Was It an Adrenocortical Adenoma or an Adrenocortical Carcinoma? Limitation of the Weiss Scoring System in Determining the Malignant Potential of Adrenocortical Tumor: Report on Two Cases.

IF 0.9 Q4 ENDOCRINOLOGY & METABOLISM
Case Reports in Endocrinology Pub Date : 2022-09-14 eCollection Date: 2022-01-01 DOI:10.1155/2022/7395050
Cheuk-Lik Wong, Chun-Kit Fok, Yuk-Kit Chan, Vicki Ho-Kee Tam, Lai-Ming Fung
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引用次数: 3

Abstract

Background: Adrenocortical carcinoma (ACC) is a rare endocrine malignancy. An accurate diagnosis of ACC is of paramount importance as it greatly impacts the management and prognosis of a patient. However, the differentiation between early stage, low-grade ACC and adrenocortical adenoma (ACA) may not always be straightforward. The recommended classification system, namely, the Weiss scoring system, is not without flaws. We herein report two cases of ACC which were initially diagnosed as ACA according to the Weiss scoring system but developed distant metastases in subsequent years. Case Presentation. Case 1: A 60-year-old Chinese woman presented with a recent onset of worsening of blood pressure control and clinical features of Cushing's syndrome. Investigations confirmed ACTH-independent endogenous hypercortisolism, and a CT abdomen showed a 6 cm right adrenal mass. Twenty-four-hour urine steroid profiling revealed co-secretion of adrenal androgens and atypical steroid metabolites. Laparoscopic right adrenalectomy was performed, and pathology of the tumor was classified as an ACA by the Weiss scoring system. Four years later, the patient presented with an abrupt onset of severe hypercortisolism and was found to have a metastatic recurrence in the liver and peritoneum. The patient received a combination of mitotane, systemic chemotherapy, and palliative debulking surgery and succumbed 8.5 years after the initial presentation due to respiratory failure with extensive pulmonary metastases. Case 2: A 68-year-old Chinese woman presented with acute bilateral pulmonary embolism and was found to have a 3 cm left adrenal mass. Hormonal workup confirmed ACTH-independent endogenous hypercortisolism, and laparoscopic left adrenalectomy revealed an ACA according to the Weiss scoring system. Five years later, she presented with recurrent hypercortisolism due to hepatic and peritoneal metastases. The patient had progressive disease despite mitotane therapy and succumbed 7 years after initial presentation.

Conclusions: Although the Weiss scoring system is recommended as the reference pathological classification system to diagnose adrenocortical carcinoma, there remain tumors of borderline malignant potential which may escape accurate classification. Various alternative classification systems and algorithms exist but none are proven to be perfect. Clinicians should recognize the potential limitation of these histological criteria and scoring systems and incorporate other clinical parameters, such as the pattern of hormonal secretion, urinary steroid profiling, and radiographic features, to improve the prognostication and surveillance strategy of these tumors.

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是肾上腺皮质腺瘤还是肾上腺皮质癌?Weiss评分系统在判断肾上腺皮质肿瘤恶性潜能方面的局限性:附两例报告。
背景:肾上腺皮质癌是一种罕见的内分泌恶性肿瘤。准确诊断ACC是至关重要的,因为它极大地影响了患者的治疗和预后。然而,早期、低级别ACC和肾上腺皮质腺瘤(ACA)之间的区别可能并不总是直截了当的。推荐的分类系统,即韦斯评分系统,并非没有缺陷。我们在此报告两例ACC,最初根据Weiss评分系统诊断为ACA,但在随后的几年中发展为远处转移。案例演示。病例1:一名60岁的中国女性,最近出现血压控制恶化和库欣综合征的临床特征。调查证实acth非依赖性内源性高皮质醇症,腹部CT显示右侧肾上腺肿块6厘米。24小时尿液类固醇分析显示肾上腺雄激素和非典型类固醇代谢物的共同分泌。行腹腔镜右肾上腺切除术,肿瘤病理按Weiss评分系统分类为ACA。四年后,患者突然出现严重的高皮质醇血症,并在肝脏和腹膜发现转移性复发。患者接受了米托坦、全身化疗和姑息性减容手术的联合治疗,并在首次就诊8.5年后因呼吸衰竭伴广泛肺转移而死亡。病例2:一名68岁的中国女性表现为急性双侧肺栓塞,发现左肾上腺有一个3厘米的肿块。激素检查证实acth非依赖性内源性高皮质醇症,根据Weiss评分系统,腹腔镜左肾上腺切除术显示ACA。5年后,由于肝脏和腹膜转移,她再次出现高皮质醇血症。尽管米托坦治疗,患者病情仍在进展,并在首次就诊7年后死亡。结论:虽然推荐使用Weiss评分系统作为诊断肾上腺皮质癌的参考病理分类系统,但仍存在交界性恶性肿瘤,可能无法准确分类。存在各种可供选择的分类系统和算法,但没有一个被证明是完美的。临床医生应该认识到这些组织学标准和评分系统的潜在局限性,并结合其他临床参数,如激素分泌模式、尿类固醇谱和放射学特征,以改善这些肿瘤的预后和监测策略。
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来源期刊
Case Reports in Endocrinology
Case Reports in Endocrinology ENDOCRINOLOGY & METABOLISM-
CiteScore
2.10
自引率
0.00%
发文量
45
审稿时长
13 weeks
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