心脏嗜铬细胞瘤临床病理分析

Cardiac pheochromocytoma: a clinicopathologic analysis

目的探讨心脏嗜铬细胞瘤的临床病理特点。方法观察和分析3例心脏嗜铬细胞瘤的病理组织学形态特征及免疫组化标记,并结合文献总结心脏嗜铬细胞瘤的临床和病理学特点。结果患者均以血压增高、多汗、头痛、心悸就诊,24 h尿儿茶酚胺测定值明显增高,早期影像学检查未能发现占位,生长受体抑素显象阳性。光镜下细胞大小差别大,核异型性明显,偶见核分裂;其中2例有周围组织浸润,1例可见片状坏死。免疫组化Ki-67、嗜铬粒素A和S-100均有明确表达,其中2例Ki-67指数>3%。结论心脏嗜铬细胞瘤极罕见,部位特殊使得诊断复杂,病理形态与肾上腺原发嗜铬细胞瘤类似。Ki-67指数>3%,组织伴有坏死,加上术后尿儿茶酚胺仍不正常,提示恶性潜能。

Objective To investigate the clinicopathologic characteristics of cardiac pheochromocytoma. Methods resected cases of cardiac pheochromecytoma from PUMC Hospital were studied clinicopathologically and immunohistechemically. Results Clinical symptoms were relevant to paroxysmal or sustained hypertension, excess 24 hour urinary catecholamine excretion. Mass was not observed on CT and MRI. All 3 patients had positive results with Tc-99m-Otretide scintigraphy. The histology of tumors cells was similar to that of adrenal pheochromocytoma. Two of the tumors showed infiltration in cardiac muscle around the tumor, and focal necrosis was seen in one case. Immunohistochemically, CgA and S-100 were positive in the tumor cells and sustentacular cells respectively, and two cases showed Ki-67 index 〉 3%. Conclusion Cardiac pheochromecytoma is a rare tumor, and its diagnosis is rather difficult and complex. The characteristics under light microscope are the same as adrenal and extra-adrenal pheochromocytoma, and Ki-67 index 〉 3 %, necrosis and sustained abnormal urinary catecholamine excretion after surgery predict malignant potential of the tumor.