Ocular adnexal lymphoma: Clinical behavior of distinct World Health Organization classification subtypes

Fung, Claire Y.; Tarbell, Nancy J.; Lucarelli, Mark J.; Goldberg, Saveli; Linggood, Rita M.; Harris, Nancy L.; Ferry, Judith A.

doi:10.1016/s0360-3016(03)00767-3

Cited by 221 publications

(210 citation statements)

References 36 publications

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“…patients with a localized relapse had a favourable outcome. This is in accordance with large series of early stage follicular lymphoma (Fung et al 2003;Guadagnolo et al 2006;Pugh et al 2010). It is noteworthy that only three of the 17 patients with limited-stage disease died of lymphoma.…”

Section: Discussionsupporting

confidence: 91%

Follicular lymphoma of the ocular adnexal region: A nation‐based study

Rasmussen

Ralfkiær

Prause

et al. 2014

Acta Ophthalmologica

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ABSTRACT.Purpose: To characterize the clinicopathological features of follicular lymphoma of the ocular adnexal region. Methods: Retrospective nation-based study of Danish patients with ocular adnexal follicular lymphoma from January 1st 1980 through December 31st 2009. Results: Twenty-four patients with ocular adnexal follicular lymphoma were identified. Fourteen (58%) of the patients were females. The median age was 63 years (range: 42-96 years). Eleven (46%) of the patients had primary ocular adnexal lymphoma, seven (29%) had an ocular adnexal lesion in conjunction with a concurrent systemic lymphoma and six patients (25%) presented with an ocular adnexal relapse. The most frequently affected sites were the lacrimal gland (38%) and the orbit (33%). Thirteen patients (54%) presented with Ann Arbor stage IE lymphoma, four (17%) had stage IIE, two patients (8%) stage IIIE, and five patients (21%) had stage IV lymphoma. Radiotherapy was primarily used in patients with primary lymphoma and those with a stage IE/IIE relapse (82%), while stage IIIE/IV lymphomas most frequently received alkylating chemotherapy (67%). Complete remission was observed in 19 of the patients (79%), but of these 11(58%) had a relapse. The 10-year overall survival for the entire cohort was 59%. The translocation t(14;18) was detected in 16 patients (16/24, 76%). Recurrence was only observed in patients with the t(14;18) (p = 0.05, log-rank). Conclusions: Ocular adnexal follicular lymphoma is more commonly found in elderly female patients. The lacrimal gland is relatively frequently involved. Radiotherapy is the treatment of choice for localized ocular adnexal follicular lymphoma providing a favourable prognosis for majority of patients.

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Section: Discussionsupporting

confidence: 91%

Follicular lymphoma of the ocular adnexal region: A nation‐based study

Rasmussen

Ralfkiær

Prause

et al. 2014

Acta Ophthalmologica

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“…The use of a higher radiation dosage gives better local control and few lymphoma relapses, but this is frequently associated with a wide range of side effects. 23,24 Not sur- prisingly, none of the cases in this study showed lymphoma relapse. In summary, we confirmed that A20 mutations were significantly associated with A20 heterozygous deletion and both were mutually exclusive from the MALT1 and IGH involved translocations.…”

Section: Correlation Of A20 Genetic Abnormalities With Clinicopatholomentioning

confidence: 54%

“…Although the cases in both studies were primarily treated by radiotherapy, there is an important difference in the total radiation dosage used between the two studies. In the previous study, the cases were treated with a total radiation dosage typically around 30 Gy, 23,24 while the cases in the present study were treated with an average total dosage of 40.7 Gy. The use of a higher radiation dosage gives better local control and few lymphoma relapses, but this is frequently associated with a wide range of side effects.…”

Section: Correlation Of A20 Genetic Abnormalities With Clinicopatholomentioning

confidence: 92%

A20 inactivation in ocular adnexal MALT lymphoma

Bi¹,

Zeng²,

Chanudet³

et al. 2011

Haematologica

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“…29,87,88 In all published series except that by Cho et al, 42 the median age was approximately 65 years. 11,13,18,23,27,28,30,36,37,39,41,[43][44][45][83][84][85] Few patients have B symptoms or a performance status (PS) greater than 1, though these findings are rarely reported in published series. 11,18,28,30,42,84 Lacrimal gland and intraorbital sites, observed in approximately 50% of patients, are the most frequent ophthalmologic sites.…”

mentioning

confidence: 99%

Ocular adnexal lymphoma: a review of clinicopathologic features and treatment options

Decaudin

Crémoux

Vincent‐Salomon

et al. 2006

Blood

133

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IntroductionLymphoproliferations of the eye, including intraocular and ocular adnexal non-Hodgkin lymphoma (NHL), constitute a heterogeneous group of neoplasms that represent less than 1% of all NHLs 1 and 5% to 15% of all extranodal sites. 2 First described in 1952, 3 they are the most frequent malignant tumors of the eye and ocular adnexae; in fact, in the Florida cancer registry, they represent up to 55% of all orbital tumors. 4 As for all NHLs, a marked increase of the incidence of ocular adnexal NHL has also been observed. 4 The clinical features and natural history of ocular adnexal lymphoma (OAL) is completely different from those of intraocular lymphoma, which emphasizes the need to distinguish between the therapeutic management of both. Intraocular lymphoma is a subset of primary central nervous system lymphoma, 5-7 and intraocular involvement is found in approximately 15% of affected patients. 8 The histologic subtype of intraocular lymphoma is almost always high grade 9,10 and it can occur in the context ofAIDS-related lymphoproliferations. In contrast, OALs-including lesions of the conjunctiva, lacrimal gland, orbit, and eyelids-are mainly low-grade tumors not associated with HIV infection. 11 Although combination chemotherapy with highdose methotrexate is the basis of treatment of primary central nervous system and intraocular lymphomas, 10,12 radiotherapy has been the standard treatment for low-grade ophthalmologic lymphomas. [13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] However, other treatment options, such as therapy with monoclonal anti-CD20 antibody, 31,32 may constitute a promising alternative to external beam irradiation and its potential toxicity. In addition, some reports have suggested a possible value of anti-Chlamydia psittaci antibiotic therapy. 33,34 In this review, which is exclusively focused on ophthalmologic lymphomas, we first present the pathologic, clinical, and prognostic factors of these lymphomas and then discuss all available treatment options in terms of safety and efficacy and their respective indications. Biology of ophthalmologic lymphomas Diagnosis of OALAs for all other forms of nodal and extranodal NHL, confirming a positive diagnosis of OAL is often long and difficult because the tumor is very small in most patients. Positive diagnosis must be based on histologic examination of a sufficient tumor sample obtained by surgical biopsy that is large enough to allow rigorous definition of lymphoproliferative disease according to the World Health Organization (WHO) classification. 35 Histopathologic examination consists of morphologic examination of cellular proliferation (cellular appearance and architectural pattern), usually combined with immunohistochemical analysis and molecular analysis.The exact modalities of biopsy depend on the anatomic site of the lesion and its relation to the eye: biopsy may be performed of the conjunctiva, an eyelid lesion, an orbital mass, or the lacrimal gland. However, sometimes patients have nodal involvement, an...

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Ocular adnexal lymphoma: Clinical behavior of distinct World Health Organization classification subtypes

Cited by 221 publications

References 36 publications

Follicular lymphoma of the ocular adnexal region: A nation‐based study

Follicular lymphoma of the ocular adnexal region: A nation‐based study

A20 inactivation in ocular adnexal MALT lymphoma

Ocular adnexal lymphoma: a review of clinicopathologic features and treatment options

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