Primary lymphoblastic B-cell lymphoma of the stomach:A case report

Primary stomach lymphoblastic B-cell lymphoma (B-LBL) is a rare tumor. We describe a primary stomach B-LBL in a 38 years old female who presented with nonspecific complaints of fatigue and vomiting for 2 mo. Gastrofiberscopy revealed a large gastric ulcer, which was successfully resected. Pathology showed a lymphoblastic cell lymphoma arising from the stomach, and there was no evidence of disease at any extrastomach site. Immunohistochemical staining and gene rearrangement studies supported that the stomach tumor was a clonal B-cell lymphoma. Therefore, the diagnosis of B-LBL was made based on the stomach specimen.

Autologous hematopoietic stem cell transplantation in chemotherapy-sensitive lymphoblastic lymphoma: treatment outcome and prognostic factor analysis

Objective： The study evaluated the effectiveness of autologous hematopoietic stem cell transplantation （AHSCT） in the treatment of lymphoblastic lymphoma （LL）. Methods： We relxospectively analyzed the data from 41 patients with chemotherapy-sensitive LL who underwent hematopoietic stem cell transplantation （HSCT） from December 1989 to December 2009 in a single institution. Results： HSCT was conducted as first-line consolidation therapy and salvage therapy in 36 and 5 patients, respectively. The median follow-up was 97.1 months （range, 24.6-173.1 months）. The 5-year overall survival （OS） and event-free survival （EFS） rate were 64% and 47% for the initially treated patients, respectively, and were both 20% for the relapsed ones. Bone marrow （BM） involvement and chemotherapy cycles prior to transplantation were identified as significant prognostic factors for EFS in multivariate analysis. Conclusions These results confirm that AHSCT is a reasonable option for chemotherapy-sensitive LL patients in first complete remission （CR1）.

PEG-asparaginase in BFM-90 regimen improves outcomes in adults with newly diagnosed lymphoblastic lymphoma

Objective： Although L-asparaginase（L-ASP） is a standard treatment for lymphoblastic lymphoma（LBL）,hypersensitivity reactions by some patients limit its application. Polyethylene glycol-conjugated asparaginase（PEGASP） has a lower immunogenicity and is a standard treatment in all pediatric acute lymphoblastic leukemia（ALL）.In this study, we investigated the efficacy and toxicity of PEG-ASP instead of L-ASP as used in the BFM-90regimen（PEG-ASP-BFM-90） for adult LBL.Methods： Between June 2012 and July 2015, we treated 30 adult patients with newly diagnosed LBL, using PEGASP-BFM-90 in a prospective, multicenter and single-arm clinical study at 5 participating institutions in China.Results： All the 30 patients, including 19 males and 11 females with a median age of 30（range： 18–62） years,completed 128 times of the PEG-ASP, with the median of 4（range： 2–6） times. Patients did not receive radiotherapy at this time. The overall response rate was 86.7%（26/30）, with 50.0%（15/30） complete response and36.7%（11/30） partial response. The 3-year overall survival was 46.0% [95% confidence interval（95% CI）,28.2%–64.8%], and the 3-year progression-free survival was 43.0%（95% CI, 25.7%–62.0%）. Major adverse events were myelosuppression, reduced fibrinogen, liver dysfunction and digestive tract toxicities. No allergic reaction and no treatment-related mortality or severe complications were recorded.Conclusions： Our clinical data and observed outcomes indicate that 1 dose of PEG-ASP can replace multiple doses of native L-ASP in BFM-90, with predominantly grade 3–4 neutropenia for adult LBL, and no therapyrelated deaths. The effect is similar to previous reports of PEG-ASP-containing regimens for adult ALL. Major advantages include less serious allergic reactions, 2–3 weeks of action duration, and convenience for patients and physicians.

