A 56-year-old woman underwent quadrantectomy with axillary dissection followed by adjuvant radiotherapy (RT) with 30 Gy for a mucinous carcinoma of the left breast (T1cN0M0). Because oestrogen and progesteron receptors were positive, she was given antihormonal therapy for 5 years. Nine years later, the patient underwent local excision of a 0.7 × 0.8 cm pigmented papule skin lesion of the operated breast. Histology revealed high-grade angiosarcoma (Fig. 1a), and immunohistochemistry was positive for CD31 (Fig. 1b) and CD34, whereas c-myc amplification was negative at fluorence in situ hybridization (FISH). Because of the history of previous RT and the typical appearance of the lesion, the tumour was presumably considered radiogenic. No residual disease was found after simple mastectomy, and adjuvant treatment was not administered. A positron emission tomography/computed tomography (PET/CT) scan performed 1 year later showed ill-defined hypermetabolism in the mediastinal lymph nodes (SUV max 11) (Fig. 2A). Video assisted thoracoscopy biopsy revealed recurrence from angiosarcoma, and she was given taxane-based chemotherapy for 6 months with complete remission. A 7.6 × 6.8 cm rounded mass in the right adrenal with heterogeneous contrast enhancement was seen 5 years later at surveillance up CT, without enlarged regional lymph nodes or other metastatic diseases (Fig. 2B). At admission, physical examination and laboratory tests including biochemical work-up were unremarkable. Open adrenalectomy was performed because of the large size and because we could not rule out the possibility of a primary tumour. Histology showed proliferation of epithelioid and atypical spindle cells, exhibiting marked nuclear pleomorphism and high mitotic rate (Fig. 3A). Immunohistochemical staining was positive for endothelial markers CD31 (Fig. 3B), CD34 (Fig. 3C) and Erythrobalst Transformation-Specific- Related Gene (Fig. 3D), whereas c-myc amplification was negative at FISH. The diagnosis was the localization of metastatic high-grade angiosarcoma. No adjuvant treatment was given, and the patient was alive without the evidence of disease 7 months after surgery. Adrenal gland involvement frequently occurs in case of disseminated disease from breast cancers, but isolated metastases are extremely rare, and the noninvasive diagnosis may be challenging. Although there are no guidelines, adrenalectomy is considered the standard of treatment for isolated metastasis, providing the opportunity to improve prognosis in selected patients with solitary lesion and slow disease progression.

中文翻译：

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转移性放射源性乳腺血管肉瘤的异常表现

一名 56 岁女性因左侧乳腺黏液癌 (T1cN0M0) 接受了象限切除术和腋窝清扫术，随后接受了 30 Gy 的辅助放疗 (RT)。由于雌激素和孕激素受体呈阳性，她接受了 5 年的抗激素治疗。九年后，患者接受了手术乳房的 0.7 × 0.8 cm 色素性丘疹皮损的局部切除术。组织学显示高级别血管肉瘤（图 1a），免疫组织化学对 CD31（图 1b）和 CD34 呈阳性，而 c-myc 扩增在原位荧光下呈阴性杂交（FISH）。由于既往放疗的病史和病变的典型外观，该肿瘤可能被认为是放射源性的。单纯乳房切除术后未发现残留病灶，未进行辅助治疗。1 年后进行的正电子发射断层扫描 / 计算机断层扫描 (PET/CT) 扫描显示纵隔淋巴结（SUV max 11）中边界不清的高代谢（图 2A）。视频辅助胸腔镜活检显示血管肉瘤复发，她接受了基于紫杉烷的化疗 6 个月，完全缓解。5 年后在 CT 监测中发现右侧肾上腺中有一个 7.6 × 6.8 cm 的圆形肿块，对比度增强不均匀，没有肿大的区域淋巴结或其他转移性疾病（图 2B）。入学时，体格检查和实验室检查，包括生化检查，均无异常。由于体积较大且我们不能排除原发肿瘤的可能性，因此进行了开放式肾上腺切除术。组织学显示上皮样细胞和非典型梭形细胞增殖，表现出明显的核多形性和高有丝分裂率（图 3A）。内皮标志物 CD31（图 3B）、CD34（图 3C）和 Erythrobalst 转化特异性相关基因（图 3D）的免疫组织化学染色呈阳性，而 FISH 的 c-myc 扩增呈阴性。诊断是转移性高级别血管肉瘤的定位。未给予辅助治疗，术后7个月患者存活且无疾病迹象。在乳腺癌播散性疾病的情况下经常发生肾上腺受累，但孤立的转移极为罕见，无创诊断可能具有挑战性。尽管没有指南，但肾上腺切除术被认为是孤立性转移灶的标准治疗方法，为某些孤立性病变和疾病进展缓慢的患者提供了改善预后的机会。