Primary splenic lymphoma is a rare lymphoproliferative disorder that involves the spleen, exhibits diverse clinical presentations, and lacks a clear consensus in terms of management strategies.

We present the case of a 52-year-old patient with a complex medical history marked by multiple chronic medical conditions. The patient was diagnosed with primary splenic lymphoma, specifically the diffuse large B-cell subtype. Treatment for our patient involved a shortened course of chemotherapy (4 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone [R-CHOP] followed by two doses of rituximab) due to issues related to compliance and treatment-related complications. This was followed by consolidative radiotherapy without resorting to splenectomy.

Remarkably, despite using a shortened course of R-CHOP, the patient achieved complete resolution, and a positron emission tomography scan conducted at the end of the 6-month posttreatment period confirmed sustained complete remission.

Primary splenic lymphoma (PSL) is characterized by lymphoma involvement confined to the spleen and hilar lymph nodes, without evidence of liver involvement or other sites. This condition is extremely uncommon, accounting for approximately 1 % of non- Hodgkin lymphomas (NHLs) and <2 % of all lymphomas. Diffuse large B-cell lymphoma (DLBCL) is the most common histological subtype of both PSLs and all NHLs. DLBCL encompasses an aggressive heterogeneous entity with distinct morphological variants.

A 68 year-old gentleman presented to the office with a 10-month history of vague left sided upper abdominal pain. Clinical examination revealed a tender left upper quadrant, evidenced with splenomegaly on radiological evaluation. The patient proceeded with a splenectomy with subsequent pathological and immunohistochemical analysis, confirming a final diagnosis of germinal center type DLBCL.

Primary splenic DLBCL is a rare variant of DLBCL, characterized by exclusive involvement of the spleen. It requires a comprehensive diagnostic evaluation to exclude lymphoma involvement in other organs and lymph nodes. Splenectomy followed by appropriate adjuvant therapy has been demonstrated as the definitive treatment strategy. This case report emphasizes the importance of considering primary splenic DLBCL as a differential diagnosis in patients presenting with splenomegaly and highlights the significance of multidisciplinary collaboration for accurate diagnosis and optimal management of this uncommon entity.

Primary Splenic DLBCL, an exceptionally rare B-Cell neoplasm variant, requires precise diagnosis due to its unique splenic involvement. Splenectomy's efficacy, adjuvant therapy, multidisciplinary collaboration, and ongoing research are crucial for optimal management.

Primary splenic lymphomas are rare with the majority of lymphomas in spleen being secondary to an extra-splenic lymphoma. We aimed to analyze the epidemiological profile of the splenic lymphoma and review the literature. This was a retrospective study including all splenectomies and splenic biopsies from 2015 to September 2021. All the cases were retrieved from Department of Pathology. Detailed histopathological, clinical and demographic evaluation was done. All the lymphomas were classified according to WHO 2016 classification. A total of 714 splenectomies were performed for a variety of benign causes, as part of tumor resections and for the diagnosis of lymphoma. Few core biopsies were also included. A total of 33 lymphomas diagnosed in the spleen, primary splenic lymphomas constituted 84.84% (n = 28) of the cohort with 5 (15.15%) having the primary site elsewhere. The primary splenic lymphomas constituted 0.28% of all the lymphomas arising at various sites. Adult population (19-65 years) formed the bulk (78.78%) with a slight male preponderance. Splenic marginal zone lymphomas (n = 15, 45.45%) comprised of major proportion of cases followed by primary splenic diffuse large B-cell lymphoma (n = 4, 12.12%). Splenectomy was the main course of treatment for SMZL with a good overall outcome, with chemotherapy ± radiotherapy forming the mainstay in other lymphomas. Lymphomas in spleen can be infiltrative or a primary, hence proper clinic-radiological and pathological evaluation is required. Appropriate management is guided by the precise and detailed evaluation by the pathologist, requiring understanding of the same.

