SYRINGOMATOUS CARCINOMA OF THE EYELID AND ORBIT - A CLINICAL AND HISTOPATHOLOGICAL CHALLENGE

Aims-To present three patients with a syringomatous carcinoma (SC). SC is a rare cutaneous neoplasm, most frequently situated on the face an d scalp and histologically characterised by an infiltrative pattern of basaloid or squamous cells, a desmoplastic stromal reaction, keratin filled cysts, and granular structures. Methods-The clinical histories of the patients with a SC were investigated retrospectively. Results-P atient 1 had a benign appearing tumour of the lower eyelid. Five tumou r excisions were necessary to remove the SC completely. Patient 2 had a tumour on the lateral part of the lower eyelid and in the medial can thal area. The histopathological findings revealed a squamous cell car cinoma, later revised as a SC. In spite of two excisions and one micro scopically controlled excision, a recurrence occurred. An exenteration orbitae was recommended. Patient 3, known to have a history of multip le malignant skin tumours after kidney transplantation and use of cycl osporin, presented with a firm mass in the eyebrow region and in the n asal area of the orbit. The pathological diagnosis of this adnexal tum our was difficult. An exenteration was recommended. Conclusions-SC is a benign appearing but extremely invasive, locally destructive, slowly growing adnexal tumour, derived from eccrine sweat glands. It is ofte n mistaken, both clinically and microscopically, for other benign and malignant entities. The tumour recurrence is high due to extensive per ineural invasion, but regional or distant metastases are rare. The loc al aggressive nature of the tumour and the high recurrence rate may ne cessitate mutilating procedures. Optimal treatment consists of a compl ete microscopically controlled surgical excision with clear surgical m argins.