Abstract

Purpose: To report a rare case of extranodal NK/T cell lymphoma (NKTL) and to compare its features with those cases previously reported. Design: Case report, observational and literature review. Methods: Complete ophthalmologic examinations followed by excisional biopsy, histopathologic examination and therapy with radiation and chemotherapy. Main Outcome measures: Evaluation of clinical presenting features and histopathologic diagnosis along with patient outcome. Results: A 22 year old female presented as a referral with right orbital swelling, decreased vision and eye pain for 5 weeks. Subsequent orbital CT and multiple biopsies resulted in a diagnosis of extranodal natural killer (NK)/T cell lymphoma (NKTL). Despite continued chemotherapy and orbital radiation the patient expired within 3 months of diagnosis. To our knowledge, only 8 cases of orbital involvement without nasal mucosal involvement are reported in the literature, the majority in patients of male gender around the fifth decade. Conclusions: Here we present an atypical and aggressive case of extranodal NK/T cell lymphoma presenting in a 22 year old Caucasian female as orbital swelling without evidence of nasal mucosal involvement. It is important to distinguish NKTL from the more common benign lymphoproliferative lesions of the orbital adnexa as prognosis of these two clinical entities varies and timely diagnosis is key. The present case demonstrates that extranodal NKTL can occur in the orbit without evidence of the more common nasal mucosal presentations and should be included in the differential diagnosis of ocular adnexal lesions suspicious for a lymphoproliferative disorder and/or an inflammatory process.

Original languageEnglish (US)
Pages (from-to)267-269
Number of pages3
JournalOrbit
Volume31
Issue number4
DOIs
StatePublished - Aug 1 2012

Fingerprint

Natural Killer T-Cells
T-Cell Lymphoma
Nose
Extranodal NK-T-Cell Lymphoma
Eye Pain
Biopsy
Drug Therapy
Lymphoproliferative Disorders
Orbit
Natural Killer Cells
Differential Diagnosis
Radiotherapy
Referral and Consultation
Outcome Assessment (Health Care)
Radiation

All Science Journal Classification (ASJC) codes

  • Ophthalmology

Cite this

@article{a7900dcb8e384d249b3a02e68b50c5a0,
title = "Orbital involvement in extranodal natural killer T cell lymphoma: An atypical case presentation and review of the literature",
abstract = "Purpose: To report a rare case of extranodal NK/T cell lymphoma (NKTL) and to compare its features with those cases previously reported. Design: Case report, observational and literature review. Methods: Complete ophthalmologic examinations followed by excisional biopsy, histopathologic examination and therapy with radiation and chemotherapy. Main Outcome measures: Evaluation of clinical presenting features and histopathologic diagnosis along with patient outcome. Results: A 22 year old female presented as a referral with right orbital swelling, decreased vision and eye pain for 5 weeks. Subsequent orbital CT and multiple biopsies resulted in a diagnosis of extranodal natural killer (NK)/T cell lymphoma (NKTL). Despite continued chemotherapy and orbital radiation the patient expired within 3 months of diagnosis. To our knowledge, only 8 cases of orbital involvement without nasal mucosal involvement are reported in the literature, the majority in patients of male gender around the fifth decade. Conclusions: Here we present an atypical and aggressive case of extranodal NK/T cell lymphoma presenting in a 22 year old Caucasian female as orbital swelling without evidence of nasal mucosal involvement. It is important to distinguish NKTL from the more common benign lymphoproliferative lesions of the orbital adnexa as prognosis of these two clinical entities varies and timely diagnosis is key. The present case demonstrates that extranodal NKTL can occur in the orbit without evidence of the more common nasal mucosal presentations and should be included in the differential diagnosis of ocular adnexal lesions suspicious for a lymphoproliferative disorder and/or an inflammatory process.",
author = "A. Ely and J. Evans and Sundstrom, {J. M.} and J. Malysz and Specht, {C. S.} and M. Wilkinson",
year = "2012",
month = "8",
day = "1",
doi = "10.3109/01676830.2011.605506",
language = "English (US)",
volume = "31",
pages = "267--269",
journal = "Orbit",
issn = "0167-6830",
publisher = "Informa Healthcare",
number = "4",

}

TY - JOUR

T1 - Orbital involvement in extranodal natural killer T cell lymphoma

T2 - An atypical case presentation and review of the literature

AU - Ely, A.

