Department of Colorectal Surgery, HwaMei Hospital, University of Chinese Academy of Sciences, No. 41 Northwest Street, Ningbo, Zhejiang, 315000, China.
Ningbo Institute of Life and Health Industry, University of Chinese Academy of Sciences, No. 41 Northwest Street, Ningbo, Zhejiang, 315000, China.
World J Surg Oncol. 2021 Dec 2;19(1):336. doi: 10.1186/s12957-021-02439-4.
Perianal Paget's disease (PPD) is a rare malignancy, often associated with an underlying adenocarcinoma and a poor prognosis.
A 69-year-old female was presented with a history of perianal pruritus for 6 months and enlarged inguinal lymph nodes in the left side. Paget cells were confirmed by pathology after a wide excision of perianal skin. Radiotherapy was performed covering the bilateral inguinal lymphatic drainage area. Hepatic metastasis was found 8 months after surgery. Hepatic artery embolization (HAE) and high-intensity focused ultrasound therapy (HIFU) were performed successively. However, hepatic metastasis happened again 3 months later. Ultrasound-guided percutaneous radiofrequency ablation (PRFA) was carried out and various means of inspection could not identify the primary tumor. In the case of rapid progression of the tumor, we gave the patient chemotherapy regimens of XELOX. After 4 cycles of chemotherapy, the tumor marker went down continuously and the hepatic metastasis stayed stable.
Hepatic metastasis from perianal Paget's disease without identified underlying carcinoma may benefit from XELOX on the basis of adenocarcinoma.
肛周派杰病（PPD）是一种罕见的恶性肿瘤，常与潜在的腺癌相关，并预后不良。
一名 69 岁女性因肛周瘙痒 6 个月和左侧腹股沟淋巴结肿大就诊。经广泛切除肛周皮肤后，病理证实为派杰细胞。行双侧腹股沟淋巴结引流区放疗。术后 8 个月发现肝转移。行肝动脉栓塞术（HAE）和高强度聚焦超声治疗（HIFU）后，3 个月后再次出现肝转移。行超声引导下经皮射频消融（PRFA），各种检查手段均未能发现原发病灶。鉴于肿瘤进展迅速，给予患者 XELOX 化疗方案。化疗 4 个周期后，肿瘤标志物持续下降，肝转移灶稳定。
无明确潜在腺癌的肛周派杰病肝转移可能从XELOX 方案中获益。
