[Primary retroperitoneal diffuse large B cell lymphoma with sclerosis mimicking idiopathic retroperitoneal fibrosis]. 2012

Mayumi Hatsuse, and Shin-ichi Fuchida, and Akira Okano, and Satoshi Murakami, and Harue Haruyama, and Chihiro Shimazaki
Department of Hematology, Kyoto Social Insurance Hospital, Japan.

A 81-year-old man with a history of gastrointestinal stromal tumor (GIST) was admitted to our hospital due to increased level of LDH, and further testing revealed a serum sIL-2R level of 15,489 U/ml. CT scan demonstrated retroperitoneal fibrosis without lymphadenopathy, and bilateral hydronephrosis. He died of aspiration pneumonia, and post-mortem biopsy revealed diffuse infiltration of medium to large-sized lymphoid cells positive for CD20, CD79a, and BCL2 in the retroperitoneal space with mild fibrosis. These findings suggested a diagnosis of primary retroperitoneal diffuse large B-cell lymphoma (PRLBCL). PRLBCL is extremely rare, and should be included in the differential diagnosis in cases of retroperitoneal fibrosis.

UI MeSH Term Description Entries
D008297 Male Males
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000369 Aged, 80 and over Persons 80 years of age and older. Oldest Old
D001344 Autopsy Postmortem examination of the body. Autopsies,Post-Mortem Examination,Postmortem Examination,Examination, Post-Mortem,Examination, Postmortem,Examinations, Post-Mortem,Examinations, Postmortem,Post Mortem Examination,Post-Mortem Examinations,Postmortem Examinations
D012185 Retroperitoneal Fibrosis A slowly progressive condition of unknown etiology, characterized by deposition of fibrous tissue in the retroperitoneal space compressing the ureters, great vessels, bile duct, and other structures. When associated with abdominal aortic aneurysm, it may be called chronic periaortitis or inflammatory perianeurysmal fibrosis. Ormond Disease,Perianeurysmal Fibrosis, Inflammatory,Periaortitis, Chronic,Fibrosis, Inflammatory Perianeurysmal,Fibrosis, Perianeurysmal Inflammatory,Idiopathic Retroperitoneal Fibrosis,Inflammatory Fibrosis, Perianeurysmal,Inflammatory Perianeurysmal Fibrosis,Ormond's Disease,Perianeurysmal Inflammatory Fibrosis,Chronic Periaortitides,Chronic Periaortitis,Disease, Ormond,Disease, Ormond's,Fibroses, Retroperitoneal,Fibrosis, Retroperitoneal,Periaortitides, Chronic,Retroperitoneal Fibroses
D012187 Retroperitoneal Space An area occupying the most posterior aspect of the ABDOMINAL CAVITY. It is bounded laterally by the borders of the quadratus lumborum muscles and extends from the DIAPHRAGM to the brim of the true PELVIS, where it continues as the pelvic extraperitoneal space. Retroperitoneal Spaces,Space, Retroperitoneal,Spaces, Retroperitoneal
D012598 Sclerosis A pathological process consisting of hardening or fibrosis of an anatomical structure, often a vessel or a nerve. Scleroses
D016403 Lymphoma, Large B-Cell, Diffuse Malignant lymphoma composed of large B lymphoid cells whose nuclear size can exceed normal macrophage nuclei, or more than twice the size of a normal lymphocyte. The pattern is predominantly diffuse. Most of these lymphomas represent the malignant counterpart of B-lymphocytes at midstage in the process of differentiation. Diffuse Large B-Cell Lymphoma,Diffuse, Large B-Cell, Lymphoma,Histiocytic Lymphoma, Diffuse,Lymphoma, Histiocytic, Diffuse,Diffuse Large-Cell Lymphoma,Histiocytic Lymphoma,Large Lymphoid Lymphoma, Diffuse,Large-Cell Lymphoma, Diffuse,Lymphoma, Diffuse Large-Cell,Lymphoma, Histiocytic,Lymphoma, Large Cell, Diffuse,Lymphoma, Large Lymphoid, Diffuse,Lymphoma, Large-Cell, Diffuse,Diffuse Histiocytic Lymphoma,Diffuse Histiocytic Lymphomas,Diffuse Large B Cell Lymphoma,Diffuse Large Cell Lymphoma,Diffuse Large-Cell Lymphomas,Histiocytic Lymphomas,Large Cell Lymphoma, Diffuse,Lymphoma, Diffuse Histiocytic,Lymphoma, Diffuse Large Cell
D017809 Fatal Outcome Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept. Fatal Outcomes,Outcome, Fatal,Outcomes, Fatal

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