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 and 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: Patient 1 had a benign appearing tumour of the lower eyelid. Five tumour 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 canthal area. The histopathological findings revealed a squamous cell carcinoma, later revised as a SC. In spite of two excisions and one microscopically controlled excision, a recurrence occurred. An exenteration orbitae was recommended. Patient 3, known to have a history of multiple malignant skin tumours after kidney transplantation and use of cyclosporin, presented with a firm mass in the eyebrow region and in the nasal area of the orbit. The pathological diagnosis of this adnexal tumour 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 often mistaken, both clinically and microscopically, for other benign and malignant entities. The tumour recurrence is high due to extensive perineural invasion, but regional or distant metastases are rare. The local aggressive nature of the tumour and the high recurrence rate may necessitate mutilating procedures. Optimal treatment consists of a complete microscopically controlled surgical excision with clear surgical margins.

Cholesterol granuloma of the frontal sinus.

It is common to see cholesterol granuloma in the mastoid air cells, less common in the orbit, and uncommon in the paranasal sinuses. Cholesterol granuloma is thought to be due to an interruption to normal aeration with impaired lymphatic drainage, resulting in a closed cavity where it may form. These expanding cysts cause bone destruction and compression of the surrounding structures that lead to clinical symptoms. Diagnosis and management of cholesterol granuloma cysts can be challenging. Magnetic resonance imaging (MRI) and computed tomographic (CT) scans are usually diagnostic. We present a rare case of cholesterol granuloma in the frontal sinus, few cases have been reported in the literature.

Amyloid deposition in the extraocular muscles.

Orbital involvement is frequent in generalised amyloidosis. However, primary localised amyloid in the orbit is rare and requires systemic investigation. We present a case with amyloid deposits localised to one extraocular muscle in whom systemic investigation has been negative.

MHC class II region, CTLA4 gene, and ophthalmopathy in patients with Graves' disease.

Up to half of patients with Graves' hyperthyroidism have signs of thyroid associated ophthalmopathy, but the factors that cause this disorder are unknown. We investigated two major genetic susceptibility loci for Graves' disease in ophthalmopathy; the MHC class II region and the cytotoxic T lymphocyte antigen-4 (CTLA4) gene. Allelic frequencies of these genes in patients with Graves' disease who did and did not have concurrent thyroid-associated ophthalmopathy did not differ, and are, therefore, unlikely to contribute to its development.