T-lymphoblastic lymphoma with cutaneous involvement

To study dermatological manifestation of T-lymphoblastic lymphoma and to help clinicians in the diagnosis, we report here the case of a 75-year-old patient who presented with violaceous nodules acquired during the last 4 wk and affecting the scalp and right arm. The diagnosis of systemic lymphoma was suggested upon the appearance of cutaneous tumors, palpable lymph nodes and general symptoms including asthenia and weight-loss. The pathology features: positive immunostaining for CD3 and terminal deoxynucleotidyl transferase(Td T) and staging, led us to the final diagnosis of T-lymphoblastic lymphoma(T-LBL) with cutaneous involvement. He received a CHOP regimen as first-line treatment. Unfortunately, the patient relapsed and died 8 mo after the treatment initiation. T-LBL may be diagnosed by skin lesions. Additional immunostaining including Td T and experienced histopathologists are needed to correctly classify this aggressive disease and discuss the correct management including bone-marrow transplantation where appropriate.

Post-Therapy Profile of BMI-for-Age of Indian Survivors of Pediatric Acute Lymphoblastic Leukemia and Non-Hodgkin’s Lymphoma

Background: Obesity in pediatric ALL survivors is a well recognized late effect. Hence the present study examines the BMI-for-age of Indian childhood ALL and NHL survivors. Method: A retrospective study of 118 ALL/NHL survivors and 138 age sex matched was carried out. From the recorded heights and weights were body mass index (BMI) was computed. The survivor data was compared with 138 controls from the data set collected by investigators previously. Results: 82.8% of patients had BMI-for-age in 5th-84th percentile (healthy) at time of diagnosis and at inclusion in the study. Comparison of BMI of survivors with matched controls was not significant. However, The mean BMI-for-age for younger patients (3 to 12 years) was significantly higher than mean BMI-for-age of matched controls. Distribution of data by time elapsed from therapy was significant. Overweight/obesity was observed among the survivors who were off therapy for two years with increase in after four years post-therapy. Conclusion: Our preliminary study indicates late effects of therapy and points to the need of long term assessment of the survivors, even though majority of them were within the normal weight range.

Graft vs host disease impacts overall survival post allogeneic hematopoietic stem cell transplantation for acute lymphoblastic leukemia/lymphoma

AIM To examine the outcome and prognostic factors for high risk patients with acute lymphoblastic leukemia/lymphoma(ALL/LBL) who underwent allogeneic hematopoietic stem cell transplantation(HCT) at our center during the period of2010-2017 METHODS After due institutional review board approval, patients with high risk ALL/LBL post HCT were identified and included. All records were retrospectively collected. Time to event analysis was calculated from the date of HCT until event of interest or last follow up with Kaplan-Meir means. Cox regression model was used for multivariable analysis calculation.RESULTS A total of 69 patients were enrolled and examined with a median age of 21(14-61). After a median follow up of 15 mo(2-87.3), the 2-year cumulative incidence of relapse, cumulative incidence of non-relapse mortality, progression free survival and overall survival(OS) were 34.1%, 10.9%, 54.9% and 62.8%,respectively. In a multivariable analysis for OS; acute graft vs host disease(GVHD) and chronic GVHD were significant with corresponding hazard ratio 4.9(1.99-12; P = 0.0007) and 0.29(0.1-0.67; P = 0.0044), respectively.CONCLUSION Allogeneic-HCT for high risk ALL/LBL resulted in promising remissions particularly for patients with cGVHD.

