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Background: Plasmablastic myeloma (PBM) is a rare, aggressive subtype of multiple myeloma (MM) with poor prognosis. On the other hand, plasmablastic lymphoma (PBL) is an aggressive B-cell lymphoma with a plasmacytic phenotype. Importantly, PBM is difficult to distinguish from PBL, because clinical features of both diseases closely overlap. We report two cases of PBM accompanied by apparent extramedullary lesions., Case: Case 1: A 38-year-old female complained of fatigue. She presented with pancytopenia, splenomegaly, a soft tissue lesion over the chest wall, and multiple osteolytic lesions. Initially, pathology of the soft tissue established a diagnosis of PBL. She received two cycles of EPOCH, leading to considerable improvement. She then received daratumumab (Dara) and lenalidomide, achieving remission for two years. Case 2: A 60-year-old male was evaluated for multiple tumors of the pancreas and retroperitoneum. A biopsy of the pancreatic tumor identified plasmacytoid cells, whereas a biopsy of the bone marrow showed no evidence of plasma cells. Therefore, he was initially diagnosed as having multiple plasmacytomas and received 3 cycles of chemotherapy with bortezomib (Bor), lenalidomide, and dexamethasone, but in vain. Once Bor was replaced to Dara, he rapidly developed panperitonitis and ascites filled with plasmablasts and eventually died of multiple organ failure., Conclusion: As there is no standard treatment for PBM, our cases raise a possibility that combination therapy with anti-myeloma and anti-lymphoma regimens may provide better outcomes. In addition, the Ki-67 proliferation index would be a useful tool to diagnose PBM., (© 2025 The Author(s). Cancer Reports published by Wiley Periodicals LLC.) more...

Richter transformation is still a serious risk in the era of innovative therapies, despite the fact that targeted therapy with Bruton's tyrosine kinase inhibitor has significantly improved the prognosis for chronic lymphocytic leukemia (CLL). We report a rare case of a 61-year-old male patient's CLL transforming into a synchronous clonal related plasmablastic lymphoma (PBL) after receiving ibrutinib. During COVID-19, the patient stopped taking ibrutinib, which caused the illness to worsen. Histology revealed that PBL was present in the right supraclavicular mass and that CLL had penetrated the bone marrow. Three cycles of CHP (cyclophosphamide, doxorubicin, and prednisone) were administered together with venetoclax and brentuximab vedotin. After receiving BCMA CAR-T cell treatment, the patient was in complete remission. For PBL transformation, a condition with a worse prognosis and few therapy choices, our results suggest the use of BCMA CAR-T and novel target agents., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.) more...

Disease Overview: Plasmablastic lymphoma (PBL) is a rare CD20-negative aggressive lymphoma with a poor prognosis under standard treatment options. Though PBL is associated with human immunodeficiency virus infection and other immunosuppressed states, it can also affect immunocompetent individuals., Diagnosis: The diagnosis requires a high clinical suspicion and pathological confirmation. EBER expression and MYC gene rearrangements are frequently detected. The differential diagnosis includes EBV+ diffuse large B-cell lymphoma, extracavitary primary effusion lymphoma, ALK+ DLBCL, and HHV8+ large B-cell lymphoma, among others., Risk Stratification: Age ≥60 years, advanced clinical stage, and high intermediate and high International Prognostic Index scores are associated with worse survival., Management: Combination chemotherapy regimens, such as EPOCH, are recommended. The addition of bortezomib, lenalidomide, or daratumumab might improve outcomes. Including PBL patients and their participation in prospective clinical trials is warranted., (© 2024 Wiley Periodicals LLC.) more...

Abstract: Plasmablastic transformation of follicular lymphoma is very rare and has been reported in only 5 cases till date. We report a case of simultaneous identification of extranodal, soft tissue plasmablastic lymphoma in the ankle and bone marrow involvement by follicular lymphoma. This unusual case presentation is a challenge for the treating physician with the patient becoming resistant to chemotherapy and succumbing to the disease within a few months of diagnosis. These cases are known to have an aggressive clinical course with very poor prognosis and survival rate of less than 6 months. This report broadens the spectrum of morphological transformation of follicular lymphoma and it may represent a new category of high-grade transformation of follicular lymphoma., (Copyright © 2023 Copyright: © 2023 Indian Journal of Pathology and Microbiology.) more...

