A case of diffuse large B-cell lymphoma transformed from immunoglobulin A-producing marginal zone B-cell lymphoma

We present a rare case of diffuse large B-cell lymphoma transformed from immunoglobulin (Ig) A-secreting marginal zone B-cell lymphoma. A 62-year-old woman was admitted to our hospital for examination of a disseminated pulmonary shadow. Gradual swelling of bilateral axilla and right inguinal lymph nodes were noted after admission. Histological examination of the lymph node biopsy specimen revealed the appearance of marginal zone B-cell lymphoma. The surface Ig of lymphoma cells was IgA-kappa, which coincided with the class of monoclonal Ig found in the patient's serum. The lymph node swelling and pulmonary shadow subsided, and the serum IgA level was normalized by 3 courses of systemic chemotherapy. However, after 4 courses of treatment, new tumor lesions at the right chest wall and left arm progressively became apparent. The biopsy specimen of the tumor showed a feature of diffuse large B-cell lymphoma. Despite intensive chemotherapy, the patient died of spreading tumor burden into the central nervous system.     

Hemobilia due to biliary intraepithelial neoplasia associated with Zollinger–Ellison syndrome

A 58-year-old man was transferred to us from his local hospital because of failure to control his gastrointestinal bleeding by endoscopic hemostasis. Abdominal imaging suggested a hypervascular tumor of the pancreatic head (36?mm diameter), and laboratory testing showed an elevated serum gastrin level (17,800?pg/mL). Gastroduodenal endoscopy revealed multiple duodenal ulcers and active bleeding from the ampulla of Vater. The selective arterial secretagogue injection test suggested a gastrinoma in the pancreatic head, but no gastrinoma in the pancreatic tail. The patient was diagnosed with solitary pancreatic head gastrinoma complicated by hemosuccus pancreaticus, and pancreaticoduodenectomy was performed. Intraoperatively, the diagnosis was changed to primary peripancreatic lymph node gastrinoma without pancreatic involvement. The gastrointestinal bleeding stopped postoperatively and serum gastrin levels returned to normal. Histological examination of the surgical specimens revealed a small submucosal gastrinoma in the duodenum (7?mm diameter). The final diagnosis was microgastrinoma of the duodenum with peripancreatic lymph node metastasis. The cause of bleeding from the ampulla of Vater was initially obscure, but eventually a hemorrhagic erosion with moderate atypia was found in the common bile duct, indicating biliary intraepithelial neoplasia (BilIN). This is the first report of hemobilia due to BilIN with gastrinoma.     

Primary gastric lymphoma with arterial bleeding

We experienced a case of primary gastric lymphoma with arterial bleeding. The case was an 88‐year‐old‐man who was admitted to our hospital with hematemesis. Gastroduodenal endoscopy revealed a gastric ulcerating tumor with arterial bleeding in the posterior wall of the angular gastric region, and a distal subtotal gastrectomy with lymph node dissection was performed. The resected tumor measured 7.0 × 3.0 cm in size with a blood vessel visible in the bottom of the ulcer. Pathologic examination confirmed a diagnosis of B‐cell malignant lymphoma of the diffuse large cell type. Metastasis was detected in nos 3 and 5 lymph nodes. According to the Ann Arbor and Naquvi classifications, the lymphoma was stage IIE and II, respectively. One year and 10 months after the operation, a computed tomography scan revealed a few swollen lymph nodes around the abdominal aorta. Recurrence of lymphoma was confirmed and chemotherapy comprising cyclophosphamide, doxorubin, vincristine and predonisolone was given at half the ordinary adult dose.     

A case of IgG4-related disease associated with diffuse large B cell lymphoma

In June 2008, a 74-year-old male was diagnosed with IgG4-related disease including histologically proven IgG4-related prostatitis, and then followed as an outpatient. In July 2011, cervical, chest, and abdominal computed tomography (CT) revealed right parotid gland swelling and lymph node enlargement of the supraclavicular, mediastinal, left hilar, porta hepatis, and para-aorta. A biopsy of the right parotid gland was performed, and we diagnosed diffuse large B-cell lymphoma (DLBCL). As malignancies are possible complications for patients with IgG4-related disease, we must be careful in the follow-up of IgG4-related disease patients.     

