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目的:探讨伴空肠转移恶性嗜铬细胞瘤的临床病理特点,提高鉴别诊断良恶性嗜铬细胞瘤的水平。方法:观察1例伴空肠转移恶性嗜铬细胞瘤的临床病理,结合免疫组化染色及电镜结果,并复习相关文献。结果:患者于行右肾上腺肿瘤切除术半年后发现空肠、肝脏及左锁骨上淋巴结转移,初诊9个月后患者死亡。肉眼观:肾上腺肿瘤大小7 cm×5 cm×3 cm,空肠黏膜下肿块大小9 cm×6 cm×6 cm。镜检:瘤组织呈弥漫性、实性、巢状结构,血窦丰富,核分裂计数>3个/10 HPF,伴有融合性坏死,见肿瘤侵犯血管、包膜及肾上腺皮质,空肠肠系膜淋巴结见转移。免疫组化均表达CgA、Syn及NSE,S-100散在阳性。超微结构见高电子密度的神经内分泌颗粒。结论:肾上腺恶性嗜铬细胞瘤转移至空肠极罕见,对恶性嗜铬细胞瘤的诊断应结合临床、手术及病理。
Objective: To investigate the clinicopathological features of malignant pheochromocytoma with jejunal metastasis and to improve the differential diagnosis of benign and malignant pheochromocytoma. Methods: One case of malignant pheochromocytoma with jejunum metastasis was observed with immunohistochemical staining and electron microscopy. The related literatures were reviewed. Results: Six months after the right adrenalectomy, the patients had jejunum, liver and left supraclavicular lymph node metastasis. The patients died after 9 months of initial diagnosis. Macroscopic: adrenal tumor size 7 cm × 5 cm × 3 cm, jejunal submucosal mass size 9 cm × 6 cm × 6 cm. Microscopic examination: tumor tissue was diffuse, solid, nested structure, rich sinusoids, nuclear fission count> 3/10 HPF, associated with fusion necrosis, see the tumor invasion of blood vessels, envelopes and adrenal cortex, see the mesenteric lymph nodes Transfer. Immunohistochemical expression of CgA, Syn and NSE, S-100 scattered positive. Ultrastructural findings of high electron density neuroendocrine granules. CONCLUSIONS: Adrenal malignant pheochromocytoma is rarely seen in the jejunum. The diagnosis of malignant pheochromocytoma should be clinically, surgically and pathologically.