Plasmablastic lymphoma is a rare subtype of large B-cell lymphoma characterised by an aggressive clinical course with frequent relapses and refractoriness to chemotherapy. It is usually associated with HIV, however, it can also be seen in immunocompetent patients. It has distinct pathological characteristics, such as plasmablastic morphology and lack of CD20 expression. These characteristics pose a clinical and pathological challenge. There is no standard of care established in this entity. In this case report, we described a novel bortezomib-based plasma cell targeted regimen in a HIV-negative patient refractory to chemotherapy. more...

Plasmablastic lymphoma (PBL) is an uncommon and aggressive type of mature B cell lymphoma rarely involving gastrointestinal (GI) tract. Here, we describe a case of PBL involving the colon in HIV/Epstein-Barr virus negative immunocompetent patient who presented with anaemia and weight loss but no significant GI symptoms. It emphasises that even in the absence of classical risk factors, one should consider possibility of this condition as this is potentially curable. Also, we would like to highlight the diagnostic and treatment challenges of such an aggressive lymphoma in a frail elderly patient with multiple comorbidities., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.) more...

Purpose: Plasmablastic lymphoma (PBL) is a rare and aggressive B-cell malignancy with a heterogenous clinical and prognostic spectrum, determined by multiple factors, including age, HIV- and MYC-status. While there exist several validated scoring systems for diffuse large B-cell lymphoma, which incorporate basic clinical features (age, lactate dehydrogenase, sites of (extranodal) involvement, stage and performance), none of these have been systematically assessed in PBL., Methods: We determined the (age-adjusted; aa)-International Prognostic Index (IPI), revised IPI (R-IPI), and National Comprehensive Cancer Network IPI (NCCN-IPI) in a comprehensive multi-center cohort (n = 78) of PBL patients. Further, all indices were comparatively investigated for model quality and concordance., Results: Univariate analysis revealed significant prognostic capabilities for all indices, all of which identified a subgroup with favorable outcome. Discriminatory power between patients with less benign prognosis and especially refractory disease exhibited significant variability. Subsequently, stratified models for each risk score were compared employing corrected Akaike's information criterion (cAIC) and Harrel's concordance index (c-index). Here, the NCCN-IPI outperformed both IPI and R-IPI regarding c-index with ambiguous cAIC results, underlining its clinical utility and suggesting it for preferential use in clinical practice., Conclusion: Our current observations support the use of the IPI and its enhanced derivatives in PBL patients. There is, however, a distinct requirement for novel prognostic tools to better delineate subgroups at risk for early relapse or refractory disease as well as late relapse. A comprehensive molecular characterization of a clinically annotated cohort of PBL patients is therefore urgently warranted., (© 2021. The Author(s).) more...

Plasmablastic lymphoma (PBL) is a rare and aggressive hematological malignancy. PBL commonly occurs in immune incompetent patients, such as those with human immunodeficiency virus (HIV), post-transplant status, or immunosenescence. Given its rarity, there is no specific standard treatment for PBL. However, small case series have shown that intensive chemotherapies combined with anti-myeloma agents such as bortezomib and lenalidomide were effective in treating PBL. Unfortunately, some fragile patients could not tolerate intensive chemotherapeutic regimens, especially the elderly patients. Here we presented a 76-year-old female PBL patient refractory to miniCHOP regimen combined with bortezomib but achieved complete remission when treated with tislelizumab combined with lenalidomide, indicating that immune therapy may be a potential treatment for PBL. To our knowledge, this is the first chemoresistant PBL patient that has been successfully treated with checkpoint inhibitor plus lenalidomide, thus providing new insight towards PBL management., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Cheng, Song, Liu, Wang, Yi, Qian, Xu, Cheng, Wang, Wang and Zhao.) more...

Plasmablastic lymphoma (PBL) is a very rare disease and it is usually considered a human immunodeficiency virus (HIV)-related B-cell lymphoma that carries a poor prognosis. It mostly involves the oral cavity, lungs, nasal cavity, gastrointestinal tract, lymph node, and skin. Therapeutic regimens like dose-adjusted etoposide, vincristine, doxorubicin, cyclophosphamide, and prednisone (DA-EPOCH) have shown better results in these aggressive lymphomas. We report a rare case of PBL in an HIV-negative patient who presented to the clinic with a complaint of left testicular swelling for 3 months. Ultrasound showed an enlarged left testicle. He underwent a left orchiectomy and the pathology showed PBL with involvement of the spermatic cord margin. Positron emission tomography scan showed hypermetabolic mediastinal and hilar lymph nodes. He was started on DA-EPOCH but showed no response. Accordingly, salvage therapy with bortezomib in addition to ifosfamide carboplatin and etoposide (B-ICE) chemotherapy was initiated with remarkable response. Several other regimens can be used in the refractory setting; however, the evidence is mostly based on retrospective analysis. more...

