Primary ocular adnexal lymphoma (POAL) is a rare extranodal lymphoma. The mucosa-associated lymphoid tissue (MALT) subtype predominates and primarily occurs after the sixth decade of life. Most studies of ocular adnexal lymphoma are from the adult population. The data and experience in pediatric patients with POAL are limited to a few cases reported in the literature. Here we describe two pediatric cases of POAL and review the literature to further help characterize the clinical features and histopathologic appearance of this uncommon lymphoma.
FreemanC, BergJW, CutlerSJ. Occurrence and prognosis of extranodal lymphomas. Cancer1972;29:252–260.
5.
HarrisNL, JaffeES, SteinH.A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood1994;84:1361–1392.
6.
JeffeES, HarrisNL, SteinH, VardimanJW. Pathology and Genetics, Tumors of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC Press, 2001.
7.
FerryJ, WhiteW, GroveA, HarrisNL (1992) “Malignant lymphoma of ocular adnexa: a spectrum of B-cell neoplasia including low grade B-cell lymphoma of MALT type (abstract 450)” Mod. Pathol.5: 77A
8.
CouplandSE, KrauseL, DelecluseHJ. Lympoproliferative lesions of the ocular adnexa: analysis of 112 cases. Ophthalmology1998;105:1430–1441.
9.
Auw-HaedrichC, CouplandSE, KappA, Schmitt-GraffA, BuchenR, WitschelH.Long term outcome of ocular adnexal lymphoma subtyped according to the REAL classification. Br J Ophthalmol2001;85:63–69.
10.
NakataM, MatsunoY, KatsumataN.Histology according to the Revised European-American Lymphoma Classification significantly predicts the prognosis of ocular adnexal lymphoma. Leuk Lymphoma1999;32:533–543.
11.
ChenPM, ChiouTJ, YuIT. Molecular analysis of mucosa-associated lymphoid tissue (MALT) lymphoma of ocular adnexa. Leuk Lymphoma2001;42:207–214.
12.
ChenPM, LiuJH, YuIT. Lack of mutations of BCL6 and BCL10 genes in mucosa-associated lymphoid tissue lymphomas of the orbital adnexa. Cancer Genet Cytogenet2000;123:44–48.
13.
JenkinsC, RoseGE, BunceC.Histological features of ocular adnexal lymphoma (REAL classification) and their association with patient morbidity and survival. Br J Ophthalmol2000;84:907–913.
14.
McKelviePA, McNabA, FrancisIC, FoxR, O'DayJ.Ocular adnexal lymphoproliferative disease: a series of 73 cases. Clin Exp Ophthalmol2001;29:387–393.
15.
MedeirosLJ, HarmonDC, LinggoodRM, HarrisNL. Immunohistologic features predict clinical behavior of orbital and conjunctival lymphoid infiltrates. Blood1989;74:2121–2129.
16.
KnowlesDM, JakobiecFA, McNallyL, BurkeJS. Lymphoid hyperplasia and malignant lymphoma occurring in the ocular adnexa (orbit, conjunctiva, and eyelids): a prospective multiparametric analysis of 108 cases during 1977 to 1987. Hum Pathol1990;21:959–973.
17.
JakobiecFA, McLeanI, FontRL (1979) “Clinicopathologic characteristics of orbital lymphoid hyperplasia” Ophthalmology86: 948–966
18.
DuM, DissTC, XuC, PengH, IsaacsonPG, PanL.Ongoing mutation in MALT lymphoma immunoglobulin gene suggests that antigen stimulation plays a role in the clonal expansion. Leukemia1996;10:1190–1197.
19.
PolitoE, GalieniP, LeccisottiA.Clinical and radiological presentation of 95 orbital lymphoid tumors. Graefes Arch Clin Exp Ophthalmol1996;234:504–509.
20.
MannamiT, YoshinoT, OshimaK.Clinical, histopathological, and immunogenetic analysis of ocular adnexal lymphoproliferative disorders: characterization of MALT lymphoma and reactive lymphoid hyperplasia. Mod Pathol2001;14:641–649.
21.
JoshiVV, GagnonGA, ChadwickEG. The spectrum of mucosa-associated lymphoid tissue lesions in pediatric patients infected with HIV: a clinicopathologic study of six cases. Am J Clin Pathol1997;107:592–600.
22.
Teruya-FeldsteinJ, TemeckBK, SloasMM. Pulmonary malignant lymphoma of mucosa-associated lymphoid tissue (MALT) arising in a pediatric HIV-positive patient. Am J Surg Pathol1995;19:357–363.
23.
GriffinCA, ZehnbauerBA, BeschornerWE, AmbinderR, MannR.t(11;18)(q21;q21) is a recurrent chromosome abnormality in small lymphocytic lymphoma. Genes Chromosomes Cancer1992;4:153–157.
24.
WotherspoonAC, PanLX, DissTC, IsaacsonPG. Cytogenetic study of B-cell lymphoma of mucosa-associated lymphoid tissue. Cancer Genet Cytogenet1992;58:35–38.
25.
DierlammJ, BaensM, WlodarskaI.The apoptosis inhibitor gene API2 and a novel 18q gene, MAT, are recurrently rearranged in the t(11;18)(q21;q21) associated mucosa-associated lymphoid tissue lymphoma. Blood1999;93:3601–3609.
26.
ZhangQ, SiebertR, YanM.Inactivating mutations and overexpression of BCL10, a caspase recruitment domain-containing gene, in MALT lymphoma with t(1;14)(p22;q32). Nat Genet1999;22:63–68.
27.
ChanJKC, NgCS, IsaacsonPG. Relationship between high-grade lymphoma and low-grade B-cell mucosa-associated lymphoid tissue lymphoma (MALToma) of the stomach. Am J Pathol1990;136:1153–1164.
28.
de JongD, BootH, Van HeerdeP, HartGA, TaalBG. Histological grading in gastric lymphoma: pretreatment criteria and clinical relevance. Gastroenterology1997;112:1466–1474.
29.
WhiteWL, FerryJA, HarrisNL, GroveASJr.Ocular adnexal lymphoma: a clinicopathologic study with identification of lymphomas of mucosa-associated lymphoid tissue type. Ophthalmology1995;102:1994–2006.
30.
CampoE, JaffeES (2002) (Abstract). Ann Oncol13(Suppl 2): 6