Primary splenic diffuse large B cell lymphoma (DLBCL) is one of the most common primary tumors in the spleen, while its prevalence is relatively low. Recently, there has been an increase in the incidence rate of primary splenic DLBCL; however, the effectiveness of various treatments for it has not been adequately described previously. The purpose of this study was to compare the effectiveness of various treatments on survival time in primary splenic DLBCL. A total of 347 patients with primary splenic DLBCL were enrolled in The Surveillance, Epidemiology, and End Results (SEER) database. These patients were subsequently divided into four subgroups according to the treatment modalities: non-treatment group (patients who had not received chemotherapy, radiotherapy, or splenectomy, n=19), splenectomy group (patients who had received splenectomy only, n=71), chemotherapy group (patients who had received chemotherapy only, n=95), and the splenectomy combined with chemotherapy group (patients who had received splenectomy and chemotherapy, n=162). The overall survival (OS) and cancer specific survival (CSS) of four treatment groups were evaluated. Compared to the splenectomy group and the non-treatment group, the OS and CSS of the splenectomy combined with chemotherapy group was extremely significantly prolonged (P<0.01). Compared with the chemotherapy group, the OS and CSS of the splenectomy combined with chemotherapy group were longer, but there was no statistical difference (P>0.05). The Cox regression analysis showed that the treatment modality was identified as an independent prognostic factor for primary splenic DLBCL. The landmark analysis shows that the overall cumulative mortality risk was significantly lower in the splenectomy combined with chemotherapy group than in the chemotherapy group within 30 months (P<0.05), and the cancer-specific mortality risk was significantly lower in the splenectomy combined with chemotherapy group than in the chemotherapy group within 19 months (P<0.05). Splenectomy combined with chemotherapy may be the most effective treatment modality for primary splenic DLBCL.

Although primary splenic lymphoma (PSL) is rare, it ranks first among splenic primary malignant cancers, and the incidence of lymphoma of spleen has gradually increased in recent years. However, the efficacy of surgery for PSL has not been clinically verified by large sample data, which has affected the formulation of relevant guidelines.

To assess whether surgery can enhance the prognosis PSL patients.

Extracted the data of patients with PSL from The Surveillance, Epidemiology, and End Results (SEER) database, and divided the patients into surgery and non-surgery group. Kaplan-Meier curves and log-rank tests were used to compare the overall survival (OS) and cancer-specific survival (CSS). The propensity score matching (PSM) was used to match the data, then compared the OS and CSS again. The COX proportional hazard regression model was used for univariate and multivariate analysis. Finally, we performed subgroup analysis in different Ahmann stages.

A sum of 2207 patients with PSL were enrolled, of which 1062 (48.1%) patients received surgery, and 1145 (51.9%) patients did not undergo surgery. Overall, patients in the surgery group had better OS and CSS. After the propensity scores matching, surgery was not statistically significant in OS and CSS. In the subgroup analysis, surgery was a protective factor for the OS and CSS in Ahmann I/II. However, surgery was no statistical significance in OS and CSS in Ahmann III. In patients with Ahmann Ⅰ/Ⅱ SMZL, surgery was a protective factor for OS and CSS. In patients with Ahmann Ⅲ SMZL, surgery was also statistically significant of OS and CSS.

Surgery can significantly improve the prognosis of patients with Ahmann Ⅰ/Ⅱ primary splenic lymphoma, but there was no survival difference in the Ahmann Ⅲ patients with or without surgery. For patients with SMZL, surgery was effective for improving OS and CSS.

B-cell lymphoma of spleen may be primary (most commonly splenic diffuse large B-cell lymphoma) or secondary (typically low-grade non-Hodgkin lymphoma). Depending on the specific lymphoma subtype, there may be a predominantly white pulp pattern of involvement, a predominantly red pulp pattern or a focal nodular pattern. Splenectomy is the ideal specimen for a multiparametric integrative diagnosis of splenic lymphoma, as it allows for a combined study of morphology, immunohistology, flow cytometry, cytogenetics, and molecular genetic techniques. This review article describes the clinicopathologic characteristics of all the relevant B-cell neoplasms that may be encountered in a splenic biopsy or a splenectomy specimen.

Splenic infiltration is often seen in diffuse large B-cell lymphoma (DLBCL). However, primary splenic DLBCL is rare and studies on its clinicopathological features are limited. We assessed 66 cases of primary splenic DLBCL and 309 control DLBCL, not otherwise specified. Hepatitis C virus antibody prevalence, B symptoms, poor performance status and CD5 positivity differed significantly between the primary splenic DLBCL and control DLBCL groups. Primary splenic DLBCL cases were classified histopathologically into two groups [white pulp pattern (n = 46), red pulp pattern (n = 20)]. Survival analysis showed no difference in overall survival between the primary splenic DLBCL and the control group, but the former had a more favourable progression-free survival. In the examination of primary splenic DLBCL, the white pulp pattern was statistically associated with a lower performance status (2-4), and a lower CD5 positivity than the red pulp pattern. In the survival analysis, the red pulp pattern demonstrated poorer overall survival. Multivariate analysis of overall survival in primary splenic DLBCL cases identified CD5 positivity as an indicator of poor prognosis. Classifying primary splenic DLBCL into white and red pulp patterns was useful in terms of clinicopathological features and overall survival.