Chronic lymphatic leukaemia (CLL) is the most common leukaemia in the Western world. Ibrutinib, a tyrosine kinase inhibitor, is the treatment of choice on relapse or p53-dysfunction. Richter's transformation to diffuse large B cell lymphoma is most often seen. However, transformation to other aggressive lymphomas as plasmablastic lymphoma (PBL) does occur. PBL is an extremely aggressive lymphoma and is usually treated using a CHOP-like regimen (cyclophosphamide, doxorubicin, vincristine and prednisone/dexamethasone), but with poor outcome. The only curative treatment is allogeneic stem cell transplant (ASCT).We report on a case of CLL treated with ibrutinib that underwent transformation to PBL. Due to high expression of CD138, we added daratumumab to the chemotherapy with a good, but transitory response. The case did not make it to an ASCT. Targeting CD138 by daratumumab may be added to chemoimmune therapy for PBL., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.) more...

Introduction: Plasmablastic lymphoma (PBL) is an uncommon and aggressive large B-cell lymphoma commonly diagnosed in human immunodeficiency viruses -positive patients. Oral cavity is the most commonly PBL affected site. Most oral PBLs presented as asymptomatic swellings, frequently associated with ulcerations and bleeding. Most cases lacked B-symptoms, suggesting a more local involvement of the disease. No standard treatment is yet for oral PBL. Five-year survival rate recorded no more than 33.5%., Patient Concerns: A 39-year-old male presented to Dental Clinic with 1 month swelling of the oral cavity, in absence of any other symptoms or signs. He followed antibiotic therapy just on suspicion of an oral abscess and later oral surgical treatment on suspicion of bone neoplasm., Diagnosis: Surgical specimen analysis highlighted a diffuse infiltrate of large-sized atypical cells with plasmablastic appearance and plasma cell phenotype. Oral cavity PBL was diagnosed. Blood tests recorded mild lymphopenia and positive human immunodeficiency viruses serology., Interventions: Patient underwent chemotherapy including intrathecal methotrexate prophylaxis, in addition to a highly active antiretroviral therapy., Outcomes: At 12 months from diagnosis, patient recorded complete hematological remission., Conclusions: Oral PBL diagnosis requires a high level of suspicion and awareness both by physicians and pathologists. They should be aware of the extent of such disease which is often mistaken as oral abscess or infected tooth, thus leading to delay the most appropriate diagnostic evaluation. As PBL is an aggressive non-Hodgkin lymphoma, a delayed diagnosis might negatively impact on both treatment and survival. more...

Incorporating bortezomib and/or lenalidomide in the management of plasmablastic lymphoma is an attractive option due to the reported high response rates. However, concerns about overlapping toxicities can deter clinicians from incorporating these novel agents into chemotherapy. In this case report we describe a patient with plasmablastic lymphoma, who received both lenalidomide and bortezomib as part of upfront treatment for a high-risk plasmablastic lymphoma. After completing intensive chemotherapy, the patient was transitioned to a regimen of daily lenalidomide and biweekly bortezomib to decrease the chance of relapse. This maintenance phase was given for 6 months and was well tolerated. Despite having multiple adverse risk factors, the patient remains in remission, 18 months following diagnosis., (Copyright© 2019, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.) more...

Plasmablastic lymphoma (PBL) is a rare subtype of diffuse large B-cell lymphoma, highly associated with HIV and Epstein-Barr virus (EBV) infections. It commonly presents in extranodal sites, often an oral mass, but reports of primary central nervous system PBL (PCNSPBL) are exceedingly rare. Here, we report on a 33-year-old man with newly diagnosed HIV infection who presented with acute-onset unilateral visual disturbance and was found to have biopsy-proven PCNSPBL. The neoplastic cells displayed a plasmacytoid appearance, with the expression of CD38 and CD138, and were positive for EBV by in situ hybridisation for EBV-encoded RNA. Systemic workup revealed the presence of Kaposi sarcoma, but no evidence of lymphoma. He is currently being treated with high-dose methotrexate, as well as antiretroviral therapy for his HIV infection, and has achieved a complete response., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.) more...

