Résumé
Syringoid eccrine carcinoma (SEC) is a rare cutaneous malignant tumor thought to be derived from eccrine sweat apparatus. It is usually present in the head, neck and trunk region, and often occurs in the fourth to seventh decades of life. A 94-year-old male patient visited our department with an 80-year history of a lesion showing a 2×2 cm sized well-demarcated round shaped erythematous to pinkish colored nodule with ulcer on his left thigh. Histological findings revealed a tumor consisted mainly of numerous small cords and nests forming luminal or tubular structures and tumor cells showing variable atypia. Some ductal structures showed tadpole appearance. On immunohistochemical staining, epithelial membrane antigen, S-100, cytokeratin 7 and carcinoembryonic antigen were reactive and Ki-67 showed less than 10% positivity. Based on these findings, the final diagnosis was made as SEC. The patient was treated with local wide excision and didn't show any recurrence during the follow-up period of 12 months. Herein, we report a very rare case of SEC which occurred on the left thigh and discuss 10 cases of SEC presented on the extremities, including our case.
Sujets)
Humains , Mâle , Antigène carcinoembryonnaire , Diagnostic , Membres , Études de suivi , Tête , Kératine-7 , Larve , Mucine-1 , Cou , Phénobarbital , Récidive , Sueur , Tumeurs des glandes sudoripares , Cuisse , UlcèreRésumé
Epidermodysplasia verruciformis (EV) is a rare, life-long heritable disease caused due to a unique susceptibility to human papilloma virus. The disseminated verrucous lesions and pityriasis versicolor-like lesions persist from early childhood and can transform into a cutaneous malignancy in a fourth of patients. Malignant transformation into syringoid eccrine carcinoma (SEC) has been reported only once so far. SEC is an extremely invasive, rare, locally destructive, slowly growing adnexal tumor. We hereby report the association of EV with SEC in a 29-year-old male.
Sujets)
Adulte , Carcinome des annexes cutanées/complications , Carcinome des annexes cutanées/anatomopathologie , Transformation cellulaire néoplasique , Épidermodysplasie verruciforme/complications , Épidermodysplasie verruciforme/anatomopathologie , Humains , Mâle , Papillomaviridae , Tumeurs des glandes sudoripares/complications , Tumeurs des glandes sudoripares/anatomopathologieRésumé
Syringoid eccrine carcinoma (SEC) is a rare adnexal tumor with some controversy surrounding its correct definition. It may also be difficult to differentiate from its benign counterpart (syringoma), other adnexal carcinomas, and cutaneous metastasis from adenocarcinomas. Histologically, the tumor is characterized by syringoma-like tadpole morphology composed of basaloid cells with ductular differentiation. However, the tumor cells are deeply invasive and often extend to subcutaneous tissue, which distinguishes this malignancy from syringoma. Moreover, the tumor cells may rarely show striking vacuolization due to intracytoplasmic glycogen accumulation, called clear cell syringoid eccrine carcinoma. To our knowledge, no case of clear cell SEC in Korea has ever been reported. Herein, we present a case of a SEC (of the clear cell variant) complemented with an immunohistochemical study, the latter revealing cytoplasmic accumulation of glycogen and the presence of intercellular and intracellular lumina in clear tumor cells, as well as diverse hallmarks of SEC.
Sujets)
Adénocarcinome , Protéines du système du complément , Cytoplasme , Glycogène , Immunohistochimie , Corée , Larve , Métastase tumorale , Grèves , Tissu sous-cutané , SyringomeRésumé
Syringoid eccrine carcinoma is a rare adnexal tumor of eccrine origin, and has metastatic potential and high recurrence rate following conventional surgical excision. Mohs micrographic surgery has been commonly used for various malignant skin cancers to minimize the defect after surgery, and to decrease the recurrence rate. We present a case of syringoid eccrine carcinoma successfully treated by Mohs micrographic surgery.