Periorbital Involvement in Early Stage Mycosis Fungoides

Abstract

Mycosis fungoides (MF) is the most common form of cutaneous T-cell lymphomas (CTCL) and accounts for approximately 50% of all CTCL (1, 2). Classically, it presents with erythematous patches on sun-protected areas 2-10 years before confirmed on skin biopsy (3). Approximately 10% of patients initially presenting with patches will subsequently progress to advanced stages of disease (4). Since its first description, MF continues to evolve with discovering of new variants (2). Periorbital involvement is a rare finding in MF and is usually associated with advanced stages of disease. In 1892, Besnier & Hallopeau reported the first patient with periorbital involvement, and extensive and ultimately, fatal disease (5). While the majority of cases describe patients with advanced stages, to date only few reports of isolated periorbital involvement, in early stage MF have been published (6-11). Herein, we report the clinical, epidemiological and histopathological presentation of 7 patients with biopsy proven early stage MF and periorbital lesions. PATIENTS AND METHODS We conducted a retrospective study of patients seen with periorbital MF lesions, at the CTCL clinic at the MD Anderson Cancer Center, Houston Texas. The study protocol was approved by the institutional review board (#PA16-0091). Additional informed consent was obtained from all individual participants for whom identifying information is included in this article. Electronic medical records and paper-based flow charts were screened for patients with periorbital involvement in MF. Clinical and histopathological data were re-evaluated. Diagnosis and staging of MF were made by a CTCL expert (M.D), according to the modified International Society of Cutaneous Lymphomas/European Organization for Research and Treatment of Cancer revisions of the TNMB Classification of MF and Sézary syndrome (1). All analyses were conducted using SPSS version 22.0 (IBM Corporation, Armonk, NY, USA). Continuous data are expressed as mean ± standard deviation (SD) and median (range), as appropriate. Basic summary statistics were used to describe demographic data, treatment modalities, and response.