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Published on: March 30, 2018
Diffuse large B-cell lymphoma with primary retroperitoneal presentation: clinico-pathologic study of nine cases
S A Pileri1, P L Zinzani, S Ascani
1The Institute of Hematology and Clinical Oncology L. & A. Seràgnoli Bologna University, Italy.
Summary
Retroperitoneal diffuse large B-cell lymphoma (PRLBCL) shows varied characteristics. Further studies are needed to understand the histogenesis and behavior of this heterogeneous group of tumors.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Diffuse large B-cell lymphoma (DLBCL) primarily presenting in the retroperitoneum (PRLBCL) is rare.
- Previous studies on PRLBCL utilized outdated diagnostic techniques.
Purpose of the Study:
- To characterize PRLBCL using modern morphologic, immunohistochemical, and molecular methods.
- To investigate the heterogeneity and potential origins of PRLBCL.
Main Methods:
- Morphologic review of nine PRLBCL cases.
- Immunohistochemical staining for cell markers (CD20, CD79a, CD3, bcl-2, bcl-6, HLA-DR, CD10).
- Molecular analysis for bcl-2 gene rearrangements.
Main Results:
- PRLBCL cases showed diffuse large cell proliferation with varying cytoplasm, sclerosis, and compartmentalization.
- Three subgroups were identified based on immunophenotype and molecular findings, suggesting potential follicular origin in some.
- Patient outcomes varied, with some achieving remission and others succumbing to the disease within months.
Conclusions:
- PRLBCL appears to be a more heterogeneous entity than previously recognized.
- Further phenotypic and molecular investigations are warranted to elucidate PRLBCL histogenesis and behavior.
- The findings prompt discussion on similarities between PRLBCL and primary mediastinal large B-cell lymphoma (PMBCL).