Plasmablastic lymphoma (PBL) is an aggressive form of non-Hodgkin's lymphoma (NHL) classically seen in patients infected with the human immunodeficiency virus, but can also be seen in other immunocompromised states such as transplant recipients, autoimmune diseases and the elderly. PBL is generally associated with a poor prognosis despite chemotherapy. There is evidence supporting the use of bortezomib in combination with standard chemotherapy to achieve durable responses in patients with PBL. We describe a patient with acquired immunodeficiency syndrome who presented with rectal pain and bright red blood per rectum. He was diagnosed with stage IVA PBL with anorectal, nodal, calvarial and hepatic involvement. Along with highly active antiretroviral therapy, he was treated with six cycles of dose adjusted etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin (DA-EPOCH) plus bortezomib resulting in durable complete remission 30 months after diagnosis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.) more...

Plasmablastic lymphoma (PBL) is a rare distinct subtype of aggressive diffuse large B-cell lymphoma and a notoriously hard to treat entity with a dismal prognosis in both HIV-negative and HIV-positive patients. Clinicians often face the question of second or third line treatment. As the treatment options with novel agents in lymphomas are rapidly evolving, more and more options beyond standard chemotherapy are available. In connection with a review of treatment options with novel lymphoma agents, we present a case report of a patient with a complete remission after the administration of brentuximab vedotin and lenalidomide. more...

Background: Plasmoblastic lymphoma is a rare and aggressive subtype of diffuse large B cell lymphoma, which occurs usually in the jaw of immunocompromised subjects., Case Presentation: We describe the occurrence of plasmoblastic lymphoma in the mediastinum and chest wall skin of an human immunodeficiency virus-negative 63-year-old Caucasian man who had had polycytemia vera 7 years before. At admission, the patient showed a superior vena cava syndrome, with persistent dyspnoea, cough, and distension of the jugular veins. Imaging findings showed a 9.7 × 8 × 5.7 cm mediastinal mass. A chest wall neoformation biopsy and ultrasound-guided fine-needle aspiration biopsy of the mediastinal mass allowed diagnosis of plasmoblastic lymphoma and establishment of an immediate chemotherapeutic regimen, with rapid remission of compression symptoms., Conclusions: Plasmoblastic lymphoma is a very uncommon, difficult to diagnose, and aggressive disease. The presented case represents the first rare mediastinal plasmoblastic lymphoma in a human immunodeficiency virus-/human herpesvirus-8-negative patient. Pathologists should be aware that this tumor does appear in sites other than the oral cavity. Fine-needle aspiration biopsy is a low-cost, repeatable, easy-to-perform technique, with a high diagnostic accuracy and with very low complication and mortality rates. Fine-needle aspiration biopsy could represent the right alternative to surgery in those patients affected by plasmoblastic lymphoma, being rapid and minimally invasive. It allowed establishment of prompt medical treatment with subsequent considerable reduction of the neoplastic tissue and resolution of the mediastinal syndrome. more...

Plasmablastic B-cell malignancies include plasmablastic lymphoma and subsets of multiple myeloma and diffuse large B-cell lymphomaDLBCL. These diseases can be difficult to diagnose and treat, and they lack well-characterized cell line models. Here, immunophenotyping and FOXP1 expression profiling identified plasmablastic characteristics in DLBCL cell lines HLY-1 and SU-DHL-9, associated with CTNNAL1, HPGD, RORA, IGF1, and/or vitamin D receptor (VDR) transcription. We demonstrated VDR protein expression in primary plasmablastic tumor cells and confirmed in cell lines expression of both VDR and the metabolic enzyme CYP27B1, which catalyzes active vitamin D3 production. Although Vdr and Cyp27b1 transcription in normal B cells were activated by interleukin 4 (IL-4) and CD40 signaling, respectively, unstimulated malignant plasmablastic cells lacking IL-4 expressed both VDR and CYP27B1. Positive autoregulation evidenced intact VDR function in all plasmablastic lines, and inhibition of growth by active vitamin D3 was both dependent on MYC protein inhibition and could be enhanced by cotreatment with a synthetic ROR ligand SR-1078. Furthermore, a VDR polymorphism, FOK1, was associated with greater vitamin D3-dependent growth inhibition. In summary, HLY-1 provides an important model of strongly plasmablastic lymphoma, and disruption of VDR pathway activity may be of therapeutic benefit in both plasmablastic lymphoma and myeloma., (Copyright © 2017 by the Endocrine Society.) more...

