Epstein-Barr virus negative primary hepatic leiomyoma: Case report and literature review

Primary hepatic leiomyoma is a neoplasm of mesen-chymal origin and occurs only rarely. Secondary to benign smooth muscle proliferation, it is usually found in adult women and is associated with Epstein-Barr virus (EBV) infection. Here, we report the 29 th case of primary hepatic leiomyoma with its unique features related to diagnosis, treatment and developmental biology. A 48-year-old man, with an immunocompromised status, complained of pain in the upper quadrant of the abdomen. Serological analysis indicated no presence of hepatitis virus, no human immunodeficiency virus, and no EBV infection. The levels of α-fetoproteinand carcinoembryonic antigen were normal. A mass was detected in segment Ⅲ of the hepatic lobe by ultrasonography and an abdominal computed tomography scan. Endoscopy had negative findings. Exploratory laparotomy found no existing extrahepatic tumor and left lateral lobectomy was performed. Pathological examination showed the mass to be a typical leiomyoma. The cells were positive for α-smooth muscle actin and desmin, and negative for the makers of gastrointestinal stromal tumor (GIST), including CD117, CD34 and DOG1 (discovered on GIST1). In situ hybridization revealed negative status for EBV-encoded small RNA. After left lateral lobectomy, the patient was not given chemotherapy or radiotherapy. During a 2-year follow- up, no sign of local recurrence or distant metastasis was observed. In conclusion, we report a rare case of primary hepatic leiomyoma in a male patient without EBV infection. Hepatic resection was curative. This case presents data to expand our knowledge concerning the complex and heterogeneous nature of primary liver leiomyoma, indicating that EBV infection is important but neither necessary nor sufficient for the development of primary liver leiomyoma.     

Thymic hyperplasia and Graves' disease: a non-fortuitous association. case report and review of literature

Thymus hyperplasia and Graves’ disease association is not well known and is probably not incidental. We report the case of a young woman affected with Graves’ disease in which a retrosternal mass was disclosed during a neck ultrasonographic-examination and confirmed by chest CT-examination. Follow-up ultrasound survey showed a decrease in the thymic mass size. Because of various antithyroid drugs allergy, a surgical procedure was performed, during which both her thyroid and thymic mass were removed. The histopathologic examination of this mass confirmed the hyperplasic nature of the thymic bulging. Ninety-one cases of thymus hyperplasia and Graves’ disease association have been reported in literature, of which 20 were histologically confirmed. Among these cases 35 showed a thymic mass regression under medical treatment alone. Accordingly, surgical procedures are most frequently unnecessary in such associations because of the thymic mass decrease incurred by antithyroid drug treatment.     

Hepatitis C virus infection and non-Hodgkin's lymphoma: a review and case report of nine patient

The role of hepatitis C virus (HCV) is well established in the development of chronic hepatitis, cirrhosis and hepatic carcinoma, as well as in mixed type II cryoglobulinemia, membranoproliferative glomerulonephritis(MPGN) and porphyria cutanea tarda (PCT). Increasing evidence has been reported of a close association of HCV infection with autoimmune and hematological processes, mainly cytopenias and lymphoproliferative disorders such as B cell non-Hodgkin's lymphoma. We describe the demographic, clinical and histopathological findings of nine patients from the Mexican population with non-Hodgkin's lymphoma and HCV infection.     

Hepatitis C virus infection in patients with B-cell non-Hodgkin's lymphoma

OBJECTIVE: As a causative role of hepatitis C virus (HCV) in B-cell lymphoproliferative disorders (LPD) has been suggested by several reports, we investigated the prevalence of HCV infection among patients with LPD at our hospital with the aim of clarifying the clinical features and the outcome for HCV antibody-positive patients with non-Hodgkin's lymphoma (NHL). METHODS: Retrospective chart review. PATIENTS: A total of 123 patients with B-cell LPD (4 with chronic lymphocytic leukemia, 17 with multiple myeloma, and 100 with B-cell NHL), 38 patients with non-B-cell LPD (5 with adult T-cell lymphoma, 8 with Hodgkin's disease, and 25 with non-B-cell NHL) and 516 patients with miscellaneous diseases other than liver diseases or LPD (control) were studied. RESULTS: HCV infection was detected in 17 of 100 patients with B-cell NHL versus none of 25 patients with non-B-cell NHL (p=0.023) and in 34 patients (6.6%) in the control group with miscellaneous diseases (p=0.0011). In HCV-positive B-cell NHL, primary liver involvement was detected in 3 of 17 patients compared to none of 83 HCV-negative patients (p=0.0019). Intermediate-grade lymphoma (Working Formulation) was the most frequent histology. Eleven of 15 HCV-positive patients achieved complete remission after chemotherapy, and 6 of 7 deaths were caused by liver-related diseases. CONCLUSION: The prevalence of HCV infection was higher in patients with B-cell NHL than in those with non-B-cell NHL and the control group. Primary liver involvement and liver-related causes of death were frequent in HCV-positive patients with B-cell NHL.     

