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.

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.

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.

Ultrasound features of primary non-Hodgkin’s lymphoma of the palatine tonsil:A case report

BACKGROUND Lymphomas are the second most common malignancy of the head and neck.In this region,the vast majority of extranodal lymphomas are located in the palatine tonsil,accounting for about 51%.Tonsillar lymphomas are aggressive tumors with intermediate-or high-grade histology.We here report a case of primary non-Hodgkin’s lymphoma of the palatine tonsil and analyze its ultrasound features.CASE SUMMARY A 40-year-old man presented with right palatine tonsil swelling for 2 mo after a cold,accompanied by dysphagia,snoring,and suffocation.He had no sore throat,fever,or history of upper respiratory tract infection or tuberculosis.The patient was generally in good health and denied other diseases.He was diagnosed with acute tonsillitis initially and treated with antibiotics for 7 d.However,there was no improvement with the treatment.Tonsil biopsy and ultrasound-guided biopsy of the biggest lymph node of the right neck showed the typical pathology of non-Hodgkin lymphoma.CONCLUSION Primary lymphoma of the tonsils is rare,and its diagnosis is challenging.Ul-trasound is a useful modality in diagnosing oropharyngeal diseases,and can clearly show the features of this tumor,but the final diagnosis should be estab-lished by histology.

Gastric Diffuse Large B-Cell Lymphoma after the Diagnosis of Primary MALT Lymphoma of the Breast One Case Report

Primary breast and gastric lymphomas as manifesta-tions of primary extranodal lymphomas are rare malignancies, and their diagnosis, prognosis, and treatment modalities remain unclear. We report for the first time the simultaneous co-occurrence of these diseases in one patient. A 60-year-old woman was diagnosed with gastric diffuse large B-cell lymphoma （DLBCL） 2.5 years after she was found to have primary mucosa-associated lymphoid tissue （MALT） lymphoma of the breast. Although the patient underwent che-motherapy, she died of leukemia that caused irreversible cytopenia of three lineages. The data show that her MALT lymphoma apparently transfigured into gastric DLBCL. This case highlights the importance of evaluating patients for Helicobacter pylori infection when they present with extranodal MALT lymphomas, except gastric ones. Positive test findings should prompt anti-H, pylori therapy to prevent MALT lymphomas from transforming into DLBCLs.

Diagnosis Error: Carcinoma or Primary Breast Lymphoma? A Case Report and Literature Review

Introduction: Breast involvement by non-Hodgkin lymphoma is particularly rare in women. We describe the case of a patient with a rapidly growing, nodule in the right breast. On ultrasonography, the nodule was suspicious for breast carcinoma. Case presentation: A breast biopsy from a 73-year-old Moroccan women answered invasive ductal carcinoma grade 3 from Elston and Ellis. Patey was performed. Microscopic examination showed lymphoid proliferation confirmed on immunohistochemical analysis. Our patient was treated with chemotherapy. Conclusions: The rarity of breast lymphomas, and the problems related to the diagnosis and therapeutic choices with these tumors require molecular techniques in association with classical histological diagnosis.

Comparative analysis of conventional ultrasound and shear wave elastography features in primary breast diffuse large B-cell lymphoma

