Prognostic value of tumour-related factors associated with canine retroperitoneal hemangiosarcoma in comparison with other anatomic presentations: A retrospective observational study.

BACKGROUND: Dogs with retroperitoneal hemangiosarcoma (HSA) exhibit variable postoperative median survival times (MST). OBJECTIVE: To retrospectively evaluate the prognostic value of selected tumour-related factors, such as tumour size, rupture, invasion into adjacent tissue, involvement of lymph node and distant metastasis, they were analysed in dogs with retroperitoneal HSA. METHODS: Ten dogs with retroperitoneal HSA managed solely with surgical excision were reviewed and compared with spleen (71) and liver (9) HSA. The Kaplan-Meier method and log-rank analysis were used compare MSTs between factors. Multivariable Cox proportional-hazard analysis was used to compare differences between arising sites. RESULTS: Retroperitoneal HSA showed comparatively longer postoperative MST compared with that of spleen and liver HSA and demonstrated significantly longer MST (p = 0.003) for tumours ≥5 cm (195 days) than <5 cm (70 days). Spleen HSA revealed significantly shorter MSTs in involvement of distant lymph nodes (23 days) and distant metastasis (39 days) than those in negative (83 days, p = 0.002 and 110 days, p < 0.001, respectively). Liver HSA also revealed significantly shorter MST (16.5 days compared with 98 days, p = 0.003) for distant metastasis. Additionally, hazard ratios (HRs) and their forest plot for overall HSA revealed as poor prognostic factors, arising sites (spleen; HR 2.78, p = 0.016 and liver; HR 3.62, p = 0.019), involvement of distant lymph nodes (HR 2.43, p = 0.014), and distant metastasis (HR 2.86, p < 0.001), and as better prognostic factor of tumour size ≥5 cm (HR 0.53, p = 0.037). CONCLUSION: In combination with overall HSA, retroperitoneal HSA shows comparatively longer postoperative MST compared to spleen and liver HSA, associated with tumour size ≥5 cm suggesting better prognostic factor.

Ethnicity and socio-economic status affects the incidence and survival of hepatosplenic T-cell lymphoma.

To address the lack of contemporary population-based epidemiological studies of hepatosplenic T-cell lymphoma (HSTCL), we undertook a population-based study of ICD-O-3-coded HSTCL in England. We used the National Cancer Registration Dataset and linked datasets on hospital admissions, Systemic Anti-Cancer Therapy, socio-demographics, comorbidities and death, identifying cases from 1 January 2013 to 31 December 2019 with survival data up to 5 January 2021. Crude and directly age-standardised incidence rates per million persons per year were calculated. Crude and adjusted incidence rate ratios compared incidence between groups using Poisson regression. A Cox proportional hazards model estimated mortality risks adjusted for age, sex, ethnicity, deprivation and allogenic stem cell transplant (allo-SCT; time varying). We identified 44 patients, mean age 42 years. Median survival was 11 months, and 1 and 5 year survivals were 48% (95% CI 29%-43%) and 22% (95% CI 12%-42%) respectively. The age-standardised incidence was 0.1 per million/year. Incidence was higher in areas with greater deprivation (0.15 per million/year), and more cases than expected were in non-White patients (39%). Non-Whites had a twofold increased risk of death (adjusted hazard ratio 2.21 [95% CI 1.03-4.78]) even after adjusting for deprivation, younger age and allo-SCT. In conclusion, ethnicity and socio-economic status affect both the incidence and survival of HSTCL.

Characteristics and survival outcomes of primary splenic cancers: A SEER population-based study.

