Conjunctival Lymphoma: An International Multicenter Retrospective Study

Marina M Kirkegaard; Peter K Rasmussen; Sarah E Coupland; Bita Esmaeli; Paul T Finger; Gerardo F Graue; Hans E Grossniklaus; Santosh G Honavar; Jwu J Khong; Penny A McKelvie; Kaustubh Mulay; Jan U Prause; Elisabeth Ralfkiaer; Lene D Sjö; Peter B Toft; Geeta K Vemuganti; Bradley A Thuro; Jeremy Curtin; Steffen Heegaard

doi:10.1001/jamaophthalmol.2015.6122

Conjunctival Lymphoma: An International Multicenter Retrospective Study

Marina M Kirkegaard, Peter K Rasmussen, Sarah E Coupland, Bita Esmaeli, Paul T Finger, Gerardo F Graue, Hans E Grossniklaus, Santosh G Honavar, Jwu J Khong, Penny A McKelvie, Kaustubh Mulay, Jan U Prause, Elisabeth Ralfkiaer, Lene D Sjö, Peter B Toft, Geeta K Vemuganti, Bradley A Thuro, Jeremy Curtin, Steffen Heegaard

51 Citations (Scopus)

Abstract

IMPORTANCE: To date, the clinical features of the various subtypes of conjunctival lymphoma (CL) have not been previously evaluated in a large cohort.

OBJECTIVE: To characterize subtype-specific clinical features of CL and their effect on patient outcome.

DESIGN, SETTING, AND PARTICIPANTS: A retrospective multicenter study was performed. Patient data were collected from January 1, 1980, through December 31, 2010. The dates of the analysis were May 15, 2015, to August 20, 2015. The median follow-up period was 43 months. Seven eye cancer centers were involved in the study. In total, 268 patients with CL were identified, 5 of whom were excluded because of missing clinical data.

MAIN OUTCOMES AND MEASURES: Overall survival, disease-specific survival, and progression-free survival were the primary end points.

RESULTS: Two hundred sixty-three patients with CL were included in the study. Their mean age was 61.3 years, and 55.1% (145 of 263) were female. All lymphomas were of B-cell type. The most frequent subtype was extranodal marginal zone lymphoma (EMZL) (68.4% [180 of 263]), followed by follicular lymphoma (FL) (16.3% [43 of 263]), mantle cell lymphoma (MCL) (6.8% [18 of 263]), and diffuse large B-cell lymphoma (DLBCL) (4.6% [12 of 263). Conjunctival lymphoma commonly manifested in elderly individuals (age range, 60-70 years old), with EMZL having a female predilection (57.8% [104 of 180]) and MCL having a marked male predominance (77.8% [14 of 18]). Unlike EMZL and FL, DLBCL and MCL were frequently secondary diseases (41.7% [5 of 12] and 88.9% [16 of 18], respectively), with MCL showing a frequent occurrence of stage IVE lymphoma (61.1% [11 of 18]) and bilateral manifestation (77.8% [14 of 18]). Localized disease (stage IE or IIE) was commonly treated with external beam radiation therapy (EBRT) with or without chemotherapy, while widespread lymphoma (stage IIIE or IVE) and MCL of any stage were managed with chemotherapy with or without EBRT. Diffuse large B-cell lymphoma and MCL had a poor prognosis, with 5-year disease-specific survival of 55.0% and 9.0%, respectively, in contrast to EMZL (97.0%) and FL (82.0%). Further survival predictors included age (EMZL), sex (FL), and Ann Arbor staging classification (EMZL and FL). The American Joint Committee on Cancer TNM staging showed limited prognostic usefulness, only being able to predict survival for patients with DLBCL.

CONCLUSIONS AND RELEVANCE: Conjunctival lymphoma consists of mainly 4 subtypes of B-cell non-Hodgkin lymphoma: EMZL, FL, MCL, and DLBCL. Mantle cell lymphoma is characterized by a particularly high frequency of secondary disease of stage IVE and bilateral manifestation. The histological subtype is the main outcome predictor, with MCL and DLBCL having a markedly poorer prognosis than EMZL and FL.

