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Multifocal mantle cell lymphoma in situ in the setting of a composite lymphoma
Caroline Sloan1, Qun-Bin Xiong1, Anne Crivaro1
1From the Department of Pathology and Laboratory Medicine, Perelman School of Medicine, Hospital of the University of Pennsylvania, Philadelphia, and.
Insights
Mantle cell lymphoma in situ (MCLIS) was multifocal and accompanied by marginal zone lymphoma (MZL). This unique case challenges the typical presentation of MCLIS, offering new insights into its pathogenesis.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Mantle cell lymphoma in situ (MCLIS) is characterized by neoplastic cells confined to mantle zones.
- MCLIS is typically histologically inapparent and lacks expansion beyond the mantle zone.
Observation:
- A unique case of MCLIS was identified in conjunction with a more prominent nodal marginal zone lymphoma (MZL).
- The MCLIS was multifocal, detected across inguinal lymph node, porta hepatis lymph node, and bone marrow.
- The neoplastic cells did not exhibit a typical mantle zone distribution, likely due to nodal architecture destruction by MZL.
Findings:
- Histologically covert MCLIS was detected using flow cytometry, immunohistochemistry, cytogenetics, and FISH.
- MCLIS was consistently associated with a histologically dominant MZL.
- The typical mantle zone growth pattern of MCLIS was absent in this case.
Implications:
- This case provides novel insights into the pathogenesis of MCLIS.
- It highlights the importance of advanced diagnostic techniques for detecting multifocal MCLIS.
- The interaction between MCLIS and MZL may influence the growth patterns and detection of MCLIS.
Objectives:
Mantle cell lymphoma in situ (MCLIS) consists of immunophenotypically defined but histologically inapparent neoplastic cells restricted to narrow mantle zones, without expansion or invasion beyond the mantle zone. We report a unique case of MCLIS associated with a much more manifest nodal marginal zone lymphoma (MZL) in an inguinal lymph node, porta hepatis lymph node, and bone marrow.
Methods:
Biopsies from all three locations were evaluated using standard H&E-stained sections, immunohistochemistry, flow cytometry, metaphase cytogenetics, and/or fluorescence in situ hybridization (FISH).
Results:
This case is unique for three reasons. First, the histologically covert mantle cell lymphoma was multifocal, detected in all three locations using one or more of flow cytometry, immunohistochemistry, cytogenetics, and FISH. Second, the MCLIS was always accompanied by a more histologically dominant MZL. Third, where evaluable, it did not grow in an appreciable mantle zone distribution, presumably due to destruction of the normal nodal architecture by the neoplastic MZL cells and the resulting absence of recognizable follicles and mantle zones.
Conclusions:
This unique case provides new insight into the pathogenesis of MCLIS.
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