Norwich Medical School, University of East Anglia, Norwich, UK.
Department of Dermatology, Norfolk and Norwich University Hospital, Norwich, UK.
Clin Exp Dermatol. 2022 Jun;47(6):1030-1035. doi: 10.1111/ced.15126. Epub 2022 Mar 20.
From the first report in 1969 to the present day, diagnosis of eccrine porocarcinoma, also known simply as porocarcinoma (PC), remains a challenge. This review presents a concise update of the history, pathogenesis, epidemiology, diagnosis, management and prognosis of this rare sweat gland neoplasm. PC differentiates towards the intraepidermal spiral ducts in the eccrine gland, is more common in people aged > 60 years and often affects the head, neck and legs. PC presents as a dome-shaped papule, plaque or nodule growing over weeks to months. The exact incidence of PC is unknown but appears to be rising. Diagnosis is difficult because of variable presentations and similar clinical and histological features to cutaneous squamous cell carcinoma. Management involves removal of the tumour, usually using wide local excision or Mohs micrographic surgery. Prognosis is poor, with PC recurring after surgery in 35% of cases. Given the lack of standardized protocols and risk profiles, further studies would help improve the understanding of PC.
自 1969 年首例报告以来,目前对大汗腺癌(又称汗管癌,PC)的诊断仍然具有挑战性。本文简要回顾了这种罕见的汗腺肿瘤的历史、发病机制、流行病学、诊断、治疗和预后。PC 向大汗腺的表皮内螺旋导管分化,多见于 >60 岁人群,常发生于头颈部和腿部。PC 表现为在数周到数月内逐渐增大的半球形丘疹、斑块或结节。PC 的确切发病率尚不清楚,但似乎呈上升趋势。由于临床表现和组织学特征与皮肤鳞状细胞癌相似,诊断较为困难。治疗包括切除肿瘤,通常采用广泛局部切除或 Mohs 显微外科手术。由于缺乏标准化的治疗方案和风险评估,因此预后较差,手术后有 35%的病例复发。鉴于目前缺乏标准化的方案和风险评估,进一步的研究将有助于提高对 PC 的认识。