Primary cardiac lymphoma in immunocompetent patients: a report of three cases and review of the literature

Primary cardiac lymphoma is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium, extremely rare in immunocompetent patients, and more frequent in immunodepressed patients. We present 3 retrospectives cases of primary cardiac lymphoma in immunocompetent patients and review 35 cases reported in the literature. Two patients were adults and one was a child. Primary cardiac lymphoma presented with constitutional symptoms in two cases and superior vena caval syndrome in one case. Diagnosis of a tumor mass was made in all cases by transthoracic echocardiography. Primary cardiac lymphoma arose in the heart right chambers in two cases. Histological diagnoses, obtained after thoracotomy, were diffuse large B-cell lymphoma in two cases, and Burkitt's lymphoma in one case. All three cases received chemotherapy, combined with radiotherapy in one patient. Of our patients, 2 are alive and asymptomatic 12 months and 33 months after diagnosis. In conclusion, diagnosis of primary cardiac lymphoma is difficult due to non-specific clinical manifestations and should be considered in patients with a cardiac mass sometimes with pericardial effusion. It is confirmed using transthoracic echocardiography and magnetic resonance imaging and certified using cytology or open biopsy. The only effective treatment is chemotherapy, but prognosis remains poor.     

A Case of Primary Adrenal Diffuse Large B-cell Lymphoma Achieving Complete Remission with Rituximab-CHOP Chemotherapy

Primary adrenal lymphoma is a very rare extranodal lymphoma; its clinical features consist of a high incidence of bilateral adrenal involvement and diffuse large B-cell lymphoma. We report a patient with primary bilateral adrenal diffuse large B-cell lyphoma who achieved complete remission with R-CHOP (rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy. A 52-yr-old man presented with fever and progressive fatigue for 3 months. Computed tomography (CT) scans of the abdomen and pelvis demonstrated large bilateral adrenal masses, and a needle biopsy of the left adrenal mass revealed diffuse large B-cell lymphoma. After 6 cycles of R-CHOP chemotherapy, CT scans showed no residual disease. To our knowledge, this is the second report to date of a patient with primary bilateral adrenal diffuse large B-cell lymphoma who achieved complete remission using R-CHOP chemotherapy.     

EBV+ diffuse large B-cell lymphoma arising within atrial myxoma in Chinese immunocompetent patient

The incidence rate of Primary cardiac lymphoma is very low. Primary cardiac lymphoma within myxoma is extremely rare disease. So far, these cases have been reported only eight in the world, which has not reported in Chinese so far. Hence, we reported the unique Chinese case of 52-year-old immunocompetent male with primary Epstein-Barr virus positive diffuse large B-cell lymphoma arising within atrial myxoma, and had no evidence of systemic lymphoma. The patient presented right sided body numbness, arm weakness no incentive and mouth twitch. A transthoracic echocardiogram revealed a large intraatrial mass, attached to the left atrial wall. The mass was removed by open thoracic surgery and subsequently diagnosed as malignant diffuse large B-cell lymphoma with myxoma by histopathology. This was the fourth case of discovered Epstein-Barr virus positive diffuse large B-cell lymphoma in a cardiac myxoma reported so far. The patient has been well by followed up for 5 months without chemotherapy. Now we discuss the importance of histodiagnosis and the proper treatment. Epstein-Barr virus positive diffuse large B-cell lymphoma arising within atrial myxoma is an extraordinary lymphoma for better prognosis, avoiding excessive treatment.     