ABSTRACT: Primary splenic cancers represent a small number of cancer cases and studies on its clinicopathological features and outcomes are limited. Splenic lymphomas and primary splenic angiosarcoma (PSA) are the 2 most common histological types of splenic cancers. This population-based study aimed to investigate the clinical characteristics and survival outcomes of patients with splenic lymphomas or PSA.Patients diagnosed with splenic lymphomas or PSA between 2000 and 2015 were identified from the Surveillance Epidemiology and End Results database of the National Cancer Institutes. Overall survival (OS) and cancer-specific survival (CSS) rates were calculated using the Kaplan-Meier method. A Cox proportional hazard models were used to identify independent predictors of cancer-specific mortality.A total of 700 patients with splenic lymphoma and 48 patients with PSA were included in this study. The median age of patients with splenic lymphoma was 65 years and 57 years for patients with PSA. For patients with splenic lymphoma, the most prevalent histological subtypes were splenic marginal zone lymphoma and diffuse large B-cell lymphoma. A total of 52.6% of the cases had stage IV disease based on the Ann Arbor staging system. Five-year OS and CSS were 76.9% and 83.4%, respectively. Multivariate analysis revealed that independent predictors of splenic lymphoma CSS included race, stage, chemotherapy, and histological subtype. However, a much shorter OS time was seen in the PSA cohort which had a 5-year OS of 11.8%, a median OS of 10.0 months and the 5-year CSS of 12.4%. Chemotherapy was correlated with better outcomes in patients with PSA. However, the survival benefits of surgery for splenic cancer were not statistically significant in our study.The current study is the largest cohort of primary splenic cancer presented in literature based on the Surveillance Epidemiology and End Results database and our large series describe the characteristics and survival outcomes of such rare diseases which may provide reliable information for further studies and clinicians.

The effect of surgery on primary splenic lymphoma: A study based on SEER database.

BACKGROUND: 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. AIM: To assess whether surgery can enhance the prognosis PSL patients. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Outcomes of Hepatosplenic T-Cell Lymphoma: The Mayo Clinic Experience.

BACKGROUND: Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma accounting for less than 1% of non-Hodgkin lymphomas. It is generally associated with poor prognosis. PATIENTS AND METHODS: We performed a cohort study of patients with HSTCL treated at the Mayo Clinic between 1996 and 2020 exploring the clinical characteristics and therapeutic outcomes. RESULTS: Twenty-two cases of HSTCL were identified with a median (range) age at diagnosis of 45.5 (15.5-80.6) years and a male predominance (15/22, 68.2%). Clinical characteristics include massive splenomegaly in 16 patients (73%), hepatic involvement in 13 (59%), and chronic immunosuppressed state in 8 (36%). Phenotypically, lymphoma cells had gamma/delta T-cell receptor expression in 18 (82%) and alpha/beta in 4 patients. Cytogenetic abnormalities included isochromosome 7q (i7q) in 8 (62%) of 13 and trisomy 8 in 4 (44%) of 9. The median (range) follow-up of surviving patients was 33 (2.5-137) months. The median progression-free and overall survival were 9.5 months (95% CI, 1.8, 16.3) and 12.4 months (95% CI, 4.9, 18.5), respectively. Long-term survival was seen in 4 (18%) of 22 patients, with survival of 55, 74, 95, and 137 months. Moreover, 3 of 4 long-term survivors had splenectomy as part of initial treatment, and 2 of 4 long-term survivors received an allogeneic hematopoietic cell transplant (allo-HCT). CONCLUSION: Liver involvement and chronic immunosuppression were associated with shorter survival. Although splenectomy and allo-HCT have anecdotal benefit in the literature, our data do not show a statistically significant benefit of splenectomy and/or allo-HCT, likely as a result of our small sample size.

Diagnostic and therapeutic splenectomy for splenic lymphomas: analysis of the National Cancer Data Base.

OBJECTIVES: To examine the use of splenectomy, chemotherapy, and subsequent overall survival (OS) in contemporary patients with splenic lymphomas. METHODS: We analyzed records of 6450 patients with various splenic lymphomas recorded in the National Cancer Data Base (2004-2013). Survival was compared using Mantel-Byer test to account for guarantee-time bias, stratified by age, sex, comorbidities, and lymphoma stage. RESULTS: Splenectomy rate was overall 58%, and varied from 49% in splenic marginal zone (SMZL) to 77% in follicular lymphoma (FL). It significantly decreased across all histologies over time (overall from 69% in 2004, to 44% in 2013). Thirty-day mortality after splenectomy was 4%. Chemotherapy use varied from 40% in FL to 76% in diffuse large B-cell lymphoma (DLBCL), but increased significantly only for SMZL and T-cell lymphomas over time. Overall, 57% of splenectomies were performed as diagnostic procedures, which was significantly less common in academic hospitals (p < 0.0001). Following a diagnostic splenectomy, chemotherapy was not administered to 29% of patients with DLBCL, 49% with mantle cell, and 42% with T-cell lymphomas. Median OS ranged from 12.4 years for FL to 1.0 year for T-cell lymphomas. We found no association between performance of splenectomy and OS across all histologies. Patients with DLBCL who did not receive chemotherapy after a diagnostic splenectomy had significantly worse OS (p = 0.001). The association between post-splenectomy chemotherapy and OS was not observed in FL or SMZL. CONCLUSION: many splenic lymphomas may be treated without surgery, but a high proportion of diagnostic splenectomies indicates an ongoing need for less invasive diagnostic modalities.