An elderly non-Hodgkin lymphoma patient with a massive tumor of the heart

We report on an elderly patient with a malignant lymphoma forming a huge mass in the heart. An 82-year-old woman became aware of general fatigue and a cough in August 1999. Her right supraclavicular, bilateral axillary, and right inguinal lymph nodes were swollen. A hypodermical mass in the right frontal chest was detected. Her left axillary lymph node was biopsied. She was diagnosed as having non-Hodgkin lymphoma, diffuse large cell type, B-cell type. Computed tomography scans showed a markedly thickened right ventricular wall of the heart, swollen lymph nodes of the mediastinum, bilateral pleural effusions, and a tumor in the spleen. Lymphoma cells were found in the pleural effusion, and the lymphoma was diagnosed as clinical stage IV. Hypofunction of the heart, ejection fraction (EF) 49%, was demonstrated with transthoracic echocardiography. EF increased to 70% after 3 courses of chemotherapy with CHOP regimen. All lesions disappeared after 6 courses of chemotherapy were completed. After consolidative radiotherapy with a total dose of 37 Gy to the mediastinum and heart, bilateral pleural effusions, elevation of the patient's lactate dehydrogenase level and soluble IL-2 receptor value were recognized, which suggested relapse of the lymphoma, although histopathological confirmation could not be realized.     

Coexistent B-cell lymphoma and cutaneous T-cell lymphoma

A 78-year-old man with a history of mycosis fungoides was referred for evaluation of a right adrenal mass. A physical examination showed the left cervical lymph node to be palpable, which was later shown to be caused by a diffuse large B-cell lymphoma. The patient was diagnosed with concurrent mycosis fungoides and a diffuse large B-cell lymphoma. Three courses of chemotherapy were performed, however, the patient died of advanced disease. Autopsy findings showed that the right adrenal and soft tissue masses had an identical B-cell origin. Although the exact mechanism remains unclear, the pathogenesis of this rare association is discussed.     

Diffuse large B-cell lymphoma presenting as a unilateral solitary round pulmonary hilar node infarction

Unilateral solitary pulmonary hilar node adenopathy is a rare presentation of diffuse large B-cell lymphoma. In this report, the authors present a case with a solitary pulmonary hilar lymph node infarction caused by diffuse large B-cell lymphoma. Enhanced CT examinations revealed a well-defined round mass with homogenous low attenuation in the left pulmonary hilum. Both radiological imaging and pathological examination can provide useful information for the interpretation of abnormalities and may enable the diagnosis of rare aetiologies.     

Malignant lymphoma presenting as Kaposi's sarcoma in a homosexual man with the acquired immunodeficiency syndrome

A homosexual man had Kaposi's sarcoma of the skin and lymph nodes. After a brief response to interferon, the patient developed new skin lesions. Massive bleeding in the gastrointestinal tract prompted endoscopy, which showed tumor involvement of the stomach, believed to be Kaposi's sarcoma. At autopsy, a diffuse lymphoma was found involving the skin and gastrointestinal tract, forming collision tumors in regions that contained Kaposi's sarcoma. Lymphomatous tissue, but not uninvolved lymph node or spleen, contained Epstein-Barr virus DNA, as shown by DNA hybridization studies. Epstein-Barr virus may play a role in the development of lymphoma in immunosuppressed patients. Unusual manifestations of tumors, such as the massive gastrointestinal bleeding seen in this case, may indicate the development of a second neoplasm.     

Splenic lymphoma of Ki-1 positive B-cell type treated with CHOP]

A 75-year old man was admitted to our hospital by complaining of his left supraclavicular lymphadenopathy. Physical examination also revealed splenomegaly and cervical lymphadenopathy. An abdominal CT scan showed paraaortic lymphadenopathy and splenomegaly with multiple low density area. A pathological diagnosis of Ki-1 lymphoma was made by the biopsied supraclavicular lymph node because the node consists of large cells with clear cytoplasms and conspicuous nucleoli and large cells were positive for IgM, lambda, CD20 and CD30 (Ki-1). The primary lesion of lymphoma was suspected to be spleen because of the radiological finding of the spleen. The patient responded to systemic chemotherapy (CHOP) with complete remission. Pathological and clinical characteristics of this B-cell Ki-1 lymphoma were discussed.     