A case of secondary myocardial lymphoma presenting with ventricular tachycardia

Malignant lymphoma can involve the cardiac cavity or myocardium as a mass. Clinical symptoms of its cardiac involvement are usually absent or nonspecific, making the diagnosis of the cardiac involvement very difficult before death. We experienced a patient with secondary myocardial non-Hodgkin's lymphoma presenting with sustained ventricular tachycardia (VT) as a primary clinical problem. A 39-yr-old woman visited our hospital because of dyspnea and palpitation for 7 days. Physical examination revealed rapid heart beat with variable intensity of the first heart sound and soft mass in the lower abdomen. VT with a cycle length of 480 msec was recorded in resting 12-lead electrocardiogram. Two well-circumscribed hypo-echogenic round masses were demonstrated in the interventricular septum and left ventricular posterior wall. Cytological examination of aspirated pericardial fluid and percutaneous needle biopsy of the abdominal mass revealed a diffuse large cell type non-Hodgkin's lymphoma. Myocardial masses and ventricular tachycardia resolved with chemotherapy using cyclophosphamide, adriamycin, vincristine and prednisone regimen. To our best knowledge, the same case as ours has not been reported previously.     

De novo CD5‐positive primary cardiac diffuse large B‐cell lymphoma diagnosed by pleural fluid cytology

Primary cardiac lymphomas are exceedingly rare. The presence and extent of the intracardiac mass is determined by echocardiography, computed tomography (CT), or magnetic resonance imaging (MRI); however, the diagnosis is established by endomyocardial biopsy or by pericardial or pleural effusion cytology. We describe the pleural effusion cytologic features of a primary cardiac lymphoma in a 55‐year‐old woman who presented with progressive shortness of breath, fatigue, mild dizziness, dull chest ache, and lower extremity edema. Transthoracic echocardiography, CT, and MRI showed a large mass centered in the right atrium and extending into the right ventricle, associated with pericardial effusion and bilateral pleural effusions. Cytologic examination of the pleural fluid showed very large pleomorphic malignant cell, some of which were binucleated and multinucleated and had anaplastic features. Flow cytometry showed a kappa monotypic population of large cells coexpressing CD5, CD19, and CD20; and immunoperoxidase stains performed on the cell block sections showed that the large neoplastic cells were positive for CD20, PAX5, CD5, and MUM1 and showed a very high proliferation rate (over 90%) by Ki67 staining. The cytologic, flow cytometry, and immunohistochemistry findings established the diagnosis of de novo CD5‐positive primary cardiac diffuse large B‐cell lymphoma (DLBCL), anaplastic variant, which was confirmed by the subsequent endomyocardial biopsy. This is, to the best of our knowledge, the first report of de novo CD5‐positive primary cardiac diffuse large B‐cell lymphoma, and the first report of the anaplastic variant of DLBCL diagnosed by effusion cytology. Diagn. Cytopathol. 2014;42:259–267. © 2012 Wiley Periodicals, Inc.     

Therapeutic comparison of chemotherapy and surgery for early stage diffuse large B-cell gastric lymphoma

PURPOSE: The use of surgery versus stomach-preserving treatment for primary gastric lymphoma has caused controversy among doctors. This retrospective, single center study aims to evaluate the efficacy and benefit of stomach-preserving treatment against surgery for early stage diffuse large B-cell lymphoma of stomach. MATERIALS AND METHODS: From August 1991 to January 2006, 43 cases of early-stage diffuse large B-cell gastric lymphoma were reviewed. RESULTS: Eleven cases were treated with chemotherapy or chemotherapy plus radiation (CT +/- RT), 17 were treated with surgery alone (OP), and 15 were treated with surgery plus adjuvant chemotherapy (OP + CT). The complete remission and response rates were 63.6% and 90.9% in those treated with CT +/- RT (7 complete responders, 3 partial responders, 1 non-responder), 100% and 100% in those treated with OP, and 100% and 100% in those treated with OP + CT, respectively. Five-year overall survival rates were 85.7%, 87.5%, and 100% in those treated by CT +/- RT, OP, and OP + CT, respectively (p=0.76). The five-year disease free survival rates were 100%, 87.5% and 100% in those treated by CT +/- RT, OP, and OP + CT, respectively (p=0.99). There was no significant difference in overall survival and disease free survival between modalities. Even though there are no definite differences in the number of complications between those treated by CT +/- RT or OP, these facts reflect little concern on complications after surgery. CONCLUSION: In preventing morbidity arising from early or late complications from surgery and promoting quality of life, chemotherapy should be a primary consideration for early stage diffuse large B-cell lymphoma of the stomach.     