Genomic landscape of T-cell lymphoblastic lymphoma

Objective: T-cell lymphoblastic lymphoma(T-LBL) is an aggressive neoplasm of precursor T cells, however,detailed genome-wide sequencing of large T-LBL cohorts has not been performed due to its rarity. The purpose of this study was to identify putative driver genes in T-LBL.Methods: To gain insight into the genetic mechanisms of T-LBL development, we performed whole-exome sequencing on 41 paired tumor-normal DNA samples from patients with T-LBL.Results: We identified 32 putative driver genes using whole-exome sequencing in 41 T-LBL cases, many of which have not previously been described in T-LBL, such as Janus kinase 3(JAK3), Janus kinase 1(JAK1), Runtrelated transcription factor 1(RUNX1) and Wilms’ tumor suppressor gene 1(WT1). When comparing the genetic alterations of T-LBL to T-cell acute lymphoblastic leukemia(T-ALL), we found that JAK-STAT and RAS pathway mutations were predominantly observed in T-LBL(58.5% and 34.1%, respectively), whereas Notch and cell cycle signaling pathways mutations were more prevalent in T-ALL. Notably, besides notch receptor 1(NOTCH1), mutational status of plant homeodomain(PHD)-like finger protein 6(PHF6) was identified as another independent factor for good prognosis. Of utmost interest is that co-existence of PHF6 and NOTCH1 mutation status might provide an alternative for early therapeutic stratification in T-LBL.Conclusions: Together, our findings will not only provide new insights into the molecular and genetic mechanisms of T-LBL, but also have tangible implications for clinical practice.

Notch 1 and NF-κB Expression and Clinical Correlation in Chinese Patients with Lymphoblastic Lymphoma

T-cell acute lymphoblastic leukemia/lymphoma (T-ALL/T-LBL) is commonly associated with Notch 1 mutations. There is limited data on the relationship between Notch l and NF-κB expression and clinical features in LBL. We evaluated the expression of Notch l and NF-κB in LBL using immunohistochemistry and analyzed their relationship with clinical characteristics, treatment results, and survival. From October 2000 to August 2008, 34 untreated patients with LBL were enrolled in the study. Median age was 11.8 years (range, 1 - 25 years). Twenty-five patients were diagnosed with T-LBL and 9 patients with B-LBL. Most patients received chemotherapy consisting of modified ALL-BFM- 90. Notch l showed high expression in 68% of T-LBL and low expression in 100% of B-LBL (p = 0.015). High expression of Notch l positively correlated with presence of a mediastinal mass but not with 5-year event free survival (EFS) in T-LBL. NF-κB showed high expression in 65% of all patients with LBL, with no difference between T- and B-LBL. NF-κB expression was higher in T-LBL patients with bulky disease and B symptom;it did not correlate with 5-year EFS in T-LBL. Expression of Notch 1 and NF-κB strongly correlated (p = 0.014) in T-LBL. Notch 1 is highly ex- pressed in T-LBL. NF-κB is highly expressed in all patients with LBL with no difference between T-LBL and B-LBL. Notch 1 expression was significantly associated with NF-κB expression in T-LBL. Notch l and NF-κB may play an important role in the development of T-LBL;further investigation is warranted.

T-cell lymphoblastic lymphoma with extensive thrombi and cardiac thrombosis:A case report and review of literature

BACKGROUND T-lymphoblastic lymphoma(T-LBL),a neoplasm of immature T-cell precursors or lymphoblasts,is a clinically aggressive disease.In general,patients with T-LBL have a poor prognosis and often have high-risk clinical features,such as mediastinal masses,central nervous system infiltration,or other indications of high tumor burden;however,extensive thrombi are not common.CASE SUMMARY A 27-year-old woman presented to the Department of General Surgery with cervical lymph node enlargement accompanied by cough,wheezing,and palpitation for 3 mo.A complete blood count showed a white blood cell count of 1.6×10^(9)/L,a hemoglobin concentration of 135 g/L,and a platelet count of 175×10^(9)/L.A biopsy sample of the lymph node mass indicated T-cell lymphoblastic lymphoma,and the bone marrow immunophenotype indicated early T-cell precursor acute lymphoblastic leukemia(ETP-ALL).Abdominal and chest enhanced computed tomography showed thrombi in the superior vena cava,inferior vena cava,right hepatic vein,azygos vein,and right atrium.The ultrasonic cardiogram showed a thrombus in the right atrium of 5.23 cm×4.21 cm.The patient was first treated with low-dose dexamethasone and lowmolecular-weight heparin followed by 2 cycles of chemotherapy.Then,the ultrasonic cardiogram showed that thrombus in the right atrium had disappeared and the patient had achieved complete cytological remission.The maintenance therapy of the patient included chidamide 30 mg/wk,and she survived for 6 mo.CONCLUSION The incidence of venous thromboembolism is high in lymphoma;however,extensive thrombi with heart thrombosis is rare.Chemotherapy is the major method of treatment for lymphoma with thrombosis.We successfully treated a patient with T-LBL complicated by extensive thrombi,including a large right atrial thrombus,with combined chemotherapy containing liposomal doxorubicin,and the patient achieved complete remission.Maintenance therapy with chidamide was also effective.

