title: Fatal necrotizing angiotropic Epstein-Barr virus-negative large B-cell lymphoma: a case report with unusual clinicopathological features in-between lymphomatoid granulomatosis and T-cell/histiocyte-rich large B-cell lymphoma.
abstract: In the spectrum of diffuse large B-cell lymphomas (DLBCL), both T-cell/histiocyte-rich large B-cell lymphoma (TCHRBCL) and most lymphomatoid granulomatosis (LG) cases are characterized by the relative rarity of the neoplastic B-cell population, with respect to the overwhelming non-neoplastic counterpart of T cells or histiocytes. Here we report a case of aggressive B-cell lymphoma with unusual clinicopathological features partially overlapping these two entities. The patient was a previously healthy 55-year-old male, presenting with a computed tomography finding of a pelvic mass, inguinal lymphadenopathies, and pulmonary nodules. Two excisional lymph node biopsies resulted inconclusive for lymphoproliferative disease. Because of a colonic perforation, the patient underwent an urgent laparotomy, which disclosed a large pelvic abscess. The pathological examination of the surgical specimen could not discriminate between a primary aggressive B-cell lymphoproliferative disorder and an abnormal inflammatory hyper-reaction. The patient developed a septic state, not resolving until death, which occurred because of an abdominal hemorrhage. A second perimortem surgical specimen consisting of a nodal mass revealed a diagnosis of an Epstein-Barr virus-negative high-grade large B-cell lymphoma with massive necrosis, angiocentric pattern of growth, and prominent T-cell infiltrate. The unique clinicopathological features did not allow to classify this tumor within any of the recognized WHO entities, potentially representing a new clinicopathological variant of DLBCL in-between TCHRBCL and LG.
authors.affiliation: From the Haematology Clinic, Department of Clinical and Molecular Sciences, Marche Polytechnic University, Ancona (AO, ST, FS, G. Gini, PL, JO); Haematopathology Unit, Department of Experimental, Diagnostic and Specialty Medicine, S. Orsola-Malpighi Hospital, University of Bologna, Bologna (ES, SAP); Pathologic Anatomy Unit, Marche Polytechnic University, Ancona (G. Goteri); and Internal Medicine Unit, Senigallia Hospital, Senigallia (AS), Italy.
authors.forename: Attilio
authors.initials: A
authors.lastname: Olivieri
authors.forename: Elena
authors.initials: E
authors.lastname: Sabattini
authors.forename: Gaia
authors.initials: G
authors.lastname: Goteri
authors.forename: Silvia
authors.initials: S
authors.lastname: Trappolini
authors.forename: Francesco
authors.initials: F
authors.lastname: Saraceni
authors.forename: Arduino
authors.initials: A
authors.lastname: Samorì
authors.forename: Guido
authors.initials: G
authors.lastname: Gini
authors.forename: Pietro
authors.initials: P
authors.lastname: Leoni
authors.forename: Jacopo
authors.initials: J
authors.lastname: Olivieri
authors.forename: Stefano A
authors.initials: SA
authors.lastname: Pileri
mesh_terms: D017809:Fatal Outcome; D006644:Histiocytes / Q000473:pathology*; D006801:Humans; D016393:Lymphoma, B-Cell / Q000473:pathology*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000473:pathology*; D008230:Lymphomatoid Granulomatosis / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D013601:T-Lymphocytes / Q000473:pathology*