AU - Evans, J.

AU - Sundstrom, J. M.

AU - Malysz, J.

AU - Specht, C. S.

AU - Wilkinson, M.

PY - 2012/8/1

Y1 - 2012/8/1

N2 - Purpose: To report a rare case of extranodal NK/T cell lymphoma (NKTL) and to compare its features with those cases previously reported. Design: Case report, observational and literature review. Methods: Complete ophthalmologic examinations followed by excisional biopsy, histopathologic examination and therapy with radiation and chemotherapy. Main Outcome measures: Evaluation of clinical presenting features and histopathologic diagnosis along with patient outcome. Results: A 22 year old female presented as a referral with right orbital swelling, decreased vision and eye pain for 5 weeks. Subsequent orbital CT and multiple biopsies resulted in a diagnosis of extranodal natural killer (NK)/T cell lymphoma (NKTL). Despite continued chemotherapy and orbital radiation the patient expired within 3 months of diagnosis. To our knowledge, only 8 cases of orbital involvement without nasal mucosal involvement are reported in the literature, the majority in patients of male gender around the fifth decade. Conclusions: Here we present an atypical and aggressive case of extranodal NK/T cell lymphoma presenting in a 22 year old Caucasian female as orbital swelling without evidence of nasal mucosal involvement. It is important to distinguish NKTL from the more common benign lymphoproliferative lesions of the orbital adnexa as prognosis of these two clinical entities varies and timely diagnosis is key. The present case demonstrates that extranodal NKTL can occur in the orbit without evidence of the more common nasal mucosal presentations and should be included in the differential diagnosis of ocular adnexal lesions suspicious for a lymphoproliferative disorder and/or an inflammatory process.

AB - Purpose: To report a rare case of extranodal NK/T cell lymphoma (NKTL) and to compare its features with those cases previously reported. Design: Case report, observational and literature review. Methods: Complete ophthalmologic examinations followed by excisional biopsy, histopathologic examination and therapy with radiation and chemotherapy. Main Outcome measures: Evaluation of clinical presenting features and histopathologic diagnosis along with patient outcome. Results: A 22 year old female presented as a referral with right orbital swelling, decreased vision and eye pain for 5 weeks. Subsequent orbital CT and multiple biopsies resulted in a diagnosis of extranodal natural killer (NK)/T cell lymphoma (NKTL). Despite continued chemotherapy and orbital radiation the patient expired within 3 months of diagnosis. To our knowledge, only 8 cases of orbital involvement without nasal mucosal involvement are reported in the literature, the majority in patients of male gender around the fifth decade. Conclusions: Here we present an atypical and aggressive case of extranodal NK/T cell lymphoma presenting in a 22 year old Caucasian female as orbital swelling without evidence of nasal mucosal involvement. It is important to distinguish NKTL from the more common benign lymphoproliferative lesions of the orbital adnexa as prognosis of these two clinical entities varies and timely diagnosis is key. The present case demonstrates that extranodal NKTL can occur in the orbit without evidence of the more common nasal mucosal presentations and should be included in the differential diagnosis of ocular adnexal lesions suspicious for a lymphoproliferative disorder and/or an inflammatory process.

UR - http://www.scopus.com/inward/record.url?scp=84863711844&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84863711844&partnerID=8YFLogxK

U2 - 10.3109/01676830.2011.605506

DO - 10.3109/01676830.2011.605506

M3 - Review article

C2 - 22681504

AN - SCOPUS:84863711844

VL - 31

SP - 267

EP - 269

JO - Orbit

JF - Orbit

SN - 0167-6830

IS - 4

ER -