Fever is a common clinical presentation of a number of diseases. A sustained unexplained fever >38.3°C lasting for >3 weeks without an established diagnosis despite intensive diagnostic evaluation is referred to as fever of unknown origin (FUO). FUO remains a clinical challenge for physicians, as it may be attributed to a wide range of disorders, mainly infections, malignancies, non-infectious inflammatory diseases and miscellaneous diseases. We herein report the case of a 59-year-old male patient who presented with prolonged unexplained fever and was found to have a diffusely enlarged hypermetabolic spleen, as shown on ¹⁸F-fluorodeoxyglucose positron emission tomography/computed tomography examination. Following splenectomy, histopathological examination revealed primary splenic lymphoma (PSL) of B-cell origin. The patient received 6 courses of systemic chemotherapy with rituximab, etoposide, cyclophosphamide, doxorubicin, vincristine and prednisone (R-ECHOP regimen) and responded well to treatment. Thus, in patients with FUO and splenomegaly, the possibility of PSL should be taken into consideration.

Primary splenic diffuse large B-cell lymphoma (PS-DLBCL), an uncommon type of non-Hodgkin lymphoma, has been investigated only in small patient series before the rituximab era. The therapeutic role of splenectomy in addition to immunochemotherapy is unknown.

The databases of 7 medical centers in Israel were searched for patients diagnosed with PS-DLBCL in 1982-2013, and clinical, treatment, and outcome data were collected for 87 patients. The mean patient age was 59.6 years; 57.5% were male.

Patients presented with abdominal pain (81%), B symptoms (59%), splenomegaly (84%), splenic masses (97%), and high lactate dehydrogenase (LDH) levels (84%); 61% had stage I or II disease. The diagnosis was made with core-needle biopsy in 46 patients and with diagnostic splenectomy in 39 patients. Eighty patients (92%) were treated with cyclophosphamide, doxorubicin, vincristine, and prednisone; 68 (78%) received rituximab. A complete response was achieved in 67 patients (77%), and a partial response was achieved in 8 (9%). At 5 years, the overall survival (OS) rate was 77%, and the progression-free survival (PFS) rate was 67%. When patients were stratified by splenectomy at diagnosis, the OS rates were 91% for splenectomized patients and 68% for nonsplenectomized patients (P = .08), and the PFS rates were 85% and 55%, respectively (P = .02). The respective values for the subgroup with early-stage disease were 96% and 63% for OS (P = .009) and 90% and 51% for PFS (P = .01). In a multivariate analysis, a low Eastern Cooperative Oncology Group performance status and splenectomy independently predicted better PFS (P < .03).

Patients with PS-DLBCL usually present with abdominal pain, high LDH levels, and a splenic mass. This study shows for the first time that splenectomy at diagnosis improves survival, specifically in patients with early-stage disease.

Paraneoplastic dermatosis is defined as both benign skin lesions and internal malignancy existing at the same time with parallel clinical courses. Herein, we report a 91-year-old male who presented as pityriasis lichenoids chronica (PLC) concomitantly with a primary splenic diffuse large B cell lymphoma. Surgical removal of the spleen cleared his skin lesions dramatically. However, seven months later, the splenic lymphoma relapsed in concordance with the recurrence of the skin lesions of PLC. To our knowledge, he is the first case that PLC is the leading presentation and paraneoplastic manifestation of primary splenic large B-cell lymphoma.

Primary splenic lymphoma (PSL) is a rare disease, and its management differs from other splenic malignancies. The purpose of the present study was to evaluate the role of 18F-FDG PET/CT in patients with PSL.

We retrospectively evaluated the data of 17 patients with PSL (median age, 46 years; range, 3-64) who had undergone 50 18F-FDG PET/CT studies. Of the 50 PET/CTs, 15 were done for primary diagnosis/staging and 35 were done for restaging. PET/CT images were evaluated both qualitatively and quantitatively [maximum standardized uptake value (SUVmax)]. Combination of clinical/imaging follow-up and/or histopathologic diagnosis was taken as reference standard.

