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球旁细胞瘤5例临床病理观察

     

摘要

Purpose To study the clinicopathologic features of juxtaglomerular cell tumor ( JGCT) . Methods The microscopic and immunohistochemical features were examined in 5 cases of JGCT, with review of related literatures. Results Four patients were fe-males and 1 was male, average aged 39 years ( range, 26-60 years) . Four patients had hypertension at diagnosis. Postoperative serum renin and aldosterone levels decreased obviously than before the operation in one patient. In one patient tumor had metastasized to liver and bone and died 14 months after operation. Microscopically, the tumors were well circumscribed and at least partially invested by a thick fibrous capsule. Entrapped tubules were identified. Numerous hemangiopericytoma-like vessels were focally present. The tumor tissue was made up of sheets of ovoid to polygonal cells with regular nuclei, distinct cell borders, moderately abundant eosinophilic cy-toplasm. Mast cells and lymphocyte were common. Thick-walled hyalinized blood vessels were obvious. Necrosis and high mitotic ac-tivity were identified in one case. Immunohistochemically, tumor cells were positive for renin, CD34, vimentin, and SMA (focally). Conclusions JGCT is a rare renal neoplasm. Its diagnosis and differential diagnosis must be established on the clinicopathologic fea-tures, immunohistochemical findings and ultrastructural features. Malignant JGCT is very rare. Its criteria are unknown, but malignant JGCT include histological vascular invasion, a large tumor size, relatively advanced patient age or child, necrosis and/or high mitotic activity.%目的:探讨球旁细胞瘤的临床病理学特征。方法采用免疫组化法对5例球旁细胞瘤进行观察并复习相关文献。结果5例中4例女性,1例男性,年龄26~60岁,平均39岁。4例伴有高血压,1例术后肾素和醛固酮水平较术前明显下降。1例术后10个月伴骨和肝多发转移,14个月后死亡。眼观:肿块边界清楚,多有完整的纤维包膜。镜检:细胞实性,成片分布,中间可见残存肾小管。部分形成血管外皮瘤样结构。间质中血管丰富,血管壁厚、玻璃样变性。同时可见肥大细胞和淋巴细胞浸润。细胞之间界限较清,胞质嗜酸性或透明,部分细胞核周有空晕,细胞核圆形,部分可见核仁,核分裂少见。1例恶性,可见凝固性坏死,明显的细胞异型以及活跃的核分裂。免疫表型:CD34、vimentin和renin均阳性,SMA部分阳性。结论球旁细胞瘤是一种罕见的肾脏肿瘤,结合临床病理学特征、免疫表型和电镜结果有助于诊断。

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