Original language	English
Journal	J A M A Ophthalmology
Volume	134
Issue number	4
Pages (from-to)	406-14
Number of pages	9
ISSN	2168-6165
DOIs	https://doi.org/10.1001/jamaophthalmol.2015.6122
Publication status	Published - Apr 2016

Keywords

Journal Article
Research Support, Non-U.S. Gov't

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1001/jamaophthalmol.2015.6122

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Kirkegaard, M. M., Rasmussen, P. K., Coupland, S. E., Esmaeli, B., Finger, P. T., Graue, G. F., Grossniklaus, H. E., Honavar, S. G., Khong, J. J., McKelvie, P. A., Mulay, K., Prause, J. U., Ralfkiaer, E., Sjö, L. D., Toft, P. B., Vemuganti, G. K., Thuro, B. A., Curtin, J., & Heegaard, S. (2016). Conjunctival Lymphoma: An International Multicenter Retrospective Study. J A M A Ophthalmology, 134(4), 406-14. https://doi.org/10.1001/jamaophthalmol.2015.6122

Kirkegaard, MM, Rasmussen, PK, Coupland, SE, Esmaeli, B, Finger, PT, Graue, GF, Grossniklaus, HE, Honavar, SG, Khong, JJ, McKelvie, PA, Mulay, K, Prause, JU, Ralfkiaer, E, Sjö, LD , Toft, PB, Vemuganti, GK, Thuro, BA, Curtin, J & Heegaard, S 2016, 'Conjunctival Lymphoma: An International Multicenter Retrospective Study', J A M A Ophthalmology, vol. 134, no. 4, pp. 406-14. https://doi.org/10.1001/jamaophthalmol.2015.6122

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abstract = "IMPORTANCE: To date, the clinical features of the various subtypes of conjunctival lymphoma (CL) have not been previously evaluated in a large cohort.OBJECTIVE: To characterize subtype-specific clinical features of CL and their effect on patient outcome.DESIGN, SETTING, AND PARTICIPANTS: A retrospective multicenter study was performed. Patient data were collected from January 1, 1980, through December 31, 2010. The dates of the analysis were May 15, 2015, to August 20, 2015. The median follow-up period was 43 months. Seven eye cancer centers were involved in the study. In total, 268 patients with CL were identified, 5 of whom were excluded because of missing clinical data.MAIN OUTCOMES AND MEASURES: Overall survival, disease-specific survival, and progression-free survival were the primary end points.RESULTS: Two hundred sixty-three patients with CL were included in the study. Their mean age was 61.3 years, and 55.1% (145 of 263) were female. All lymphomas were of B-cell type. The most frequent subtype was extranodal marginal zone lymphoma (EMZL) (68.4% [180 of 263]), followed by follicular lymphoma (FL) (16.3% [43 of 263]), mantle cell lymphoma (MCL) (6.8% [18 of 263]), and diffuse large B-cell lymphoma (DLBCL) (4.6% [12 of 263). Conjunctival lymphoma commonly manifested in elderly individuals (age range, 60-70 years old), with EMZL having a female predilection (57.8% [104 of 180]) and MCL having a marked male predominance (77.8% [14 of 18]). Unlike EMZL and FL, DLBCL and MCL were frequently secondary diseases (41.7% [5 of 12] and 88.9% [16 of 18], respectively), with MCL showing a frequent occurrence of stage IVE lymphoma (61.1% [11 of 18]) and bilateral manifestation (77.8% [14 of 18]). Localized disease (stage IE or IIE) was commonly treated with external beam radiation therapy (EBRT) with or without chemotherapy, while widespread lymphoma (stage IIIE or IVE) and MCL of any stage were managed with chemotherapy with or without EBRT. Diffuse large B-cell lymphoma and MCL had a poor prognosis, with 5-year disease-specific survival of 55.0% and 9.0%, respectively, in contrast to EMZL (97.0%) and FL (82.0%). Further survival predictors included age (EMZL), sex (FL), and Ann Arbor staging classification (EMZL and FL). The American Joint Committee on Cancer TNM staging showed limited prognostic usefulness, only being able to predict survival for patients with DLBCL.CONCLUSIONS AND RELEVANCE: Conjunctival lymphoma consists of mainly 4 subtypes of B-cell non-Hodgkin lymphoma: EMZL, FL, MCL, and DLBCL. Mantle cell lymphoma is characterized by a particularly high frequency of secondary disease of stage IVE and bilateral manifestation. The histological subtype is the main outcome predictor, with MCL and DLBCL having a markedly poorer prognosis than EMZL and FL.",

