Fibrin-associated Large B-cell Lymphoma Research Articles

From the archives of MD Anderson Cancer Center. EBV-positive fibrin-associated large B-cell lymphoma in an ovarian leiomyoma with cystic degeneration: A case report and discussion of differential diagnosis

Fibrin-associated large B-cell lymphoma (FA-LBCL) is a rare type of lymphoma usually associated with Epstein-Barr virus (EBV) infection. We report a case incidentally detected in a right ovarian mass of a 53-year-old woman. The patient presented with bloating and weight gain over 8 months. Imaging studies showed a 20.7 cm, complex right adnexal mass. Total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. Macroscopic examination revealed a 25 × 18.5 × 9.5 cm predominantly cystic right ovarian mass with focal solid areas. Microscopically, most of the mass was a leiomyoma with hyaline necrosis and extensive cystic degeneration. In areas, the cyst showed focally necrotic, fibrinous material associated with small aggregates of round and atypical lymphoid cells with prominent karyorrhexis and mitotic activity These large cells were confined within the cystic spaces. Immunohistochemical analysis showed that the atypical cells were positive for CD20, CD30, CD79a and MUM1/IRF4, and were negative for CD3, CD10 and BCL6 supporting B-cell lineage. In situ hybridization for Epstein-Barr virus-encoded RNA (EBER ISH) was also positive in the atypical cells supporting the diagnosis of EBV-positive fibrin-associated large B-cell lymphoma. The patient subsequently received four cycles of chemotherapy using rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP). Computed tomography (CT) scan of the neck, chest, abdomen and pelvis 5 months after the last chemotherapy cycle showed no evidence of disease. After a follow-up of 37 months, the patient is alive with no evidence of disease. This report is being used to discuss the salient features of this rare entity and its differential diagnosis.

Fibrin-Associated Large B-Cell Lymphoma in a Young Patient in the Setting of Pleural Effusion

Abstract Introduction/Objective Fibrin-associated large B-cell lymphoma (FA-LBCL) is a proliferation of EBV-positive large B- cells in association with fibrinous debris, typically in the context of prostheses, hematomas, pseudocysts and myxomas. It is a newly described entity and is extremely rare, with no deaths reported as a direct result. It is indolent and not visible on imaging, instead being found incidentally on microscopy. It is mainly a diagnosis of exclusion and is characterized by sheets of neoplastic lymphocytes contained within a fibrin background. The median age of presentation is 55, with 75% of cases being reported among elderly patients. The prognosis is excellent and surgery is considered curative. It needs to be distinguished from malignant lymphomas, mainly diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI), which is also EBV-positive but occurs in elderly patients with a longstanding history of inflammation. The two have identical immunohistochemical profiles but vastly different clinical presentations and outcomes. Methods/Case Report A 35-year-old male with end-stage renal disease and a history of recurrent pleural effusions presented with dyspnea. CT showed a large loculated right-sided pleural effusion with no lymphadenopathy. Pleural fluid cytology was negative for any malignant cells. He underwent decortication to relieve pleural rind constriction. Microscopy revealed small layers of fibrin with an infiltrate of atypical, EBV-positive B-cells with a Ki-67 index of 60%. As the morphological findings and clinical presentation did not fit other known lymphomas, the case was signed out as FA-LBCL. The patient has followed up with oncology and PET scans have been negative for any malignancy. He is currently on the waiting list for a renal transplant. Results (if a Case Study enter NA) NA Conclusion FA-LBCL is a rare benign neoplasm, identified incidentally and cured by surgical resection. It is mainly diagnosed in the elderly, but the number of cases is limited and the age distribution may be skewed. This case involved a much younger patient and is the first known diagnosis in the pleural setting. It is important to differentiate this entity from DLBCL-CI, which is aggressive and requires intense oncological treatment. In addition, patients with FA-LBCL can undergo immediate solid organ transplantation, whereas those with DLBCL-CI need to be in remission for at least three years before receiving a transplant.

