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.

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.

Rare primary colonic T cell lymphoma with curative resection by endoscopic submucosal dissection:A case report

BACKGROUND The gastrointestinal tract is a well-known extranodal site of lymphoma.B-cell lymph-oma is the most common type,while T-cell lymphoma is uncommon.Primary gastrointestinal lymphoma mainly occurs in the stomach and small intestine,and the colon is less frequently involved,especially in females.CASE SUMMARY A 45-year-old woman was admitted to our hospital for physical examination.Gastroenteroscopy revealed a visible pedunculated polyp in the transverse colon,for which endoscopic submucosal dissection(ESD)was performed.Pathology suggested highly active proliferation of T lymphocytes with atypical hyperplasia.CONCLUSION A middle-aged female patient was found to have colonic T-cell lymphoma by endoscopy.The lesion was successfully removed by ESD,and the surgical margin was negative.It is essential to raise awareness of colonic T-cell lymphoma and choose the appropriate treatment.

Heterochronous multiple primary prostate cancer and lymphoma:A case report

BACKGROUND Multiple primary malignant tumors(MPMTs)are rare type of cancer,especially when solid tumors are the first and lymphoma is the second primary malignancy.We report a patient with heterochronous MPMTs consisting of prostate cancer and rectal diffuse large B-cell lymphoma(DLBCL).CASE SUMMARY We report a 77-year-old male patient diagnosed with prostate cancer who was treated with radiation therapy and one year of endocrine therapy with bicalutamide(50 mg per day)and an extended-release implant of goserelin(1/28 d).Seven years later,rectal DLBCL with lung metastases was found.CONCLUSION Although rare,the possibility of prostate cancer combined with a double primary cancer of DLBCL can provide a deeper understanding.

Bruton’s tyrosine kinase inhibitors in primary central nervous system lymphoma:New hopes on the horizon

In this editorial,we comment on the article by Wang et al.This manuscript explores the potential synergistic effects of combining zanubrutinib,a novel oral inhibitor of Bruton’s tyrosine kinase,with high-dose methotrexate(HD-MTX)as a therapeutic intervention for primary central nervous system lymphoma(PCNSL).The study involves a retrospective analysis of 19 PCNSL patients,highlighting clinicopathological characteristics,treatment outcomes,and genomic biomarkers.The results indicate the combination’s good tolerance and strong antitumor activity,with an 84.2%overall response rate.The authors emphasize the potential of zanubrutinib to modulate key genomic features of PCNSL,particularly mutations in myeloid differentiation primary response 88 and cluster of differentiation 79B.Furthermore,the study investigates the role of circulating tumor DNA in cerebrospinal fluid for disease surveillance and treatment response monitoring.In essence,the study provides valuable insights into the potential of combining zanubrutinib with HD-MTX as a frontline therapeutic regimen for PCNSL.The findings underscore the importance of exploring alternative treatment modalities and monitoring genomic and liquid biopsy markers to optimize patient outcomes.While the findings suggest promise,the study’s limitations should be considered,and further research is needed to establish the clinical relevance of this therapeutic approach for PCNSL.

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.

Computed tomography-based radiomics combined with machine learning allows differentiation between primary intestinal lymphoma and Crohn's disease

BACKGROUND Due to similar clinical manifestations and imaging signs,differential diagnosis of primary intestinal lymphoma(PIL)and Crohn's disease(CD)is a challenge in clinical practice.AIM To investigate the ability of radiomics combined with machine learning methods to differentiate PIL from CD.METHODS We collected contrast-enhanced computed tomography(CECT)and clinical data from 120 patients form center 1.A total of 944 features were extracted singlephase images of CECT scans.Using the last absolute shrinkage and selection operator model,the best predictive radiographic features and clinical indications were screened.Data from 54 patients were collected at center 2 as an external validation set to verify the robustness of the model.The area under the receiver operating characteristic curve,accuracy,sensitivity and specificity were used for evaluation.RESULTS A total of five machine learning models were built to distinguish PIL from CD.Based on the results from the test group,most models performed well with a large area under the curve(AUC)(>0.850)and high accuracy(>0.900).The combined clinical and radiomics model(AUC=1.000,accuracy=1.000)was the best model among all models.CONCLUSION Based on machine learning,a model combining clinical data with radiologic features was constructed that can effectively differentiate PIL from CD.

