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Makoto Imazeki, Yoshihiko Usui, Kinya Tsubota, Kazuhiko Umazume, Rei Nemoto, Hiroshi Goto; Clinical features of primary vitreoretinal lymphomas with lesions involving organs other than central nervous system. Invest. Ophthalmol. Vis. Sci. 2018;59(9):5582.
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© ARVO (1962-2015); The Authors (2016-present)
Most of the primary vitreoretinal lymphomas, a high-grade diffuse large B cell lymphoma, develop central nervous system (CNS) lymphoma within a few years after onset, and the prognosis is poor with 5-year survival rate of around 60-70% in Japan. On the other hand, development of vitreoretinal lymphomas in organs other than CNS is rare. We report the clinical features of primary vitreoretinal lymphomas in which lymphomas are detected in extra-CNS organs.
Patients diagnosed with primary vitreoretinal lymphoma at the Department of Ophthalmology, Tokyo Medical University Hospital between 1999 and 2017, and subsequently developed lymphomas in organs other than CNS were studied. Medical records of the patients were reviewed retrospectively to extract data of patient background and clinical features.
Of 95 patients diagnosed with vitreoretinal lymphoma at our department during the study period, 8 patients (8.4%) developed lymphomas in organs other than CNS after the diagnosis of vitreoretinal lymphoma. The 8 patients comprised 3 males and 5 females, with mean age of ocular onset at 68.2 ± 5.47 years. Mean observation period was 59.3 ± 28.7 months. The sites of extra-CNS lymphoma were cervical lymph nodes in 3 patients, and inguinal lymph node, bone marrow, mandibular lymph node, orbit, paranasal sinus, skin, and heart in 1 patient each (with overlapping). Five of 8 patients also developed lymphoma in CNS. In 8 patients, the 1-year survival rate was 88% and 5-year survival rate was 50%.
In patients diagnosed with primary vitreoretinal lymphoma, lymphoma may also develop in organs other than CNS. After confirming the diagnosis of primary vitreoretinal lymphoma, not only CNS but also systemic management is required over a long period.
This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.
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