Orbital plasmablastic lymphoma: a clinico-pathological correlation of a rare disease and review of literature

Abstract

Ocular involvement by plasmablastic lymphoma is extremely rare with very few reports in the literature. Its morphological and immunological resemblance to plasma cell myeloma makes it a diagnostic challenge, while its clinical course, which is characterized by recurrence and death, makes therapy a challenge for clinicians. We present three cases of plasmablastic lymphoma, each of which has distinct clinicoradiological features, and we also review the literature on orbital plasmablastic lymphomas.

Orbital plasmablastic lymphoma

“…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.…”

“…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.…”

Radiological-Pathological Correlation in Plasmablastic Lymphoma in an Immunocompromised Patient

“…The most common localizations are extra-nodal sites, usually the oral cavity and the jaw; the ocular involvement is extremely rare. Few cases had been described so far in which there is a primary involvement of the orbit [ 3 – 7 ]. MRI findings of our case correspond to those found on the literature; consisting on an intermediate signal on T1-weighted images and iso-hyperintense signal on T2-weighted images with variable postcontrast enhancement [ 3 – 7 ].…”

“…Plasmablastic lymphoma is frequently resistant to therapy and often rapidly fatal [ 1 , 2 ]. Ocular involvement is extremely rare with very few cases reported in the literature [ 3 – 7 ]. We present a case of orbital plasmablastic lymphoma whose MRI characteristics might be observed in different entities that have to be considered in the differential diagnosis for orbital mass lesions.…”

Extraoral Plasmablastic Lymphoma Detected Using Ascitic Fluid Cytology and Flow Cytometry: A Case Report with a Review of the Literature

“…However, upon review, in fact, we found it to be the most common site for EoPL, with a total of 12 cases being identified from the available literature [6,16,17,20,21,22,23]. The other common sites include the stomach, skin, cervical lymph node and orbit, with a similar incidence [10,11,15,17,24,25,26,27,28,29,30,31,32,33,34]. A few cases of CNS involvement have also been reported [33,35].…”