Molina-Ruiz Ana-María, Llamas-Velasco Mar, Rütten Arno, Cerroni Lorenzo, Requena Luis
*Department of Dermatology, Fundación Jiménez Díaz †Department of Dermatology, Hospital Universitario de la Princesa, Universidad Autónoma, Madrid, Spain ‡Dermatopathology Friedrichshafen, Friedrichshafen, Germany §Dermatopathology Research Unit, Department of Dermatology, Medical University of Graz, Graz, Austria.
Am J Surg Pathol. 2016 Mar;40(3):410-8. doi: 10.1097/PAS.0000000000000547.
Digital papillary carcinoma (DPC) is a rare, underreported, and often misdiagnosed malignant tumor of the sweat glands. It is often located on the digits and toes and most commonly occurs in male individuals in their fifties to seventies. Because of lack of pain, slow growth, and an inconspicuous appearance, clinical diagnosis is often missed or delayed. In contrast, apocrine hidrocystoma (AH) is a cystic adenoma that arises from the apocrine secretory coil, and it is extremely rare for AHs to develop on the digits. We report 7 cases of DPC, including clinical course, histopathologic and immunohistochemical findings, and therapeutic approach in which the initial histopathologic diagnosis in all cases was AH or cystadenoma. However, complete excision of the neoplasms led to a final diagnosis of DPC. After an adequate treatment, no recurrence or metastasis was found in any of the cases described. All the cases studied showed similar histopathologic and immunohistochemical findings. The initial incisional biopsy showed large unilocular or multilocular cystic spaces situated within the dermis, lined by a double layer of epithelial cells with tiny papillary structures. No cellular atypia, necrosis, or pleomorphism was observed. However, complete excision revealed neoplastic lesions involving the dermis and/or subcutis, with an infiltrative pattern and papillary projections into luminal spaces. Immunoperoxidase studies showed positivity for CK7, S-100 protein, CEA, p63, smooth muscle actin, and calponin. DPC is a rare but life-threatening malignancy, therefore it is important to be able to identify such a lesion both clinically and histopathologically, treat it, and monitor the patient for the tumor's potential recurrence and metastasis. Pathologists and dermatopathologists should be aware that a histopathologic diagnosis of AH or cystadenoma on the fingers and toes should be established with caution, because probably those lesions represent the superficial and cystic component of an underlying DPC, and a wider excision should be performed.
指状乳头状癌(DPC)是一种罕见、报道不足且常被误诊的汗腺恶性肿瘤。它常位于手指和脚趾,最常见于50至70岁的男性个体。由于无痛、生长缓慢且外观不明显,临床诊断常被漏诊或延误。相比之下,顶泌汗腺囊瘤(AH)是一种起源于顶泌汗腺分泌蟠管的囊性腺瘤,在手指上发生极为罕见。我们报告7例DPC,包括临床病程、组织病理学和免疫组化结果以及治疗方法,所有病例最初的组织病理学诊断均为AH或囊腺瘤。然而,肿瘤的完整切除导致最终诊断为DPC。经过充分治疗,所描述的任何病例均未发现复发或转移。所有研究病例均显示出相似的组织病理学和免疫组化结果。最初的切开活检显示真皮内有大的单房或多房囊性间隙,内衬双层上皮细胞,有微小乳头结构。未观察到细胞异型性、坏死或多形性。然而,完整切除显示肿瘤病变累及真皮和/或皮下组织,呈浸润性生长模式,并有乳头状突起伸入管腔间隙。免疫过氧化物酶研究显示CK7、S-100蛋白、癌胚抗原(CEA)、p63、平滑肌肌动蛋白和钙调蛋白呈阳性。DPC是一种罕见但危及生命的恶性肿瘤,因此能够在临床和组织病理学上识别此类病变、进行治疗并监测患者是否有肿瘤潜在的复发和转移非常重要。病理学家和皮肤病理学家应意识到,对手指和脚趾上AH或囊腺瘤的组织病理学诊断应谨慎确立,因为这些病变可能代表潜在DPC的浅表和囊性成分,应进行更广泛的切除。