BACKGROUND Primary breast diffuse large B-cell lymphoma(PB-DLBCL)is a rare subtype of non-Hodgkin lymphoma that accounts for<3%of extranodal lymphomas and 1%of breast tumors.Its diagnosis and management are challenging because of its rarity,heterogeneity,and aggressive behavior.Conventional ultrasound(US)is the first-line imaging modality for breast lesions;however,it has limited specificity and accuracy for PB-DLBCL.Shear wave elastography(SWE)is a novel US technique that measures tissue stiffness and may reflect the histological characteristics and biological behavior of breast lesions.AIM To compare the conventional US and SWE features of PB-DLBCL and evaluate their diagnostic performance and prognostic value.METHODS We retrospectively reviewed the clinical data and US images of 32 patients with pathologically confirmed PB-DLBCL who underwent conventional US and SWE before treatment.We analyzed conventional US features(shape,margin,orientation,echo,posterior acoustic features,calcification,and vascularity)and SWE features(mean elasticity value,standard deviation,minimum elasticity value,maximum elasticity value,and lesion-to-fat ratio)of the PB-DLBCL lesions.Using receiver operating characteristic curve analysis,we determined the optimal cutoff values and diagnostic performance of conventional US and SWE features.We also performed a survival analysis to assess the prognostic value of conventional US and SWE features.RESULTS The results showed that the PB-DLBCL lesions were mostly irregular in shape(84.4%),microlobulated or spiculated in margins(75%),parallel in orientation(65.6%),hypoechoic in echo(87.5%),and had posterior acoustic enhancement(65.6%).Calcification was rare(6.3%)and vascularity was variable(31.3%avascular,37.5%hypovascular,and 31.3%hypervascular).The mean elasticity value of PB-DLBCL lesions was significantly higher than that of benign breast lesions(113.4±46.9 kPa vs 27.8±16.4 kPa,P<0.001).The optimal cutoff value of the mean elasticity for distinguishing PB-DLBCL from benign breast lesions was 54.5 kPa,with a sensitivity of 93.8%,specificity of 92.9%,positive predictive value of 93.8%,negative predictive value of 92.9%,and accuracy of 93.3%.The mean elasticity value was also significantly correlated with Ki-67 expression level(r=0.612,P<0.001),which is a marker of tumor proliferation and aggressiveness.Survival analysis showed that patients with higher mean elasticity values(>54.5 kPa)had worse overall survival(OS)and progression-free survival(PFS)than those with lower mean elasticity values(<54.5 kPa)(P=0.038 for OS and P=0.027 for PFS).CONCLUSION Conventional US and SWE provide useful information for diagnosing and forecasting PB-DLBCL.SWE excels in distinguishing PB-DLBCL from benign breast lesions,reflects tumor proliferation and aggressiveness,and improves disease management.

Rare Primary Diffuse Large B-Cell Lymphoma of a Male Breast

Background: Breast lymphomas are typical extranodal types of lymphoma, also known as Extranodal-lymphoma (ENL), which occur extremely infrequently, aggregating into a very small proportion of malignant breast tumors. The rarity of breast lymphomas is attributed to the scant lymphoid tissue content of the chest wall. Aims of Study: This case report is aimed at providing an up-to-date review of the literature on breast lymphomas for clinicians to, therefore, consider the possibility of this disease entity while treating a breast mass. Case Presentation: A case was reported of a 52-year man with chief mammary non-Hodgkin breast ENL when fine-needle aspiration cytology (FNAC) was not leading to a firm conclusion or result. Following an incisional biopsy, he was found to have a primary breast lymphoma. Later, the patient was diagnosed with the diffuse large B-cell type of lymphoma also known as non-Hodgkin’s Lymphoma (NHL). He had a complete reduction and disappearances of all the signs and symptoms of the disease after a course of neoadjuvant chemotherapy: Cyclophosphamide, Doxorubicin, Vincristine and Prednisolone (CHOP). CONCLUSION: Based on the above case presentation, it is vital for health care professionals and oncologists to recognize the disease by assessing the breast mass accurately with more entities so that proper diagnosis via core biopsy (incisional biopsy) can eliminate the PBL before further treatment is required.

Primary Ovarian Non-Hodgkin’s Lymphoma: Retrospective Study of 16 Patients

Background: Primary non-Hodgkin’s lymphoma (PONHL) of the ovary is rare disease. Ovarian involvement by NHL is usually secondary, as a part of systemic disease. It is frequently diagnosed as ovarian carcinoma which causes a significant delay in diagnosis and management. Objective: To analyze, to report and to better understand the clinico-pathologic features and results of treatment, and prognostic factors of these tumors. Material & methods: This was a retrospective single institutional work that included 16 cases of primary ovarian non-Hodgkin lymphoma (PONHL) treated in National Cancer Institute-Cairo University from January 2010 till January 2015. All available medical data including the clinical and pathological characteristics, treatment, and outcomes of patients with PONHL are analyzed. Results: Data from 16 patients are obtained. The patient’s age ranges from 14 to 55 years (mean 28 years). Ascites is the most common manifestation (75%). Tumor size ranges from 5 to 24 cm (mean 13.1). LDH is elevated in all cases (mean 644 U/L) and CA-125 is elevated in only 4 cases (25%) especially when there is an extensive peritoneal irritation. Ten cases (62.5%) are bilateral with stage IV-E according to the Ann Arbor staging system. The remaining six cases (37.5%) are unilateral with Ann Arbor stage I-E. There are no stage II-E or III-E in the current study. Tumors are classified according to the World Health Organization as follows: diffuse large B-cell lymphoma (10 cases) (62.5%), Burkitt’s lymphoma (5 cases) (31%) and only one case of B-lymphoblastic lymphoma/leukemia. All the tumors are of B-cell lineage and are all CD20 positive. All Burkitt’s lymphoma cases show higher Ki67 index (4 cases are 100% and one is 88%). The case of B-lymphoblastic lymphoma/leukemia is positive for TDT & CD 10. Surgery is the main treatment modality for primary diagnosis and for staging, although chemotherapy should have been the primary treatment because it is one of the most chemosensitive tumors. Follow-up period ranges from 3 months to 5 years (mean 33 months). Ten patients are alive without disease. Two cases experienced relapse and one case died during chemotherapy treatment. The remaining three cases died from other causes than disease. The median overall survival time was not reached yet;however, the mean overall survival was 46.8 monthes;median progression free survival was 36 months. Conclusion: Most patients with PONHL present with symptoms attributable to an ovarian mass which necessitates extensive surgical staging that is not mandatory for lymphomas. More studies will be needed to better define and treat this rare entity.