Primary hepatic lymphoma presenting as pyogenic liver abscess:A case report

BACKGROUND Primary hepatic lymphoma(PHL)is a lymphoproliferative disorder confined to the liver without peripheral lymph node involvement and bone marrow invasion.PHL is extremely rare in clinical practice.The etiology and pathogenesis of PHL are largely unknown.There are no common standard protocols or guidelines for the treatment of PHL.CASE SUMMARY We report the case of a 66-year-old man who presented with fever and abdominal pain for three weeks.Computed tomography and magnetic resonance imaging scans showed a pyogenic liver abscess.The patient underwent a right posterior hepatectomy.The surgical pathology revealed aggressive B-cell lymphoma,with a primary consideration of diffuse large B-cell lymphoma of non-germinal center origin.CONCLUSION This article reviews the characteristics,mechanism and treatment of PHL and provides insight into the diagnosis of PHL.

Primary pancreatic lymphoma:A case report and review of literature

BACKGROUND Primary pancreatic lymphoma(PPL)is a rare tumor that mimics pancreatic adenocarcinoma,leading to diagnostic and therapeutic challenges.PPL accounts for 0.2%of all pancreatic tumors and is typically treated with chemotherapy.However,the long-term survival rates for PPL with chemotherapy and radiotherapy alone are unsatisfactory.Due to the improvements in pancreatic surgery,there is a need to reevaluate the treatment strategies for PPL.CASE SUMMARY A 62-year-old male presented to our clinic.A biopsy was unsuccessful,and the imaging was suggestive of pancreatic adenocarcinoma.Therefore,subtotal splenopancreatectomy was performed and histopathology was performed.He was then diagnosed with primary pancreatic diffuse large B-cell lymphoma.He received adjuvant chemotherapy and radiotherapy.Currently,the patient is alive with no evidence of disease 36 months after surgery.CONCLUSION The potential role of surgery in the treatment of PPL should be emphasized and added in the management protocol of early stage lymphoma.

Diagnostic and management challenges in primary cutaneous anaplastic large cell lymphoma with necrosis,inflammation,and surgical intervention:A case report

BACKGROUND Primary cutaneous anaplastic large cell lymphoma(PC-ALCL)poses significant diagnostic difficulties due to its similarity in the appearance of skin lesions with chronic inflammatory disorders and other dermatological conditions.This study aims to investigate these challenges by conducting a comprehensive analysis of a case presenting with PC-ALCL,emphasizing the necessity of accurate differentiation for appropriate management.CASE SUMMARY An 89-year-old female patient with diabetes and hypertension presented with arm and abdominal ulcerated mass lesions.Diagnostic procedures included skin biopsies,histopathological assessments,and immunohistochemistry,complemented by advanced imaging techniques to confirm the diagnosis.The patient’s lesions were determined as PC-ALCL,characterized by necrosis,chronic inflammation,and a distinct immunophenotypic profile,including CD30,CD3,CD4,and EBER,CD56,MUM-1,Ki 67-positive in>80%of tumor cells,CD10,but negative for anaplastic lymphoma kinase,CD5,CD20,PAX-5,Bcl-2,Bcl-6,CD8,and CD15.Recurrence was not reported at the 6-month follow-up.CONCLUSION Accurate PC-ALCL differentiation from similar conditions is crucial for effective management and requires a multidisciplinary approach.

High-dose methotrexate and zanubrutinib combination therapy for primary central nervous system lymphoma

In this editorial I comment on the article,published in the current issue of the World Journal of Clinical Oncology.Primary central nervous system lymphoma(PCNSL)is a disease of elderly and immunocompromised patients.The authors reported clinical results of 19 patients with PCNSL treated with zanubrutinib/high dose methotrexate(HD-MTX)until disease progression.They demonstrated that the combination of zanubrutinib with HD-MTX led to a marked clinical response and tolerability among these patients.They also observed that cerebrospinal fluid liquid biopsy to detect circulating tumor DNA may be a good option for evaluating treatment response and tumor burden in patients with PCNSL.PCNSL is a challenging disease for treatment as these patients present with different neurological states and comorbidities.Treatment has evolved over the years from whole brain radiotherapy to HD-MTX followed by autologous stem cell transplant.Gradually,treatment of patients with PCNSL is going to become individualized.

Primary effusion lymphoma-like lymphoma in a patient with inflammatory bowel disease

A 77-year-old man with inflammatory bowel disease (IBD) and who was treated with anti-tumor necrosis factor (TNF), 6-mercaptopurine and corticosteroids, presented with primary effusion lymphoma-like lymphoma (PEL-like lymphoma) with massive ascites. The patient&#x02019;s clinical course was complicated by acute renal insufficiency and hypotension, which led to death within 2 wk. In general, patients with IBD may have an increased risk for development of lymphoma, which is frequently associated with immunosuppressive and/or anti-TNF antibody therapies. PEL is a rare subset of lymphoma localized to serous body cavities, lacks tumor mass or nodal involvement, and is associated with infection by human herpes virus 8 (HHV-8). Primary neoplastic effusion may also be present in patients with large B-cell lymphoma without evidence of human immunodeficiency virus or HHV-8 infections. This type of lymphoma is classified as PEL-like lymphoma. Both PEL and PEL-like lymphoma types have been reported in patients undergoing immunosuppressive therapy, but to the best of our knowledge, the case described herein represents the first PEL-like lymphoma occurring in a patient with IBD.