Acute myelomonocytic leukemia and T-lymphoblastic lymphoma as simultaneous bilineage hematologic malignancy treated with decitabine:A case report

BACKGROUND Simultaneous bilineage hematologic malignancies are rare;however,several cases of acute myeloid leukemia(AML)and T-lymphoblastic lymphoma(T-LBL)cooccurrence have been reported.A standard treatment for simultaneous AML and T-LBL has not yet been established,and its prognosis is very poor.Further studies to develop standard treatments are required to increase patient survival rates.CASE SUMMARY A 69-year-old man complaining of pleuritic chest pain visited the emergency room.Computed tomography revealed multiple enlarged lymph nodes(LNs)in the neck and groin and pulmonary thromboembolism with pulmonary infarction.Furthermore,a peripheral blood smear performed due to leukocytosis revealed circulating blasts.Acute myelomonocytic leukemia(AMML)was diagnosed after bone marrow examination,and T-LBL positivity for terminal deoxynucleotidyl transferase,cluster of differentiation(CD)34,and CD4 was confirmed by cervical LN biopsy.Decitabine and dexamethasone were administered because he could not receive intensive chemotherapy due to poor performance status.Complete remission of AMML and T-LBL was achieved after 4 cycles of decitabine plus dexamethasone.CONCLUSION We report the therapeutic effect of decitabine,a hypomethylating agent(HMA),in patients with concurrent bilineage hematologic malignancies and suggest that further studies are required to evaluate the therapeutic effect of HMAs on both lymphoid and bilineage hematologic malignancies.

Disseminated Presentation of Primary Lymphoblastic Lymphoma of the Bone in a Pediatric Patient

Primary non-Hodgkin’s lymphoma of the bone (PLB) is extremely rare in the pediatric population with less than 100 cases reported in the English literature. Most commonly, patients present with atraumatic bone pain and grossly normal radiographic findings. PLB is in the histopathological class of “small round cell tumors of bone”, as with most common bone tumors. The diagnosis is confirmed by immunohistochemical or flow cytometry based detection of tumor-specific proteins. We present a case of stage IV PLB of B-lymphoblastic type with an excellent response to chemotherapy to increase awareness among general pediatricians and pathologists about the importance of making the correct diagnosis, given the excellent prognosis for this disease.

CD34-Negative B-Lymphoblastic Leukemia/Lymphoma Presenting as Cutenous Lesions at Infancy: A Case Report

Acute lymphoblastic leukemia/lymphoma is a highly aggressive neoplasm of precursor lymphoid (blast) cells. There are 2 main subtypes based on lymphoid lineage;B lymphoblastic leukemia/lymphoma (B-ALL/LBL) and T lymphoblastic leukemia/lymphoma (T-ALL/LBL). B-ALL/LBL commonly presents with fever, fatigue, bone or joint pain, bleeding or anorexia (signs of bone marrow infiltration), lymphadenopathy, hepatosplenomegaly, involvement of skin, soft tissue and testes, with a predilection for the central nervous system. Immature cell markers, such as CD34 and TdT, can help to differentiate lymphoblasts from Burkitt lymphoma which, is considered a mature high-grade B cell lymphoma that mimics lymphoblastic lymphoma/leukemia. Unfavorable prognostic factors include: infancy and adult age of diagnosis, high white blood cell count, slow response to initial therapy, central nervous system involvement at the time of diagnosis and Minimal residual disease after therapy. We present a case report of a 4 months old infant seen at a Tertiary Hospital with a rare presentation of CD34 Negative B-lymphoblastic leukemia/lymphoma presenting as cutaneous lesions in infancy.

