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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.

Background: Intraoperative radiotherapy (IORT) has gained momentum for early stage and favorable breast cancers (BC). The 21-gene recurrence assay guides treatment of hormone positive and node-negative BC. Methods: Analysis of 82 invasive BC treated with breast conservation surgery (BCS) and IORT 2013–2015. Data collection included patient demographics, tumor characteristics, nodal status, recurrence test (RS) and adjuvant therapy. Results: The mean age was 68. Tumors were stage Ia (86.6%), 3.6% Ib and 9.8% IIa. Of 50 patients (61.0%) with RS testing, 72% (n=36) were low risk (RS 0–17), with 28% (n=14) at intermediate risk (RS 18–30). The 39% (n=32) of patients without RS testing, were more likely to have smaller tumors (1.3 vs. 0.9 cm) and age >70 (P Conclusions: Most patients selected for IORT based on clinical features were indeed low risk based on RS. Given the limited long-term clinical outcome and safety data of this technique, additional investigation is needed.