Primary central nervous system lymphoma presenting as a single choroidal lesion mimicking metastasis:A case report

BACKGROUND Primary choroidal lymphoma is usually an indolent B-cell lymphoma and rarely progresses to extraocular sites.Herein,we report a case of primary choroidal lymphoma diagnosed as diffuse large B-cell lymphoma(DLBL),which progressed to the brain parenchyma after 4 mo.CASE SUMMARY A 78-year-old man presented with diminution of vision in his right eye.A choroidal lesion suspected of metastatic lesion was observed in the right eye by ophthalmologic examination.To discover the primary tumor,imaging investigations were performed but no malignant lesion was detected.After 4 mo,the patient returned to the clinic presenting with neurological symptoms.Brain magnetic resonance imaging revealed an abnormal contrast-enhancing mass in the left cerebellum.A stereotactic biopsy was performed,and DLBL was confirmed.The patient received the high dose methotrexate-based chemotherapy and he achieved complete remission.CONCLUSION Primary choroidal lymphoma is usually known to have a benign clinical course without systemic involvement.We present a rare case of primary choroidal lymphoma diagnosed as DLBL that progressed to the brain parenchyma within months.

Primary gastric non-Hodgkin lymphomas:Recent advances regarding disease pathogenesis and treatment

Primary gastric lymphomas(PGLs)are distinct lymphoproliferative neoplasms described as heterogeneous entities clinically and molecularly.Their main histological types are diffuse large B-cell lymphoma(DLBCL)or mucosaassociated lymphoma tissue.PGL has been one of the main fields of clinical research of our group in recent years.Although gastric DLBCLs are frequent,sufficient data to guide optimal care are scarce.Until today,a multidisciplinary approach has been applied,including chemotherapy,surgery,radiotherapy or a combination of these treatments.In this minireview article,we provide an overview of the clinical manifestations,diagnosis and staging of these diseases,along with their molecular pathogenesis and the most important related clinical published series.We then discuss the scientific gaps,perils and pitfalls that exist regarding the aforementioned studies,in parallel with the unmet need for future research and comment on the proper methodology for such retrospective studies.Aiming to fill this gap,we retrospectively evaluated the trends in clinical presentation,management and outcome among 165 patients with DLBCL PGL who were seen in our institutions in 1980-2014.The study cohort was divided into two subgroups,comparing the main 2 therapeutic options[cyclophosphamide doxorubicin vincristine prednisone(CHOP)vs rituximab-CHOP(R-CHOP)].A better outcome with immunochemotherapy(R-CHOP)was observed.In the next 2 mo,we will present the update of our study with the same basic conclusion.

Primary bone anaplastic lymphoma kinase positive anaplastic largecell lymphoma: A case report and review of the literature

BACKGROUND Primary bone lymphoma(PBL)is an uncommon extranodal disease that represents approximately 1%-3%of lymphomas.Anaplastic lymphoma kinase(ALK)positive anaplastic large-cell lymphoma(ALCL)is an extremely rare type of PBL.The aim of this report is describe the symptoms,diagnosis,and treatment of primary bone ALK-positive ALCL.CASE SUMMARY A 66-year-old man presented to our hospital with neck and shoulder pain and intermittent fever that lasted for 1 mo.After extensive evaluation,positron emission tomography-computed tomography(CT)examination showed multiple osteolytic bone lesions without other sites lesions.CT-guided biopsy of the T10 vertebral body was performed,and the pathology results showed that neoplastic cells were positive for ALK-1,CD30,and CD3.A diagnosis of primary bone ALK positive ALCL was ultimately made.The patient was in partial response after four cycle soft cyclophosphamide,doxorubicin,vincristine,and prednisone chemotherapy,and we planned to repeat the biopsy and radiological examination after completion of the fifth cycle of therapy.CONCLUSION Primary bone ALK positive ALCL is a rare disease and physicians should keep in mind that ALCL can present with isolated osseous involvement without nodal involvement,and lymphoma should be considered in the differential diagnosis of primary bone lesions.