鼻腔及鼻窦非霍奇金淋巴瘤的CT诊断

目的：探讨鼻腔及鼻窦非霍奇金淋巴瘤（NHL）的CT特点,提高诊断准确性。方法：回顾性分析24例经病理证实的鼻腔及鼻窦NHL的CT表现。结果：24例中16例位于单侧,8例累及双侧。原发于鼻前庭2例、鼻腔6例、鼻窦9例、鼻腔鼻窦7例。病灶肿块表现15例,浸润表现9例。24例中22例病灶密度相对均匀,2病灶密度相对不均,增强后部分强化。原发于鼻腔的淋巴瘤1例见骨质破坏,原发于鼻窦和鼻腔鼻窦的有6例见骨质破坏;常累及邻近结构。结论：鼻腔鼻窦NHL的CT表现缺乏特征性征象,但有一些相对特异性征象有助于鼻腔鼻窦NHL的诊断。根据CT的相对特征性表现,结合临床,有助于及时诊断该类疾病。     

Gastric and pulmonary lymphoma presenting as a solitary pulmonary nodule

The common presentations of lymphoma are widespread lymphadenopathy or development of constitutional symptoms. This paper presents a case of a patient who presented with a solitary mass detected on chest X-ray and underwent FDG-PET for further evaluation of this mass. FDG-PET is a commonly utilised technique to assess solitary nodules as it not only allows characterisation of the lesion but can also detect nodal and extra-thoracic disease with greater accuracy than the standard CT. In this case, FDG-PET demonstrated abnormal activity in the lung nodule and at the gastro-oesophageal junction. Biopsies confirmed Non-Hodgkin's Lymphoma at both sites. The value of FDG-PET in this case was the determination of previous unsuspected disease in an unusual presentation of lymphoma and as a useful tool for monitoring the therapeutic effect post chemotherapy.     

原发性中枢神经系统恶性淋巴瘤MR表现及其病理学基础

目的 研究原发性中枢神经系统恶性淋巴瘤(PCNSL)的MR表现及其病理学基础。方法 分析13例手术病理证实的原发性中枢神经系统恶性淋巴瘤的临床病理及MR表现。结果 13例中单发肿瘤4例，多发肿瘤9例，共计36个病灶。13例病变均累及幕上，其中8例病灶位于深部脑白质近脑室旁。肿瘤平均最大径为3．2cm。T1WI略低信号28个，T2WI等信号24个。28个病灶呈均匀强化。肿瘤水肿及占位效应相对较轻。2例PCNSL行MR动态增强扫描，早期强化均不明显，时间-信号强度曲线呈缓慢上升型。病理上肿瘤细胞弥漫分布，瘤细胞大小较一致，细胞质少，细胞核大，染色质颗粒粗，可见瘤细胞围绕血管呈袖套样浸润，少见明显的出血及片状坏死，未见钙化，病理均为非霍奇金淋巴瘤。结论 原发性中枢神经系统恶性淋巴瘤的病理基础决定其MR增强形态、占位程度以及肿瘤发生部位具有一定特征，运用不同的MR影像学检查方法和技术，在多数情况下可以做出术前正确诊断。     

Clinicopathological features of primary hepatic diffuse large B-cell lymphoma: a report of seven cases and a literature review

