Specific location of ocular adnexal lymphoma and mortality: an international multicentre retrospective study

Abstract

AimsTo examine whether the specific location of ocular adnexal lymphoma (OAL) and the American Joint Committee on Cancer (AJCC) TNM tumour stage are prognostic factors for mortality in the main OAL subtypes.MethodsClinical and survival data were retrospectively collected from seven international eye cancer centres. All patients from 1980 to 2017 with histologically verified primary or secondary OAL were included. Cox regression was used to compare the ocular adnexal tumour locations on all-cause mortality and disease-specific mortality.ResultsOAL was identified in 1168 patients. The most frequent lymphoma subtypes were extranodal marginal zone B-cell lymphoma (EMZL) (n=688, 59%); follicular lymphoma (FL) (n=150, 13%); diffuse large B-cell lymphoma (DLBCL) (n=131, 11%); and mantle cell lymphoma (MCL) (n=89, 8%). AJCC/TNM tumour-stage (T-stage) was significantly associated with disease-specific mortality in primary ocular adnexal EMZL and increased through T-categories from T1 to T3 disease. No associations between AJCC/TNM T-stage and mortality were found in primary ocular adnexal FL, DLBCL, or MCL. EMZL located in the eyelid had a significantly increased disease-specific mortality compared with orbital and conjunctival EMZL, in both primary EMZL and the full EMZL cohort. In DLBCL, eyelid location had a significantly higher disease-specific mortality compared with an orbital or lacrimal gland location.ConclusionDisease-specific mortality is associated with AJCC/TNM T-stage in primary ocular adnexal EMZL patients. Lymphoma of the eyelid has the highest disease-specific mortality in primary EMZL, and in the full cohort of EMZL and DLBCL patients.

Eyelid involvement of primary cutaneous lymphomas: A clinical perspective

“…The distribution of eyelid lymphomas in all the mentioned studies and reviews 3–7 differs from the general distribution of PCLs with MF as by far the most common type 8,9 . However, in routine dermatological practice the great majority of MF cases are diagnosed with histopathological examination performed from elsewhere other than the periocular region, 10 eyelid lesions of MF usually do not worsen the vision of the eyes and do not enhance the therapeutic decision which may explain the absence or very low frequency of MF diagnosis in ophthalmology or pathology centre‐originated studies 1,4–7 . There are not many large series reported from dermatology centres focusing on PCLs on the eyelids.…”

“… 4 However, the authors remarked that some of the cutaneous T‐cell lymphoma (CTCL) subtypes of the eyelids may have been examined and treated in non‐ophthalmological centres and thus may not be included in this study 4 . In a recent international study from different eye cancer centres evaluating 1168 primary or secondary OALs, the most frequent lymphoma types were EMZL (59%), follicular lymphoma (13%), diffuse large B‐cell lymphoma (11%) and mantle cell lymphoma (8%), whereas MF constituted only 0.9% 5 . In a study reported from a pathology centre evaluating OALs in 99 patients of whom 63.6% were EMZL without any stated MF patients 6 .…”

“…In a review article regarding 199 lymphomas of the eyelids which have been published until 2016, 56% of cases were B‐cell origin and others were T‐cell origin of whom 25 (12.6%) were MF 3 . The distribution of eyelid lymphomas in all the mentioned studies and reviews 3–7 differs from the general distribution of PCLs with MF as by far the most common type 8,9 . However, in routine dermatological practice the great majority of MF cases are diagnosed with histopathological examination performed from elsewhere other than the periocular region, 10 eyelid lesions of MF usually do not worsen the vision of the eyes and do not enhance the therapeutic decision which may explain the absence or very low frequency of MF diagnosis in ophthalmology or pathology centre‐originated studies 1,4–7 .…”

Clinical characteristics and treatment outcomes of secondary ocular adnexal lymphoma

“…2,3 Based on the sites of primary lesion and invasion mode, OAL can be divided into two categories: primary OAL and secondary OAL. 4 The first refers to a biopsy-verified primary lymphoma limited to the ocular adnexal region with or without the involvement of adjacent lymph nodes or tissues. The latter refers to disseminated lymphoma with ocular adnexal and systemic involvement or ocular adnexal relapse of previously diagnosed systemic lymphoma.…”

“…However, it is one of the most common ophthalmic tumours, with an incidence rate increasing on an annual basis 2,3 . Based on the sites of primary lesion and invasion mode, OAL can be divided into two categories: primary OAL and secondary OAL 4 . The first refers to a biopsy‐verified primary lymphoma limited to the ocular adnexal region with or without the involvement of adjacent lymph nodes or tissues.…”