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超敏性埃勒斯-当洛综合征侵袭性导管癌的治疗:一例报告

Management of invasive ductal carcinoma in hypermobile Ehlers-Danlos syndrome: a case report.

作者信息

Deshpande Nikita, Murti Stephanie, Singh Rashi, Coker Cameron, Rudra Sonali, Paudel Nitika, Fan Kenneth L, Greenwalt Ian T

机构信息

Georgetown University School of Medicine, Washington, DC, USA.

Kansas City University School of Medicine, Kansas City, MO, USA.

出版信息

Gland Surg. 2021 Sep;10(9):2861-2866. doi: 10.21037/gs-21-380.

Abstract

Literature is sparse regarding the management and long-term outcomes of breast cancer in patients with Ehlers-Danlos syndrome (EDS). Of the EDS subtypes, hypermobile Ehlers-Danlos Syndrome (hEDS) is associated with cardiovascular dysautonomia which manifests as spontaneous episodes of tachycardia and hypotension. Given this clinical autonomic system impact, hEDS is known to have significant intraoperative risk and postoperative complications. However, outcomes of hEDS patients have not been specifically studied in the field of breast cancer surgery. Here we present a case of a 62-year-old female with hEDS and node-positive invasive ductal breast carcinoma. Given the patient's medical history of hEDS, close attention was given to the patient's intraoperative vital signs and predisposition for poor wound healing. The patient underwent left Goldilocks mastectomy with left axillary lymph node dissection. Due to cardiac comorbidities, she was not a candidate for neoadjuvant or adjuvant chemotherapy. The patient tolerated adjuvant radiation and endocrine therapy without side effects, and has remained free of local, regional, and distant cancer recurrence following treatment. This case report highlights a literature gap in the surgical and radiation therapy management of breast cancer in patients with hEDS. Although breast surgery and radiation therapy in patients with invasive breast cancer and hEDS can be a safe management option, we discuss how perioperative complications must be cautiously navigated and how treatment must be tailored to individuals' specific hEDS variant to ensure optimal patient safety and positive long-term outcomes.

摘要

关于埃勒斯-当洛综合征(EDS)患者乳腺癌的管理和长期预后,相关文献较少。在EDS的亚型中,活动过度型埃勒斯-当洛综合征(hEDS)与心血管自主神经功能障碍有关,表现为心动过速和低血压的自发发作。鉴于这种对临床自主神经系统的影响,hEDS已知具有显著的术中风险和术后并发症。然而,hEDS患者在乳腺癌手术领域的预后尚未得到专门研究。在此,我们报告一例62岁患有hEDS且淋巴结阳性的浸润性导管乳腺癌女性患者。鉴于患者有hEDS病史,术中密切关注患者生命体征以及伤口愈合不良的易感性。患者接受了左侧Goldilocks乳房切除术及左侧腋窝淋巴结清扫术。由于存在心脏合并症,她不适合进行新辅助或辅助化疗。患者耐受了辅助放疗和内分泌治疗且无副作用,治疗后未出现局部、区域和远处癌症复发。本病例报告突出了hEDS患者乳腺癌手术和放疗管理方面的文献空白。尽管浸润性乳腺癌合并hEDS患者的乳房手术和放疗可能是一种安全的管理选择,但我们讨论了如何谨慎应对围手术期并发症,以及如何根据个体特定的hEDS变体调整治疗方案,以确保患者的最佳安全和良好的长期预后。

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本文引用的文献

1
Treatment of the wide open wound in the Ehlers-Danlos syndrome.埃勒斯-当洛综合征中开放性伤口的治疗。
Arch Craniofac Surg. 2019 Apr;20(2):130-133. doi: 10.7181/acfs.2018.02334. Epub 2019 Apr 20.

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