Section of Dermatology, Department of Health Sciences, University of Florence, Florence, Italy.
Unit of Dermatology, S. Jacopo Hospital, AUSL Toscana Centro, Pistoia, Italy.
Int J Dermatol. 2022 Sep;61(9):1098-1105. doi: 10.1111/ijd.16129. Epub 2022 Feb 28.
Porocarcinoma (PC) is a rare cutaneous adnexal tumor with a variable metastatic potential. Given the paucity of data, guidelines and specific recommendations for PC are not yet well-established. In this study, we evaluate the disease-specific characteristics and outcome of this rare and often underestimated tumor.
A retrospective study of the epidemiological, clinical, and dermoscopic characteristics among cases of histopathologically diagnosed PC, collected from the database of two skin cancer clinics in Italy (Firenze, Pistoia) from 2000 to 2020, was conducted.
Among the 52 patients with 53 tumors, 31 were men (59.6%) and 21 were women (40.4%) with an age range of 49-96 years (median age 82 years). The most common locations were the head/neck region in men (34% in men vs. 17% in women) and the lower limb in women (17% in women vs. 9% in men). Forty-eight cases (91%) underwent local excision. Of these patients, two (4%) experienced local recurrence, and one (2%) developed a second PC on a different anatomical site 1 month after the primary tumor's excision. Lymph node metastases were present in three cases (6%). Two of them have been treated surgically with adjuvant radiotherapy (both are disease-free after a 2-year follow-up period), whereas the third case developed visceral metastases followed by PC-related death.
This study, with 52 patients with 53 tumors covering a follow-up period of more than 5 years, shows a less aggressive behavior of PC with 4% local recurrence, 6% nodal metastases, and 2% mortality.
派杰氏病（PC）是一种罕见的皮肤附属器肿瘤，具有不同的转移潜能。由于数据有限，PC 的指南和具体建议尚未得到很好的确立。在这项研究中，我们评估了这种罕见且常常被低估的肿瘤的疾病特异性特征和结果。
对 2000 年至 2020 年间意大利两家皮肤癌诊所（佛罗伦萨、皮斯托亚）的数据库中经组织病理学诊断为 PC 的病例的流行病学、临床和皮肤镜学特征进行了回顾性研究。
在 52 名患者的 53 个肿瘤中，男性 31 例（59.6%），女性 21 例（40.4%），年龄范围为 49-96 岁（中位年龄 82 岁）。最常见的部位是男性的头颈部（34%在男性中，17%在女性中）和女性的下肢（17%在女性中，9%在男性中）。48 例（91%）患者行局部切除术。这些患者中，有 2 例（4%）出现局部复发，1 例（2%）在原发性肿瘤切除后 1 个月在不同解剖部位出现第二例 PC。有 3 例（6%）出现淋巴结转移。其中 2 例接受了手术和辅助放疗（均在 2 年随访期内无病），而第 3 例发生了内脏转移，随后死于 PC 相关疾病。
这项研究共纳入了 52 名患者的 53 个肿瘤，随访时间超过 5 年，结果显示 PC 的侵袭性较低，局部复发率为 4%，淋巴结转移率为 6%，死亡率为 2%。
