|Year : 2007 | Volume
| Issue : 2 | Page : 70-72
Primary orbital mantle cell lymphoma
Imtiaz A Chaudhry1, Osama Al-Sheikh1, Hind Al-Katan2
1 Oculoplastic and Orbit Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia
2 Pathology Department, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia
|Date of Web Publication||11-Nov-2009|
Imtiaz A Chaudhry
King Khaled Eye Specialist Hospital, PO Box 7191, Riyadh 11462
Source of Support: None, Conflict of Interest: None
| Abstract|| |
The clinical, imaging, and histopathological features of a periocular region tumor in a middle-aged man are described. A diagnosis of mantle cell lymphoma is made, based on immunohistochemical staining results.
Keywords: orbit, mantle cell lymphoma
|How to cite this article:|
Chaudhry IA, Al-Sheikh O, Al-Katan H. Primary orbital mantle cell lymphoma. Middle East Afr J Ophthalmol 2007;14:70-2
|How to cite this URL:|
Chaudhry IA, Al-Sheikh O, Al-Katan H. Primary orbital mantle cell lymphoma. Middle East Afr J Ophthalmol [serial online] 2007 [cited 2019 Aug 19];14:70-2. Available from: http://www.meajo.org/text.asp?2007/14/2/70/57710
The International Lymphoma Study Group in 1992 coined the term mantle cell lymphoma (MCL) to describe a subtype of B-cell non-Hodgkin lymphoma, which was originally referred to as diffuse germinocytoma.  The tumor is composed of lymphocytes with nuclei that are very irregular in shape and often cleaved with very fine chromatin and very small-to-absent nucleoli with scant and slightly basophilic cytoplasm. It is one of the rare B-cell lymphoma subtypes occurring in the periocular region, with only a few well-recorded cases from the series reported in the ophthalmic literature. ,,,,, For example, from a recent clinical and pathologic study of 69 cases of periocular lymphoproliferative disorders, only 1 case of MCL was recorded.  We describe clinical, imaging, and histopathological features of a periocular region tumor, which was diagnosed as MCL by immunochemical staining, in a middle-aged man.
| Case Report|| |
A 50-year-old Saudi businessman with no past history of trauma or surgery was referred for evaluation of a slowly progressive, painless swelling under his left lower eyelid over a 1-year period. He was a nonsmoker, and his family history was noncontributory.
On examination, the lesion was palpable, nontender, and irregular, and had extended further into the left lower orbit without any obvious proptosis [Figure 1]a. There was no associated lymphadenopathy. His visual acuity was 20/20 in both eyes, and intraocular pressures were normal. There was no relative afferent pupillary defect. Slit-lamp examination of the eyelids, conjunctiva, cornea, and fundus was normal.
A computed tomography (CT) scan of the head and orbits revealed a homogenous mass extending from the anterior of the left lower orbit up to the equator of the eyeball [Figure 1]b,c. The complete blood count (CBC), biochemistry profile, and chest X-ray were normal.
Surgical excision of the lesion was performed through a subciliary incision [Figure 1]d. A combination of blunt and sharp dissections was performed along the orbicularis plane down to the tumor, which had irregular borders that were extensively attached to the surrounding tissues. The tumor was removed as a single piece and was then submitted for pathological analysis.
On gross examination, the tumor was an irregular, tan-colored piece of tissue, measuring 35 x 20 x 12 mm [Figure 2]a. On microscopic examination, the tumor had diffuse atypical lymphocytic proliferation in the form of large nodules that were separated by thin connective tissue septae with significant capillary proliferation [Figure 2]b. Abnormal proliferation showed multiple lymphoid follicles with some germinal centers surrounded by a diffuse proliferation of mature small lymphocytes. Some large cells and cells with plasmacytic differentiation were also observed. The proliferating lymphocytes infiltrated adjacent fat, skeletal muscle, and nerves in all of the sections studied. The infiltration of lymphocytes extended to the lateral and medial ends of the surgical excision. This pattern of atypical lymphoid infiltration was highly suggestive of low-grade extranodal lymphoma. Immunohistochemical stains showed diffuse staining with CD3 [Figure 2]c and CD20 [Figure 2]d. Staining for CD43 was equivocal. There was no staining performed for CD5, CD23, cyclin D1, CD10, and BCL-6. Staining for kappa and lambda light chains was noncontributory. The immunophenotype, combined with the morphology, was found to be most compatible with mantle cell lymphoma.
| Discussion|| |
Ocular adnexal lymphoproliferative disorders can masquerade as a variety of conditions presenting with similar clinical and radiological features. Periocular lymphomas represent the malignant end of the spectrum of lymphoproliferative disorders, which account for less than 5% of cases of systemic non-Hodgkin lymphomas.  According to the Revised European-American Lymphoma (REAL) classification, these are predominantly extranodal marginal zone B-cell lymphomas, but they can also include diffuse large cell B-cell lymphomas and follicular lymphomas; less common B-cell lymphoma subtypes include lymphoplasmocytic lymphoma/immunocytoma, plasmocytoma, immunoblastic lymphoma, and mantle cell lymphoma.  Only a few series have described the clinicopathologic features of MCL in the periocular region. ,,,,, Research has shown that MCL is a disease of the elderly, is associated with a male predominance, has an aggressive clinical course, and tends to be seen initially with widespread advanced disease.  Our case may be considered unusual because the patient had a primary orbital lesion without any associated systemic findings.
Among the standard methods utilized for differentiating lymphoproliferative disorders involving the periocular region include a histopathological examination, flow cytometry, cytogenetics, and immunohistochemical staining. Newer diagnostic techniques, such as the real-time polymerase chain reaction (RT-PCR) and fluorescent in situ hybridization (FISH), have become useful in the differential diagnosis of lymphoproliferative disorders. Rapid diagnosis by genetic analysis utilizing FISH on a touch preparation of the orbital MCL that identifies the classic t(11;14) translocation associated with MCL has been found to be very effective.  In a comparative analysis of FISH, RT-PCR, PCR, and immunohistochemical stains, FISH analysis may be the most sensitive method for the diagnosis of MCL because of its very high specificity and sensitivity. The final diagnosis of orbital MCL may be based on the combination of various diagnostic techniques.
The overall prognosis for patients with MCL in the periocular region has been found to be discouraging compared with other much more common extranodal marginal zone B-cell lymphomas in the same region. 
Unlike our patient with primary orbital MCL, most studies have found that patients with MCL present late in life with late-stage bone marrow involvement and poor response to treatment. , Radiotherapy alone is a highly effective modality in the curative management of primary orbital lymphomas, including MCL.  Current treatment for advanced-stage MCL has been limited to palliative therapy, mainly in the form of chemotherapeutic regimens that may include cyclophosphamide, vincristine, hydroxydaunorubicin, mitoxantrone, or chlorambucil in combination with prednisone.  In recent years, the most promising treatment option has been found to be an allogeneic bone marrow graft. The curative effect of allogeneic bone marrow transplantation relies on a combination of chemoradiation and a graft-versus-leukemia/lymphoma effect. 
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[Figure 1], [Figure 2]