Purulent Pleurisy Revealing Lymphoblastic Lymphoma Type T: A Pediatric Case Report

Lymphoma is a very common cancer in the pediatric population, and its modes of revelation are very variable. Case report: A 7-year-old patient was admitted for purulent pleurisy, whose cytological study of the pleural fluid isolated lymphoblastic cells in favor of a T-type lymphoblastic lymphoma, a very rare mode of revelation in current practice. Conclusion: Pediatric lymphoma takes on different aspects, and it is important to look for it systematically in order to ensure adequate management.

Absence of enhancement in a lesion does not preclude primary central nervous system T-cell lymphoma:A case report

BACKGROUND Primary central nervous system lymphoma(PCNSL)is a non-Hodgkin lymphoma that originates in the central nervous system(CNS)and is exclusively limited to the CNS.Although most PCNSLs are diffuse large B-cell lymphomas,primary CNS T-cell lymphomas(PCNSTLs)are rare.PCNSTLs typically demonstrate some degree of enhancement on contrast-enhanced magnetic resonance imaging(MRI).To the best of our knowledge,non-enhancing PCNSTL has not been reported previously.CASE SUMMARY A 69-year-old male presented to the neurology department with complaints of mild cognitive impairment and gradual onset of left lower leg weakness over a span of two weeks.Initial MRI showed asymmetric T2-hyperintense lesions within the brain.No enhancement was observed on the contrast-enhanced T1 image.The initial diagnosis was neuro-Behçet’s disease.Despite high-dose steroid therapy,no alterations in the lesions were identified on initial MRI.The patient’s symptoms deteriorated further.An MRI performed one month after the initial scan revealed an increased lesion extent.Subsequently,brain biopsy confirmed the diagnosis of PCNSTL.The patient underwent definitive combined chemoradiotherapy.However,the patient developed bacteremia and died of septic shock approximately three months after diagnosis.CONCLUSION The absence of enhancement in the lesion did not rule out PCNSTL.A biopsy approach is advisable for pathological confirmation.

Marginal zone lymphoma with severe rashes: A case report

BACKGROUND Marginal zone lymphoma(MZL)is an indolent subtype of non-Hodgkin lymphoma(NHL),which is rare clinically with severe rashes as the initial symptom.CASE SUMMARY This study reports a case of MZL with generalized skin rashes accompanied by pruritus and purulent discharge.First-line treatment with rituximab combined with zanubrutinib had poor effects.However,after switching to obinutuzumab combined with zanubrutinib,the case was alleviated,and the rashes disappeared.CONCLUSION For patients with advanced stage MZL not benefiting from type I anti-CD20 monoclonal antibody(mAb)combination therapy,switching to a type II anti-CD20 mAb combination regimen may be considered.This approach may provide a new perspective in the treatment of MZL.

Transformed gastric mucosa-associated lymphoid tissue lymphoma originating in the colon and developing metachronously after Helicobacter pylori eradication:A case report

