PDF(Pubmed)
Abstract：
Metastatic pheochromocytoma/paraganglioma (MPP) is a rare endocrine tumor that originates from extra-adrenal chromaffin cells such as the paraganglia cells of sympathetic and parasympathetic nerves. It usually causes multiple solid tumors and exhibits strong aggressiveness with poor prognosis, with a reported 5-year survival rate of less than 50%. Cases of brain and retroperitoneal metastases at the initial diagnosis have not yet been reported. We report a 41-year-old male patient initially diagnosed with MPP in the brain and retroperitoneum who underwent multi-disciplinary collaborative surgery and simultaneous removal of two tumors at our center. Postoperative pathology revealed infiltrative growth of a skull base tumor. The patient chose to receive the tyrosine kinase inhibitor sunitinib as a targeted treatment. A 3-month follow-up after surgery showed that the patient recovered well without signs of metastasis or recurrence. We present multi-disciplinary surgery under similar circumstances for enhanced treatment and postoperative management. The patient demonstrates a favorable prognosis during postoperative follow-up, indicating that simultaneous multidisciplinary surgery may offer greater benefits for MPP patients.
摘要：
转移性嗜铬细胞瘤/副神经节瘤（MPP）是一种罕见的内分泌肿瘤，起源于肾上腺外嗜铬细胞，例如交感神经和副交感神经的副神经节细胞。它通常会导致多个实体瘤，并表现出强烈的侵袭性，预后不良。据报道，5年生存率低于50％。尚未报道最初诊断时脑和腹膜后转移的病例。我们报告了一名41岁的男性患者，最初被诊断为大脑和腹膜后MPP，他在我们中心接受了多学科协作手术并同时切除了两个肿瘤。术后病理显示颅底肿瘤浸润性生长。患者选择接受酪氨酸激酶抑制剂舒尼替尼作为靶向治疗。术后3个月随访显示，患者恢复良好，无转移或复发迹象。我们提出了类似情况下的多学科手术，以加强治疗和术后管理。患者在术后随访期间表现出良好的预后，表明同时进行多学科手术可能为MPP患者带来更大的益处.
