Pan Zenggang, Repertinger Susan, Deng Caishu, Sharma Poonam
Department of Pathology, Creighton University Medical Center, 601 N. 30th Street, Omaha, NE 68131, USA.
Endocr Pathol. 2008 Summer;19(2):133-8. doi: 10.1007/s12022-008-9016-4.
Adrenal pheochromocytomas are rare catecholamine-secreting tumors that originate from chromaffin cells in the adrenal medulla, and giant pheochromocytomas with cystic changes are particularly rare. We report a case of a 46-year-old man who presented with episodic hypertension and headache. Radiographic studies showed an 18-cm cystic mass in the left upper quadrant of the abdomen; further workups, which included light microscopy, immunohistochemical, and electron microscopic analysis, revealed a pheochromocytoma of the left adrenal gland. Cytogenetic analysis and genetic mutation analyses for von-Hippel-Lindau (VHL), rearranged during transfection (RET), and succinate dehydrogenase complex subunit B (SDHB) genes were also performed but failed to reveal any abnormalities within the tumor cells.
肾上腺嗜铬细胞瘤是一种罕见的分泌儿茶酚胺的肿瘤,起源于肾上腺髓质的嗜铬细胞,而伴有囊性变的巨大嗜铬细胞瘤则尤为罕见。我们报告一例46岁男性患者,其表现为发作性高血压和头痛。影像学检查显示腹部左上象限有一个18厘米的囊性肿块;进一步检查,包括光学显微镜检查、免疫组织化学和电子显微镜分析,发现是左肾上腺嗜铬细胞瘤。还进行了针对冯-希佩尔-林道(VHL)、转染期间重排(RET)和琥珀酸脱氢酶复合物亚基B(SDHB)基因的细胞遗传学分析和基因突变分析,但未发现肿瘤细胞内有任何异常。