肛周派杰氏病——一种侵袭性疾病。

Perianal Paget's-an aggressive disease.

机构信息

Division of Colon and Rectal Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota, USA.

出版信息

Colorectal Dis. 2023 Jun;25(6):1213-1221. doi: 10.1111/codi.16549. Epub 2023 Mar 21.

DOI:10.1111/codi.16549

PMID:36945125

Abstract

AIM

Perianal Paget's disease (PAPD) is a rare disorder with a predisposition to anal and colorectal malignancies and an unclear prognosis. Our previous 25-year series demonstrated a non-aggressive nature. This study aims to describe our updated institutional experience.

METHODS

This is a retrospective review of all patients diagnosed with primary PAPD from 1991 to 2021. A prospectively maintained institutional database was searched which included demographics, clinical and pathological manifestations, treatment methods, recurrence, oncological outcome and mortality.

RESULTS

Thirty patients were diagnosed with PAPD. Fifteen were women (50%); the average age at diagnosis was 71 ± 10.7 years, and the average lesion size was 3.7 ± 2.6 cm. At diagnosis, 12 (40%) were harbouring invasive anal adenocarcinoma. Eight (27%) developed adenocarcinomas concurrent with PAPD recurrence at a mean interval of 9 ± 4.4 years (range 1.9-14.8). The Kaplan-Meier curve estimated overall survival of 93%, 86%, 82%, 65% and 56% at 1, 3, 5, 10 and 15 years, respectively. Median survival was 16 years. Six (20%) had disease-related mortality. Initially, nine (30%) were treated with abdominoperineal resection (APR), 15 (50%) underwent local resection, three (10%) were treated with radiotherapy, two (7%) received only topical therapy and one (3%) chose observation. Fifteen (50%) experienced recurrence of PAPD, two after undergoing APR. Five (17%) had persistent disease until death. Only 10 (33%) did not experience PAPD recurrence, seven of whom underwent APR. The mean follow-up time was 9.2 ± 6.2 years.

CONCLUSIONS

Perianal Paget's disease is an aggressive entity with high rates of synchronous anal adenocarcinoma at diagnosis and development of metachronous adenocarcinoma later in life.

摘要

目的

肛周派杰病（PAPD）是一种罕见疾病，易发生肛门和结直肠恶性肿瘤，且预后不明。我们之前的 25 年系列研究表明该病性质并不具有侵袭性。本研究旨在描述我们机构的最新经验。

方法

这是一项对 1991 年至 2021 年间所有诊断为原发性 PAPD 的患者进行的回顾性研究。通过前瞻性维护的机构数据库搜索了患者的人口统计学、临床和病理表现、治疗方法、复发、肿瘤学结果和死亡率等数据。

结果

共诊断出 30 例 PAPD 患者。其中 15 例为女性（50%）；诊断时的平均年龄为 71±10.7 岁，平均病变大小为 3.7±2.6cm。诊断时，12 例（40%）合并有侵袭性肛门腺癌。8 例（27%）在 PAPD 复发时同时发生腺癌，平均间隔时间为 9±4.4 年（范围 1.9-14.8 年）。Kaplan-Meier 曲线估计总生存率分别为 1 年时 93%、3 年时 86%、5 年时 82%、10 年时 65%和 15 年时 56%。中位生存期为 16 年。6 例（20%）患者因疾病相关死亡。最初，9 例（30%）接受了腹会阴联合切除术（APR），15 例（50%）接受了局部切除术，3 例（10%）接受了放疗，2 例（7%）仅接受了局部治疗，1 例（3%）选择了观察。15 例（50%）出现 PAPD 复发，其中 2 例在接受 APR 后复发。5 例（17%）疾病持续存在直至死亡。只有 10 例（33%）未发生 PAPD 复发，其中 7 例接受了 APR。平均随访时间为 9.2±6.2 年。