We studied the imaging and histopathological features of primary hepatic diffuse large B-cell lymphoma in order to explore the clinicopathological features, diagnosis, differential diagnoses, and treatment. Immunolabelling was performed in seven cases of primary hepatic diffuse large B-cell lymphoma using histological and immunohistochemical techniques. The clinical manifestations; imaging, histopathological, and immunohistochemical features; treatment; and prognosis of primary hepatic diffuse large B-cell lymphoma were observed and analyzed in light of the relevant literature. The average age of the seven patients was 63.4 years. Moreover, bulge of the upper right abdomen and progressive athrepsia and anemia were observed in all seven patients. Computed tomography (CT) revealed the presence of multiple solid hypodense lesions. Further, CT also revealed an enhanced irregular focus. Histopathological analysis revealed the following characteristics: heavy infiltration composed mainly of medium-sized round cells with a lightly stained cytoplasm, prominent nucleoli and vesicular nuclei, nuclear fission and visible sky star phenomena. The tumor cells showed diffuse expression of CD19, CD20, and CD79a, with the percentage of Ki67-positive cells being 75%-80%. All these findings indicated that primary hepatic diffuse large B-cell lymphoma is rare and generally has a poor prognosis. Biopsy and immunohistochemical staining are helpful in its diagnosis. Further, the differential diagnoses include secondary liver diffuse large B-cell lymphoma, low/undifferentiated cancer of the liver, hepatoblastoma, leukemia of the liver, and other tumors. Early surgery and chemotherapy can have a good curative effect.     

CT and multimodal imaging findings of primary orbital Ewing's sarcoma involving the middle cranial fossa: a case report

This report details the CT and MR imaging findings of a primary orbital Ewing's sarcoma case involving the middle cranial fossa in a 13-year old boy. CT showed an ill-circumscribed homogeneous soft-tissue density mass with needle-like bone reaction. On MRI, the mass showed homogeneous iso-intensity on T1WI, heterogeneous hyperintensity on T2WI, and marked homogeneous enhancement. Meanwhile, the mass was hyper-intense on DWI, and ADC value was 575×10-6 mm2/s. The mass showed as high rCBV and rCBF, prolonged MTT based on DCSPWI, and wash-out pattern of TIC derived from the DCE-MRI. Our case suggests that functional MRI modalities, including DWI, DSC-PWI or DCE-MR, could provide additional information for differential diagnoses. Both CT and MRI should be performed and comprehensively analyzed for limiting differential diagnoses, determining lesion extension and facilitating operative approach.     

Primary cardiac plasmablastic (diffuse large B-cell) lymphoma mimicking left ventricular aneurysm with mural thrombus.

Primary cardiac lymphoma is rare and is usually of the non-Hodgkin type. By definition, it involves only the heart and the pericardium, with no evidence of extracardiac disease. Primary cardiac lymphoma accounts for 1% of primary cardiac tumors and 0.5% of extranodal lymphomas [Gowda RM, Kahn RA. Clinical perspectives of primary cardiac lymphoma. Angiology 2003;54(5):599-604]. On the other hand, secondary cardiac involvement can occur in approximately 20% of patients with disseminated extracardiac lymphoma [Gowda RM, Kahn RA. Clinical perspectives of primary cardiac lymphoma. Angiology 2003;54(5):599-604]. The majority of primary cardiac lymphomas are diffuse large B-cell lymphomas with centroblastic or immunoblastic cytologic features. Herein, we describe an exceptional case of a primary cardiac plasmablastic variant of diffuse large B-cell lymphoma (Epstein-Barr virus-positive) presenting in an immunocompetent host with chest pain that mimicked a left ventricular apical thrombus.     