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author = "Kirkegaard, {Marina M} and Rasmussen, {Peter K} and Coupland, {Sarah E} and Bita Esmaeli and Finger, {Paul T} and Graue, {Gerardo F} and Grossniklaus, {Hans E} and Honavar, {Santosh G} and Khong, {Jwu J} and McKelvie, {Penny A} and Kaustubh Mulay and Prause, {Jan U} and Elisabeth Ralfkiaer and Sj{\"o}, {Lene D} and Toft, {Peter B} and Vemuganti, {Geeta K} and Thuro, {Bradley A} and Jeremy Curtin and Steffen Heegaard",

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doi = "10.1001/jamaophthalmol.2015.6122",

language = "English",

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TY - JOUR

T1 - Conjunctival Lymphoma

T2 - An International Multicenter Retrospective Study

AU - Kirkegaard, Marina M

AU - Rasmussen, Peter K

AU - Coupland, Sarah E

AU - Esmaeli, Bita

AU - Finger, Paul T

AU - Graue, Gerardo F

AU - Grossniklaus, Hans E

AU - Honavar, Santosh G

AU - Khong, Jwu J

AU - McKelvie, Penny A

AU - Mulay, Kaustubh

AU - Prause, Jan U

AU - Ralfkiaer, Elisabeth

AU - Sjö, Lene D

AU - Toft, Peter B

AU - Vemuganti, Geeta K

AU - Thuro, Bradley A

AU - Curtin, Jeremy

AU - Heegaard, Steffen

PY - 2016/4

Y1 - 2016/4

N2 - IMPORTANCE: To date, the clinical features of the various subtypes of conjunctival lymphoma (CL) have not been previously evaluated in a large cohort.OBJECTIVE: To characterize subtype-specific clinical features of CL and their effect on patient outcome.DESIGN, SETTING, AND PARTICIPANTS: A retrospective multicenter study was performed. Patient data were collected from January 1, 1980, through December 31, 2010. The dates of the analysis were May 15, 2015, to August 20, 2015. The median follow-up period was 43 months. Seven eye cancer centers were involved in the study. In total, 268 patients with CL were identified, 5 of whom were excluded because of missing clinical data.MAIN OUTCOMES AND MEASURES: Overall survival, disease-specific survival, and progression-free survival were the primary end points.RESULTS: Two hundred sixty-three patients with CL were included in the study. Their mean age was 61.3 years, and 55.1% (145 of 263) were female. All lymphomas were of B-cell type. The most frequent subtype was extranodal marginal zone lymphoma (EMZL) (68.4% [180 of 263]), followed by follicular lymphoma (FL) (16.3% [43 of 263]), mantle cell lymphoma (MCL) (6.8% [18 of 263]), and diffuse large B-cell lymphoma (DLBCL) (4.6% [12 of 263). Conjunctival lymphoma commonly manifested in elderly individuals (age range, 60-70 years old), with EMZL having a female predilection (57.8% [104 of 180]) and MCL having a marked male predominance (77.8% [14 of 18]). Unlike EMZL and FL, DLBCL and MCL were frequently secondary diseases (41.7% [5 of 12] and 88.9% [16 of 18], respectively), with MCL showing a frequent occurrence of stage IVE lymphoma (61.1% [11 of 18]) and bilateral manifestation (77.8% [14 of 18]). Localized disease (stage IE or IIE) was commonly treated with external beam radiation therapy (EBRT) with or without chemotherapy, while widespread lymphoma (stage IIIE or IVE) and MCL of any stage were managed with chemotherapy with or without EBRT. Diffuse large B-cell lymphoma and MCL had a poor prognosis, with 5-year disease-specific survival of 55.0% and 9.0%, respectively, in contrast to EMZL (97.0%) and FL (82.0%). Further survival predictors included age (EMZL), sex (FL), and Ann Arbor staging classification (EMZL and FL). The American Joint Committee on Cancer TNM staging showed limited prognostic usefulness, only being able to predict survival for patients with DLBCL.CONCLUSIONS AND RELEVANCE: Conjunctival lymphoma consists of mainly 4 subtypes of B-cell non-Hodgkin lymphoma: EMZL, FL, MCL, and DLBCL. Mantle cell lymphoma is characterized by a particularly high frequency of secondary disease of stage IVE and bilateral manifestation. The histological subtype is the main outcome predictor, with MCL and DLBCL having a markedly poorer prognosis than EMZL and FL.

