肛周Paget病合并肺腺癌和肛管癌1例并文献复习

Perianal Paget’s disease complicated with lung adenocarcinoma and anal canal carcinoma:A case report and literature review

肛周Paget病(perianal Paget’s disease,PPD)是一种罕见皮肤恶性肿瘤。本文报道1例PPD合并肺腺癌和肛管癌的患者资料。患者为76岁女性,反复下腹部疼痛伴肛周瘙痒5年。体格检查示右侧肛周皮肤5 cm×6 cm大小菜花状新生物,局部皮肤破溃并有少量液体渗出,累及左侧肛周。胸膝位,距肛缘2 cm直肠5~6点直肠黏膜下触及质硬包块。胸部CT示双肺多发病变,考虑转移瘤。氟代脱氧葡萄糖-正电子发射断层显像(fluorodeoxyglucosepositron emission tomography and computed tomography,FDG-PET/CT)提示双肺多发代谢增高结节,全身多发代谢增高淋巴结,左侧臀部小片皮肤早期FDG摄取增高,直肠肛管区FDG摄取增高。支气管镜下右上叶前段开口新生物活检,组织病理学提示肺腺癌。最终诊断为PPD合并肺腺癌、肛管癌、全身多发淋巴结转移。PPD合并消化道肿瘤和其他恶性肿瘤的发生率高,完善结肠镜检查、FDG-PET/CT、组织病理学及免疫组织化学检查,能早期识别局部淋巴结和远处受累,鉴别诊断潜在恶性肿瘤。目前治疗手段多样化,可术后联合或单一化学治疗、放射治疗、靶向治疗及光动力学治疗等,且随着治疗的进展,需重新评估外科手术的范围和有效性。

Perianal Paget’s disease(PPD)is a rare malignant cutaneous tumor.This paper reported a case of PPD complicated by lung adenocarcinoma and anal canal cancer.The patient,a 76-year-old female,had been experiencing recurrent lower abdominal pain and perianal pruritus for the past 5 years.Upon physical examination,a cauliflower-like neoplasm in size of 5 cm×6 cm was observed on the right perianal skin,with local skin ulceration and a small amount of fluid discharge.The left perianal skin was also involved.In thoracoknee position,a hard mass was palpable in the rectal submucosa at 5-6 points 2 cm from the anal verge.Chest CT revealed multiple lesions in both lungs,indication of metastatic tumors.Further evaluation with fluorodeoxyglucose positron emission tomography and computed tomography(FDG-PET/CT)indicated multiple hypermetabolic nodules in the lungs,hypermetabolic lymph nodes throughout the body,early FDG uptake in a small patch of skin on the left hip,and increased FDG uptake in the anorectal region.Histopathological examination confirmed the diagnosis of lung adenocarcinoma.This resulted in the patient being diagnosed with PPD,lung adenocarcinoma,anal canal cancer,and systemic multiple lymph node metastasis.The combination of PPD with gastrointestinal tumors and other metachronous malignant tumors is highly prevalent.Colonoscopy,FDG-PET/CT,histopathology,and immunohistochemistry play crucial roles in early identification of local lymph node and distant involvement,facilitating the evaluation of potential malignant tumors and differential diagnosis.Treating methods for PPD are currently diverse,including postoperative combined or single chemotherapy,radiotherapy,targeted therapy,and photodynamic therapy.As trerapeutical options continue to develop,the extent and efficacy of surgery need to be reassessed.