Division of Endocrine Surgery, Leeds Teaching Hospitals NHS Trust, Leeds, UK.
Division of Endocrine Surgery, University Surgical Cluster, National University Health System, Singapore, Singapore.
J Surg Oncol. 2023 Oct;128(5):764-768. doi: 10.1002/jso.27388. Epub 2023 Jul 5.
Predicting malignancy among pheochromocytoma paragangliomas (PPGLs) remains a challenge, with only limited understanding of the clinical and molecular characteristics. It has been suggested that reduced avidity of a PPGL on Ga-DOTATATE PET/CT could be a sign of not only altered metabolic activity, but also of increased biologic aggressiveness, possibly due to loss of SSTR-expression.
Retrospective cohort review.
Thirty-seven patients who underwent treatment for PPGL at a tertiary institution over the period 2010-2022, had their biochemical, radiological, and clinicopathological variables collected.
Five of 37 (13%) patients (5 males) with a mean age of 42 years were found to have malignant PPGLs. The mean size of the tumors were 5.4 cm, with 4 located in the paraaortic area and 1 in right adrenal. Functional imaging with Ga-DOTATATE PET/CT showed a mean SUV of 4.5. Four of 5 patients underwent open resection of the tumors under general anesthesia following preoperative alpha blockade with oral phenoxybenzamine. The mean PASS score of the excised tumors was 5.5 in keeping with biologically aggressive tumors, with evidence of necrosis. All but 1 patient had germline SDHB-mutation (Deletion Exon 1). Postintervention after a mean follow-up of 31 months, 2 of 5 (40%) patient developed spinal metastasis and 1 patients (25%) died of cardiac complications.
A non-highly avid PPGL on DOTATE scan should be considered as possibly having necrosis of tumors indicating a more aggressive tumor-biology. There might be a subgroup of patients in whom FDG-PET scan should be considered to gain additional information.
预测嗜铬细胞瘤/副神经节瘤（PPGL）的恶性程度仍然具有挑战性，目前对其临床和分子特征的了解有限。有研究表明，PPGL 在 Ga-DOTATATE PET/CT 上的摄取减少不仅可能是代谢活性改变的标志，而且可能是生物侵袭性增加的标志，这可能是由于 SSTR 表达缺失所致。
回顾性队列研究。
对 2010 年至 2022 年期间在一家三级医疗机构接受治疗的 37 例 PPGL 患者进行了生化、影像学和临床病理变量的收集。
在 37 例患者中，有 5 例（5 例男性，平均年龄 42 岁）被发现患有恶性 PPGL。肿瘤的平均大小为 5.4cm，其中 4 例位于腹主动脉旁，1 例位于右肾上腺。Ga-DOTATATE PET/CT 功能性成像显示平均 SUV 为 4.5。4 例患者在术前口服酚苄明进行α受体阻断后，在全身麻醉下接受了肿瘤的开放切除术。切除肿瘤的平均 PASS 评分为 5.5，提示为具有生物侵袭性的肿瘤，并伴有坏死。除 1 例患者外，所有患者均携带种系 SDHB 突变（外显子 1 缺失）。平均随访 31 个月后，5 例患者中有 2 例（40%）发生脊柱转移，1 例（25%）患者死于心脏并发症。
在 DOTATE 扫描上摄取不高的 PPGL 应被视为可能存在肿瘤坏死，表明肿瘤生物学更具侵袭性。可能存在一部分患者需要进行 FDG-PET 扫描以获得更多信息。