Clinical Characteristics and Prognostic Factors of Primary Splenic Angiosarcoma: A Retrospective Clinical Analysis from China.

BACKGROUND/AIMS: Primary splenic angiosarcoma is an aggressive malignancy originating from endothelial cells with a particularly poor outcome despite radical therapy. Owing to its extremely low incidence, available data for splenic angiosarcoma are limited. The present study aimed to address this limitation by presenting a thorough retrospective analysis of Chinese primary splenic angiosarcoma patients over a 53-year period (1963-2016). METHODS: To determine the characteristics of Chinese primary splenic angiosarcoma and identify factors that impact the outcomes of this histology, we retrospectively retrieved reports of 110 Chinese primary splenic angiosarcoma cases published between 1963-2012. RESULTS: In total, 61 males and 49 females diagnosed with primary splenic angiosarcoma were included in the present study. The median age at diagnosis was 50 years (range 2.5-76 years). Of these patients, 25.5% had received prior radiotherapy. The rate of splenic rupture was 59.11%. The 1-year overall survival rate was 19.1% with a median overall survival time of 8.1 months. Age, gender, and radiation history showed no correlation with survival rate. However, by univariate analysis, we found that significant adverse predictors of survival were splenic rupture before surgery and large tumor size (> 5 cm), while adjuvant chemotherapy was a favorable predictor. Furthermore, multivariate analysis revealed that splenic rupture and adjuvant chemotherapy were independent adverse and favorable predictors, respectively. CONCLUSION: Our large series describes and confirms the characteristics and poor prognosis of Chinese primary splenic angiosarcoma, thus indicating a critical role for early diagnosis and surgical intervention (prior to rupture) in management, and highlights the promising potential of adjuvant chemotherapy for improving the outcome in these cases.

Clinicopathological features of splenic tumours of lymphoid tissue.

BACKGROUND: To study the effects of splenectomy on treatment and diagnosis of tumours of lymphoid tissue of the spleen. METHODS: Fifty-three cases were reviewed from Peking University People's Hospital from 2002 to 2017. According to WHO classification of tumours of haematopoietic and lymphoid tissues (2008) and classification updated (2016), the cases were studied by microscopy, immunohistochemistry and in situ hybridization, combined with the bone marrow biopsy and clinical examination. RESULTS: In 53 cases, the male to female ratio was 3.4:1, the mean age was 55.4 years old, the median survival time was 17.0 months, and all patients present with variable degree of splenomegaly. The elevated percentage of lymphocyte in peripheral blood can be seen in 22 cases, and elevated LDH level in 24 cases. Abnormal blood counts can be seen in 26 cases before operation, and 22 cases remission to normal level partly or completely after operation. The clinical symptoms included abdominal pain or distension, fatigue, fever, and weight loss, etc. Seventeen cases present with lymphoadenopathy of abdomen or other sites. Fourteen cases were stage I or II, whereas 6 were stage III, 28 were stage IV. Forty-three cases were splenic B-cell marginal zone lymphoma (SMZL)(48.8%,21/43), DLBCL(23.3%,10/43), splenic diffuse red pulp small B-cell lymphoma (SDRPSBL)(11.6%,5/43), mantle cell lymphoma (MCL)(9.3%,4/43), follicular lymphoma (FL)(4.7%,2/43), composite lymphoma (CL, DLBCL and classical Hodgkin lymphoma)(2.3%,1/43) in turn, and the remaining 10 cases were chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL/SLL) (n = 4), hairy cell leukaemia (HCL) (n = 1), hepatosplenic T-cell lymphoma (HSTL) (n = 5). The survival period of SMZL and DLBCL was 25.7, 18.6 months, respectively. Thirteen cases were dead (27.1%, 13/48). The chemotherapy protocol included Hyper-CVAD A/B with/without R (Rituximab), COP, CHOP with/without R etc. The prognosis of those with elevated LDH level, high clinical staging, B symptom, and older than 60 year old was obviously worse, and the prognosis of DLBCL was worse than that of SMZL. CONCLUSIONS: Most splenic lymphoid tumors present with splenomegaly and abnormal blood counts, and complete or part remission of blood counts can be seen after splenectomy, and splenectomy is also helpful for pathological diagnosis. The most common pathological types are SMZL and DLBCL. The definite diagnosis can be made by combining with clinical features, histopathology, immunophenotype, genetics, bone marrow biopsy and laboratory examination.