Rationale: Plasmablastic lymphoma (PBL) is a rare subtype of human immunodeficiency virus (HIV)-related non-Hodgkin's lymphoma that predominantly manifests in the oral cavity., Patient Concerns: Three cases of HIV-negative PBL were reported., Diagnoses: HIV-negative PBL INTERVENTIONS:: The patient had undergone chemotherapy., Outcomes: Clinical outcomes were very poor in Cases 1 and 3; Case 2, whose diagnosis suggested no bone marrow involvement, is still alive., Lessons Subsections: These cases served to broaden the reported clinical spectrum of HIV-negative PBL. Clinicians and pathologists need to be familiar with lymphoma in the identified extra-oral PBL variation and there levant differential diagnosis procedures for this particular disease. more...

Introduction: Plasmablastic lymphoma (PBL) is a rare and aggressive type of mature B-cell lymphoma, which is usually associated with HIV infection. The most common site of PBL is the oral cavity. Involvement of the gastrointestinal (GI) tract is rare, and literature is limited to few case reports and case series., Aims: To retrospectively analyze the presentation, clinical findings, and outcome of patients presenting to our institute with a diagnosis of PBL involving the GI tract., Materials and Methods: A retrospective observational study was conducted at our institute from February 2008 to January 2015 on consecutive patients presenting with PBL involving the GI tract. The data were compared to various case reports and series published in peer-reviewed journals., Results: There were four patients diagnosed with PBL of the GI tract; three male and one female. The location of involvement was in the stomach, ileocecal junction, ascending colon, and rectum. Only one patient was HIV-positive and was on combination antiretroviral therapy since 2 years. Among the three immunocompetent patients, only one survived with therapy; however, the patient relapsed within 6 months of completion of treatment., Conclusion: PBL was seen to have a uniformly aggressive clinical course with poor outcomes even with optimal treatment. The prognosis of immunocompetent patients appears to be worse than that of HIV-AIDS patients. Although the most common histologies seen with GI lymphomas are mucosa-associated lymphoid tissue type lymphomas or diffuse large B-cell lymphoma, rarer and more aggressive histologies like PBL need to be kept in mind. more...

Objective: To evaluate the clinical features and outcomes of HIV-negtive plasmablastic lymphoma (PBL). Methods: Nine patients with HIV-negtive PBL were diagnosed and treated between January 2006 and January 2016. The clinical and follow-up data were analyzed retrospectively. Results: The median age was 56 years (range 30-77 years) with a male-to-female ratio of 2∶1. Nobody had underlying diseases associated with immunosuppression. Primary extra nodal diseases were observed in 7 cases and only 1 patient had oral involvement. Two patients were in earlystage and 7 in advanced stage by the Ann Arbor stage system. Ki-67 index was 60%-90% in the 9 cases, and 80% or higher in 7 cases. Epstein-Barr virus-encoded RNA expression (EBER) was detected in 4 cases, and 2 of them were positive. Chemotherapy was documented in 9 patients, from which 8 received the cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP)-like regimens as the first-line chemotherapy and responses were observed in 5 (1 complete, 4 partial responses). Three elderly patients were treated with CHOP combined with thalidomide, and 2 of them achieved partial responses. One patient, failed three chemotherapy regimens, accepted thalidomide combined with etopside and achieved stable disease for 10 months. One patient with early stage had disease-free survival of 61.9 months after treatment. The other eight patients experienced recurrence or progression after the first-line chemotherapy, and 6 of them died of disease progression within 2 years after the diagnosis. Conclusion: The HIV-negative PBL patients in this study did not have an apparent association with immunosuppression. Primary extra nodal diseases were common, but only 1 patient had oral involvement. Most patients had advanced stage and poor prognosis. Effectiveness of thalidomide in the treatment of PBL deserves further investigation. more...

