关键词: Cushing’s syndrome adrenal tumor mixedcorticomedullary tumor pheochromocytoma

来  源:   DOI:10.3390/medicina59091539   PDF(Pubmed)

Abstract:
Adrenal mixed corticomedullary tumors (MCMTs) are composed of an admixture of cortical and medullary cells. Owing to the presence of two distinct components of different embryonic lineage, these tumors are extremely rare. Less than 30 tumors of this type have been reported to date. MCMTs have varied presentation including hypertension, Cushing syndrome or even as adrenal incidentalomas. Also noted is a slightly higher female preponderance. We report a case of a 26-year-old female who was evaluated for uncontrolled hypertension. A renal ultrasound followed by MRI abdomen revealed a 9.3 × 8.1 × 7.0 cm partially cystic, partially solid enhancing mass in the region of/replacing the left adrenal gland. Hormonal work-up was significant for elevated catecholamines concerning pheochromocytoma. She underwent laparoscopic left adrenalectomy, with adequate pre-operative adrenergic blockade. Histology and immunochemical testing were consistent with a mixed corticomedullary tumor. She was monitored annually for recurrence of the tumor. We also performed a comprehensive review of literature of the cases published so far to the best of our knowledge.
摘要:
肾上腺混合性皮质髓质肿瘤(MCMT)由皮质和髓质细胞的混合物组成。由于存在不同胚胎谱系的两种不同成分,这些肿瘤极为罕见。迄今为止,已经报道了不到30种这种类型的肿瘤。MCMT有不同的表现,包括高血压,库欣综合征或甚至作为肾上腺偶发瘤。还注意到女性的优势略高。我们报告了一例26岁女性,接受了不受控制的高血压评估。肾脏超声,然后MRI腹部显示9.3×8.1×7.0cm部分囊性,部分固体增强肿块/替换左肾上腺区域。激素检查对于与嗜铬细胞瘤有关的儿茶酚胺升高具有重要意义。她接受了腹腔镜左肾上腺切除术,有足够的术前肾上腺素能阻滞。组织学和免疫化学测试与混合皮质髓质肿瘤一致。每年对她进行肿瘤复发监测。据我们所知,我们还对迄今为止发表的病例文献进行了全面审查。
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