Successful treatment with rituximab of IgG4-related disease coexisting with adult-onset asthma and periocular xanthogranuloma.

Immunoglobulin G4-related disease (IgG4RD) is a systemic fibro-inflammatory disease of unknown aetiology, which is characterized by tumefactive lymphoplasmatocytic infiltrative lesions, with a predominance of IgG4 positive plasma cells, fibrosis and obliterative phlebitis. Adult-onset asthma and periocular xanthogranuloma (AAPOX) is a rare disease of unknown aetiology characterized by violaceous or yellow cutaneous papules and nodules usually accompanied with adult-onset asthma. We report a case of IgG4RD associated with AAPOX. We also conducted a literature search with keywords including IgG4RD and AAPOX. A 36-year-old woman presented with bilateral exophthalmos and periorbital oedema. Keratoconjunctivitis sicca, painless left parotid gland and submandibular left lymph node enlargement were also noted. Two and half years ago biopsy of yellow plaques of her right lower eyelid confirmed periorbital xanthogranuloma. She underwent parotid gland biopsy which showed IgG4RD. She was treated with steroids and two cycles of rituximab with complete remission. The literature review revealed 8 articles describing 14 cases with IgG4RD and AAPOX, 9 men and 5 women (ratio M:F = 1.8:1) were affected. The age at diagnosis was greater in men compared to women. In the majority of patients, ophthalmic presentation included bilateral upper and lower eyelid swelling while systemic features were asthma, lacrimal and parotid involvement, lymphadenopathy, sclerosing pancreatitis and sclerosing cholangitis. Prednisone and rituximab were effective treatments. It has to be clarified whether the association between IgG4RD and AAPOX represents shared pathophysiology, a common underlying cause or coincidence. Rituximab added to steroids resulted in complete remission of the two entities.

Immunohistochemical Study of Vasculogenic Mimicry and Angiogenesis in Melanocytic Tumors of the Eye and the Periocular Area.

BACKGROUND/AIM: The ability of a tumor to grow requires a sufficient blood supply. Microvascular density is considered the standard for assessing the neovasculature. Tumor cell vasculogenic mimicry refers to the formation of tumor cell-lined vessels that contribute to tumor neovascularization. The aim of the present work was to study angiogenesis and vasculogenic mimicry in benign and malignant melanocytic tumors of the eye and the periocular region. PATIENTS AND METHODS: Histological sections from 118 patients were studied. Eighty-eight of the patients had nevi while the remaining 30 had malignant melanomas. Microvascular density was assessed by using antibodies against the endothelial cell markers CD31 and CD34. Vascular-like channels between neoplastic cells, that were not lined by endothelial cells and thus were negative for CD31 and CD34, represented areas of vasculogenic mimicry. RESULTS: Angiogenesis was more pronounced in melanomas compared to melanocytic nevi and was increased in melanomas with high mitotic index and/or epithelioid cell preponderance compared to melanomas with low mitotic index and/or spindle cell predominance. Vasculogenic mimicry was observed in many melanomas, while it was evident in the minority of benign nevi as well. CONCLUSION: The existence of vasculogenic mimicry in benign nevi might have prognostic implications.

Vasculogenic mimicry: lessons from melanocytic tumors.

Tumor cell vasculogenic mimicry refers to the formation of tumor cell-lined vessels that contribute to tumor neovascularization and nutrient and oxygen supply. These tumor cells express many endothelial and stem cell markers, resulting in them having a unique phenotype. This phenomenon is observed in a variety of neoplasms, such as glioblastomas and sarcomas, as well as breast, ovarian, liver and lung carcinomas. It is also evident in melanocytic lesions, regardless of their benign or malignant nature. The biochemical and molecular events that regulate vasculogenic mimicry provide opportunities for development of novel forms of tumor-targeted treatments. Furthermore, the presence of this process in a tumor might have prognostic implications.

Eyelid Tumors at the University Eye Clinic of Ioannina, Greece: A 30-year Retrospective Study.

AIMS: The aim was to describe the epidemiology of primary eyelid tumors over a 30-year period at the Ioannina University Eye Clinic, Greece. MATERIALS AND METHODS: This retrospective case series examined the histopathology of eyelid tumor specimens of patients treated between 1983 and 2012. Data were collected on patient age, gender, location of the lesion, extent of tumor excision and recurrence. Descriptive and inferential statistics were used to describe the study subgroups. RESULTS: A total of 851 eyelid tumors comprised the study sample. There were 351 (41.2%) malignant cases and 500 (58.8%) benign cases. For malignant tumors, there were 86% basal cell carcinomas, 7% squamous cell carcinomas, and 7% basosquamous cell carcinomas. The benign eyelid lesions were comprised of 20% cysts, 18% seborrheic keratosis, 13% nevi, and 13% papillomas. Benign eyelid lesions occurred with equal frequency in the upper and lower lids. Malignant lesions were more frequently located in the lower lid. The mean age at diagnosis was 49 ± 1.45 years for patients with benign lesions and 67 ± 1.6 years for patients with malignant lesions. CONCLUSIONS: In this Greek cohort, benign eyelid lesions affected mostly young individuals, and malignant lesions occurred predominantly in elderly patients. Males and females were equally affected by malignant lesions, and recurrence after surgical excision was rare.