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由产生促肾上腺皮质激素的胸腺典型类癌伴局部侵犯和区域淋巴结转移引起的库欣综合征:一例报告

Cushing's syndrome caused by ACTH-producing thymic typical carcinoid with local invasion and regional lymph node metastasis: a case report.

作者信息

Fujiwara Wakako, Haruki Tomohiro, Kidokoro Yoshiteru, Ohno Takashi, Yurugi Yohei, Miwa Ken, Taniguchi Yuji, Nakamura Hiroshige

机构信息

Division of General Thoracic Surgery, Department of Surgery, Faculty of Medicine, Tottori University, 36-1, Nishi-cho, Yonago, 683-8504, Japan.

出版信息

Surg Case Rep. 2018 Jun 11;4(1):55. doi: 10.1186/s40792-018-0459-7.

Abstract

BACKGROUND

Ectopic ACTH-producing thymic carcinoid tumors are rare, but often behave aggressively with local invasion and distant metastasis. We herein report a case of ACTH-producing thymic typical carcinoid tumor with lymph node metastasis treated by surgery and postoperative radiation therapy.

CASE PRESENTATION

A 61-year-old woman was admitted to be evaluated for hypoglycemia and hypokalemia. Laboratory data revealed elevation of serum cortisol and ACTH levels. Overnight administration of 8 mg dexamethasone did not suppress plasma ACTH. Chest CT demonstrated a tumor of 30 mm in diameter and enlargement of the lymph node at the anterior mediastinum. Ectopic ACTH syndrome was suspected and total thymectomy and lymph node dissection were performed. The histopathological examination indicated typical carcinoid tumor and mediastinal lymph node metastasis, and immunohistochemical staining was positive for ACTH. The plasma ACTH level decreased immediately after surgery. She received postoperative radiation therapy of 60 Gy.

CONCLUSION

Ectopic ACTH-producing thymic typical carcinoid tumors are rare, and it is important to consider this disease and perform appropriate treatment.

摘要

背景

异位分泌促肾上腺皮质激素(ACTH)的胸腺类癌肿瘤罕见,但常具有侵袭性,可发生局部侵犯和远处转移。我们在此报告1例分泌ACTH的胸腺典型类癌伴淋巴结转移患者,经手术及术后放射治疗。

病例介绍

一名61岁女性因低血糖和低钾血症入院评估。实验室检查显示血清皮质醇和ACTH水平升高。夜间给予8mg地塞米松未能抑制血浆ACTH。胸部CT显示直径30mm的肿瘤及前纵隔淋巴结肿大。怀疑为异位ACTH综合征,遂行全胸腺切除术及淋巴结清扫术。组织病理学检查显示为典型类癌肿瘤及纵隔淋巴结转移,免疫组化染色ACTH呈阳性。术后血浆ACTH水平立即下降。患者接受了60Gy的术后放射治疗。

结论

异位分泌ACTH的胸腺典型类癌肿瘤罕见,考虑此病并进行适当治疗很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/22de/5995764/d7566b3c5269/40792_2018_459_Fig1_HTML.jpg

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