Primary non-Hodgkin' s lymphoma of the pancreas:report of six cases

Objective To evaluate the diagnosis and treatment of primary non-hodgkin’ s lymphoma (PNHL) of the pancreas. Methods The therapeutic result of 6 cases admitted from January 1980 to March 2000 was reviewed retrospectively. Results The lesion was located in the head of the pancreas in 2 cases and the body and/or tail in 4, respectively. Clinical manifestations included epigastric discomfort, loss of weight, abdominal mass and jaundice. Two cases underwent Whipple’ s procedure and distal pancreatectomy respectively, the other 4 underwent laparotomy and biopsy. B cell non-Hodgkin’ s lymphoma of 5 cases and T cell non-Hodgkin’s lymphoma of one were verified by pathology. One patient lost follow-up, the other 5 patients survived 58, 49, 22, 13 and 4 months respectively. Conclusion The clinical manifestation and radiological features of PNHL of the pancreas are not specific. Comprehensive therapy including surgery is beneficial to the long-term survival of patients with PNHL of the pancreas. 4 refs.

Primary esophageal lymphoma in immunocompetent patients:Two case reports and literature review

Primary lymphoma that involves the esophagus is very rare,with fewer than 30 cases reported in the Englishlanguage literature.Non-Hodgkin lymphoma accounts for most of the cases.Esophageal lymphomas have varied radiological appearances,which poses diagnostic difficulty.We report two cases of histopathologically confirmed primary diffuse large B-cell esophageal lymphoma and describe their radiological features,and briefly review the literature.

Synchronous breast cancer and breast lymphoma:two case reports and literature review

Synchronous breast cancer and breast lymphoma are rare. It is of high rate of misdiagnosis in clinical practice. Here we present two cases with this presentation. They are both middle-aged women, with stage I invasive ductal carcinoma of the breast. One patient happened to have primary breast lymphoma （PBL）; the other was secondary breast lymphoma （SBL）. Their pathology and immunohistochemistry （IHC） findings supported the diagnosis of multiple primary carcinoma. Both patients had a surgery. Then they both received CHOP regime chemotherapy and subsequent endocrine therapy.

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).

Primary hepatic peripheral T-cell lymphoma associated with Epstein-Barr viral infection

Primary hepatic peripheral T-cell lymphoma(H-PTCL) is one of the rarest forms of non-Hodgkin lymphoma. We report a patient who presented with worsening jaundice, abdominal pain, and vomiting. Laboratory values were significant for elevated total bilirubin, alkaline phosphatase, and liver aminotransferases. Following a liver biopsy, histopathology revealed several large dense clusters of atypical T-lymphocytes which were CD2+, CD3+, CD5+, CD7-, CD4+, CD8-, CD56-, CD57-, CD30+ by immunohistochemistry. The proliferation index was approximately 70% by labeling for ki67/mib1. The above histological profile was consistent with peripheral T-cell lymphoma of the liver. Epstein-Barr viral serology indicated a remote infection, a likely risk factor for PTCL. Bone marrow biopsy was negative for malignancy, further supporting hepatic origin.

Primary Liver Lymphoma: A Case Report and Literature Review

Primary non-Hodgkin lymphoma of liver is a very rare malignancy. The disease is poorly understood and few clinical studies have been conducted to help elucidate the natural course of disease, pathogenesis, optimal therapy, response to therapy, and survival. Here, we present an interesting case of primary non-Hodgkin lymphoma (NHL) originating in liver. A literature review of clinical features, diagnosis, and management is also provided.

