Montes-Torres Andrea, Pérez-Plaza Alejandra, Llamas-Velasco Mar, Gordillo Carlos, De Argila Diego, García-García Carmen, Fraga Javier, Marín Alicia, Ballesteros Ana Isabel, Daudén Esteban
Department of Dermatology, Hospital Universitario de La Princesa, Madrid, Spain.
Department of Pathology, Hospital Universitario de La Princesa, Madrid, Spain.
Int J Dermatol. 2016 Mar;55(3):e156-60. doi: 10.1111/ijd.13121. Epub 2015 Nov 13.
Eccrine porocarcinoma (EPC) is an uncommon malignant neoplasm that originates in the intraepidermal portion of the eccrine sweat duct. Although porocarcinoma is a slow-growing tumor, up to 20% of cases can metastasize to regional lymph nodes, thus increasing mortality.
We describe the clinical and histopathological features and clinical course of three cases of extensive metastatic EPC diagnosed in our department over the last 10 years.
All three patients were women aged 89-96 years. They had numerous skin tumors on the left leg that were histologically and immunohistochemically diagnosed as metastatic EPC. Only one patient had a history of primary porocarcinoma, which had been excised 6 years earlier. The remaining two patients had a previous lesion diagnosed as squamous cell carcinoma. We treated the patients with palliative radiotherapy and/or chemotherapy. Only one patient is currently alive.
The cases of cutaneous and regional metastatic EPC we present occurred in elderly women with major involvement of the left leg. The third case is noteworthy, as the patient presented a long latency period before metastases appeared. Difficulties in the clinical diagnosis--and occasionally histological diagnosis--of primary EPC could delay more aggressive treatment, although optimal treatment does not always guarantee a good prognosis.
小汗腺汗孔癌（EPC）是一种罕见的恶性肿瘤，起源于小汗腺导管的表皮内部分。尽管汗孔癌是一种生长缓慢的肿瘤，但高达20%的病例可转移至区域淋巴结，从而增加死亡率。
我们描述了过去10年在我科诊断的3例广泛转移性EPC的临床、组织病理学特征及临床病程。
所有3例患者均为89 - 96岁女性。她们左腿有大量皮肤肿瘤，经组织学和免疫组织化学诊断为转移性EPC。仅1例患者有原发性汗孔癌病史，6年前已切除。其余2例患者既往病变诊断为鳞状细胞癌。我们对患者进行了姑息性放疗和/或化疗。目前仅1例患者存活。
我们报道的皮肤及区域转移性EPC病例发生在老年女性，主要累及左腿。第3例值得注意，因为该患者在转移出现前有很长的潜伏期。原发性EPC临床诊断——偶尔还有组织学诊断——的困难可能会延迟更积极的治疗，尽管最佳治疗并不总能保证良好预后。