Hepatocellular carcinoma with duodenal invasion and metastasis

Background and Aim: Hepatocellular carcinoma (HCC) is the leading cause of cancer‐related deaths in Taiwan. HCC with duodenal involvement are rare and are associated with a poor prognosis. The purpose of this retrospective study was to collect clinical information and data regarding survival following various treatments. Methods: Between 1996 and 2009, 21 cases (17 men) were diagnosed with HCC and duodenal invasion and metastases by diagnostic imaging, endoscopy with biopsy, or surgically collected specimens sent to pathology. The clinical course was analyzed from the patients' medical records. Results: Gastrointestinal bleeding was reported in 18/21 patients. Diagnostic imaging showed that the majority of cases involved direct tumor invasion (predominantly from the right liver lobe) and six cases from metastasis. Tumor mass and ulcerations were the most common features noted on endoscopy. In addition to the component therapy and medication treatment, panendoscopic hemostasis, surgery, transcatheter arterial embolization, and radiotherapy were performed for the management of duodenal involvement and gastrointestinal bleeding. Survival duration after duodenal involvement ranged from 0.2 to 57.8 months (mean 10.5 months). Conclusions: Gastrointestinal bleeding in advanced HCC should raise suspicions of duodenal involvement. HCC can involve the duodenum by direct invasion (from either the left or right liver lobes) or metastasis. The prognosis for HCC patients with duodenal involvement is poor, but is improved by supportive care and application of various treatment modalities.     

Prevalence of Common Non-Hodgkin Lymphomas and Subtypes of Hodgkin Lymphoma by Nodal Site of Involvement: A Systematic Retrospective Review of 938 Cases

Non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL) represent a heterogeneous group of malignant lymphoid tumors, which have distinct histological and/or biological characteristics with preferential nodal involvement. However, none of the previous studies have assessed the prevalence of common NHL and HL subtypes at each nodal site of involvement.The aim of our study was to determine the prevalence of HL and NHL subtypes depending on their nodal sites of involvement.We conducted a single-center retrospective study of 938 lymphoma cases diagnosed in the Pathology Department of Toulouse Purpan Hospital in France between 2001 and 2008, taking into account the site that corresponded to the diagnostic biopsy.The most frequent sites were cervical lymph nodes (36.8% of all cases), inguinal lymph nodes (16.4%), axillary lymph nodes (11.9%), and supraclavicular lymph nodes (11%). We found an unexpected association between intraparotid nodes and nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) and between inguinal nodes and follicular lymphoma. The risk of having classical Hodgkin lymphoma (CHL) was 15 times greater in patients with mediastinal lymphoma compared to those with other sites of involvement. Regarding HL, nodal and extranodal mediastinal sites and supraclavicular nodes were more likely to be involved by nodular sclerosis Hodgkin lymphoma (NSCHL). In addition, intra-abdominal lymph nodes were more frequently involved by lymphocyte depleted Hodgkin lymphoma compared to inguinal nodes where NLPHL predominated.Our study shows that some lymph node sites have a disproportionate prevalence of specific subtypes of lymphoma. Identifying these sites may aid to diagnose and better elucidate the pathogenesis of these tumors.     

Successfully treated massive pulmonary thromboembolism and thrombus in the right atrium due to diffuse malignant lymphoma: a case report

A 40-year-old man presented with massive pulmonary embolism related to diffuse large B cell lymphoma. His hemodynamic state worsened rapidly to shock after sudden onset of dyspnea. Echocardiography and thoracic computed tomography indicated pulmonary thromboembolism due to deep venous thromboembolism, associated with a mass in the anterior mediastinum and a 5 x 8 cm mass in the left pelvis compressing the left femoral vein. He underwent emergent surgery to remove a huge thrombus from the right atrium through the bilateral pulmonary arteries. Soon after this surgery, his hemodynamic state recovered and excision of the left cervical lymph node revealed diffuse large B cell lymphoma. Venous compression by the lymphoma mass had caused hemostasis and thrombus formation in the present case.     

