PDF(Pubmed)
Abstract：
Secondary tumors of the ampulla of Vater are exceedingly rare and associated with relatively poor prognosis. Tumors of the ampulla are classified into four distinct subtypes based on the location and involvement of surrounding structures. Most reported cases are of renal cell or malignant skin melanoma primary with only five previously reported cases of breast primary found in a literature review. We present a 72-year-old woman with metastatic breast cancer to the ampulla of Vater as well as multiple bones. She had a history of breast cancer status post bilateral mastectomy and chemo 27 years prior. She presented to the hospital with altered mental status and was found to have an acute liver injury. Magnetic resonance cholangiopancreatography revealed a distended gallbladder and an indeterminate left retroperitoneal mass concerning for cystic or necrotic lymphadenopathy. Endoscopy then showed an edematous and erythematous periampullary region, which was biopsied and returned positive for carcinoma. Immunohistochemical staining of the retroperitoneal mass returned positive for keratin, estrogen receptor, GATA3, and MOC31 and negative for progesterone receptor, WT1, calretinin, and E-cadherin. The periampullary region\'s immunohistochemistry returned positive for pankeratin (AE1/AE3) and CD138 and negative for CD45 and S100, supporting a diagnosis of primary breast carcinoma. The average time from diagnosis of breast cancer to metastasis was found to be 2.5 years. Endoscopic visual presentation of metastatic cancer to the ampulla is indistinguishable from that of primary cancers. Thus, a biopsy with cytology and immunohistochemical analysis is necessary for diagnosis. Management of secondary ampullary tumors requires a multidisciplinary team, including gastroenterology, surgery, oncology, and often palliative care. Secondary tumors have been found to be treated by any combination of Whipple\'s resections, chemotherapy, drainage/stenting, and endoscopic ampullectomy.
摘要：
Vater壶腹继发性肿瘤极为罕见，预后相对较差。根据周围结构的位置和受累，壶腹肿瘤分为四种不同的亚型。大多数报道的病例是原发性肾细胞或恶性皮肤黑色素瘤，在文献综述中仅发现了5例先前报道的乳腺原发性病例。我们介绍了一名72岁的女性，患有转移性乳腺癌的Vater壶腹以及多个骨骼。27年前，她在双侧乳房切除术和化疗后有乳腺癌病史。她因精神状态改变被送往医院，被发现患有急性肝损伤。磁共振胰胆管造影显示胆囊扩张和不确定的左腹膜后肿块，涉及囊性或坏死性淋巴结病。内镜检查显示壶腹周围水肿和红斑，活检结果为癌症阳性。腹膜后肿块的免疫组织化学染色显示角蛋白呈阳性，雌激素受体，GATA3和MOC31，孕激素受体阴性，WT1，钙，和E-cadherin.壶腹周围区域的免疫组织化学恢复为pankeratin（AE1/AE3）和CD138阳性，而CD45和S100阴性，支持诊断为原发性乳腺癌。从乳腺癌诊断到转移的平均时间为2.5年。壶腹转移性癌症的内窥镜视觉表现与原发性癌症的表现没有区别。因此，活检与细胞学和免疫组织化学分析是必要的诊断。继发性壶腹部肿瘤的治疗需要多学科团队，包括胃肠病学,手术,肿瘤学,通常是姑息治疗。已发现继发性肿瘤可以通过Whipple切除的任何组合进行治疗，化疗,引流/支架,和内镜下的壶腹切除术。