Progressively Enlarging Goiter:Case Reports of Primary Thyroid Lymphoma and Literature Review

Primary thyroid lymphoma(PTL)is an exceptionally rare and highly aggressive potentially curable malignant disease.We report three typical cases of PTL referred to our hospital.All three cases had long history of Hashimoto’s thyroiditis,and presented with progressively enlarging neck mass.The first two cases were confirmed by surgical biopsy to be diffuse large B cell lymphoma,and received radiotherapy combined with chemotherapy,or received only chemotherapy.The third case was confirmed by core needle biopsy to be mucosa-associated lymphoid tissue lymphoma,and received radiotherapy.In summary,confirmation of PTL diagnosis is essential for further clinical decisions.Core biopsy should be one of the most important methods to make the diagnosis of PTL,while the use of fine needle aspiration cytology alone is still limited in diagnosing PTL.

Primary pancreatic anaplastic large cell lymphoma, ALK negative: A case report

We present the fourth case of a primary pancreatic anaplastic large cell lymphoma (ALCL), ALK-. An 80-year-old man was admitted to our clinic for further investigation of a fever of unknown origin. He noted anorexia, weight loss and fatigue. His laboratory tests showed anemia and a great elevation of ESR, LDH, and β2 microglobulin. In CT and MRI scan, a soft tissue mass in the pancreas was observed. A repeated endoscopy after his admission revealed an ulcerated mass-like deformity of the duodenal bulb. Explorative laparotomy confirmed a diffuse spread of an unresectable malignant pancreatic mass extending to the adjacent organs. Duodenal and surgical biopsies identified an ALCL of T-cell lineage, ALK-. The patient died in the Intensive Care Unit due to hemodynamic instability.Our case is the first one indicating that primary pancreatic lymphoma should be suspected in a patient with pancreatic mass and elevated serum LDH and β2 microglobulin.

Primary cardiac lymphoma:a case report and review of the literature

Primary cardiac lymphoma (PCL) is an extremely rare neoplasm and usually defined as a non-Hodgkin's lymphoma being exclusively located in the heart and/or the pericardium. It is particularly difficult to diagnose this rare disease due to its nonspecific clinical manifestations. The prognosis of this disease is poor. In this diffuse large B-cell lymphoma, the patient finally died of heart failure after 2 courses of chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone).

Individualized Treatment and Palliative Care for A90-Year-Old Patient with Primary Gastric Diffuse Large-B Cell Lymphoma:4 Year Follow-up and Inspiration

A 90-year-old man was diagnosed with primary gastric diffuse large B-cell lymphoma(PGDLBL)by PET/CT examination,gastroscopy,biopsy and histopathological analysis at a regular physical check in April,2016.The patient received R-CO chemotherapy(rituximab,cyclophosphamide,and vincristine)and radiotherapy subsequently,with enteral nutritional treatment through 3-cavity nasogastric tube due to development of pyloric obstruction.To satisfy patient's strong desire of eating by himself,we performed surgery of exploratory laparotomy and Roux-en-Y gastric bypass(RGB)to relieve pylorus obstruction.Postoperatively,the patient resumed oral feeding,supplemented by nasogastric tube feeding at 1350-1550 Kcal daily.He is now 94 years old with fairly well nutrition and normal communication.The outcome of 4 year follow-up suggests that nutritional treatment and palliative medicine are important for improving prognosis and life-quality of very elderly patients with end-stage tumors apart from the effective chemotherapy,radiotherapy,and surgery.

Imaging of hemorrhagic primary central nervous system lymphoma: A case report

BACKGROUND A primary central nervous system lymphoma(PCNSL)presenting with massive hemorrhage is a rare occurrence that is difficult to distinguish from a high-grade glioblastoma.Comprehensive descriptions of the imaging characteristics of such tumors have not yet been reported.Herein,we reported a case of a PCNSL with massive hemorrhage by presenting the imaging features of computed tomography(CT)imaging and structural and perfusion magnetic resonance imaging(MRI).CASE SUMMARY A 48-year-old man presented with headache lasting for 10 d.CT of the brain showed a round,heterogeneous,high-density lesion with surrounding edema in the right temporal lobe.For further diagnosis,a series of MRI examinations of the brain were subsequently performed,and a hemorrhagic lesion with ring-like enhancement was determined.The whole lesion was relatively hypoperfused on arterial spin labeling images.Surgical resection of the lesion and histopathological examination confirmed that the lesion was a diffuse large B-cell lymphoma with massive hemorrhage.CONCLUSION PCNSLs with hemorrhage occur very rarely,and structural and perfusion MRI examinations are requested exceedingly rarely.This case provided insight into some characteristics of a hemorrhagic lymphoma on CT and MRI examinations.Perfusion MRI examination may be useful for the differential diagnosis of PCNSLs and other brain tumors.