Torresan Francesca, Beber Arianna, Schiavone Donatella, Zovato Stefania, Galuppini Francesca, Crimì Filippo, Ceccato Filippo, Iacobone Maurizio
Endocrine Surgery Unit, Department of Surgery, Oncology and Gastroenterology, University of Padova, 35128 Padova, Italy.
Familial Cancer Clinic, Veneto Institute of Cancer, Istituto Oncologico Veneto IOV IRCCS, 35128 Padova, Italy.
Cancers (Basel). 2023 May 24;15(11):2890. doi: 10.3390/cancers15112890.
The prognosis of pheochromocytoma and sympathetic paraganglioma (PHEO/sPGL) is difficult to predict at the time of diagnosis and long-term follow-up data are scarce, especially for apparently benign and sporadic variants. The aim of the study was to analyze the long-term outcomes in PHEO/sPGL patients.
A monocentric series of 170 patients who underwent surgery for PHEO/sPGL was analyzed.
The study cohort included 91 female and 79 males with a median age of 48 years (range 6-83). The majority of PHEO/sPGL cases were considered apparently benign at the time of diagnosis; evident malignant behavior was found in 5% of cases. The overall 10-year risk of recurrence was 13%, but it rose up to 33% at 30 years. The risk of new tumor recurrence was higher in patients with hereditary tumors, but the risk was still significant in patients with apparently sporadic variants (20-year risk: 38% vs. 6.5%, respectively; < 0.0001). The risk of metastatic recurrence was higher in patients with locally aggressive tumors at diagnosis, but the risk was present also in apparently benign variants (5-year risk: 100% vs. 1%, respectively; < 0.0001).
Lifelong follow-up is required not only for hereditary PHEO/sPGL but also for apparently benign and sporadic tumors at diagnosis because of the risk of long-term recurrent disease.
嗜铬细胞瘤和交感神经副神经节瘤（PHEO/sPGL）的预后在诊断时难以预测，长期随访数据稀缺，尤其是对于明显良性和散发性变体。本研究的目的是分析PHEO/sPGL患者的长期预后。
分析了一组170例接受PHEO/sPGL手术的单中心患者。
研究队列包括91名女性和79名男性，中位年龄为48岁（范围6 - 83岁）。大多数PHEO/sPGL病例在诊断时被认为明显良性；5%的病例发现有明显的恶性行为。总体10年复发风险为13%，但在30年时升至33%。遗传性肿瘤患者新肿瘤复发风险更高，但在明显散发性变体患者中风险仍然显著（20年风险：分别为38%和6.5%；<0.0001）。诊断时局部侵袭性肿瘤患者转移复发风险更高，但在明显良性变体中也存在该风险（5年风险：分别为100%和1%；<0.0001）。
由于存在长期复发疾病的风险，不仅遗传性PHEO/sPGL，而且诊断时明显良性和散发性肿瘤都需要终身随访。