Should rituximab replace splenectomy in the management of splenic marginal zone lymphoma?

BACKGROUND: SMZL is a relatively rare low grade B-cell lymphoma, characterized usually by an indolent clinical behavior. Since there is no prospective randomized trials to establish the best treatment approach, decision on therapeutic management should be based on the available retrospective series. Based on these data, rituximab and splenectomy appear to be the most effective. Splenectomy represented the standard treatment modality until early 2000s. More than 90% of the patients present quick amelioration of splenomegaly related symptoms along with improvement of cytopenias related to hypersplenism. The median progression free survival was 8.25 years in the largest series of patients published so far, while the median 5- and 10- year OS were 84% and 67%, respectively. Responses to splenectomy are not complete since extrasplenic disease persists. Patients with heavy bone marrow infiltration, lymphadenopathy or other disease localization besides the spleen are not good candidates for splenectomy. Furthermore splenectomy is a major surgical procedure accompanied by acute perioperative complications as well as late toxicities mainly due to infections. For that reasons splenectomy is not appropriate for elderly patients or patients with comorbidities with a high surgical risk. On the other hand rituximab monotherapy displays high efficacy with minimal toxicity. Several published series have shown an ORR more than 90%, with high CR rates (∼50%). The 10-year PFS and OS were 63% and 85%, respectively in a series of 104 SMZL patients. The role of rituximab maintenance has been investigated by only one group. Based on these data, maintenance with rituximab further improved the quality of responses by increasing significantly the CR rates (from 42% at the end of induction to 71% at the end of maintenance treatment), as well as the duration of responses: 7-year PFS was 75% for those patients who received maintenance vs 39% for those who did not (p < 0.0004). However no difference in OS has been noticed between the two groups, so far. Summarizing the above data, it is obvious that Rituximab monotherapy is associated with high response rates, long response duration and favorable safety profile, rendering it as the treatment of choice in SMZL.

Does thalidomide prolong survival in dogs with splenic haemangiosarcoma?

OBJECTIVES: To investigate thalidomide as an adjuvant treatment for canine haemangiosarcoma. MATERIALS AND METHODS: Fifteen dogs with splenic haemangiosarcoma, initially treated by splenectomy, were included. Following recovery from surgery, all dogs received thalidomide continuously until their death. Tumour stage was established using CT scans of the chest and abdomen immediately before starting thalidomide treatment and again three months later. Cause of death was confirmed by post mortem examination. RESULTS: The median survival time of dogs receiving thalidomide was 172 days (95% confidence interval: 93 to 250 days). Five dogs (33% of the population receiving thalidomide) survived more than 1 year (range 458 to 660 days) after surgery. Dogs with stage 2 disease that received thalidomide also had a longer survival time than dogs with stage 3 disease (median survival time 303 versus 40 days). Of 15 dogs, 13 died from metastatic haemangiosarcoma. CLINICAL SIGNIFICANCE: Treatment using thalidomide may improve survival of dogs with splenic haemangiosarcoma and should be considered a possible adjuvant therapy.