Agarwal Shubham, Alkaissi Hussam, Pacak Karel, Mosquera Izurieta Jorge Esteban, Dackiw Alan P, Oltmann Sarah C, Nwariaku Fiemu, Jia Liwei, Roden Mary Grace, Hamidi Oksana
Division of Endocrinology and Metabolism, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA.
National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892, USA.
J Endocr Soc. 2025 Feb 26;9(4):bvaf036. doi: 10.1210/jendso/bvaf036. eCollection 2025 Mar 3.
Cells derived from neural crest populate several organs. A particular precursor cell, sympathogonia, gives rise to pheochromoblasts and neuroblasts. Due to common origin, tumors originating from pheochromoblasts, such as pheochromocytoma (PHEO) and paraganglioma (PGL), may rarely coexist with ganglioneuroma (GN).
We evaluated clinical, biochemical, and radiological characteristics of patients with composite PHEO/PGL and GN (PPGL-GN) and compared them to patients with PHEO.
In this retrospective, dual-center, observational, case-control study, we identified patients with PPGL-GN. Similarly, we identified a control group of patients with PHEO who underwent laparoscopic adrenalectomy. All diagnoses were confirmed on histology. Descriptive statistics were used to summarize demographic and clinical data.
We identified 19 consecutive patients with PPGL-GN and 86 patients with PHEO. Patients with PPGL-GN, compared to those with PHEO, were younger (aged 46.0 vs 50.8 years; = .03), had higher rate of underlying genetic disorders (47.4% vs 23.2%; = .03), and had fewer functioning tumors (89.5% vs 100%; = .002). There was no difference in the median radiological tumor size or the precontrast computed tomography density. Disease recurrence (at another site) was noted in 15.8% of PPGL-GN patients who had a median follow up of 14.6 months, as opposed to no disease recurrence in patients with PHEO. There was no documented recurrence at the tumor bed and no metastasis in both groups.
Patients with PPGL-GN were younger and had a higher occurrence of underlying genetic disorders compared to PHEO. However, PPGL-GN was radiologically indistinguishable from PHEO. The higher observed disease recurrence of PPGL-GN reinforces vigilant postoperative follow-up.
源自神经嵴的细胞分布于多个器官。一种特殊的前体细胞，即交感神经原细胞，可分化为嗜铬母细胞和成神经细胞。由于起源相同，源自嗜铬母细胞的肿瘤，如嗜铬细胞瘤（PHEO）和副神经节瘤（PGL），可能很少与神经节神经瘤（GN）共存。
我们评估了复合型嗜铬细胞瘤/副神经节瘤与神经节神经瘤（PPGL-GN）患者的临床、生化和放射学特征，并将其与嗜铬细胞瘤患者进行比较。
在这项回顾性、双中心、观察性病例对照研究中，我们确定了PPGL-GN患者。同样，我们确定了一组接受腹腔镜肾上腺切除术的嗜铬细胞瘤患者作为对照组。所有诊断均经组织学证实。描述性统计用于总结人口统计学和临床数据。
我们连续确定了19例PPGL-GN患者和86例嗜铬细胞瘤患者。与嗜铬细胞瘤患者相比，PPGL-GN患者更年轻（年龄分别为46.0岁和50.8岁；P = 0.03），潜在遗传疾病发生率更高（47.4%对23.2%；P = 0.03），功能性肿瘤更少（89.5%对100%；P = 0.002）。放射学肿瘤大小中位数或增强前计算机断层扫描密度无差异。PPGL-GN患者中有15.8%出现疾病复发（在另一部位），中位随访时间为14.6个月，而嗜铬细胞瘤患者无疾病复发。两组在肿瘤床均无记录到的复发，也无转移。
与嗜铬细胞瘤相比，PPGL-GN患者更年轻，潜在遗传疾病发生率更高。然而，PPGL-GN在放射学上与嗜铬细胞瘤无法区分。PPGL-GN观察到的较高疾病复发率强化了术后的密切随访。