Composite diffuse large B-cell lymphoma and classical Hodgkin’s lymphoma of the stomach:Case report and literature review

The combination of classical Hodgkin’s lymphoma(cHL)and non-Hodgkin lymphoma coexisting in the same patient is not common,especially in one extranodal location.Here we present a rare case of composite diffuse large B-cell lymphoma(DLBCL)and cHL occurring simultaneously in the stomach of a 53-year-old female who presented with upper abdominal discomfort and gas pain.Surgery was performed and the disease was diagnosed pathologically as composite lymphoma of DLBCL and cHL using hematoxylin-eosin and immunohistochemical staining.Epstein-Barr virus(EBV)infection was not detected by in situ hybridization for EBV-encoded RNA or immunohistochemistry for EBV latent membrane protein-1.Polymerase chain reaction analysis from the two distinct components of the tumor demonstrated clonal immunoglobulinκlight chain gene rearrangements.The patient died approximately 11 mo after diagnosis in spite of receiving eight courses of the CHOP and two courses of the rituximab-CHOP(RCHOP) chemotherapy regimen.This case report showed that the two distinct components,DLBCL and cHL,appeared to originate from the same clonal progenitor cell,and that EBV infection was not essential for transformation during the course of tumorigenesis.     

Hepatitis C virus and diffuse large B-cell lymphoma: Pathogenesis,behavior and treatment

A significant association between hepatitis C virus (HCV) infection and B-cell lymphoma has been reported by epidemiological studies, most of them describing a strong relationship between indolent lymphomas and HCV. Furthermore, the curative potential of antiviral therapy on HCV related indolent lymphomas supports a specific role for the virus in lymphomagenesis. These observations are reinforced by numerous laboratory experiments that led to several hypothetical models of B-cell transformation by HCV. Diffuse large B-cell lymphoma (DLBCL), the most common lymphoma subtype in the western countries, has been associated to HCV infection despite its aggressive nature. This association seems particularly prominent in some geographical areas. Clinical presentation of HCV-associated DLBCL has consistently been reported to differ from the HCV-negative counterpart. Nevertheless, histopathology, tolerance to standard-of-care chemo-immunotherapy (R-CHOP or CHOP-like regimens) and final outcome of HCV-positive DLBCL patients is still matter of debate. Addition of rituximab has been described to enhance viral replication but the probability of severe hepatic complications remains low, with some exceptions (i.e., hepatitis B virus or immune immunodeficiency virus co-infected patients, presence of grade > 2 transaminases elevation, cirrhosis or hepatocarcinoma). HCV viral load in this setting is not necessarily directly associated with liver damage. Overall, treatment of HCV associated DLBCL should be performed in an interdisciplinary approach with hepatologists and hematologists with close monitoring of liver function. Available reports reveal that the final outcome of HCV-positive DLBCL that receive standard immunochemotherapy is not inferior to their HCV-negative counterpart. This review summarizes data on epidemiology, pathogenesis and therapeutic approach on HCV-associated DLBCL. Several issues that are matter of debate like clinical management of patients with transaminase elevation, criteria for discontinuing or starting immuno-chemotherapy, as well as the exact role of monoclonal antibodies will be analyzed.     

Hepatitis C virus infection and B-cell non-Hodgkin's lymphoma: a cross-sectional study in Lyon, France

