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肾上腺成熟畸胎瘤的恶性转化:1例罕见病例报告及文献复习

Malignant transformation of a mature teratoma of the adrenal gland: A rare case report and literature review.

作者信息

Niu Miao, Liu Ailian, Zhao Ying, Feng Lu

机构信息

The First Affiliated Hospital of Dalian Medical University-Radiology Department The First Affiliated Hospital of Dalian Medical University-Pathology Department, Liaoning, Dalian, China.

出版信息

Medicine (Baltimore). 2017 Nov;96(45):e8333. doi: 10.1097/MD.0000000000008333.

Abstract

RATIONALE

Adrenal mature TMT is very rare. So far, only two cases have been reported.

PATIENT

We report a rare case of malignant transformation of a mature teratoma in the right adrenal gland of a 36-year-old Chinese female. The patient had been asymptomatic until physical exam identified a palpable mass in the right abdomen. Abdominal non-enhanced computed tomography (CT) scan showed a huge tumor with mixed density in right adrenal region, which had cystic components, fat, calcifications, and separations. Contrast-enhanced CT showed significant enhancement of the cyst wall and septations as well as mural nodules. In addition, the patient had four abnormal laboratory results, including cortisol 773.58 mol/L ↑ (reference range 118.6 to 618 mol/L), adrenocorticotropic hormone (ACTH) 70.980 pg/ml ↑ (reference range 7.2 to 63.3 pg/ml), aldosterone 317.84 pg/ml ↑ (reference range prone: 49.3-175 pg/ml; Standing: 34.7-275 pg/ml; 24h urine 2.84-33.99 ug/d), and vanillyl mandelic acid (VMA) 22.38 mg / 24 h ↑ (reference range 0-13.6 mg / 24 h) which to our knowledge have not been reported by any adrenal mature teratoma's reference literature.

DIGNOSES

It was initially diagnosed as a right adrenal hamartoma by the radiologist. Final pathology confirmed it as malignant transformation of a right adrenal mature teratoma, which is extremely rare.

INTERVENTIONS

Laparoscopic resection is usually the treatment of choice for adrenal teratoma and our case report is no exception.

OUTCOMES

This article is a case report, no outcomes.

LESSONS

Non-enhanced CT findings of a mass with cystic change, fat, calcification and separation are suggestive of a teratoma. If there is significant enhancement of the cyst wall and septations as well as mural nodules in contrast-enhanced CT, then adrenal TMT should be considered. In addition, abnormal levels of hormones including cortisol, ACTH, aldosterone and VMA may also aid in the diagnosis of adrenal TMT.

摘要

原理

肾上腺成熟畸胎瘤非常罕见。迄今为止,仅报道过两例。

患者

我们报告了一例罕见的36岁中国女性右肾上腺成熟畸胎瘤恶变病例。该患者在体检发现右腹部可触及肿块之前一直无症状。腹部非增强计算机断层扫描(CT)显示右肾上腺区域有一个密度混合的巨大肿瘤,有囊性成分、脂肪、钙化和分隔。增强CT显示囊壁、分隔及壁结节有明显强化。此外,该患者有四项实验室检查结果异常,包括皮质醇773.58 μmol/L↑(参考范围118.6至618 μmol/L)、促肾上腺皮质激素(ACTH)70.980 pg/ml↑(参考范围7.2至63.3 pg/ml)、醛固酮317.84 pg/ml↑(参考范围卧位:49.3 - 175 pg/ml;立位:34.7 - 275 pg/ml;24小时尿2.84 - 33.99 μg/d)以及香草扁桃酸(VMA)22.38 mg / 24 h↑(参考范围0 - 13.6 mg / 24 h),据我们所知,任何肾上腺成熟畸胎瘤的参考文献均未报道过这些情况。

诊断

放射科医生最初诊断为右肾上腺错构瘤。最终病理证实为右肾上腺成熟畸胎瘤恶变,极为罕见。

干预措施

腹腔镜切除术通常是肾上腺畸胎瘤的首选治疗方法,我们的病例报告也不例外。

结果

本文为病例报告,无结果。

经验教训

非增强CT显示肿块有囊性改变、脂肪、钙化和分隔提示为畸胎瘤。如果增强CT显示囊壁、分隔及壁结节有明显强化,则应考虑肾上腺畸胎瘤恶变。此外,包括皮质醇、ACTH、醛固酮和VMA在内的激素水平异常也可能有助于肾上腺畸胎瘤恶变的诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7caa/5690708/6e695aeaa685/medi-96-e8333-g002.jpg

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