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Angiocentric and intravascular lymphomas.

D Tomasini1, E Berti

  • 1Unit of Dermatology, Busto Arsizio Hospital, Busto Arsizio, Varese, Italy - emilio.berti@unimib.it.

Giornale Italiano Di Dermatologia E Venereologia : Organo Ufficiale, Societa Italiana Di Dermatologia E Sifilografia
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Angiocentric and intravascular lymphomas are rare, aggressive B, T, or NK/T cell cancers often involving multiple organs and frequently linked to Epstein-Barr virus (EBV). Early diagnosis is crucial to differentiate them from inflammatory conditions.

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Area of Science:

  • Hematology
  • Oncology
  • Pathology

Background:

  • Angiocentric and intravascular lymphomas encompass diverse subtypes characterized by neoplastic lymphocytes within blood vessels or inflammation centered on blood vessels.
  • These lymphomas often exhibit multiorgan involvement, a poor prognosis, and frequent Epstein-Barr virus (EBV) integration.
  • Early-stage presentations can be misdiagnosed as inflammatory diseases, complicating timely diagnosis and treatment.

Purpose of the Study:

  • To provide a comprehensive review of intravascular and angiocentric lymphomas.
  • To detail histopathologic, immunophenotypic, and molecular data for accurate diagnosis.
  • To differentiate these lymphomas from other lymphoproliferative disorders and vasculitides with overlapping features.

Main Methods:

  • Review of histopathologic, immunophenotypic, and molecular data.
  • Comparison with other lymphoproliferative disorders and vasculitides.
  • Analysis of treatment approaches and outcomes.

Main Results:

  • These lymphomas are histopathologically defined by neoplastic lymphocyte infiltration of blood vessels or perivascular inflammation.
  • Common features include multiorgan involvement, poor prognosis, and frequent EBV association.
  • Distinguishing these from inflammatory conditions and other lymphoid infiltrates is critical for appropriate management.

Conclusions:

  • Accurate histopathologic, immunophenotypic, and molecular characterization is essential for diagnosing angiocentric and intravascular lymphomas.
  • Understanding these features aids in differentiating them from mimics like vasculitis and secondary vascular infiltration.
  • Therapeutic strategies may involve chemotherapy, potentially combined with antiviral agents for EBV-related cases.