Porter Katherine R, Shroff Seema, Tran Tien-Anh, Neychev Vladimir
Miscellaneous, University of Central Florida College of Medicine, Orlando, USA.
Pathology, AdventHealth, Orlando, USA.
Cureus. 2019 Aug 27;11(8):e5500. doi: 10.7759/cureus.5500.
A 28-year-old woman with a past medical history significant for cervical cancer was diagnosed with a 2.5 cm adrenal tumor but was lost to follow-up. Two years later, she presented to the emergency room with worsening right upper abdominal and flank pain. The computed tomography (CT) and magnetic resonance imaging (MRI) of the abdomen and pelvis revealed that the right adrenal mass had nearly doubled in size (4.3 cm), was heterogeneous with calcifications, central necrosis and actively uptaking the intravenous (IV) contrast with a delayed washout. The biochemical workup was negative for hyperaldosteronism, hypercortisolism, and pheochromocytoma. She reported an unintentional body weight loss of 40 pounds. Adrenocortical carcinoma or a metastatic malignancy was high on the differential diagnoses list. She underwent a successful laparoscopic adrenalectomy, and final pathology revealed a benign extra-adrenal combined ganglioneurofibroma and schwannoma. These rare benign malignancies alone or in combination may closely mimic the clinical and imaging characteristics of adrenal malignancy and pose a diagnostic and therapeutic dilemma to surgeons as well as cause a significant distress to patients and their families. Thus, it is important to thoroughly document and report these cases in order to increase awareness and improve our understanding of the biology, natural history and management of these extremely rare tumors.
一名28岁有宫颈癌病史的女性被诊断出患有一个2.5厘米的肾上腺肿瘤,但失访了。两年后,她因右上腹和侧腹疼痛加重就诊于急诊室。腹部和盆腔的计算机断层扫描(CT)及磁共振成像(MRI)显示,右侧肾上腺肿块大小几乎翻倍(4.3厘米),呈不均匀性,有钙化、中央坏死,且静脉注射造影剂后有强化及延迟廓清。生化检查排除了醛固酮增多症、皮质醇增多症和嗜铬细胞瘤。她自述体重无意中减轻了40磅。鉴别诊断中肾上腺皮质癌或转移性恶性肿瘤的可能性很大。她接受了成功的腹腔镜肾上腺切除术,最终病理显示为良性肾上腺外复合型神经节神经纤维瘤和神经鞘瘤。这些罕见的良性肿瘤单独或合并存在时,可能酷似肾上腺恶性肿瘤的临床和影像学特征,给外科医生带来诊断和治疗上的难题,也给患者及其家属造成极大困扰。因此,全面记录和报告这些病例很重要,以便提高认识,并增进我们对这些极其罕见肿瘤的生物学特性、自然病程及治疗的了解。