Conjunctival MALT Lymphoma


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Conjunctival MALT Lymphoma

  1. 1. Doheny Eye Institute Grand Rounds Ying Pan, MD
  2. 2. History of Present Illness <ul><li>CC: 8 months of progressively enlarging growth in right eye </li></ul><ul><li>HPI: 24 year old Hispanic man from jail presented with increasing right eye redness since he was kicked in the eye 8 months ago. +Ocular irritation and foreign body sensation, but denied discharge, bleeding, pain or changes in vision. His symptoms did not improve with topical drops and ointments. </li></ul>
  3. 3. History POH:none PMH: none PSH: none Meds: none NKDA SH: Jail for 1 year FH: unremarkable ROS: 10 LB weight loss, occasional night sweats
  4. 4. Exam <ul><li>VA: 20/25 OU </li></ul><ul><li>Pupils: Round and reactive to light, no APD </li></ul><ul><li>Motility: Full OU </li></ul><ul><li>IOP (Tp): 12 OD , 15 OS </li></ul><ul><li>CVF: Full OU </li></ul>
  5. 5. Exam
  6. 6. Exam
  7. 10. H & E
  8. 11. H & E
  9. 12. H & E
  10. 13. Immunohistochemical staining CD 3 (T cell) CD 20 (B cell)
  11. 14. Immunohistochemical staining CD 21 Ki-67
  12. 15. Mucosa-Associated Lymphatic Tissue <ul><li>characteristic arrangement of lymphoid tissues in the mucosa </li></ul><ul><li>conjunctiva-associated lymphoid tissue </li></ul><ul><li>-minimal organized lymphatic tissue </li></ul><ul><li>-small number of lymphocytes </li></ul><ul><li> -few mast cells & langerhans cells </li></ul>
  13. 16. MALT Lymphoma <ul><li>spectrum from benign hyperplasia to malignant lymphoma </li></ul><ul><li>common site: gastrointestinal tract </li></ul><ul><li>extraintestinal locations: tonsils, skin, breast, lung, orbit </li></ul>
  14. 17. Conjunctival MALT Lymphoma <ul><li>Mainly occurs in the fifth to sixth decades </li></ul><ul><li>Bilateral involvement: 20% </li></ul><ul><li>Systemic disease: 30% </li></ul><ul><ul><li>117 conjunctival lymphoid tumors </li></ul></ul><ul><ul><li>(Shields et al. Ophthalmology 2001) </li></ul></ul><ul><ul><ul><li>unilateral disease: 11 of 64 (17%) </li></ul></ul></ul><ul><ul><ul><li>bilateral disease: 25 of 53 (47%) </li></ul></ul></ul><ul><li>Favorable prognosis </li></ul><ul><ul><li>Minimally aggressive </li></ul></ul><ul><ul><li>Localize course </li></ul></ul><ul><ul><li>Favorable response to treatment </li></ul></ul><ul><ul><li>Recurrence </li></ul></ul>
  15. 18. Standard Treatment <ul><li>Localized </li></ul><ul><ul><li>Excision </li></ul></ul><ul><ul><li>Cryotherapy </li></ul></ul><ul><ul><li>Radiotherapy (tumor control: 89-100%) </li></ul></ul><ul><ul><ul><li>50 patients with ocular adnexal lymphoma </li></ul></ul></ul><ul><ul><ul><li>( Uno et al. Cancer 2003 ) </li></ul></ul></ul><ul><ul><ul><ul><li>49 patients: tumor control at 24 months </li></ul></ul></ul></ul><ul><ul><ul><ul><li>6 patients: disease reoccurrence from 4-97 months </li></ul></ul></ul></ul><ul><li>Systemic </li></ul><ul><ul><li>Chemotherapy (+/- excision) </li></ul></ul>
  16. 19. Observation ( Matsuo & Yoshino, Ophthalmology 2004) <ul><ul><li>13 patients with Conjunctival MALT Lymphoma </li></ul></ul><ul><ul><li> </li></ul></ul><ul><ul><li>excisional biopsy leaving small lesion behind </li></ul></ul><ul><li>Observation (8) Radiation (5) </li></ul><ul><li>Tumor Tumor Tumor </li></ul><ul><li>Regression (7) Regrowth (1) Regression (5) </li></ul><ul><li>at 5 years at 3 years </li></ul>