The clinicopathological analysis of 303 cases with malignant lymphoma classified according to the World Health Organization classification system in a single institute of Taiwan

Several reports have shown a different distribution of malignant lymphoma (ML) in Asian and Western populations. The purpose of our survey was to elucidate whether there are substantial differences in the frequencies of subtypes of ML between different geographical areas. All entities diagnosed as ML between June 1995 and December 2007 were selected according to the 2008 World Health Organization (WHO) classification and searched for clinical outcomes. The cases were retrieved and reviewed by a panel of clinical haematologists and haematopathologists. A total of 303 patients with ML were identified for retrospective analysis. Of the 303 patients with ML, 278 patients (91.7%) had non-Hodgkin’s lymphoma (NHL), and 25 (9.2%) had Hodgkin’s lymphoma. Of the 278 patients with NHL, 223 (73.6%) had lymphoma of B-cell lineage, and 55 (18.1%) had lymphoma of T-cell lineage. One hundred and thirty-seven patients were diagnosed with diffuse large B-cell lymphoma, which was the most common B-cell lineage subtype and accounted for 45.2% of patients with NHL. Peripheral T-cell lymphomas were the most frequent subset of the T-cell neoplasms, comprising 10.6% of ML. Extranodal involvement was found in 125 (44.9%) of the 278 patients with NHL, and the lymph node was the site of primary involvement in 153 patients (55.1%). Fifty-nine (47.2%) of the 125 patients with extranodal presentation had gastrointestinal tract involvement. Outcome was worse in patients with extranodal NHL than in those with nodal NHL through the entire follow-up period; the difference in survival rates was significant. Our findings clarify the applicability and prognostic relevance of the WHO classification system and provide further information about the incidence of various lymphoma subtypes in Taiwan. Primary extranodal NHL was associated with a worse prognosis and distinct characteristics compared with nodal NHL. The outcome of different types of extranodal NHL should be investigated further.     

Tracheal ulcer due to Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly

A 74-year-old man was referred to our hospital because of a tracheal stenosis circumscribed with soft tissue density and a left pulmonary nodule. Open biopsy of a right submandibular lymph node revealed diffuse large B-cell lymphoma, and the malignant cells were positive for Epstein-Barr virus gene products. Bronchofiberscopy revealed a tracheal necrotizing ulcer. After chemotherapy, the tracheal ulcer resolved. To our knowledge, this is the first report of a case of Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly with a tracheal ulcer.     

Diffuse large B-cell lymphoma showing CNS invasion by CD30-positive multinuclear giant cells mimicking the clinical features of progressive multifocal leukoencephalopathy

We report a 55-year-old woman with diffuse large B-cell lymphoma showing central nervous system (CNS) infiltration by CD30-positive lymphoma cells. The patient was admitted with pleural effusion, ascites and a large mass in the abdominal cavity. Southern blot analysis of DNA extracted from the ascites revealed IgJH rearrangement, and therefore she was initially diagnosed as B-cell neoplasia. She received combined chemotherapy (DICE and CHOP regimens), and achieved a transient clinical response. Three months later, she developed various neurological abnormalities, and brain magnetic resonance imaging revealed diffuse infiltration of the cerebral white matter. We considered the possibility of CNS involvement by the lymphoma or progressive multifocal leukoencephalopathy (PML), and began a course of anti-virus therapy and radiation therapy. Because multiple lumbar punctures demonstrated large multinuclear lymphoma cells in the cerebrospinal fluid, a diagnosis of metastatic CNS lymphoma was made. Immunohistochemistry revealed that these lymphoma cells were reactive with anti-CD30 antibody. Although the radiation therapy was temporarily effective against the CNS involvement, the patient died of systemic invasion of the lymphoma cells. The final diagnosis was diffuse large B-cell lymphoma on the basis of pathologic findings, immunohistochemistry, and Southern blot analysis using a mesenteric lymph node obtained at autopsy. Cytospin preparations and immunohistochemistry of specimens obtained from frequent lumbar punctures were useful for differentiating CNS lymphoma from PML.     

