Extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue-type of the orbit and ocular adnexa.
Non-Hodgkin's lymphomas of the orbit and ocular adnexa (OOA), the majority of which are extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue-type (MALT lymphomas), are a rare disorder. The aim of this study was to evaluate the clinical features and treatment outcomes and complications in patients with MALT lymphoma of OOA. Thirty-seven patients with a histologically verified diagnosis of MALT lymphoma of OOA were included in this retrospective, observational case study. There were 22 (59%) men and 15 (41%) women, with a median age of 44 years (range, 21-80 years). The most common presenting complaint was a slowly growing orbital mass. The stages were IA(E) in 74%, IA(EE) (bilateral involvement) in 18%, IIIA(E) in 6%, and IVA(E) in 3%. None of the patients had an elevated value of LHD or beta(2)-microglobulin. Surgical resection alone was attempted as the sole treatment in two patients, but tumor recurred 19 and 24 months after surgery. Radiotherapy, with a median tumor dose of 3,060 cGy, was administered in 29 patients; all of the patients achieved complete remission, and none of them had severe later complications. Combination chemotherapy alone was employed in three patients with stage IIIA(E) or IVA(E). With a median follow-up duration of 21 months, the 3-year overall survival (OS) rate and event-free survival (EFS) rate were 97% and 86%, respectively. Subgroup analysis of the patients with localized disease, who received radiotherapy as an initial treatment modality, revealed that 3-year OS rate and EFS rate were 100% and 93%, respectively. All disease recurrences were documented histologically as MALT lymphoma. In conclusion complete staging evaluation is needed to select an adequate treatment modality. Radiotherapy alone can produce excellent local control and survival in patients with localized MALT lymphoma of OOA. Systemic chemotherapy should be considered in patients with advanced stages or systemic manifestation.
Lee
Jae-Lyun
JL
Department of Medicine, Yeungnam University College of Medicine, Nam-gu, 705-717 Daegu, South Korea.
Kim
Min-Kyoung
MK
Lee
Kyung Hee
KH
Hyun
Myung Soo
MS
Chung
Hwa Sun
HS
Kim
Dong Suk
DS
Shin
Sei One
SO
Cho
Hee Soon
HS
Bae
Sung Hwa
SH
Ryoo
Hun Mo
HM
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003131:Combined Modality Therapy; D005134:Eye Neoplasms / Q000150:complications / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000150:complications / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009918:Orbital Neoplasms / Q000150:complications / Q000473:pathology / Q000628:therapy; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome