异无统计学意义。
巨大囊性嗜铬细胞瘤和副神经节瘤（PPGL）罕见，其临床表现不典型。此外，由于存在占据较大空间的相邻器官，影像学表现难以识别。因此，这些不明来源的巨大囊性病变常被误诊。早期生化检查结合影像学特征对于准确判断肿瘤性质和降低误诊率至关重要。
