Natural killer/T-cell lymphoma with ocular and adnexal involvement.
OBJECTIVE
To review the clinical, radiological, and histopathologic features in 8 patients with natural killer/T-cell lymphoma (NKTL) involving the orbit and/or ocular adnexa, and to describe the responses of these patients to various treatment regimens.


METHODS
Retrospective observational case series.


METHODS
Eight patients (5 male, 3 female) with NKTL involving the orbit and/or ocular adnexa were identified from 1999 through 2005. The mean age at presentation was 45 years (range, 26-65).


METHODS
We retrospectively identified patients with NKTL of the ocular adnexa treated in the authors' medical centers from 1999 through 2004 using computerized diagnostic index retrieval. The clinical records and radiologic studies were analyzed to define modes of presentation and progression, response to therapy, and areas of anatomic involvement. Histopathologic findings, including the presence of CD3, CD56, and Epstein-Barr virus-encoded mRNA in each patient, were reviewed.


METHODS
Time of survival from presentation to last known follow-up and tumor-related death.


RESULTS
Four of the 8 patients (50%) with NKTL involving the orbit or ocular adnexa had systemic involvement at presentation. Five of the 8 patients (62.5%) had concurrent sinonasal involvement, whereas 3 (37.5%) had orbital involvement alone. All lesions demonstrated CD3, CD56, and/or Epstein-Barr virus positivity on immunopathology studies. Therapy consisted of various chemotherapeutic regimens typically employed in the treatment of non-Hodgkins lymphoma, steroids, surgical intervention, and radiation. Seven (87.5%) patients died 5 weeks to 13 months after presentation, and 1 (12.5%) is alive without disease (5-year follow-up).


CONCLUSIONS
Natural killer/T-cell orbital lymphoma is a rare Epstein-Barr virus-associated neoplasm that may occur with or without associated sinonasal involvement. Our series, the largest cohort reported to date, demonstrates the high lethality of this condition despite aggressive conventional therapy, suggesting that new treatment options should be considered early in the course of treatment of patients with this disorder.
Woog
John J
JJ
Mayo Clinic, Rochester, Minnesota, USA.
Kim
Yoon Duck
YD
Yeatts
R Patrick
RP
Kim
Stella
S
Esmaeli
Bita
B
Kikkawa
Don
D
Lee
H B Harold
HB
Korn
Bobby S
BS
Punja
Karim
K
Habermann
Thomas M
TM
Colgan
Joseph P
JP
Salomao
Diva
D
Cameron
J Douglas
JD
D000328:Adult; D000368:Aged; D017252:CD3 Complex / Q000378:metabolism; D019002:CD56 Antigen / Q000378:metabolism; D003131:Combined Modality Therapy; D020031:Epstein-Barr Virus Infections / Q000000981:diagnostic imaging / Q000378:metabolism / Q000401:mortality / Q000473:pathology; D005142:Eyelid Neoplasms / Q000000981:diagnostic imaging / Q000401:mortality / Q000473:pathology* / Q000821:virology; D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology*; D007766:Lacrimal Apparatus Diseases / Q000000981:diagnostic imaging / Q000401:mortality / Q000473:pathology* / Q000821:virology; D016399:Lymphoma, T-Cell / Q000000981:diagnostic imaging / Q000401:mortality / Q000473:pathology* / Q000821:virology; D008297:Male; D008875:Middle Aged; D009918:Orbital Neoplasms / Q000000981:diagnostic imaging / Q000401:mortality / Q000473:pathology* / Q000821:virology; D010255:Paranasal Sinus Neoplasms / Q000000981:diagnostic imaging / Q000401:mortality / Q000473:pathology* / Q000821:virology; D012189:Retrospective Studies; D015996:Survival Rate; D014057:Tomography, X-Ray Computed
D017252:CD3 Complex; D019002:CD56 Antigen