Nandakumar Devika, Rajan Remya, Pai Rekha, Sigamani Elanthenral, Kapoor Nitin, Thomas Nihal, Paul Thomas, Jebasingh Felix, Cherian Kripa, Paul M J, Jacob Anish, Thomas Shawn, Sen Supriya, Manipadam Marie Therese, Chandramohan Anuradha, Singh Ashish, Hephzibah Julie, Kumar Santhosh, Santhanam Prisca, Asha Hesarghatta Shyamasunder
Department of Endocrinology, Diabetes and Metabolism, Christian Medical College, Vellore, Tamil Nadu, India.
Department of Molecular Pathology, Christian Medical College, Vellore, Tamil Nadu, India.
Clin Endocrinol (Oxf). 2025 Oct;103(4):480-486. doi: 10.1111/cen.70007. Epub 2025 Jul 30.
Malignant pheochromocytoma and paraganglioma (mPPGL) are not so common and the factors predicting the metastatic behavior are not well understood.
This retrospective cohort study included 218 patients with PPGL managed at our center over a period of 11 years (2013-2024). The clinical profile and treatment outcomes of patients with mPPGL were studied and were compared with non-metastatic PPGL(non-mPPGL).
Thirty-six patients had mPPGL and the median age at diagnosis of metastases was 39.5 years(range 10-62). Twenty-one(58.3%) patients had synchronous metastases and 15(41.7%) developed metachronous metastases after a median duration of 76 months(range 13-270 months) from the diagnosis of the primary tumor. Metastases were detected in 100% of patients who had 18FDG(4/4) and 68 Ga DOTATATE(4/4) PET-CT, 97.2% (35/36) with CT/MRI and 79.3%(23/29) with 131I MIBG scan. Surgery was the primary treatment in 78%, and 131I MIBG therapy was administered to 19(52.8%) patients. Eleven patients succumbed due to metastatic disease and among them nine died within a year of diagnosis of metastases, the median survival at last follow-up was 31.5 months (range 3-96). On comparing mPPGL(n = 36) and non-mPPGL(n = 182), we found that patients with mPPGL had larger tumors (8.8 ± 5.2 vs. 6.3 ± 3.3, p = 0.001), had less frequent adrenergic symptoms and more often had extra-adrenal tumors.
Malignant PPGLs had a variable clinical course and were amenable to multimodal therapeutic strategies with a favorable outcome in about 67% of the patients. Although mPPGL had larger tumor diameter compared to non-mPPGL, no particular size cut-off could accurately predict metastases. Adjuvant 131I MIBG therapy is a useful treatment option in resource limited settings.
恶性嗜铬细胞瘤和副神经节瘤（mPPGL）并不常见，且预测其转移行为的因素尚不清楚。
这项回顾性队列研究纳入了在我们中心11年（2013 - 2024年）期间接受治疗的218例PPGL患者。对mPPGL患者的临床特征和治疗结果进行了研究，并与非转移性PPGL（non - mPPGL）进行了比较。
36例患者患有mPPGL，转移诊断时的中位年龄为39.5岁（范围10 - 62岁）。21例（58.3%）患者发生同步转移，15例（41.7%）在原发性肿瘤诊断后中位76个月（范围13 - 270个月）发生异时转移。100%接受18FDG（4/4）和68Ga DOTATATE（4/4）PET - CT检查的患者检测到转移，CT/MRI检测到转移的比例为97.2%（35/36），131I MIBG扫描检测到转移的比例为79.3%（23/29）。78%的患者以手术作为主要治疗方法，19例（52.8%）患者接受了131I MIBG治疗。11例患者因转移性疾病死亡，其中9例在转移诊断后一年内死亡，最后一次随访时的中位生存期为31.5个月（范围3 - 96个月）。比较mPPGL（n = 36）和non - mPPGL（n = 182），我们发现mPPGL患者的肿瘤更大（8.8 ± 5.2 vs. 6.3 ± 3.3，p = 0.001），肾上腺素能症状较少，且肾上腺外肿瘤更为常见。
恶性PPGL临床病程多变，可采用多模式治疗策略，约67%的患者预后良好。虽然与non - mPPGL相比，mPPGL的肿瘤直径更大，但没有特定的大小阈值能够准确预测转移。在资源有限的情况下，辅助性131I MIBG治疗是一种有用的治疗选择。
