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Intravascular lymphomatosis presenting within angiolipomas
M E Smith1, M D Stamatakos, T S Neuhauser
1Department of Pathology, Wilford Hall Medical Center, Lackland Air Force Base, TX 78236-5300, USA.
Annals of Diagnostic Pathology
|April 11, 2001
Summary
This case report details intravascular lymphomatosis found within an angiolipoma. This aggressive cancer, often diagnosed postmortem, presents varied symptoms and requires consideration in vascular invasion diagnoses.
Area of Science:
- Oncology
- Pathology
- Hematology
Background:
- Intravascular lymphomatosis (IVL) is a rare, aggressive hematologic malignancy characterized by the proliferation of lymphoid cells within blood vessels.
- It can affect any organ system, with common presentations involving the central nervous system and skin.
- Diagnosis is often challenging and frequently made postmortem due to its insidious onset and diverse clinical manifestations.
Observation:
- A case of IVL was identified within an angiolipoma in a 73-year-old woman with a history of breast cancer.
- Microscopic examination revealed vascular spaces filled with atypical, pleomorphic cells expressing CD45RB and CD20.
- The patient later developed widespread lesions and neurological symptoms, ultimately succumbing to the disease.
Findings:
- Immunohistochemical analysis confirmed the atypical cells as B-cell lymphocytes, consistent with IVL.
- The angiolipoma served as an unusual site for the initial presentation of IVL.
- The patient's progression to widespread disease and CNS involvement highlights the aggressive nature of IVL.
Implications:
- This case underscores the importance of considering IVL in the differential diagnosis of vascular lesions, especially when atypical cells are present.
- Recognizing the protean manifestations of IVL is crucial for earlier diagnosis and potential intervention.
- The findings emphasize the need for vigilance in evaluating vascular anomalies, particularly in patients with a history of malignancy.