  17. 20. Local Chemotherapy with Interferon  ( Blasti et al. Ophthalmology 2001 ) <ul><li>Interferon: resistance to viral infections, inhibit cell proliferation and modulate immune response. </li></ul><ul><ul><ul><ul><li>4 patients with conjunctival MALT Lymphoma </li></ul></ul></ul></ul><ul><ul><li>Subconjunctival interferon injection (1,500,000IU 3X/wk for 4 wks) </li></ul></ul><ul><ul><li>Tumor regression (3) Recurrence/systemic disease </li></ul></ul><ul><ul><li>follow up time: 12-36 months at 11 months </li></ul></ul>
  18. 21. Antibiotics ( Ferreri et at. J Natl Cancer Inst 2004 ) <ul><li>Predisposing condition (ie autoimmune, infection) </li></ul><ul><li> acquisition of MALT </li></ul><ul><li> reactive lymphoid tissue </li></ul><ul><li> Lymphoma </li></ul><ul><li>Link between chlamydia psittaci infection and chronic antigenic stimulation  Lymphoma? </li></ul>
  19. 22. Antibiotics ( Ferreri et at. J Natl Cancer Inst 2004 ) <ul><ul><li>Chlamydia psittaci DNA in ocular tissue </li></ul></ul><ul><ul><ul><li>Ocular adnexal lymphoma: 32 of 40 (80%) </li></ul></ul></ul><ul><ul><ul><li>Reactive lymphadenopathy: 3 of 26 (12%) </li></ul></ul></ul><ul><ul><ul><li>Nonneoplastic samples: 0 of 20 (0%) </li></ul></ul></ul><ul><ul><li>8 patients with C psittaci positive MALT lymphoma of adnexal- Doxycycline (3 wk regimen) </li></ul></ul><ul><ul><ul><li>At 12 months follow up: </li></ul></ul></ul><ul><ul><ul><li>Complete response: 2 </li></ul></ul></ul><ul><ul><ul><li>> 50% tumor reduction: 2 </li></ul></ul></ul><ul><ul><ul><li>< 50% tumor reduction: 2 </li></ul></ul></ul><ul><ul><ul><li>Minimal reduction: 1 </li></ul></ul></ul><ul><ul><ul><li>Tumor progression: 0 </li></ul></ul></ul>
  20. 23. Patient Up-Date <ul><li>Systemic Evaluation </li></ul><ul><li>Blood </li></ul><ul><li>Bone marrow biopsy </li></ul><ul><li>CT Scan (head, thorax, abdomen) </li></ul><ul><li>Chlamydia Psittaci IGM and IGG </li></ul>
  21. 24. References <ul><ul><li>Blasti et al. Local Chemotherapy with Interferon  for conjunctival mucosa-associated lymphoid tissue lymphoma. Ophthalmology 2001; 108: 559-562 </li></ul></ul><ul><ul><li>Ferreri et at. Evidence for an association between Chlamydia psittaci and ocular adnexal lymphomas. J Natl Cancer Inst 2004; 96: 586-94. </li></ul></ul><ul><ul><li>  </li></ul></ul><ul><ul><li>Isaacson PG et al. Extranodal marginal zone B cell lymphoma of mucosa associated lymphoid tissue. In: Jaffe ES, Harris NL. Stein H, et al. editors. WHO classification of tumours. Pathology and genetics of haematopoietic and lymphoid tissues. Lyon: IARC press, 2001: 157-160. </li></ul></ul><ul><ul><li>  </li></ul></ul><ul><ul><li>Knowles L, Jakobei FA, McNally NS, Burke JS. 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 Pathol 1990; 21 pp 959-973. </li></ul></ul><ul><ul><li>Le Qt et al. Primary radiotherapy for localized orbital MALT lymphoma. Int J Radiot Oncol Biol Phys 2002; 53: 657-663. </li></ul></ul><ul><ul><li>Shields et al. Conjunctival lymphoid tumors: clinical analysis of 117 cases and relationship to systemic lymphoma. Ophthalmology 2001; 108:979-984 Matsuo T, Yoshimo T. Long-term follow up results of observation or radiation for conjunctival malignant lymphoma. Ophthalmology 2004; 111:1233-1237. </li></ul></ul><ul><ul><li>  </li></ul></ul><ul><ul><li>Uno et al. Radiotherapy for extranodal, marginal zone, B cell lymphoma of mucosa-associated lymphoid tissue originating in the ocular adnexa. Cancer 2003; 93: 865-71. </li></ul></ul><ul><ul><li>  </li></ul></ul><ul><ul><li>  </li></ul></ul><ul><ul><li>  </li></ul></ul><ul><ul><li>. </li></ul></ul>