Pehlivan Umur Anil, Oktay Kadir, Onan Hasan Bilen, Acikalin Arbil, Keser Celalet, Paydas Semra
Department of Radiology, Van Başkale State Hospital, Van, Turkey.
Department of Neurosurgery, Çukurova University School of Medicine, Adana, Turkey.
Curr Med Imaging. 2022;18(13):1439-1442. doi: 10.2174/1573405618666220602121607.
Paragangliomas are tumors of neuroendocrine origin, may appear in different localizations, and are related to the autonomic nervous system. Paragangliomas are generally asymptomatic and may rarely appear with adrenergic symptoms, and clinical findings depend on the catecholamines they secrete. Extra-adrenal paragangliomas are mostly benign, like all paragangliomas. Malignancy criteria consist of local recurrence, metastasis after total resection, and presence of distant metastasis during primary diagnosis.
This report presents the case of a 31-year-old man with jugular paraganglioma, multiple skeletal metastases, and a long-segment tumor thrombus. Imaging procedures showed a continuous tumor thrombus extending from the posterior fossa to the right atrium and metastases in C2, T1, T6, T8, L5, and right humerus. Histopathological assessment of the metastasis in C2 identified malignant paraganglioma. Curative surgery was not an option for this patient, hence combined chemotherapy was given.
In cases of malignant paraganglioma with multiple distant metastases, chemotherapy and radiotherapy are feasible treatment methods.
副神经节瘤是神经内分泌起源的肿瘤,可出现在不同部位,与自主神经系统有关。副神经节瘤通常无症状,很少出现肾上腺素能症状,临床症状取决于其分泌的儿茶酚胺。与所有副神经节瘤一样,肾上腺外副神经节瘤大多为良性。恶性标准包括局部复发、全切除术后转移以及初诊时存在远处转移。
本报告介绍了一名31岁男性患者,患有颈静脉副神经节瘤、多处骨骼转移及长节段肿瘤血栓。影像学检查显示,肿瘤血栓连续,从后颅窝延伸至右心房,C2、T1、T6、T8、L5和右肱骨有转移灶。对C2转移灶的组织病理学评估确诊为恶性副神经节瘤。该患者无法进行根治性手术,因此给予联合化疗。
对于伴有多处远处转移的恶性副神经节瘤病例,化疗和放疗是可行的治疗方法。