Orbital and adnexal involvement in systemic non-Hodgkin's lymphoma

Abstract

Background. The orbit is rarely a secondary site of lymphoma dissemination, and only few reports exist on the course and characteristics of involvement in these sites. Methods. The authors retrospectively reviewed the records of 187 consecutive patients with systemic non‐Hodgkin's lymphoma (NHL) diagnosed and treated at Beilinson Medical Center between 1986 and 1992. Results. Ten patients (5.3% of those with NHL) had orbital or adnexal involvement or both. Histologically, six had intermediate‐grade lymphoma, three had diffuse small cleaved cell lymphoma, and one had nodular small cleaved cell lymphoma. In all 10 patients, the lymphoma was widespread, and in 6, there were two or more other extranodal sites of involvement, mainly, bone marrow (six) and skin (three). The orbital involvement was found either at presentation or as late as 53 months after primary diagnosis. Various therapeutic approaches were chosen, from local orbital irradiation to different mild to aggressive chemotherapeutic protocols. Complete regression of the orbital or adnexal involvement or both was observed in 9 of 10 patients, but in 6 the systemic disease either persisted or recurred at other sites. Conclusions. Orbital or adnexal involvement or both by NHL may appear at any time during the course of the disease. It responds well to either chemotherapy or radiation therapy with prolonged local remission. The results of this study strongly suggest that every patient with NHL in whom any periorbital or orbital mass, ptosis, proptosis or lid edema develops should be suspected of having orbital lymphoma involvement until proven otherwise.

Review article: Mucosa-associated lymphoid tissue (MALT)-type lymphoma of ocular adnexa. Biology and treatment

“…Specifically, primary OALs (POALs) represent approximately 1-2% of all lymphomas (Bairey et al, 1994) and 5-15% of all extranodal-type lymphomas (Lambo et al, 1993).…”

“…In fact, ocular adnexa are affected in an estimated 5% of all NHL patients (Bairey et al, 1994;White et al, 1995); of these, about 80-85% are indolent lymphomas (Follicular or Marginal Zone Lymphomas of MALT type) while 15-20% are aggressive lymphomas (Diffuse Large B Cell or Mantle Cell Lymphomas) (Rath et al, 2014). In this review we focus on the biological and clinical features of POALs of MALT type (POAML) as well as on the proposed managements for these type of Lymphoma representing the most common lymphoma arising in ocular adnexa.…”

Non-Hodgkin’s lymphomas of the ocular adnexa, their diagnostics and outpatient monitoring

“…По некоторым данным, на долю экстранодальных НХЛ приходится от 24 до 48 % всех НХЛ; НХЛ орбиты, глаза и его придаточного аппарата составляют от всех экстранодальных лимфом 4,1-8 % [1,[5][6][7], а по мне-нию S. Coupland и других авторов, -от 8 до 14 % [8][9][10][11]. При диссеминированных формах лимфом вовле-чение в процесс тканей орбиты и конъюнктивы встречается редко -у 5,3 % больных [7,12].…”

“…При диссеминированных формах лимфом вовле-чение в процесс тканей орбиты и конъюнктивы встречается редко -у 5,3 % больных [7,12].…”

Non-Hodgkin lymphoma in the orbita: A case report and review of the literature

“…Zamanla PET-CT, kemik iliği biyopsisi, gastrointestinal endoskopik girişimler gibi tanısal yöntemlerdeki hızlı gelişmelerle birlikte oküler veya orbital lenfomalı birçok olguda vücudun başka bölgelerinde de lenfoid tutulumlar gösterilmiştir. [9][10][11] Bu nedenle primer lenfomaların önceden bildirilen oranlardan daha dü-şük olabileceği iddia edilmektedir. [12] Jakobiec ve ark.…”