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Editor,—In spite of its rare occurrence there is a well established association between the acquired immune deficiency syndrome (AIDS) and lymphoma, which most of the time is a highly malignant B cell type affecting the central nervous system (CNS).1
Rare cases of lymphomas involving paranasal sinus, orbit, and intraocular structures have been described. This report describes a case of primary lymphoma of the maxillary sinus with orbital and intraocular secondary involvement.
A 28 year old man with AIDS presented with right proptosis and recurrent sinusitis. His CD4+ count was 3 cells ×109/l and viral load 1 343 145 copies. Skull and orbital computed tomograph (CT) scans demonstrated right maxillary sinus opacification, mild right proptosis, and an anterior superotemporal mass in the right orbit. Cerebral image was unremarkable. The ocular examination disclosed right eye proptosis, ptosis, and a visible mass on the temporal area of superior right lid, restricting spontaneous lid opening (Fig 1). There was bilateral lid oedema but no mass could be found on the left side. Right eye movements were restricted. Pupillary reflexes were normal in both eyes, and visual acuity reached 6/12 right eye and 6/6 left eye. Fundus examination revealed a left choroidal lesion measuring approximately one disc diameter superior to the disc (Fig 2). Lid infiltration prevented right fundus examination, but a B-scan revealed a serous retinal detachment and an intraocular mass.
Since a lymphoma was highly suspected specific treatment was started, and 5 days after the first chemotherapy, a significant reduction in the size of the mass was noted. Examination of the right fundus disclosed a large temporal scar and surprisingly, the left choroidal lesion had completely disappeared.
One month after the last chemotherapy, a drastic recurrence of the right mass occurred, causing total ophthalmoplegia, paralytic mydriasis, and amaurosis. An elevated lesion was found on the bulbar conjunctiva from which a tissue specimen was easily obtained. Left visual acuity had also decreased to 6/60. At this moment, an extensive yellowish temporal choroidal lesion was found in the right fundus. Left fundus examination disclosed a serous retinal detachment involving the superior retina along the supratemporal arcade. CT scan showed that the right orbital mass expanded towards the apex, with cavernous sinus involvement. It also detected a left retro-ocular mass associated with a posterior scleral thickening, as well as multiple cerebral lesions. The patient's condition rapidly deteriorated and he died a few days later. Postmortem examination was not performed.
The specimen removed from the right bulbar conjunctiva revealed a large cell, poorly differentiated neoplasm. Neoplastic cells with plasmocytoid differentiation were found in a scattered distribution. Immunohistochemical studies were done using the indirect imunoperoxidase method with diaminobenzidine (DAB), and the antigen retrieval method using heat or protease. The following markers were used: CD3, CD20, CD30, Kappa, Lambda, Vimentin, Citoceratina pan, HMB45, Protrína S-100. The tumour cells showed nucleolar immunopositivity for anti-CD20 antibody.
Classically, the AIDS associated lymphomas are of the B cell type and involve the CNS and the abdominal cavity, as opposed to the preferential ganglion involvement seen in immune competent patients.1-3 Such cases usually develop at advanced disease stage. Primary maxillary sinus lymphoma is rare, as is secondary orbital involvement.4 Malignant paranasal sinus tumour secondarily involves the orbit in 45% of the cases, but lymphomas are uncommon.5 Primary and secondary orbital lymphomas are rare, and bilateral occurrence is very unlikely.6 Rare cases of intraocular primary lymphomas were also previously described.78 Our patient had bilateral involvement of the sinuses, orbit, and intraocular structures, which was confirmed by CT scan, and although we didn't obtain a specimen from the choroidal lesions for histological confirmation, the disappearance of the left lesion and the scarring on the right fundus just after treatment supports lymphoma as the diagnosis for the intraocular lesion. The immunohistochemistry was CD20 (L26) positive, and negative for all the other markers (CD3, CD30, Lambda, Kappa, S-100 protein, HMB45), confirming the B cell origin.
Non-Hodgkin's lymphoma in HIV infected patients tends to be of high grade malignancy, being very aggressive most of the times, with a mean survival of around 8 months.49 Our patient died after 3 months in spite of initial good response to chemotherapy.
Lymphoma treatment is still controversial. Radiotherapy has demonstrated good results for treatment of both systemic disease and in cases of orbital and intraocular involvement. On the other hand, chemotherapy has been good for systemic disease but has not been successfully used for intraocular disease.10Interestingly, our patient had a significant improvement after chemotherapy, but that was shortly followed by an intense reactivation, which was probably due to the high grade of malignancy.
Isolated or associated paranasal sinus, orbital, and intraocular lymphomas are considered to be very rare tumours, and even though their incidence has decreased in the HAART era, clinicians should be aware of this potential manifestation of non-Hodgkin's lymphoma.