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肾上腺皮质癌中的副肿瘤综合征:意想不到的模仿者。

Paraneoplastic Syndrome in Adrenocortical Carcinoma: The Unexpected Mime.

作者信息

Nava Suarez Corina, Prater Janna, Mayrin Jane, Vorokhib Galyna, Corrado Minimo

机构信息

Endocrinology, Jefferson Einstein Philadelphia Hospital, Philadelphia, USA.

Pathology, Jefferson Einstein Philadelphia Hospital, Philadelphia, USA.

出版信息

Cureus. 2024 Jun 30;16(6):e63534. doi: 10.7759/cureus.63534. eCollection 2024 Jun.

DOI:10.7759/cureus.63534
PMID:39086789
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11288974/
Abstract

Adrenocortical carcinoma (ACC) is a malignancy of the adrenal cortex with a high morbidity and mortality. More than half of the cases are functional tumors. As different hormones can be co-secreted above physiologic levels, it causes a very broad variety of symptoms and makes differentiating from more common entities hard. Here we present a case of a patient with a newly diagnosed ACC who initially presented with acute pulmonary embolism and recurrent deep vein thromboses (DVT) in the setting of hypercortisolism. Imaging showed a left adrenal mass invading adjacent structures including a nonocclusive thrombus in the left renal vein. Intravenous anticoagulation and thrombectomy were initially performed, followed by removal of the tumor and adjacent metastatic disease. Pathology confirmed ACC. The patient underwent left adrenalectomy, left nephrectomy, splenectomy, distal pancreatectomy, and caval thrombectomy with inferior vena cava (IVC) filter placement. Intravenous anticoagulation and glucocorticoid replacement were also administered as part of the treatment plan. Unfortunately, the patient had multiple episodes of bleeding and thrombosis and was eventually discharged to hospice care. DVT in the setting of ACC can be caused by increased hypercoagulability from hypercortisolism, direct venous thrombosis, or vascular invasion. Thrombosis, especially in the inferior vena cava, has been associated with poor prognosis and survival rates. Clinicians should be aware of this rare complication given its immediate therapeutic repercussions and prognostic value.

摘要

肾上腺皮质癌(ACC)是一种肾上腺皮质的恶性肿瘤,发病率和死亡率都很高。超过半数的病例为功能性肿瘤。由于不同激素可在生理水平以上共同分泌,其会引发多种多样的症状,且难以与更常见的疾病相鉴别。在此,我们报告一例新诊断为ACC的患者,该患者最初表现为急性肺栓塞和在皮质醇增多症背景下的复发性深静脉血栓形成(DVT)。影像学检查显示左侧肾上腺肿块侵犯相邻结构,包括左肾静脉内的非闭塞性血栓。最初进行了静脉抗凝和血栓切除术,随后切除了肿瘤及相邻的转移性病变。病理证实为ACC。患者接受了左肾上腺切除术、左肾切除术、脾切除术、远端胰腺切除术以及下腔静脉(IVC)滤器置入下腔静脉血栓切除术。静脉抗凝和糖皮质激素替代治疗也作为治疗方案的一部分予以实施。不幸的是,患者出现了多次出血和血栓形成事件,最终出院接受临终关怀。ACC背景下的DVT可能由皮质醇增多症导致的高凝状态增加、直接静脉血栓形成或血管侵犯引起。血栓形成,尤其是下腔静脉血栓形成,与预后不良和生存率相关。鉴于其直接的治疗影响和预后价值,临床医生应意识到这种罕见的并发症。

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本文引用的文献

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A Case of Oncocytic Adrenal Cortical Neoplasm with Uncertain Malignant Potential Turned Out to Be Oncocytic Adrenal Cortical Carcinoma with Distant Metastasis: Could Pathology Do Better Initially?一例具有不确定恶性潜能的嗜酸细胞瘤样肾上腺皮质肿瘤最终被诊断为具有远处转移的嗜酸细胞瘤样肾上腺皮质癌:最初病理科能否做得更好?
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Adrenal Incidentaloma.肾上腺偶发瘤
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CUSHING'S SYNDROME, A RISK FACTOR FOR VENOUS THROMBOEMBOLISM IS A CANDIDATE FOR GUIDELINES.
库欣综合征作为静脉血栓栓塞的一个风险因素,是指南的适用对象。
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