RESUMEN
Porocarcinoma (PC) is a rare adnexal tumor, mainly found in the elderly. The tumor arises from the acrosyringium of eccrine sweat glands. The risk of lymph node and distant metastasis is high. Differential diagnosis with squamous cell carcinoma is difficult, although NUT expression and YAP1 fusion products can be very useful for diagnosis. Currently, wide local excision is the main surgical treatment, although Mohs micrographic surgery is promising. To date, there is no consensus regarding the role of sentinel lymph node biopsy and consequential lymph node dissection. No guidelines exist for radiotherapy, which is mostly performed based on tumor characteristics and excision margins. Only a few studies report systemic treatment for advanced PC, although therapy with pembrolizumab and EGFR inhibitors show promise. In this review, we discuss epidemiology, clinical features, histopathological features, immunohistochemistry and fusion products, surgical management and survival outcomes according to stage, surgical management, radiotherapy and systemic therapy.
Asunto(s)
Porocarcinoma Ecrino , Humanos , Porocarcinoma Ecrino/patología , Porocarcinoma Ecrino/terapia , Porocarcinoma Ecrino/metabolismo , Porocarcinoma Ecrino/diagnóstico , Inmunohistoquímica , Neoplasias de las Glándulas Sudoríparas/patología , Neoplasias de las Glándulas Sudoríparas/terapia , Neoplasias de las Glándulas Sudoríparas/metabolismo , Neoplasias de las Glándulas Sudoríparas/diagnóstico , Biomarcadores de Tumor/metabolismo , Proteínas Señalizadoras YAPAsunto(s)
Porocarcinoma Ecrino , Neoplasias de las Glándulas Sudoríparas , Humanos , Porocarcinoma Ecrino/diagnóstico , Porocarcinoma Ecrino/cirugía , Porocarcinoma Ecrino/patología , Neoplasias de las Glándulas Sudoríparas/diagnóstico , Neoplasias de las Glándulas Sudoríparas/cirugía , Neoplasias de las Glándulas Sudoríparas/patologíaRESUMEN
Abstract: Eccrine poromas and porocarcinomas are adnexal tumors derived from the sweat duct epithelium. However, eccrine poroma is benign in nature, whilst eccrine porocarcinoma is regarded as its malignant counterpart. We report the case of a man who presented simultaneously with an eccrine poroma and eccrine porocarcinoma. Both lesions had no clear distinctive features, enhancing the need of high-level clinical suspicion together with surgical excision and histopathology for prompt diagnosis confirmation.
Asunto(s)
Humanos , Masculino , Anciano , Neoplasias de las Glándulas Sudoríparas/patología , Poroma/patología , Porocarcinoma Ecrino/patología , Neoplasias Primarias Múltiples/patología , Neoplasias de las Glándulas Sudoríparas/diagnóstico , Biopsia , Diagnóstico Diferencial , Poroma/diagnóstico , Porocarcinoma Ecrino/diagnóstico , Neoplasias Primarias Múltiples/diagnósticoRESUMEN
Los tumores anexiales de la piel representan un grupo heterogéneo de entidades de baja frecuencia de presentación. Su origen es controvertido, quizás a partir de células madres pluripotenciales, aceptándose principalmente dos líneas embriológicas: pilosebáceo-apócrina y ecrina. Se los clasifica en cuatro grupos principales: tumores del folículo piloso, tumores sebáceos, tumores apócrinos y tumores ecrinos. Su distribución anatómica refleja áreas con mayor densidad de anexos cutáneos, presentándose en forma única o múltiple.
Skin adnexal tumors are represented as a heterogeneous group of entities of low frequency range. Its origin is controversial, perhaps from pluripotent stem cells, mainly from two embryological lines: pilosebaceous-apocrine and eccrine. They are classified as four main groups: tumors of the hair follicle, sebaceous tumors, apocrine tumors and eccrine tumors. Their anatomic distribution reflects areas with the highest density of cutaneous adnexal, arising in either single or multiple forms.