原发性乳腺淋巴瘤MRI表现及文献复习

目的 探讨原发性乳腺淋巴瘤(primary breast lymphoma,PBL)的MRI特征,以提高对该病的认识。方法 选取2例PBL患者的临床、影像及病理结果,并通过检索文献,总结PBL的MRI表现。结果 本文1例为弥漫性大B细胞瘤,1例为T淋巴母细胞淋巴瘤,结合文献表明原发性乳腺淋巴瘤表现为边界清晰/模糊的圆形、卵圆形或分叶状肿块,ADC值较低,增强后可呈均匀/不均匀强化,增强后期可见勾边样强化,TIC强化曲线多为Ⅱ型或Ⅲ型,肿瘤内部可见血管穿行/贴边征及分隔样强化。结论 结合患者的病史及MRI表现,有助于PBL的诊断。

原发性乳腺淋巴瘤误诊误治分析

目的探讨原发性乳腺淋巴瘤(PBL)的临床表现及误诊原因、防范措施。方法回顾性分析3例曾误诊为乳腺癌的PBL的临床资料。结果3例均以乳腺肿块为首发症状就诊,初期皆误诊为乳腺癌,误诊时间9~31 d。2例行乳腺癌改良根治术,1例行单侧乳房切除加前哨淋巴结活检术。3例均经病理加免疫组织化学检查确诊PBL,术后给予化疗和放疗。随访至今,1例术后3年余复发后死亡,2例无复发。结论PBL发病率极低,且缺乏特异性临床表现及医技检查,易误诊。临床遇及类似本文患者时需考虑到PBL,及时完善肿物粗针穿刺或切除活组织病理检查,并认真分析病情和仔细鉴别诊断,以减少或避免误诊误治。

原发性乳腺非霍奇金淋巴瘤的临床特征和诊疗分析

目的探讨原发性乳腺非霍奇金淋巴瘤的临床特征及诊治手段。方法回顾性分析2000年1月至2024年3月襄阳市中心医院收治的13例原发性乳腺非霍奇金淋巴瘤患者的临床资料。结果13例患者均以乳腺包块为首发症状,确诊为原发性乳腺非霍奇金淋巴瘤,3年生存率为76.9%、5年生存率为61.5%。结论原发性乳腺非霍奇金淋巴瘤发病率极低,预后较乳腺癌差,手术治疗没有生存获益,病理确诊后应进行全身综合性治疗。

Primary spinal epidural non-Hodgkin’s lymphoma presentedwith spinal cord compression syndrome

The spinal epidural space is an uncommon presenting site in primary non-Hodgkin’s lymphomas,especially for children.A boy suffered spinal cord compression syndrome caused by primary spinal epidural non-Hodgkin’s lymphoma.Thoracolumbar magnetic reso-nance imaging(MRI)demonstrated an intraspinal mass.An operation was performed with gross total tumor removal.Histological examination revealed a non-Hodgkin’s B-cell lymphoma.Bone marrow aspiration was negative for lymphoma involvement.No other therapies(chemotherapy and/or radiotherapy)were per-formed according to the parents’opinion.The patient died approximately one year after the operation due to brain metastases.The clinical course and imaging features were discussed with a review of literatures.

原发性乳腺恶性淋巴瘤15例临床病理分析

背景与目的原发性乳腺恶性淋巴瘤(primarybreastlymphoma,PBL)是一种罕见疾病,易误诊,且治疗上仍存在争议。本研究旨在总结分析PBL的临床病理特点及治疗经验,以提高对本病的认识。方法回顾性分析我院1986年1月至2003年12月诊治的15例PBL患者的临床和病理资料。结果15例患者中位年龄39岁。93.3%(14/15)患者为B细胞性,6.7%(1/15)为T细胞性;40%(6/15)为弥漫大B细胞型,26.6%(4/15)为粘膜相关淋巴样组织淋巴瘤。8例局部切除联合化放疗综合治疗患者至今无局部复发,中位随访34.5个月(4～214个月)。6例低度恶性PBL,2例未行化疗者均短期复发。4例接受化疗者中2例无复发,2例复发后解救化、放疗持续缓解至今。本组患者总的3年和5年生存率分别为88.9%和66.7%。结论PBL多为B细胞性,弥漫大B细胞型与粘膜相关淋巴样组织型为最常见类型。PBL采用局部切除术联合放、化疗综合治疗可有效防止局部复发,根治术不是必需的。无论何种病理类型PBL都应看作全身性疾病。