November 2002: a 72-year-old woman with a pineal gland mass

The November 2002 COM. A 72-year-old immunocompetent woman presented with recent confusion, memory loss, visual and a gait disturbance. MRI scans demonstrated a T2 hypointense, gadolinium enhancing mass in her pineal region. A whole body CT scan identified a large retroperitoneal mass and significant lymphadenopathy. She underwent a biopsy of the pineal lesion. The intraoperative smear contained discohesive, basophilic malignant cells of moderate size that had only scant cytoplasm, an increased nuclear-to-cytoplasmic ratio and irregular, hyperchromatic nuclei containing prominent nucleoli. Mitotic figures, apoptotic and lymphoglandular bodies were frequent. Histologic examination showed malignant cells infiltrating and almost completely replacing the residual pineal gland. The tumor cells immunoreacted with LCA and CD-20. Scattered CD3 and UCHL-1 positive cells were also present. The diagnosis was stage IV, high grade, large B-cell lymphoma involving the pineal gland and retroperitoneum. Four cycles of systemic chemotherapy significantly reduced the patient's retroperitoneal mass. However, a repeat head MRI scan continued to show gadolinium-enhancement in the pineal region, now with extension into contiguous brain parenchyma and the subependymal region. The patient died 4 months after presentation. At autopsy, the lymphoma encased the pineal region and disseminated along the ventricular walls and into the subarachnoid spread. Pineal-region tumors are rare in the elderly population, and although unusual, lymphomas should be considered in this context. The behavior of this circumventricular organ lymphoma was comparable to both peripheral and primary central nervous system lymphomas.     

Diagnosis of primary cerebral lymphoma with particular reference to CT-guided stereotactic biopsy

Summary In establishing the histological diagnosis of primary cerebral lymphoma, stereotactic brain tumour biopsy is the method of choice as the mainstay of therapy is radiation and chemotherapy. This study describes the histopathology and diagnostic immunohistochemistry of 54 primary brain lymphomas in a mainly non-AIDS population. The stereotactic biopsies were performed using the Leksell CT stereotactic frame and a spiral needle which procured about 10-mm-long tissue cylinders. Usually, three successive biopsy cylinders were taken along the target trajectory. Histological examination revealed the prevalence of high-grade non-Hodgkin's lymphoma of the polymorphous centroblastic type. The series did not include any low-grade lymphomas or T-cell lymphomas. L-26 immunohistochemistry resulted in a positive staining of the blasts, thus confirming the B-cell origin of primary brain lymphomas. Small reactive T-lymphocytes and monohistiocytic cells were also found within and at the periphery of the lymphomas and in areas of degeneration. In the biopsies of nine patients, who had shown significant reduction of the lesions on the CT scans, after corticosteroid medication, regressive tissue changes were predominant and consisted of T-lymphocytes, macrophages, and occasionally bizarre reactive astrocytes.     

Lung intravascular large B-cell lymphoma with ground glass opacities on chest computed tomography: a case report

Intravascular large B-cell lymphoma (IVLBCL) is a rare type of extranodal large B-cell lymphoma characterized by the selective growth of lymphoma cells within the lumina of vessels, particularly within capillaries, with exception of larger arteries and veins. The authors reported a case of a 45-year-old woman who was admitted in hospital with refractory fever, cough and progressive dyspnea despite of receiving broad-spectrum antibiotics. Computed tomography (CT) of the lung showed bilateral patchy ground-glass opacities with some pleural effusion in the left lung. A CT-guided percutaneous lung biopsy was performed and primary pulmonary intravascular large B-cell lymphoma was diagnosed by histopathology, immunophenotype and fluorescence in situ hybridization. The patient’s general status was improved after chemotherapy with R-CHOP. CT-guided percutaneous biopsy of lung is a safe and accurate diagnostic procedure in IVLBCL.     

