Plasmablastic lymphoma of nose and para-nasal sinuses in an immunocompetent patient
DOI:
https://doi.org/10.18203/issn.2454-5929.ijohns20211589Keywords:
Plasmablastic lymphoma, Sino-nasal, Neoplasm, Immuno-competent, Chemotherapy, RadiotherapyAbstract
Plasmablastic lymphoma (PbL) is a rare and aggressive neoplasm with heterogeneous clinical, histological and genetic features. It has been reported in both immuno-compromised and immuno-competent patients and commonly presents in the extra-nodal regions, but it is more heterogeneous in immuno-competent patients. Its clinical course is aggressive with early dissemination and poor response to therapy. A 61 year old male presented with left sided nasal bleeding and nasal obstruction for last 6 months, snoring and disturbed sleep for last 2 months. On examination, there was fullness over left side of nose and obliteration of left naso-facial groove. A mass lesion was seen in the left nasal cavity on endoscopy. There was bulge in the soft palate and the mass extended into the oropharynx. CECT showed a locally aggressive sino-nasal mass. Histopathology and immuno-histo-chemistry (IHC) of the biopsy were suggestive of plasmablastic lymphoma. A debulking surgery was done to relieve his obstructive symptoms. The patient received 3 cycles of EPOCH chemotherapy and radiotherapy to local site. At 9 months of follow up he presented with recurrence of the mass. Plasmablastic lymphoma presents in patients other than those with HIV. Awareness about its unique characteristics is crucial for establishing a correct diagnosis. Currently available treatments are ineffective in achieving long term remission and prognosis remains unfavourable with high incidence of recurrence
Metrics
References
Delecluse HJ, Anagnostopoulos I, Dallenbach F, Hummel M, Marafioti T, Schneider U, et al. Plasmablastic lymphomas of the oral cavity: a new entity associated with the human immunodeficiency virus infection. Blood. 1997;89(4):1413-20.
Morscio J, Dierickx D, Nijs J, Verhoef G, Bittoun E, Vanoeteren X, et al. Clinicopathologic comparison of plasmablastic lymphoma in HIV-positive, immunocompetent, and posttransplant patients: single center series of 25 cases and meta-analysis of 277 reported cases. Am J Surg Pathol. 2014;38(7):875-86.
Chicuellar NR, Sufyan W, Mahendran S. Unilateral maxillary sinus plasmablastic lymphoma in an immunocompetent patient. an unusual occurrence report and literature review. Ear Nose Throat J. 2020:145561320963715.
Lopez A, Abrisqueta P. Plasmablastic lymphoma: current perspectives. Blood Lymphat Cancer. 2018;8:63-70.
Castillo JJ, Bibas M, Miranda RN. The biology and treatment of plasmablastic lymphoma. Blood. 2015;125(15):2323-30.
Tchernonog E, Faurie P, Coppo P, Monjanel H, Bonnet A, Génin MA, et al. Clinical characteristics and prognostic factors of plasmablastic lymphoma patients: analysis of 135 patients from the LYSA group. Ann Oncol. 2017;28(4):843-8.
Qunaj L, Castillo JJ, Olszewski AJ. Survival of patients with CD20-negative variants of large B-cell lymphoma: an analysis of the national cancer data base. Leuk Lymphoma. 2018;59(6):1-9.
Habesoglu TE, Habesoglu M, Surmeli M, Uresin T, Egeli E. Unilateral sinonasal symptoms. J Craniofac Surg. 2010;21(6):2019-22.
Laurent C, Gravelle P, Péricart S, Fabiani B, Coppo P, Brousset P, et al. EBV infection promotes tumor infiltrating leucocyte and immune escape in plasmablastic lymphoma according to gene expression profiling. Hematol Oncol. 2017;35:297-8.
Shakri NM, Husain S, Zahedi FD, Tan GC. Nasal plasmablastic lymphoma in an HIV-negative immunocompetent patient. Medeni Med J. 2020;35(1):71.
Chapman J, Gentles AJ, Sujoy V, Vega F, Dumur CI, Blevins TL, et al. Gene expression analysis of plasmablastic lymphoma identifies downregulation of B-cell receptor signaling and additional unique transcriptional programs. Leukemia. 2015;29(11):2270-3.
Phipps C, Yeoh KW, Lee YS, Nagarajan C, Gopalakrishnan S, Ho LP, et al. Durable remission is achievable with localized treatment and reduction of immunosuppression in limited stage EBV-related plasmablastic lymphoma. Ann Hematol. 2017;96(11):1959-60.
Castillo JJ, Reagan JL. Plasmablastic lymphoma: a systematic review. Sci World J. 2011;11:687-96.
Guerrero-Garcia TA, Mogollon RJ, Castillo JJ. Bortezomib in plasmablastic lymphoma: a glimpse of hope for a hard-to-treat disease. Leuk Res. 2017;62:12-6.
