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Dabska tumor arising in lymphangioma circumscriptum
Patrick O Emanuel1, Robert Lin, Lester Silver
1Department of Dermatopathology, Mount Sinai Medical Center, New York, NY 10128, USA. patrick.emanuel@mssm.edu
Journal of Cutaneous Pathology
|December 22, 2007
Summary
This case report details a rare Dabska tumor (DT) found within a congenital lymphangioma circumscriptum in a young female. The study highlights the tumor
Area of Science:
- Vascular Oncology
- Dermatopathology
- Pediatric Oncology
Background:
- Lymphangioma circumscriptum is a benign congenital vascular malformation.
- Dabska tumor (DT) is a rare vascular neoplasm of intermediate malignant potential, often of lymphatic origin.
- DT has been previously described within lymphangiomas, but not specifically lymphangioma circumscriptum.
Observation:
- A 14-year-old female presented with a large congenital lymphangioma circumscriptum on her thigh.
- Biopsy revealed intravascular papillary projections with atypical endothelial cells within the lymphangioma's vascular channels, consistent with DT.
- The lymphangioma circumscriptum recurred during a 9-year follow-up period.
Findings:
- This is the first documented case of Dabska tumor arising in a pre-existing lymphangioma circumscriptum.
- The histological findings confirmed the coexistence of both vascular anomalies.
- No recurrence or metastasis of the Dabska tumor was observed during the follow-up period.
Implications:
- This case expands the understanding of Dabska tumor's association with lymphangioma circumscriptum.
- It underscores the importance of vigilant histopathological examination of lymphangiomas for potential malignant transformation.
- Long-term monitoring is crucial for patients with lymphangioma circumscriptum, even after recurrence, to detect any neoplastic changes.