BACKGROUND Helicobacter pylori(H.pylori)eradication treatment for primary gastric mucosaassociated lymphoid tissue(MALT)lymphoma has already been established.However,t(11;18)(q21;q21)/API2-MALT1 translocation-positive lesions are a type of primary gastric MALT lymphoma in which a response to eradication treatment is difficult to achieve.In addition,trisomy 18 may be associated with diffuse large B-cell lymphoma(DLBCL)transformation of gastric MALT lymphoma.CASE SUMMARY A 66-year-old man was diagnosed with MALT lymphoma in the ascending colon by colonoscopy and biopsy.Two years later,esophagogastroduodenoscopy revealed chronic atrophic gastritis that was positive for H.pylori,and eradication treatment was administered.Two years and nine months later(at the age of 70),a new ulcerative lesion suggestive of MALT lymphoma appeared in the gastric body,and six months later,a similar lesion was also found in the fundus.One year later(4 years and 3 months after H.pylori eradication),at the age of 72,the lesion in the gastric body had become deeper and had propagated.A biopsy revealed a pathological diagnosis of DLBCL.Both MALT lymphoma lesions in the ascending colon and DLBCL lesions in the stomach were positive for the t(11;18)(q21;q21)/API2-MALT1 translocation,and trisomy 18q21 was also detected.After 6 courses of R-CHOP(rituximab,cyclophosphamide,doxorubicin,vincristine and prednisone)chemotherapy,all of the above lesions disappeared[complete remission(CR)],and CR has been maintained for more than 3 years.In addition,both the colonic and gastric lesions were proven to have the same clonality.CONCLUSION Because the patient had a MALT1 translocation with trisomy 18q21,it was thought that this gastric MALT lymphoma developed independently of H.pylori infection and progressed.

Clinical study of chemotherapy-related cognitive impairment in patients with non-Hodgkin lymphoma

BACKGROUND Chemotherapy for malignant tumors can cause brain changes and cognitive impairment,leading to chemotherapy-induced cognitive impairment(CICI).Current research on CICI has focused on breast cancer and Hodgkin’s lymphoma.Whether patients with non-Hodgkin’s lymphoma(NHL)undergoing chemo-therapy have cognitive impairment has not been fully investigated.therapy have cognitive impairment has not been fully investigated.AIM To investigate whether NHL patients undergoing chemotherapy had cognitive impairments.METHODS The study included 100 NHL patients who were required to complete a compre-hensive psychological scale including the Brief Psychiatric Examination Scale(MMSE)at two time points:before chemotherapy and within 2 wk of two chemo-therapy courses.A language proficiency test(VFT),Symbol Number Pattern Test(SDMT),Clock Drawing Test(CDT),Abbreviated Daily Cognition Scale(ECog-12),Prospective and Retrospective Memory Questionnaire,and Karnofsky Perfor-mance Status were used to assess cognitive changes before and after chemo-therapy.RESULTS The VFT scores for before treatment(BT)and after treatment(AT)groups were 45.20±15.62,and 42.30±17.53,respectively(t-2.16,P<0.05).The CDT scores were 8(3.5-9.25)for BT and 7(2.5-9)for AT groups(Z-2.1,P<0.05).Retrospective memory scores were 13.5(9-17)for BT and 15(13-18)for AT(Z-3.7,P<0.01).The prospective memory scores were 12.63±3.61 for BT and 14.43±4.32 for AT groups(t-4.97,P<0.01).The ECog-12 scores were 1.71(1.25-2.08)for BT and 1.79(1.42-2.08)for AT groups(Z-2.84,P<0.01).The SDMT and MMSE values did not show a significant difference between BT and AT groups.CONCLUSION Compared to the AT group,the BT group showed impaired language,memory,and subjective cognition,but objec-tive cognition and execution were not significantly affected.

Rare primary gastric peripheral T-cell lymphoma not otherwise specified:A case report

BACKGROUND Gastrointestinal lymphoma typically arises in the stomach,small bowel,or colorectum and is usually a B-cell lymphoma.However,primary T-cell lymp-homas originating in the stomach are particularly rare.Gastric peripheral T-cell lymphoma-not otherwise specified(PTCL-NOS)is an extremely rare subtype.CASE SUMMARY We report a 63-year-old male presenting with epigastric pain.Esophagogastro-duodenoscopy revealed a large ulcerative lesion in the gastric cardia.Biopsy and immunohistochemical profiling confirmed PTCL-NOS.Imaging indicated stage II disease involving the stomach and intra-abdominal lymph nodes.The patient is planned to undergo cyclophosphamide,doxorubicin,vincristine,and prednisone or cyclophosphamide,doxorubicin,vincristine,prednisone,and etoposide chemo-therapy.CONCLUSION This case highlights the necessity of considering PTCL-NOS in differential diag-noses of gastric lesions.Comprehensive histopathological and immunohistoche-mical analysis is crucial for accurate diagnosis and guiding treatment.

