Patel Erica, Chen Yufei, Fan Xuemo, Liu Ning-Ai, Westreich Anne Marie, Reyes Kathryn, Labadzhyan Artak
Division of Endocrinology and Metabolism, Cedars-Sinai Medical Center, Los Angeles, California.
Division of Surgery, Cedars-Sinai Medical Center, Cedars-Sinai Medical Center, Los Angeles, California.
AACE Clin Case Rep. 2022 Dec 2;9(1):17-20. doi: 10.1016/j.aace.2022.11.005. eCollection 2023 Jan-Feb.
BACKGROUND/OBJECTIVE: Adrenal corticomedullary mixed tumor (CMMT) are extremely rare single adrenal tumor masses containing a mixture of adrenal cortical adenoma and pheochromocytoma cells.
A 52-year-old woman presented with clinical and biochemical evidence of cortisol and catecholamine excess and was found to have an adrenal CMMT with intermixed chromaffin, cortical adenoma, and ganglioneuroma components. She underwent a successful unilateral adrenalectomy with subsequent improvement in her symptoms.
We report the first case of a patient with a CMMT that had symptoms of both catecholamine and cortisol excess from her tumor. Typically, patients with similar tumors have signs of cortisol excess; however, the pheochromocytoma portion is clinically silent. Although most CMMT contain 2 distinct cell types, this is the third ever described case of a single adrenal CMMT containing 3 unique cellular components: (1) intermixed chromaffin, (2) cortical adenoma, and (3) ganglioneuroma cells.
Our understanding of these rare tumors is limited, and this case serves to broaden our knowledge about their clinical, biochemical, and pathologic features.
背景/目的:肾上腺皮质髓质混合瘤(CMMT)是极为罕见的单一肾上腺肿瘤块,包含肾上腺皮质腺瘤和嗜铬细胞瘤细胞的混合物。
一名52岁女性出现皮质醇和儿茶酚胺过量的临床及生化证据,被发现患有肾上腺CMMT,其包含嗜铬细胞、皮质腺瘤和神经节神经瘤成分。她接受了成功的单侧肾上腺切除术,随后症状有所改善。
我们报告了首例患有CMMT的患者,其肿瘤同时出现儿茶酚胺和皮质醇过量症状。通常,患有类似肿瘤的患者有皮质醇过量的体征;然而,嗜铬细胞瘤部分在临床上无症状。尽管大多数CMMT包含2种不同细胞类型,但这是第三例被描述的单一肾上腺CMMT,包含3种独特细胞成分:(1)嗜铬细胞混合,(2)皮质腺瘤,(3)神经节神经瘤细胞。
我们对这些罕见肿瘤的了解有限,该病例有助于拓宽我们对其临床、生化和病理特征的认识。