AB - IMPORTANCE: To date, the clinical features of the various subtypes of conjunctival lymphoma (CL) have not been previously evaluated in a large cohort.OBJECTIVE: To characterize subtype-specific clinical features of CL and their effect on patient outcome.DESIGN, SETTING, AND PARTICIPANTS: A retrospective multicenter study was performed. Patient data were collected from January 1, 1980, through December 31, 2010. The dates of the analysis were May 15, 2015, to August 20, 2015. The median follow-up period was 43 months. Seven eye cancer centers were involved in the study. In total, 268 patients with CL were identified, 5 of whom were excluded because of missing clinical data.MAIN OUTCOMES AND MEASURES: Overall survival, disease-specific survival, and progression-free survival were the primary end points.RESULTS: Two hundred sixty-three patients with CL were included in the study. Their mean age was 61.3 years, and 55.1% (145 of 263) were female. All lymphomas were of B-cell type. The most frequent subtype was extranodal marginal zone lymphoma (EMZL) (68.4% [180 of 263]), followed by follicular lymphoma (FL) (16.3% [43 of 263]), mantle cell lymphoma (MCL) (6.8% [18 of 263]), and diffuse large B-cell lymphoma (DLBCL) (4.6% [12 of 263). Conjunctival lymphoma commonly manifested in elderly individuals (age range, 60-70 years old), with EMZL having a female predilection (57.8% [104 of 180]) and MCL having a marked male predominance (77.8% [14 of 18]). Unlike EMZL and FL, DLBCL and MCL were frequently secondary diseases (41.7% [5 of 12] and 88.9% [16 of 18], respectively), with MCL showing a frequent occurrence of stage IVE lymphoma (61.1% [11 of 18]) and bilateral manifestation (77.8% [14 of 18]). Localized disease (stage IE or IIE) was commonly treated with external beam radiation therapy (EBRT) with or without chemotherapy, while widespread lymphoma (stage IIIE or IVE) and MCL of any stage were managed with chemotherapy with or without EBRT. Diffuse large B-cell lymphoma and MCL had a poor prognosis, with 5-year disease-specific survival of 55.0% and 9.0%, respectively, in contrast to EMZL (97.0%) and FL (82.0%). Further survival predictors included age (EMZL), sex (FL), and Ann Arbor staging classification (EMZL and FL). The American Joint Committee on Cancer TNM staging showed limited prognostic usefulness, only being able to predict survival for patients with DLBCL.CONCLUSIONS AND RELEVANCE: Conjunctival lymphoma consists of mainly 4 subtypes of B-cell non-Hodgkin lymphoma: EMZL, FL, MCL, and DLBCL. Mantle cell lymphoma is characterized by a particularly high frequency of secondary disease of stage IVE and bilateral manifestation. The histological subtype is the main outcome predictor, with MCL and DLBCL having a markedly poorer prognosis than EMZL and FL.

KW - Journal Article

KW - Research Support, Non-U.S. Gov't

U2 - 10.1001/jamaophthalmol.2015.6122

DO - 10.1001/jamaophthalmol.2015.6122

M3 - Journal article

C2 - 26891973

SN - 2168-6165

VL - 134

SP - 406

EP - 414

JO - J A M A Ophthalmology

JF - J A M A Ophthalmology

IS - 4

ER -

Conjunctival Lymphoma: An International Multicenter Retrospective Study

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