Primary pancreatic peripheral T-cell lymphoma:A case report

BACKGROUND Primary pancreatic lymphoma(PPL)is an exceedingly rare tumor with limited mention in scientific literature.The clinical manifestations of PPL are often nonspecific,making it challenging to distinguish this disease from other panc-reatic-related diseases.Chemotherapy remains the primary treatment for these individuals.CASE SUMMARY In this case study,we present the clinical details of a 62-year-old woman who initially presented with vomiting,abdominal pain,and dorsal pain.On further evaluation through positron emission tomography-computed tomography,the patient was considered to have a pancreatic head mass.However,subsequent endoscopic ultrasonography-guided fine needle aspiration(EUS-FNA)revealed that the patient had pancreatic peripheral T-cell lymphoma,not otherwise specified(PTCL-NOS).There was a substantial decrease in the size of the pancreatic mass after the patient underwent a cycle of chemotherapy comprised of brentuximab vedotin,decitabine,and oxaliplatin(brentuximab vedotin and Gemox).The patient had significant improvement in radiological findings at the end of the first cycle.CONCLUSION Primary pancreatic PTCL-NOS is a malignant and heterogeneous lymphoma,in which the clinical manifestations are often nonspecific.It is difficult to diagnose,and the prognosis is poor.Imaging can only be used for auxiliary diagnosis of other diseases.With the help of immunostaining,EUS-FNA could be used to aid in the diagnosis of PPL.After a clear diagnosis,chemotherapy is still the first-line treatment for such patients,and surgical resection is not recommended.A large number of recent studies have shown that the CD30 antibody drug has potential as a therapy for several types of lymphoma.However,identifying new CD30-targeted therapies for different types of lymphoma is urgently needed.In the future,further research on antitumor therapy should be carried out to improve the survival prognosis of such patients.

Primary lymphomas of the genitourinary tract:A population-based study

Objective:We performed a population-based analysis focusing on primary extranodal lymphoma of either testis,kidney,bladder or prostate(PGUL).Methods:We identified all cases of localized testis,renal,bladder and prostate primary lymphomas(PL)versus primary testis,kidney,bladder and prostate cancers within the Surveillance,Epidemiology,and End Results database(1998e2015).Estimated annual proportion change methodology(EAPC),multivariable logistic regression models,cumulative incidence plots and multivariable competing risks regression models were used.Results:The rates of testis-PL,renal-PL,bladder-PL and prostate-PL were 3.04%,0.22%,0.18%and 0.01%,respectively.Patients with PGUL were older and more frequently Caucasian.Annual rates significantly decreased for renal-PL(EAPC:5.6%;pZ0.004)and prostate-PL(EAPC:3.6%;pZ0.03).In multivariable logistic regression models,older ager independently predicted testis-PL(odds ratio[OR]:16.4;p<0.001)and renal-PL(OR:3.5;p<0.001),while female gender independently predicted bladder-PL(OR:5.5;p<0.001).In surgically treated patients,cumulative incidence plots showed significantly higher 10-year cancer-specific mortality(CSM)rates for testis-PL,renal-PL and prostate-PL versus their primary genitourinary tumors.In multivariable competing risks regression models,only testis-PL(hazard ratio[HR]:16.7;p<0.001)and renal-PL(HR:2.52;p<0.001)independently predicted higher CSM rates.Conclusion:PGUL rates are extremely low and on the decrease in kidney and prostate but stable in testis and bladder.Relative to primary genitourinary tumors,PGUL are associated with worse CSM for testis-PL and renal-PL but not for bladder-PL and prostate-PL,even after adjustment for other-cause mortality.