McCune-Albright综合征继发性动脉瘤性骨囊肿。

Q3 Medicine
Symeon Tournis, Alexia Balanika, Panayiotis D Megaloikonomos, Andreas F Mavrogenis
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引用次数: 7

摘要

多骨赘纤维发育不良合并卡萨劳-莱斑疹和内分泌功能亢进症构成一种罕见的临床症状,称为麦库恩-奥尔布赖特综合征。动脉瘤性骨囊肿是肿瘤样囊性病变,由充满血液的腔室组成。它们可能是原发病变或继发于其他病理;最常见的是骨巨细胞瘤。然而,麦丘内-奥尔布赖特综合征的继发性动脉瘤性骨囊肿是例外。我们报告一名28岁女性患有麦库恩-奥尔布赖特综合征。她在3个月大时经历了性早熟。在童年时期,她经历了多次长骨骨折,面部畸形和进行性视力和听力障碍。一年前,她经历了一个痛苦的,逐渐扩大的骨病变,包括右髂骨,耻骨和坐骨,并伴有磨玻璃样,间隔,边缘硬化,骨内扇贝和吹出扩张,导致局部皮质变薄。ct引导下盆腔病变穿刺活检显示动脉瘤样骨囊肿。推荐选择性动脉栓塞,但患者及其亲属不同意继续治疗,此后仍对其进行密切监测。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Secondary aneurysmal bone cyst in McCune-Albright syndrome.

Secondary aneurysmal bone cyst in McCune-Albright syndrome.

Secondary aneurysmal bone cyst in McCune-Albright syndrome.

Secondary aneurysmal bone cyst in McCune-Albright syndrome.

Polyostotic fibrous dysplasia in combination with caféau-lait macules and hyperfunctioning endocrinopathies consists of a rare clinical condition termed as McCune-Albright syndrome. Aneurysmal bone cysts are tumor-like cystic lesions, composed of blood-filled compartments. They may occur as primary lesions or secondary to other pathologies; most commonly giant cell tumors of bone. However, secondary aneurysmal bone cysts in McCune-Albright syndrome are exceptional. We present a 28-year-old female with McCune-Albright syndrome. She experienced precocious puberty at age 3 months. In childhood, she experienced multiple long bone fractures, facial deformity and progressive visual and hearing impairment. One year ago, she experienced a painful, gradually enlarging bone lesion involving the right ilium, pubic and ischial bone with groundglass appearance, septa, marginal sclerosis, endosteal scalloping and blow-out expansion resulting in localized thinning of the cortex. CT-guided needle biopsy of the pelvic lesion showed aneurysmal bone cyst. Selective arterial embolization was recommended, however, the patient and her relatives did not consent to proceed to treatment, and she remained in close surveillance thereafter.

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来源期刊
Clinical Cases in Mineral and Bone Metabolism
Clinical Cases in Mineral and Bone Metabolism ENDOCRINOLOGY & METABOLISM-
CiteScore
2.60
自引率
0.00%
发文量
0
期刊介绍: The Journal encourages the submission of case reports and clinical vignettes that provide new and exciting insights into the pathophysiology and characteristics of disorders related to skeletal function and mineral metabolism and/or highlight pratical diagnostic and /or therapeutic considerations.
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