Case report: EBV-related eye orbits and sinuses lymphohistiocytic infiltration responsive to rituximab in a patient with X lymphoproliferative syndrome type 1.

Background And Aims: X lymphoproliferative syndrome type 1 (XLP1) is a rare inborn error of immunity due to mutations of , encoding for slam-associated protein (SAP). The clinical phenotype includes severe mononucleosis, hemophagocytic lymphohistiocytosis (HLH), and B-cell lymphomas.

Methods: We report the case of a child affected with XLP1 who presented with an incomplete HLH, triggered by Epstein-Barr virus (EBV) and treated with rituximab, involving orbits and paranasal sinuses.

A rare case of bilateral conjunctival Kaposi's sarcoma in a HIV-negative patient.

Purpose: Kaposi's sarcoma (KS) is a rare multi-centric vascular neoplasm, first described by Moritz Kaposi in 1872. It can appear in four different forms: classic, endemic, HIV-related and post-transplant form. We present an uncommon case of seronegative HIV infection patient with skin KS involving conjunctiva of both eyes.

Primary cutaneous anaplastic large-cell lymphoma of the eyelid: report of two cases and review of the literature.

Purpose: Two new cases of primary cutaneous CD30+ anaplastic large-cell lymphoma (cALCL) of the eyelid are reported; these are analysed alongside existing cases to identify challenges relating to the diagnosis and management of such rare lesions.

Material And Methods: A review of existing literature on the PubMed database is conducted using the keywords: 'eyelid lymphoid proliferations', 'lymphoma of the eyelid', and 'primary cutaneous CD30+, ALK-anaplastic large-cell lymphoma of the eyelid'. Two new cases of cALCL are reported.

The role of ultrasonography in differential diagnosis of orbital lesions.

Aims: The aim of this study is to assess the value of ultrasound in the differential diagnosis of orbital lesions.

Methods: One hundred and thirty-eight patients with clinical features of an orbital mass were examined by orbital ultrasound prior to undergoing surgery, from January 2000 to January 2017. All patients underwent excisional or incisional orbital biopsy.

An analysis of 2,480 space-occupying lesions of the orbit from 1976 to 2011.

Purpose: To evaluate the frequencies of orbital space-occupying lesions seen at single orbital unit in a period of 35 years.

Methods: In this retrospective case series, the authors reviewed the medical records of 2,480 consecutive patients referred to the authors' Orbital Unit for evaluation of an orbital mass between 1976 and 2011. The final diagnosis in each case was established by a combination of history, ocular findings, diagnostic imaging, and histopathologic analysis, when available.

Risk factors for orbital exenteration in periocular Basal cell carcinoma.

Purpose: To present the proportion of patients with periocular basal cell carcinoma (BCC) who underwent orbital exenteration and to evaluate the significance of the following risk factors: initial tumor site, pathologic features, and initial treatment.

Design: Retrospective, comparative, interventional case series.

Methods: Charts of all patients with BCC referred to Orbital Unit of the University of Naples "Federico II" between 1984 and 2003 were reviewed.