Plasmablastic lymphoma (PBL) represents a rare type of non-Hodgkin lymphoma associated with human immunodeficiency virus (HIV) infection. The impact of highly active antiretroviral therapy (HAART) in this tumor is poorly known due to its small incidence. This study reports a case of a 33-year-old HIV-positive woman who was referred to the Stomatology Department complaining about a painful gingival growth and cervical nodule both with 20 days of evolution. The lesions appeared 7 months after the patient stopped HAART. The final diagnosis was PBL. After resuming HAART for 45 days, the gingival lesion presented complete remission. The patient continued with HAART alongside chemotherapy. At 24 months follow-up, the patient was stable. The dental surgeon plays an essential role in orientation and retention in care of HIV patients once the adherence of HAART seems to play an important role in PBL development and response to treatment. more...

Background: It has been known that plasmablastic lymphoma (PBL) is a neoplasm of immunocompromised patients occurring in soft tissue of oral cavity or in the vicinity whereas bone is an unlikely site to harbor PBL. However, its occurrence is increasingly being reported in immunocompetent individuals in either osseous or extra-oral sites. To our best knowledge, F-18 FDG PET/CT findings of PBL involving bones in an immunocompetent patient have not been reported, yet ., Case Summary: We report a case of PBL involving multiple bones in an immunocompetent patient. Features of different imaging modalities including F-18 Fluoro-deoxy glucose (FDG) positron emission tomography/computed tomography (PET/CT) were correlated well as findings of osteosarcoma in mandible with metastatic lesions. However, the histopathology and immunohistochemistry (IHC) of bone tissues from 2 separate biopsy sites revealed features of PBL., Conclusion: awareness to F-18 FDG PET/CT findings of PBL involving bones in an immunocompetent patient may prevent misdiagnosis. more...

Plasmablastic lymphoma is a very rare B-cell lymphoma typically associated with immunosuppression: It occurs primarily in the oral cavity, although some cases were reported in other organs and tissues.To date, only 10 cases of primary cutaneous plasmablastic lymphoma have been described. Clinically, primary cutaneous plasmablastic lymphoma presents as non-specific cutaneous lesions (purple nodules, erythematous infiltrated plaques). In previously described cases, as in this case, histology and immunohistochemistry are required to make the diagnosis. Owing to the rarity of this entity, there is no established therapy, which makes its management an individualized, patient-based decision. more...

Background: Plasmablastic lymphoma (PBL) is a rare subtype of non-Hodgkin's lymphoma. Characterized by its aggressive nature and plasmacytic differentiation, PBL remains a therapeutic and diagnostic challenge; it generally has a poor prognosis with very few long-term survivors and most patients dying within 2 years from initial presentation. PBL has been reported in several other countries; however, there have been no reported cases from Saudi Arabia. Here, we report 8 cases of PBL depicting the clinical presentation, immunocompetency, immunphenotypic characterization, diagnostic challenges and treatment outcome., Methods: The medical records were reviewed for clinical presentation, staging, laboratory data, radiological studies, treatments, and outcomes. A broad immunohistochemical panel consisting of CD45, CD3, CD20, CD79a, Pax5, CD38, CD138, MUM1, EMA, Kappa, Lambda, CD 56, CD30, Bcl-2, Bcl-6, Alk-1, Ki-67, EBV-LMP-1, and HHV8 was performed., Results: The tumors predominantly exhibited immunoblastic/plasmablastic or plasmacytic morphologic features and had a plasma cell-like immunophenotype. All cases were immunoreactive for CD38, CD138 and MUM1 confirming plasma cell differentiation of the tumor cells. CD20 was negative for all cases; whereas CD79a and Pax5 were weakly positive in 2cases. All 8 cases were EBV-LMP-1/EBER-1 negative, and 1 case was HHV8 positive. Similar to previously published studies, PBL in Saudi Arabia is characterized by male predominance (6/8), median age 51.5 years (mean age 46 years), associated with early dissemination, poor response to therapy, and limited survival (average survival time, 6.4 months, median overall survival 5.5 months). However, it does have some unique features. It occurs more commonly in immunocompetent persons (6/8, 75%), is not associated with EBV infection (0/8), and nodal involvement (either primary or secondary) is common among patients (6/8). In addition, extra-oral sites are more common than oral/nasal cavities (7/8) and the c-myc gene is not common (1/8, 12.5%)., Conclusion: It appears that PBL is heterogeneous in terms of clinical presentation and morphology. PBL is a therapeutic challenge with a clinical course that is characterized by its high rate of relapse and death. To date, treatment responses are usually partial and temporary. Therapies that are more intensive than CHOP do not seem to prolong survival. Further research is needed to understand the biology and molecular pathogenesis of PBL in order to improve therapies., Virtual Slides: The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1465801416161912. more...