MALToma: 92 year old woman with painless conjunctival mass

By Hari Bodhireddy, MSIII

Texas Tech University Health Sciences Center School of Medicine, Lubbock, TX

Chief Complaint: 92 year old woman complaining of a speck in her eye, OS.

History of Presenting Illness: Patient has had a slow-growing, painless lesion on her bulbar conjunctiva OS for an unknown period of time. She has occasional episodes of side-by-side double vision of 15 minutes duration late in the afternoon, which is corrected when either eye is covered.

Medical History/Ocular History: Arthritis of unknown type, age-related macular degeneration, esotropia, bilateral cataract surgery

Social History: Non-drinker, non-smoker

Family History: none reported

Medications/Allergies: oral pain meds for arthritis, no known drug allergies

Review of Systems: Worsening joint pain, denies any additional symptoms (such as nausea/vomiting/dizziness/headache/fever/chills/weight loss)

Ocular Exam:

Visual acuity – OD 20/40, OS 20/30, pinhole no improvement
Pupils – pupils are 3mm OU, with 4+ reactivity OU, and no APD OU
Tonometry – 14 OD and 14 OS at 15:55
External Eye Exam – normal
Motility – EOM’s normal
Visual Fields – CVF’s full OU
Slit Lamp Examination – Salmon-colored 3mm conjunctival lesion in superior nasal aspect of bulbar conjunctiva OS, PCIOL’s in good position OU, remainder of slit lamp exam normal
Dilated Fundus Examination – 1+ RPE change OU, 2+ drusen OU, optic nerve C/D 0.2 OU, remainder of dilated fundus exam unremarkable

Diagnosis: Conjunctival mucosa-associated lymphoid tissue lymphoma (MALToma)

Key Facts

- 92 year old female with history of slow-growing mass on bulbar conjunctiva OS
- No similar lesions elsewhere or in other eye
- Possible history of autoimmune disease (arthritis, unknown type)
- Negative history for any hematolymphoid process such as total WBC count elevation, organomegaly, or lymphadenopathy- Extranodal lymphoid cell neoplasm with characteristic immunohistochemical staining

Discussion

Patients with MALTomas present with an indolent, extranodal disease that is usually localized (stage I or II), and a history of infection or autoimmune disease is common. The most common site of involvement is the stomach, but other sites known to be involved are the orbit, lung, skin, salivary gland, breast, and thyroid [1]. Current literature classifies conjunctival MALTomas as ocular adnexal lymphomas of mucosa-associated lymphoid tissue-type (OAMLs).

Ocular adnexal lymphomas represent only 6% of all primary extranodal non-Hodgkin lymphomas, but they characterize 26% of all tumors occurring in the ocular adnexa [2]. Histological diagnosis of ocular lymphoma was made in 24 patients from 1986 to 2003, with only 2 of those cases involving the conjunctiva and the majority involving the orbit. The median age of the patients was 62 years, with a 2:1 female predominance [3].

The patient’s lesion grossly resembled a benign cyst, and a histological evaluation was sought. H&E histology showed monotonous small-sized lymphoid cells infiltrating the conjunctival epithelium and the subjacent stroma (Figure 1). The differential diagnosis at this point was small cell lymphocytic lymphoma/leukemia, follicular lymphoma, mantle cell lymphoma and MALToma.

Figure 1. H&E stain showing monotonous small-sized lymphoid cells infiltrating the conjunctival epithelium (right edge of picture) and the subjacent stroma (bulk of picture).

Flow cytometry performed on peripheral blood was negative for any monoclonal hematolymphoid cell population. This finding, coupled with the absence of lymphadenopathy or organomegaly, helped rule out small cell lymphocytic lymphoma/leukemia.

A panel of immunohistochemical stains was performed on the paraffin-embedded tissue specimen. A stain for cyclin D1 (mantle cell marker) was negative, helping to rule out mantle cell lymphoma. Negative staining was seen for CD10 and Bcl-2 (follicular center cell markers), ruling out follicular lymphoma. A stain for CD20 (B-cell marker) showed strong membrane positivity within the lymphoid cells (Figure 2) confirming lymphoma of B-cell origin with negative staining seen for CD3 and CD5 (T-cell markers). These results and the close association with conjunctival mucosa helped establish the diagnosis of primary conjunctival MALToma.

Figure 2. Immunohistochemical stain for CD20 showing strong membrane positivity within the lymphoid cells.

Complete surgical resection of the patient’s lesion was performed. Adjuvant therapy was not initiated, and a “watchful waiting” strategy was implemented. A retrospective study spanning 30 years and involving 36 patients and 41 eyes provides evidence suggesting that survival is not affected when initial approaches such as local radiotherapy following surgery are instituted, compared to no initial therapy [4].

References

1. Liu NS, Medeiros LJ, McLaughlin P. The Indolent Lymphomas. In MD Anderson Manual of Medical Oncology; Kantarjian HM, Wolff RA, Koller CA, Eds.; The McGraw-Hill Companies, Inc: New York, 2006; Chapter 5.

2. Rosado MF et al. Ocular adnexal lymphoma: a clinicopathologic study of a large cohort of patients with no evidence for an association with Chlamydia psittaci. Blood. 2006 Jan 15; 107(2): 467-472.

3. Nola M et al. Outcome and Prognostic Factors in Ocular Adnexal Lymphoma. Croat Med J. 2004; 45(3): 328-332.

4. Tanimoto K et al. Long-term follow-up results of no initial therapy for
ocular adnexal MALT lymphoma. Ann Oncol. 2006 Jan; 17(1): 135-140.