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Published on: May 1, 2015
Lymphangiosarcoma complicating extensive congenital mixed vascular malformations.
Rola Al Dhaybi1, Mehdi Agoumi, Julie Powell
1Division of Dermatology, CHU Sainte Justine, University of Montreal, Montreal, Quebec, Canada. roladhaybi@yahoo.com
Lymphatic Research and Biology
|September 25, 2010
Summary
Pediatric hepatic angiosarcoma, a rare vascular tumor, may arise from extensive congenital vascular malformations. This case highlights a fatal outcome in an infant with complex malformations and subsequent angiosarcoma.
Area of Science:
- Pediatric Oncology
- Vascular Malformations
- Pathology
Background:
- Pediatric hepatic angiosarcoma is an exceptionally rare malignancy.
- Existing vascular lesions may precede the development of hepatic angiosarcoma in children.
Observation:
- A newborn presented with extensive limb and trunk cutaneous mixed vascular malformations and thrombocytopenia.
- Initial treatment with compressive therapy regressed cutaneous lesions.
- At 9 months, the infant developed hepatosplenic nodules, initially responsive to aggressive treatment but later progressing.
Findings:
- Autopsy revealed hepatic angiosarcoma with widespread metastasis (spleen, lungs, peritoneum, etc.).
- Extensive cutaneous and visceral complex capillaro-lymphatico-venous malformations were noted.
- The patient died at 19 months of age.
Implications:
- This case suggests a potential link between extensive congenital mixed vascular malformations and the development of pediatric hepatic angiosarcoma.
- Chronic lymphedema secondary to malformations may be a predisposing factor.
- Further research is needed to understand this rare association and guide potential therapeutic strategies.