Splenic cyst deroofing complicated with B lymphoma

BackgroundSplenic cysts are uncommon and very rarely malignant therefore their treatment isn’t standardized. In case of symptomatic cysts different surgical approaches have been suggested. Primary malignant lymphoma of the spleen comprises less than 1% of non-Hodgkin’s lymphomas. To our knowledge, only 203 cases of splenic large B-cell lymphoma (LBCL) have been reported to date and only 2 of them were fibrin-associated splenic cysts.Case presentation27-year-old model with a 19 × 13 cm splenic cyst without data of malignancy in the preliminary study and therefore treated with laparoscopic deroofing. After histological diagnosis of LBCL with a fibrin/EBV-associated splenic pseudocyst, the patient received 4 cycles of Rituximab and a laparoscopic splenectomy was performed due to resurgence of the pseudocyst. No evidence of malignancy has been found during follow up (EBV viral load every 3 months during the first year, PET-CT every 6 months during the first year and annual afterwards) performed after the splenectomy.Discussion and conclusionsThe value of tumor markers and radiology for diagnosis of splenic cysts is put into question.Only 60 cases of Fibrin-associated LBCL (FA-LBCL) have been described in the literature therefore there are no treatment guidelines for them even though surgery together with systemic treatment has been the prevalent route with good results in most cases.

Next Generation Sequencing Analysis of Fibrin-associated Large B-cell Lymphoma Reveals Pathogenic Single Nucleotide Variants.

Fibrin-associated large B-cell lymphoma (FA-LBCL) is a newly identified subtype of Epstein-Barr virus (EBV)-associated lymphoma. Arising within fibrinous material in confined spaces, FA-LBCL is associated with chronic inflammation. We herein report histopathologic features and molecular alterations of three cases of FA-LBCL to refine this new disease entity. We performed immunohistochemical staining for CD3, CD20, CD10, Bcl-2, Bcl-6, MUM-1, CD10, and c-Myc and in situ hybridization for EBV-encoded RNA. Additionally, targeted DNA sequencing was conducted using commercially available gene panels. Three cases of FA-LBCL developed underlying lesions of retroperitoneal cyst, cardiac myxoma, and pancreatic cyst. Histopathologic features of these lesions were characterized by aggregates of atypical large cells in a background of fibrinous cellular debris. Atypical lymphoid cells were positive for CD20, Bcl-2, MUM-1, and EBV-in situ hybridization, negative for CD10, and variably positive for Bcl-6 and c-Myc. NGS analysis revealed the presence of pathogenic mutations in BRIP1, SOCS1, and KRAS. This is the first report of NGS analysis in FA-LBCL cases. It provides precise clinicopathological and molecular traits and allows its recognition as a new entity.

Fibrin-associated large B-cell lymphoma arising in an endovascular graft: first case report in Korea.

Fibrin-associated large B-cell lymphoma (FA-LBCL) is an extremely rare subtype of LBCL that consists of microscopic aggregates of atypical large B cells in the background of fibrin. Here, we report the first case of FA-LBCL in Korea. A 57-year-old male presented with a large amount of thrombus in the thoracic aorta during follow-up for graft replacement of the thoracoabdominal aorta 8 years prior. The removed thrombus, measuring 4.3 × 3.1 cm, histologically exhibited eosinophilic fibrinous material with several small clusters of atypical lymphoid cells at the periphery. The atypical cells were positive for CD20 by immunohistochemistry and for Epstein-Barr virus by in situ hybridization. The Ki-67 proliferation rate was 85%. The patient was still alive with no recurrence at the 7-year follow-up after thrombectomy. Although the diagnosis can be very difficult and challenging due to its paucicellular features, pathologists should be aware of FALBCL, which has likely been underestimated in routine evaluations of thrombi.

Fibrin-associated large B-cell lymphoma around vascular prosthesis mimicking recurrent abdominal aortic aneurysm

Fibrin-associated large B-cell lymphoma around vascular prosthesis mimicking recurrent abdominal aortic aneurysm

Fibrin-associated large B-cell lymphoma: first case report within a cerebral artery aneurysm and literature review

BackgroundFibrin-associated diffuse large B-cell lymphoma (FA-DLBCL) is a rare Epstein-Barr virus (EBV) positive lymphoproliferative disorder included in the current World Health Organization (WHO) classification. It arises within fibrinous material in the context of hematomas, pseudocysts, cardiac myxoma or in relation with prosthetic devices. In these clinical settings the diagnosis requires an high index of suspicion, because it does not form a mass itself, being composed of small foci of neoplastic cells. Despite overlapping features with diffuse large B-cell lymphoma associated with chronic inflammation, it deserves a separate classification, being not mass-forming and often following an indolent course.Case presentationA 64-year-old immunocompetent woman required medical care for cerebral hemorrhage. Computed Tomography (CT) angiography identified an aneurysm in the left middle cerebral artery. A FA-DLBCL was incidentally identified within thrombotic material in the context of the arterial aneurysm. After surgical removal, it followed a benign course with no further treatment.ConclusionsThe current case represents the first report of FA-DLBCL identified in a cerebral artery aneurysm, expanding the clinicopathologic spectrum of this rare entity. A complete literature review is additionally made.