OBJECTIVES: The role of hepatitis C virus (HCV) infection in the pathogenesis of non-Hodgkin's lymphoma (NHL) is controversial. A high prevalence of HCV infection in patients with NHL has been reported in Italy and Japan. By contrast, several studies in Northern Europe and Canada have not found any increased prevalence of HCV in B-cell NHL, suggesting a possible geographic variation. We sought to determine whether such an association could be found in patients treated in the Rhone-Alpes region in south-east France. Our main interest was to identify histological subtypes preferentially linked to HCV. METHODS: We determined the prevalence of anti-HCV antibodies in 212 consecutive patients with B-cell NHL diagnosed in our institution between January 1997 and December 1998. The comparison group comprised 974 patients tested for HCV before transfusion at the same hospital during the same period. RESULTS: Anti-HCV antibodies were found in six (2.8%) NHL patients. The distribution by histopathological category was as follows: three gastric mucosa-associated lymphoid tissue (MALT) lymphomas, one marginal lymphoma and two diffuse large-cell lymphomas. Anti-HCV antibodies were found in 20 (2%) of 974 comparison patients. Overall, there was a positive but non-significant trend towards an association between NHL and HCV infection (odds ratio 1.31; 95% confidence interval 0.51-3.36). However, the prevalence of HCV antibodies was significantly higher in MALT lymphoma patients than in the comparison group (odds ratio 9.87; 95% confidence interval 2.59-37.69). CONCLUSIONS: To our knowledge, this is the first French study to show an association between HCV and MALT lymphoma. These results, although derived from a small number of patients, suggest a possible role of HCV in gastric MALT lymphomagenesis.     

Pancreatic T/histiocyte-rich large B-cell lymphoma: A case report and review of literature

Primary pancreatic lymphoma(PPL)is an extremely rare form of extranodal malignant lymphoma.The most common histological subtype of PPL is diffuse large B cell lymphoma(DLBCL).In rare cases,PPL can also present as follicular lymphoma,small lymphocytic lymphoma,and T cell lymphoma either of non-Hodgkin’s lymphoma or of Hodgkin’s lymphoma.T-cell/histiocyterich large B-cell lymphoma(T/HRBCL)is an uncommon morphologic variant of DLBCL with aggressive clinical course,it is predominantly a nodal disease,but extranodal sites such as bone marrow,liver,and spleen can be involved.Pancreatic involvement of T/HRBCL was not presented before.Herein,we report a 48-year-old male who was hospitalized with complaints of jaundice,dark brown urine,pale stools,and nausea.The radiological evaluation revealed a pancreatic head mass and,following operative biopsy,the tumor was diagnosed as T/HRBCL.The patient achieved remission after six cycles of CHOP chemotherapy.Therefore,T/HRBCL can be treated similarly to the stage-matched DLBCL and both of them get equivalent outcomes after chemotherapy.     

Intravascular large B-cell lymphoma with involvement of the abdominal subcutis: a case report and literature review

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of diffuse large B-cell lymphoma (DLBCL). It commonly presents with a variety of symptoms due to occlusion of small vessels by tumor cells in different organ systems. Clinically patients may present with generalized symptoms such as fever and malaise. In western patients, there is a ‘cutaneous variant’ of IVLBCL, which demonstrates cutaneous involvement only. However, Asian patients show hemophagocytosis, which is typical of the ‘Asian variant’. Here we report a case of IVLBCL in a Chinese individual who presented with a huge mass in the subcutis of the abdomen. Wenjuan Yin, Min Li are contributed equally to this work.     

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

We report here a case of an older woman, 90 years old on admission, who presented with general deterioration, fever, abdominal pain, large hepatic mass, and was found to have an extra-nodal large B-cell lymphoma of the liver. The patient was successfully treated with multi-agent chemotherapy and followed up for 2 years with no recurrence of the disease. To the best of our knowledge this is the oldest patient reported with such a primary extra-nodal hepatic lymphoma and a remarkably favourable response to chemotherapy.     

Prevalence of hepatitis C virus infection in B-cell non-Hodgkin's lymphoma: systematic review and meta-analysis

BACKGROUND & AIMS: The aim of our study was to conduct a systematic review of studies evaluating prevalence of hepatitis C virus (HCV) infection in B-cell non-Hodgkin's lymphoma (B-NHL) and to perform a meta-analysis of case-control studies comparing this prevalence with that of a reference group. METHODS: Data sources: Electronic databases and the Cochrane Controlled Trials Register. Study selection: Studies evaluating prevalence of HCV infection in patients with B-NHL. Studies comparing HCV prevalence in B-NHL (cases) and in a reference group (controls) were included in the meta-analysis. Data extraction: Author/country, diagnostic method (serology/PCR), control type, matching/design, and VHC prevalence. Data synthesis: Prevalence of HCV infection and meta-analysis combining the odds ratios (OR). RESULTS: Forty-eight studies (5542 patients) were identified. Mean HCV infection prevalence was 13% (95% CI: 12%-14%), which was higher in Italy (20%) and Japan (14%). Ten studies compared HCV prevalence in B-NHL (17%) and healthy controls (1.5%) (OR: 10.8; 95% CI: 7.4-16), results being homogeneous; OR increased up to 14.1 when only Italian studies were considered. Sixteen studies compared HCV prevalence in B-NHL (13%) and in other hematologic malignancies (2.9%) (OR: 4.2; 95% CI: 2.5-7), also with homogeneous results; OR increased up to 7.8 when subanalysis included only Italian studies. CONCLUSIONS: HCV prevalence in patients with B-NHL is approximately 15%, higher than that reported not only in general population (1.5%) but also in patients with other hematologic malignancies (2.9%), suggesting a role of HCV in the etiology of B-NHL. The striking geographic variation in this association suggests that genetic and/or environmental factors are also involved in the pathogenesis of this disorder.     

