Discussion :
Ocular adnexal lymphoma (OAL) is a rare manifestation of non-Hodgkin’s
lymphoma (NHL). It represents 1%–2% of all NHL and 5%–10% of all
extranodal lymphomas [2]. About 25%–30% of OALs involve the
conjunctiva [2]. Conjunctival lymphomas are most commonly extranodal
marginal zone lymphoma [previously termed mucosal associated lymphoid
tissue (MALT)] (68%), follicular lymphoma (16%), mantle cell
lymphoma (7%), and diffuse large B-cell lymphoma (5%). Whereas the
T-cell and T/natural killer-cell lymphoma type are rare and very
aggressive; this subtype presents only 1% to 3% of all lymphomas in
these sites [3]. Up to 80% of T-cell NHL in the conjunctiva appear
as secondary lymphomas [4].
Few cases of primary orbital peripheral T-cell lymphoma were reported in
literature [5–9] (Table 1). Clinical features of conjunctival
lymphoma are non-specific and the lesions can look nearly identical to
other more benign processes. Biopsies and further studies are
fundamental to make the diagnosis. Our case was an unusual site of T
cell lymphoma; thus, the diagnosis was totally unexpected. As well as
the patient did not have the widespread and systemic disease symptoms
frequently associated withthis type of lymphoma. There by careful
histopathological examinationis very important for prompt confirmation
of the diagnosis and early commencement of treatment. However, patients
should have a complete medical check and an extented evaluation of the
systemic disease. There is no consensus on the treatment of these
lymphomas. The management of conjunctival lymphoma relay on location,
extent of periocular involvement, systemic staging of the disease, and
general health of the patient [10]. The Modalities include complete
surgical resection if possible; external beam radiotherapy and specifc
systemic chemotherapy protocols. We opted for the last strategy in our
case because the data is scarce. He was treated by CHOP chemotherapy
(cyclophosphamide, doxorubicin, vincristine, prednisone). T-cell
lymphoma is considered extremely rare in conjunctiva with poor
prognosis. Approximately, half of patients progress or relapse during
the first year after diagnostic [7]. Our case illustrates the
unfavorable evolution and the aggressiveness of the disease with
meningeal relapse during the treatment. The patient died within 4,3
months of diagnosis.