Lymphangiosarcoma complicating extensive congenital mixed vascular malformations
| Autor: | Julie Powell, Josée Dubois, Victor Kokta, Mehdi Agoumi, Rola Al Dhaybi |
|---|---|
| Rok vydání: | 2010 |
| Předmět: |
Vincristine
medicine.medical_specialty Vascular Malformations Autopsy Skin Diseases Vascular Fatal Outcome Upper trunk Prednisone medicine Lymphangiosarcoma Humans business.industry Infant Newborn Infant medicine.disease Response to treatment Surgery Lymphatic system medicine.anatomical_structure Female Cardiology and Cardiovascular Medicine Cutaneous vascular malformations business medicine.drug |
| Zdroj: | Lymphatic research and biology. 8(3) |
| ISSN: | 1557-8585 |
| Popis: | Pediatric hepatic angiosarcoma is a very rare malignant vascular tumor. A few cases have shown pediatric hepatic angiosarcoma occurring on a background of preexisting vascular lesions. We report the case of a newborn girl who presented extensive limbs and upper trunk cutaneous mixed vascular malformations at birth. These malformations were associated with thrombocytopenia. Cutaneous biopsies revealed complex vascular malformations with a significant lymphatic component. Compressive body suit therapy led to regression of the limbs' cutaneous vascular malformations. At the age of 9 months, the patient presented multiple heterogeneous hepatosplenic nodules. Aggressive treatment with prednisone, vincristine, and hepatosplenic embolizations resulted in initial improvement of the hepatosplenic lesions for few months, followed by an increase of the lesions with failure of response to treatment despite adding alpha-interferon-2b to treatment. The patient died at the age of 19 months. The autopsy's pathological examination revealed a hepatic-based angiosarcoma with plurimetastatic dissemination to the spleen, lungs, peritoneum, pleura, mesenteric linings as well as the serosa of the stomach and small intestine. Multiple cutaneous and visceral complex capillaro-lymphatico-venous malformations were also identified. We hypothesize that these multiple extensive mixed vascular malformations were associated with chronic lymphedema which probably predisposed to the development of the angiosarcoma in our patient. |
| Databáze: | OpenAIRE |
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