Primary cutaneous B-cell lymphoma: an association of chronic hepatitis C infection.

Primary cutaneous B-cell lymphoma is a low-grade malignancy, distinct from other lymphomas in terms of biological activity and response to treatment. We describe a 77-year-old woman with a five-year history of chronic hepatitis C infection who developed a lower-limb lesion over a period of 3 months which was diagnosed as a high-grade cutaneous B-cell lymphoma. Despite a lack of definitive evidence implicating hepatitis C virus (HCV) in the aetiology of lymphomas, there is considerable research which establishes a strong association between these two diseases. On the basis of published research and the demonstration of HCV RNA in the lymphomatous tissue, we consider this to be a rare case of primary cutaneous lymphoma in association with hepatitis C.     

Primary intravascular large B-cell lymphoma of the lung: a review and case report

Objective

To investigate the clinicopathological features of primary intravascular large B-cell lymphoma (IVLBCL) of the lung.

Methods

Histopathological and clinical data based on lung biopsy were analyzed and used to diagnose a patient with IVLBCL of the lung.

Results

Fever and respiratory symptoms were the main presentations, lung biopsy revealed lymphoma cells in the lumen of small blood vessels. Tumor cells expressed Bcl-2, the Bcl-6, CD20, Ki67, MUM-1, Pax5, CD, CD30, and vascular endothelial CD34.

Conclusions

Primary pulmonary IVLBCL of the lung is extremely rare, on chest CT it manifests as diffuse ground glass shadow, or nodular consolidations in the lung, lactate dehydrogenase and C-reactive protein was found to increase, fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) is an important and significant diagnostic modality in its early diagnosis. Also, bronchial lung biopsy has the advantage of less trauma and high sensitive rate. R-CHOP is the main treatment for lung primary pulmonary IVLBCL of the lung; however, its prognosis is relatively poor.     

Primary sellar lymphoma: intravascular large B-cell lymphoma diagnosed as a double cancer and improved with chemotherapy,and literature review of primary parasellar lymphoma

Lymphoma is one of the causative factors of hypothalamus–pituitary dysfunction, and intravascular large B-cell lymphoma (IVLBCL) is a subtype of primary extranodal neoplasm. A 69-year-old woman visited our hospital because of general fatigue. We diagnosed her with presumable non-functional primary pituitary adenoma and subsequent dysfunction. Eight months after, the patient revisited our hospital because of dyspnea. Though we conducted systemic investigations including chest and abdomen enhanced computer tomography, transbronchial lung biopsy, and bone marrow biopsy, the diagnosis was not confirmed. Inadvertently, a breast cancer was found, and the surgical specimen proved that the patient had double cancer—adenocarcinoma and IVLBCL. Rituximab, cyclophosphamide, adriamycin, vincristine, and prednisolone regimen was initiated, and complete remission was achieved. Notably, the sellar mass returned to normal size with improved function. We reviewed 32 patients with primary parasellar lymphoma. In affected sites, both sellar and pituitary stalk (6.7%), both hypothalamus and pituitary stalk (6.7%), only sellar (63.3%), only pituitary stalk (6.7%), only hypothalamus (13.3%), and only clivus (3.3%) were observed. In hypothalamus–pituitary dysfunction, both anterior and posterior dysfunction (20.7%), only anterior dysfunction (58.6%), only posterior dysfunction (3.4%), and no dysfunction (17.2%) were observed. It seemed that hypothalamic lesion is related to both anterior and posterior dysfunction, while sellar lesion is related to mainly anterior dysfunction. In cranial nerve dysfunction, 2nd nerve dysfunction (45.2%) and 6th nerve dysfunction (35.5%) were frequently observed. It seemed that sellar lesion is related to both 2nd and 6th nerve dysfunction, while hypothalamic lesion is related to mainly 2nd nerve dysfunction.