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伴有假性血管瘤样间质增生的特发性巨乳症:一例报告

Idiopathic gigantomastia with Pseudoangiomatous stromal hyperplasia: A case report.

作者信息

Vashistha Aakansha, Rundla Manish, Khan Farukh, Om Prabha

机构信息

Department of General Surgery, Sawai Mansingh Hospital, Jaipur, India.

Department of General Surgery, Sawai Mansingh Hospital, Jaipur, India.

出版信息

Int J Surg Case Rep. 2020;77:915-919. doi: 10.1016/j.ijscr.2020.09.151. Epub 2020 Sep 25.

Abstract

BACKGROUND

Gigantomastia is a rare condition characterized by excessive diffuse enlargement of both breasts and can be physically and psychosocially disabling for the patient. Despite an extensive search, the etiology remains poorly understood with most common being pubertal and gestational gigantomastia, with incidence of Idiopathic gigantomastia associated with bilateral PASH being extremely rare.

METHODS

A 37 year old lady with bilateral gigantomastia and severe back pain with a normal radiological, hormonal and histopathological evaluation underwent reduction mammoplasty with objective of weight and volume reduction of the breasts along with aesthetic enhancement.

RESULTS

The excised specimen weighed 3.5 and 5 kg respectively in left and right breast with uneventful post operative period and symptomatic relief to the patient. The histopathology was suggestive of macromastia with pseudoangiomatous stromal hyperplasia like areas with focally PR positive status on IHC.

CONCLUSION

Most commonly etiological factor for gigantomastia is endogenous hormone stimulation. While idiopathic gigantomastia is rare those associated with PASH are still rarer with around 13 cases reported in the literature till date. PASH is a beingn mesenchymal proliferative lesion of the breast, mostly found in premenopausal women and rarely manifests clinically. Reduction mammoplasty can make a significant improvement in life of such young patients with explained risk of probability of recurrence. Among the various techniques available inverted T scar pattern with superiomedical pedicle are preferred as the learning curve is shorter, have greater versatility, and is reproducible with consistent results.

摘要

背景

巨乳症是一种罕见的病症,其特征为双侧乳房过度弥漫性增大,会给患者带来身体和心理社会方面的功能障碍。尽管进行了广泛的研究,但病因仍知之甚少,最常见的是青春期和妊娠期巨乳症,特发性巨乳症合并双侧乳晕下纤维性假血管瘤病极为罕见。

方法

一名37岁双侧巨乳症且伴有严重背痛的女性,其放射学、激素和组织病理学评估均正常,接受了乳房缩小成形术,目的是减轻乳房重量和体积并改善美观。

结果

切除标本左、右乳房分别重3.5千克和5千克,术后恢复顺利,患者症状缓解。组织病理学提示为巨乳症,伴有类似假血管瘤样间质增生区域,免疫组化显示局部孕激素受体(PR)呈阳性。

结论

巨乳症最常见的病因是内源性激素刺激。特发性巨乳症罕见,而合并乳晕下纤维性假血管瘤病的则更为罕见,迄今为止文献报道约13例。乳晕下纤维性假血管瘤病是一种良性乳腺间质增生性病变,多见于绝经前女性,临床罕见。乳房缩小成形术可显著改善此类年轻患者的生活质量,但有复发风险。在可用的各种技术中,带内侧上蒂的倒T形瘢痕模式更受青睐,因为其学习曲线较短,适用性更强,且可重复操作,效果一致。

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