Hashimoto Hiroki, Kaku-Ito Yumiko, Furue Masutaka, Ito Takamichi
Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Front Oncol. 2021 Apr 15;11:642919. doi: 10.3389/fonc.2021.642919. eCollection 2021.
Extramammary Paget's disease (EMPD) sometimes spreads from the skin to mucosal areas, and curative surgical excision of these areas is challenging. The aim of this study is to analyze the impact of mucosal involvement and surgical treatment on the survival of patients with EMPD.
We conducted a retrospective review of 217 patients with EMPD. We also assessed the associations between tumor involvement in boundary areas (anal canal, external urethral meatus, vaginal introitus), prognostic factors, and survival in 198 patients treated with curative surgery.
Of 217 patients, 75 (34.6%) had mucosal boundary area involvement. Lesions in these areas were associated with frequent lymphovascular invasion ( = 0.042), lymph node metastasis ( = 0.0002), incomplete excision ( < 0.0001), and locoregional recurrence ( < 0.0001). Boundary area involvement was an independent prognostic factor associated with disease-specific survival, per multivariate analysis (HR: 11.87, = 0.027). Incomplete excision was not significantly correlated with disease-specific survival (HR: 1.05, = 0.96).
Boundary area tumor involvement was a major risk factor for incomplete excision, local recurrence, and poor survival outcomes. However, incomplete removal of primary tumors was not significantly associated with poor prognosis. A less invasive surgical approach for preserving anogenital and urinary functions may be acceptable as the first-line treatment for resectable EMPD.
乳腺外佩吉特病（EMPD）有时会从皮肤扩散至黏膜区域，对这些区域进行根治性手术切除具有挑战性。本研究的目的是分析黏膜受累及手术治疗对EMPD患者生存的影响。
我们对217例EMPD患者进行了回顾性研究。我们还评估了198例接受根治性手术治疗患者的肿瘤在边界区域（肛管、尿道外口、阴道口）的累及情况、预后因素与生存之间的关联。
在217例患者中，75例（34.6%）有黏膜边界区域受累。这些区域的病变与频繁的淋巴管侵犯（P = 0.042）、淋巴结转移（P = 0.0002）、切除不完全（P < 0.0001）及局部区域复发（P < 0.0001）相关。根据多因素分析，边界区域受累是与疾病特异性生存相关的独立预后因素（HR：11.87，P = 0.027）。切除不完全与疾病特异性生存无显著相关性（HR：1.05，P = 0.96）。
边界区域肿瘤累及是切除不完全、局部复发及生存不良结局的主要危险因素。然而，原发肿瘤切除不完全与预后不良无显著关联。一种保留肛门生殖和泌尿功能的侵入性较小的手术方法作为可切除EMPD的一线治疗可能是可以接受的。
