If you don't remember your password, you can reset it by entering your email address and clicking the Reset Password button. You will then receive an email that contains a secure link for resetting your password
If the address matches a valid account an email will be sent to __email__ with instructions for resetting your password
The authors present a rare case of conjunctival MCL in a female patient. Collection and evaluation of protected patient health information complied with the Health Insurance Portability and Accountability Act.
Case Report
A 61-year-old female presented with a painless, salmon-colored conjunctival lesion approximately 8 × 5 mm in size in the medial canthal region of the right eye (Fig. 1). The mass had been present for 2 weeks, and at her follow-up visit 1 month later it appeared to have grown. She had no visual symptoms or complaints in the right eye, although she had been experiencing flashes and floaters in the left eye, which was found to be a posterior vitreous detachment. Ophthalmic examination was otherwise within normal limits. No palpable or visible posterior extension could be appreciated. Patient denied B-symptoms such as fever, night sweats, or lymphadenopathy. She had no history of malignancy.
Fig. 1Right eye in abduction showing a salmon-colored conjunctival lesion near the medial canthus, approximately 8 × 5 mm in size.
There was high clinical suspicion of lymphoma, and the patient subsequently underwent biopsy of the right conjunctival lesion. Histopathology showed soft tissue infiltration of fairly uniform atypical lymphocytes with slightly irregular nuclear membranes and focally vesicular chromatin without prominent nucleoli. Occasional mitoses were identified. Immunohistochemical staining showed CD20+, CD5+, Bcl-2+, CyclinD1+, CD10−, CD23−, and 50% Ki-67 proliferation consistent with MCL. Computed tomography (CT) of orbits showed no other ocular involvement. Staging positron emission tomography (PET) and CT showed multiple small, mildly hypermetabolic axillary, mediastinal, hilar, retroperitoneal, mesenteric, and external iliac lymph nodes as well as splenomegaly. Bone marrow biopsy showed 10% MCL involvement.
She subsequently started rituximab, bendamustine, and cytarabine for stage IV MCL but was switched to rituximab and bendamustine after severe cytopenias after the second cycle. PET/CT showed complete remission with resolution of lymphadenopathy and splenomegaly after the third cycle. Remission was maintained after completion of 6 cycles. She did not require maintenance therapy. Marrow biopsy after completion of chemotherapy was normocellular. She subsequently developed rituximab-induced delayed neutropenia, which resolved with filgrastim. As of 10 months after completion of chemotherapy, she continues to be in remission.
Discussion
MCL is a rare type of B-cell NHL that accounts for approximately 3%–5% of conjunctival lymphomas.
It is a high-grade, aggressive lymphoma that typically presents in the eighth decade. Males are 6 times more likely to be diagnosed with ocular adnexal MCL than females.
At the genetic level, MCL is characterized by the t(11;14)(q13;32) translocation, which causes overexpression of cyclin D-1 and subsequent cellular proliferation and dysregulation.
The mainstay of treatment for MCL is chemotherapy, usually involving R-CHOP (rituximab, cyclophosphamide, hydroxydaunorubicin, vincristine, prednisone), although a combination of rituximab, bendamustine, and cytarabine is a valid alternative for older patients.
Combination of rituximab, bendamustine, and cytarabine for patients with mantle-cell non-Hodgkin lymphoma ineligible for intensive regimens or autologous transplantation.
J Clin Oncol Off J Am Soc Clin Oncol.2013; 31: 1442-1449
However, a different study showed that patients who receive rituximab-containing regimens have a 3-year survival rate of 57% compared to 40% for patients who received regimens without rituximab.
Although conjunctival MCL overwhelmingly present in male patients, female sex does not necessarily preclude MCL from the differential diagnosis of conjunctival lymphomas.
Disclosure
The authors have no proprietary or commercial interest in any materials discussed in this article.
References
Coupland S.E.
Hellmich M.
Auw-Haedrich C.
Lee W.R.
Stein H.
Prognostic value of cell-cycle markers in ocular adnexal lymphoma: an assessment of 230 cases.
Graefes Arch Clin Exp Ophthalmol Albrecht Von Graefes Arch Klin Exp Ophthalmol.2004; 242: 130-145
Combination of rituximab, bendamustine, and cytarabine for patients with mantle-cell non-Hodgkin lymphoma ineligible for intensive regimens or autologous transplantation.
J Clin Oncol Off J Am Soc Clin Oncol.2013; 31: 1442-1449
30732-9&id=gr1.jpg){kind=link}