Primäre renale Manifestation bei malignen Lymphomen und Leukämie

Primary manifestation of malignant lymphoma and/or leukaemia rarely occurs in the kidney. It can be the cause of a hitherto unexplained acute renal failure or it is incidentally detected as shown in the three cases under report.1.A 68-year-old man was operated on because of a symptomatic tumour in his right kidney. At nephrectomy, a conventional (clear cell) renal cell carcinoma was found simultaneously with an occult mantle cell lymphoma infiltrating the adjacent renal and extrarenal tissue. Clinical follow-up uncovered nodal and bone marrow involvement, so that a primary renal manifestation of mantle cell lymphoma was apparent.2.A 69-year-old man with suspected vertebral metastasis underwent partial renal resection because of a mass in his left kidney. Histologically and immunohistochemically, the renal infiltration was diagnosed as a precursor B-lymphoblastic lymphoma. After chemotherapy and irradiation, leukaemic blood cell counts with 50% lymphoblasts proved a primary renal manifestation of precursor B-lymphoblastic leukaemia/lymphoma.3.A 13-year-old boy presented clinically with renal failure, enlarged kidneys, and normal urinalysis. Renal biopsy showed a diffuse interstitial infiltration with atypical T-lymphoblasts compressing tubules and surrounding preserved glomeruli. Subsequent clinical bone marrow smears presented 60% T-lymphoblasts, so that the final diagnosis of a primary renal manifestation of acute T-lymphoblastic leukaemia of mature thymic cortex type was made. Immediate chemotherapy resulted in total recovery of renal function and bone marrow findings.     

Primary effusion lymphoma with cardiac involvement in HIV positive patient-complete response and long survival with chemotherapy and HAART

Primary effusion lymphoma (PEL) is a rare type of lymphoma related to herpesvirus-8 (HHV-8), and considered an AIDS-defining condition. The authors describe a case of PEL with cardiac involvement occurring in an HIV-positive patient treated with HAART and chemotherapy, who achieved complete remission and long survival.     

Posttransplant primary cutaneous Ki-1 (CD30)+/CD56+ anaplastic large cell lymphoma

An anaplastic large cell lymphoma that was negative for Epstein-Barr virus and positive for Ki-1 (CD30) presented as a polypoid scalp mass in a 56-year-old man 16 years after renal transplantation. The lymphoma was of the CD4+ cytotoxic T-cell lineage, and the tumor cells also expressed CD56. Despite reduction in the dose of immunosuppression and localized radiotherapy, the tumor had rapidly progressed to involve the soft tissue of the right hand. Systemic chemotherapy induced complete regression of the soft tissue lesion. This case illustrates that posttransplant primary cutaneous CD30+ anaplastic large cell lymphomas may assume an aggressive clinical course but can still be controlled by systemic chemotherapy.     

A primary cardiac sarcoma preoperatively presented as a benign left atrial myxoma

Primary cardiac sarcomas are extremely rare. We report a case of a primary cardiac sarcoma with myxoid change, which originally presented as a benign cardiac myxoma on a two- dimensional echocardiogram. On operating, the mass was found to extend into the posterior left atrial wall, the left pulmonary vein, and the mitral valve. The patient underwent wide resection of the left atrium, a mitral valve replacement and a left pneumonectomy. The histological diagnosis was of an undifferentiated primary cardiac sarcoma. The patient had postoperative chemotherapy. The patient expired 11 months after surgery due to a recurrence of the cardiac sarcoma. Although most tumors that develop in the left atrium are benign myxomas, we should make a preoperative differential diagnosis.     

A 3D Hermite-based multiscale local active contour method with elliptical shape constraints for segmentation of cardiac MR and CT volumes

Analysis of cardiac images is a fundamental task to diagnose heart problems. Left ventricle (LV) is one of the most important heart structures used for cardiac evaluation. In this work, we propose a novel 3D hierarchical multiscale segmentation method based on a local active contour (AC) model and the Hermite transform (HT) for LV analysis in cardiac magnetic resonance (MR) and computed tomography (CT) volumes in short axis view. Features such as directional edges, texture, and intensities are analyzed using the multiscale HT space. A local AC model is configured using the HT coefficients and geometrical constraints. The endocardial and epicardial boundaries are used for evaluation. Segmentation of the endocardium is controlled using elliptical shape constraints. The final endocardial shape is used to define the geometrical constraints for segmentation of the epicardium. We follow the assumption that epicardial and endocardial shapes are similar in volumes with short axis view. An initialization scheme based on a fuzzy C-means algorithm and mathematical morphology was designed. The algorithm performance was evaluated using cardiac MR and CT volumes in short axis view demonstrating the feasibility of the proposed method.