Orbital plasmablastic lymphoma.

Plasmablastic lymphoma is an unusual and aggressive form of diffuse large B-cell lymphoma, which arises more commonly within the oronasal mucosa. It should be considered as a differential diagnosis for rapidly growing periorbital lesions, particularly in the context of HIV positivity.

Case Presentation

A 45‐year‐old HIV‐positive man presented with a 2‐week history of swelling of the right lower eyelid. Magnetic resonance imaging revealed a plaque of enhancing soft tissue at the inferolateral border of the orbit (A). Incisional biopsy showed skin (B) with normal epidermis and Grenz zone (star), overlying an infiltrate of large atypical lymphoid cells (C) with mitotic figures (arrow). On immunohistochemical staining, lymphocytes were positive for MUM1 (D), CD38 (E), but negative for CD20. Ki67 proliferative index (F) and EBER in situ hybridization for Epstein–Barr virus (G) were strongly positive, signifying plasmablastic lymphoma (Fig. 1).

Figure 1

Magnetic resonance imaging and incisional biopsy histopathology

This neoplasm, a rare and aggressive subtype of DLBCL, has more frequently been described originating from the oral and sinonasal of HIV‐positive males 1, 2, but has also been reported in immunocompetent patients 2, 3, 4. In this case, the primary lesion appears to be within the orbital mucosa. There is no standardized therapy and most published cases included systemic treatment with an individualized intensive chemotherapy regime similar to that of other lymphomas 1, 2. Prognosis is poor with an overall median survival of 8 months 5.

Our patient shows some improvement at 3 months postdiagnosis, having been commenced on cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisolone (CHOP protocol) chemotherapy.

Authorship

EB and FR: contributed to the writing and approval of the final manuscript.