Ocular MALT lymphoma

Epidemiology

MALT lymphoma is the most common ocular adnexal lymphoma, accounting for 25% of all non-gastric MALT lymphomas. They occur across a wide age range in adults, without a sex bias.

Macroscopic appearances

The lymphoma may involve the lachrymal gland, conjunctival mucosa or eyelid. Some cases also show bone marrow involvement.

Histopathology

The tumour is diffuse or vaguely nodular. Residual follicles may be present. The neoplastic cells are monocytoid, centrocytic or lymphoplasmacytoid. A low proportion of immunoblasts or centroblasts may be present but no tin clusters of more than 20 such cells.

Immunohistochemistry

 

CD5

negative

  
 

CD10

0/39

  
 

CD20

39/39

  
 

CD23

negative

  
 

CD38

8/39

  

CD43

12/36

CD79a

39/39

bcl-2

39/39

bcl-6

0/39

cytoplasmic bcl-10

8/39

nuclear bcl-10

11/37

bcl-XL - high level expression

6/39

light chain restriction

32/33

MUM1

4/39

Ki-67 - high level expression

9/39

CDK1

11/39

CDK2

0/39

CDK4

12/39

CDK6

14/39

CyclinD1

0/39

CyclinD3

6/39

CyclinE

7/39

CicA

4/39

p16 - high expression

17/39

p18 - high expression

22/31

p21 - high expression

4/39

p27 - high expression

25/39

p53

0/39

Hdm2

12/39

Bax - high level expression

30/39

pLkBa

19/39

Caspase 3

1/38

Survivin - high level expression

6/38

TUNEL - high level expression

28/36

 

 

HCV seropositivity

5/10

t(11;18)

0/36

t(14;18)

5/37

Several of these alterations related to the regulation of apoptosis: caspase 3 activity is abolished and IkBa activity increased. The presence of a large cell component is associated with increased expression of survivin, cyclin A, cyclin E; KI67 and p16 expression are reduced1.

Management

Good control may be obtained by either radiotherapy or chemotherapy.

Prognosis

Most ocular MALT lymphomas have an indolent course. Recurrence after treatment may be local or contralateral. Some cases disseminate. The presence of large cells does not predict behaviour.

References

1 Franco R, Camacho FI, Caleo A, et al. Nuclear bcl10 expression characterizes a group of ocular adnexa MALT lymphomas with shorter failure-free survival. Mod Pathol 2006; 19:1055-1067

 

This page last revised 12.8.2006.

©SMUHT/PW Bishop