Lymph node histology simulating T-cell lymphoma in adult-onset Still's disease

Summary A patient with adult-onset Still's disease (AOSD) underwent lymph node biopsy as part of initial evaluation for fever of unknown origin. The lymph node histology showed a massive, diffuse immunoblastic hyperplasia, simulating T-cell lymphoma. This nodal histology differs from rheumatoid arthritis, where mostly a follicular B-cell reaction predominates. Evaluating fever of unknown origin when one is unacquainted with this massive immunoblastic hyperplasia can lead to the wrong diagnosis of T-cell lymphoma in patients with AOSD.     

Pulmonary metastasis of extranodal high malignancy B-cell non-Hodgkin lymphoma of the bulbus duodeni and pylorus of the stomach

We report a 71-year-old female patient with repeated vomitus, meteorism, epigastric pain and reflux for more than four month. She had a palpable mass in the upper abdomen and lost 7 kg of weight during the last four months. Chest X-ray showed two masses, 2 cm and 3 cm in diameter, in the left and right lower lung. A stenosing polypoid mucosal swelling in the antrum and the duodenal bulb. The pulmonal masses were biopsied under CT-guidance. Biopsy proved a high malignant B-cell non-Hodgkin's lymphoma of the stomach. The masses in the lung were identified as metastases of the gastrointestinal lymphoma. In conclusion on this tumor was an extranodal non-Hodgkin's lymphoma stadium BE IV according to Musshoff. A CHOP-chemotherapy was initiated. Restaging after three cycles of CHOP revealed a complete remission. Primary gastrointestinal non-Hodgkin's lymphomas are relatively rare neoplasms of the abdomen. Unusual and interesting in this case ist the metastatic pattern involving the lung periphery without local lymph node metastases.     

A Case Report of the Histologic Transformation of Primary Follicular Lymphoma of the Duodenum

A 46-year-old woman underwent upper endoscopy for evaluation of anemia, which revealed whitish granules at the duodenal papilla, diagnosed as duodenal follicular lymphoma (DFL) by biopsy. Computed tomography and abdominal ultrasonography revealed that follicular lymphoma was confined to the duodenum. Seven years after the diagnosis, fluorine-18 fluorodeoxyglucose positron emission tomography scanning revealed multiple lesions including in bone marrow and lymph nodes. Bone marrow biopsy of the right iliac bone revealed diffuse large B-cell lymphoma, indicating systemic dissemination and histologic transformation of the DFL. The patient responded to chemotherapy and has been progression-free for 2.5 years. Although DFL is usually indolent even without any treatment, systemic dissemination with histologic transformation can occur. This case suggests that the life-time follow-up that is usually done for patients with nodal follicular lymphoma should be provided to patients with DFL.     

Use of a B-type Natriuretic Peptide in Evaluating the Treatment Response of a Relapsed Lymphoma with Cardiac Involvement

A 50-year-old woman received a diagnosis of diffuse large B-cell non-Hodgkin's lymphoma in 2000 and achieved complete remission. In April 2004, echocardiography and computed tomography examinations identified a tumor attached to the tricuspid valve and protruding within the right atrium. Bone marrow and lymph node biopsies showed a relapse of large cell lymphoma. The patient had a markedly elevated level of B-type natriuretic peptide (BNP) but a normal level of cardiac troponin I. The follow-up evaluation of the BNP level after chemotherapy showed that it had returned to within normal limits, and an echocardiogram showed regression of the tumor. Use of the BNP level as a monitor in the treatment of cardiac lymphoma has never been reported. This article is the first to report the use of BNP monitoring before and after chemotherapy to evaluate a patient with an unusual relapsed lymphoma with cardiac involvement.     

Review of lymphoma in the duodenum: An update of diagnosis and management

The presentation, subtype, and macroscopic images of lymphoma vary depending on the site of the tumor within the gastrointestinal tract. We searched PubMed for publications between January 1, 2012 and October 10, 2022, and retrieved 130 articles relating to duodenal lymphoma. A further 22 articles were added based on the manual screening of relevant articles, yielding 152 articles for full-text review. The most predominant primary duodenal lymphoma was follicular lymphoma. In this review, we provide an update of the diagnosis and man-agement of representative lymphoma subtypes occurring in the duodenum: Follicular lymphoma, diffuse large B-cell lymphoma, extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue, mantle cell lymphoma, and T-cell lymphomas.