Bharti J N
All India Institute of Medical Science, Department of Pathology & Lab Medicine, Mangalagiri, Guntur, India.
Acta Endocrinol (Buchar). 2024 Apr-Jun;20(2):236-238. doi: 10.4183/aeb.2024.236. Epub 2025 Jan 18.
Urinary Bladder paraganglioma accounts for 0.06% of all bladder tumors and 1% of all pheochromocytoma. Most tumors are localized at the dome or trigone and are unifocal. The presenting complaints are painless hematuria, micturition syncope. It may be sporadic or associated with hereditary predisposition syndromes such as Hereditary Paraganglioma-Pheochromocytoma Syndrome.
A 70-year-old male presented with complaints of hematuria. The physical examination was unremarkable. The CT scan revealed an enhancing mass in the lateral wall of bladder. The mass was resected, and histopathological examination showed features of tumor cells arranged in Zell ballen pattern. Tumor cells show mild nuclear pleomorphism, round to oval vesicular nuclei, prominent nucleoli and moderate to abundant granular basophilic cytoplasm. Rare mitosis and no necrosis were noted. On immunohistochemistry, tumor cells were immunoreactive to Synaptophysin, S-100P and negative for CK7 suggesting Paraganglioma. The patient is doing fine after two years of follow up. Due to its rarity, there are no recommendations for treatment and monitoring but, their risk of malignancy forces a long-term follow up. Initial management included early reassessment by cystoscopy, transurethral bladder resection (TURB) and imaging.
膀胱副神经节瘤占所有膀胱肿瘤的0.06%,占所有嗜铬细胞瘤的1%。大多数肿瘤位于膀胱顶部或三角区,且为单病灶。主要症状有无痛性血尿、排尿性晕厥。它可能是散发性的,也可能与遗传性易患综合征有关,如遗传性副神经节瘤-嗜铬细胞瘤综合征。
一名70岁男性因血尿就诊。体格检查无异常。CT扫描显示膀胱侧壁有一强化肿块。切除肿块后,组织病理学检查显示肿瘤细胞呈巢状排列。肿瘤细胞显示轻度核多形性,圆形至椭圆形泡状核,核仁突出,胞质呈中度至丰富的颗粒状嗜碱性。可见罕见的有丝分裂,无坏死。免疫组化显示,肿瘤细胞对突触素、S-100P呈免疫反应性,对CK7呈阴性,提示为副神经节瘤。随访两年后,患者情况良好。由于其罕见性,目前尚无治疗和监测建议,但因其恶性风险,需要长期随访。初始治疗包括早期通过膀胱镜检查、经尿道膀胱切除术(TURB)和影像学进行重新评估。