Of the total 50 F-FDG PET/CT studies, 27 studies (54%) were positive and 23 (46%) were negative for disease. PET/CT was true positive in 25 studies, true negative in 22 studies, false positive in 2 studies, and false negative in 1 study. Overall, per study-based sensitivity was 96.2%, specificity was 91.7%, and accuracy was 94%. In those studies performed for diagnosis/staging (15/50), PET/CT was true positive in all 15, with an accuracy of 100%. In those studies performed for restaging (35/50), sensitivity was 90.9%, specificity was 91.7%, and accuracy was 91.4%. No significant difference was seen in the accuracy of PET/CT between staging and restaging groups (P=0.601). On 18F-FDG PET/CT, splenic involvement was seen in 24 studies, lymph nodal involvement in 5 studies, liver involvement in 3 studies, and bone marrow involvement in 1 study. More than one site of disease was seen in 9 PET/CT studies. On semiquantitative analysis, the overall mean splenic lesion SUVmax was 6.9±7.9 (median, 4.6; range, 1.3-29). The mean value of ratio of SUVmax of splenic lesions and SUVliver was 4.4±5.5 (median, 2.4; range, 0.9-24.2). On comparison of areas under the curve, the mean value of ratio of SUVmax of splenic lesions and SUVliver was found to be superior to SUVmax alone for this differentiation of involvement from normal spleen (P=0.018).

18F-FDG PET/CT seems useful in patients with primary splenic lymphoma and shows high diagnostic accuracy.

A 80-year-old woman was hospitalized due to anemia and fever. Computed tomography depicted a solitary concentric, sharply marginated lesion of the spleen. Laparotomy identified a creamy-white soft tumor. Histopathological examination confirmed the diagnosis of non-Hodgkin B-cells anaplastic lymphoma. Extended range of pathological manifestations explains the variety of radiological appearances and difficulty in accurate diagnostic process of primary splenic lymphoma.

Primary Hepatic (PHL) and Primary Splenic (PSL) non-Hodgkin's Lymphoma are rare entities. Small series of PHL and PSL have been reported, suggesting a non-fortuitous association with Hepatitis C Virus (HCV) infection. The prognosis is believed to be dismal, with early recurrence and short survival.

We retrospectively reviewed all PHL and PSL patients diagnosed at our institution between 1990 and 2005.

Twenty-five adult patients were identified, six with PHL and 19 with PSL. Twenty-four patients had a B-cell lymphoma, defined as Diffuse Large B-cell lymphoma in 18. The prevalence of HCV infection was 68% among PSL and 66% among PHL. Combination chemotherapy was the mainstay of treatment for PHL and PSL; all but one patient with PSL underwent splenectomy before chemotherapy. Complete remission was achieved in all the cases after frontline therapy; only four patients relapsed but responded to additional chemotherapy courses. Most patients presented with aggressive histological subtypes; 92% were alive at a median follow up of 79 months. HCV infection did not appear to influence the results of therapy.

Our study confirms the rarity of PHL and PSL, shows a high prevalence of HCV infection, and demonstrates that the outcome of patients with PHL and PSL may be favourable.

Diffuse large B-cell lymphoma is the most common form of lymphoma. It usually begins in the lymph nodes; up to 40% may have an extranodal presentation. According to a definition of primary extranodal lymphoma with presentation only in extranodal sites, there are reports of large B-cell lymphomas limited to liver or spleen as separate entities, and to date there have been only three documented cases of primary hepatosplenic presentation. This paper reports a fourth case. Due to a review of the literature and the clinical course of the case reported, we conclude that primary hepatosplenic large B-cell lymphoma has been found predominantly in females older than 60 years. The patients reported had <2 months of evolution prior to diagnosis, prominent B symptoms, splenomegaly in three and hepatomegaly in two, none with lymph node involvement. All had thrombocytopenia and abnormal liver function tests; three had anemia and elevated serum lactic dehydrogenase levels, two with hemophagocytosis in bone marrow. Because of the previously mentioned data, it can be stated that primary hepatosplenic lymphoma is an uncommon and aggressive form of disease that requires immediate recognition and treatment.

Primary splenic lymphoma (PSL) is rare with a reported incidence of less than 1%. Diffuse large cell pathology has been reported in 22-23% of the cases and is felt to have poor outcome. This study reports a 50 year old male who presented with fever and weakness. He was found to have a mass lesion in the spleen documented by CT scan. A splenectomy was performed which showed non-Hodgkin's lymphoma. Immunohistological studies showed a positivity for CD20 and CD30.