Capillary‐Lymphatic Malformation, Kaposiform Hemangioendothelioma and Delayed Kasabach‐Merritt Phenomenon

:  According to International Society for the Study of Vascular Anomalies classification, vascular anomalies are mainly divided into two groups: vascular tumors and vascular malformations. Nevertheless, a small group of patients present clinical and/or histopathologic overlapping features. We report...

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Veröffentlicht in:Pediatric dermatology 2011-07, Vol.28 (4), p.439-443
Hauptverfasser: del Pozo, Jesús, López‐Gutiérrez, Juan Carlos, Gómez‐Tellado, Manuel, Nistal, Manuel, Soler, Rafaela, Sacristán, Felipe, Tovar, J.A.
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container_end_page 443
container_issue 4
container_start_page 439
container_title Pediatric dermatology
container_volume 28
creator del Pozo, Jesús
López‐Gutiérrez, Juan Carlos
Gómez‐Tellado, Manuel
Nistal, Manuel
Soler, Rafaela
Sacristán, Felipe
Tovar, J.A.
description :  According to International Society for the Study of Vascular Anomalies classification, vascular anomalies are mainly divided into two groups: vascular tumors and vascular malformations. Nevertheless, a small group of patients present clinical and/or histopathologic overlapping features. We report a case of a 4‐month‐old boy that presented a vascular lesion on his right buttock with involvement of abdominal wall muscles, abdominal cavity and drainage to primitive iliac by a tortuous drainage vein. Surgery was performed and histopathology demonstrated a combined vascular malformation. Six months later he developed a thrombocytopenia and repeat magnetic resonance imaging revealed a new solid mass involving the majority of the abdominal cavity. At 18 months of age the patient developed a Kasabach‐Merrit phenomenon and treatment with vincristine, interferon and then acetyl‐salicilic acid and dypiridamol was started. No response in platelet counts was obtained and one more surgery was perfomed. On this occasion a histopathologic study revealed vascular malformation areas intermingled with areas of kaposiform hemangioendothelioma. This patient demonstrates the Kasabach‐Merritt phenomenon with kaposiform hemangioendothelioma arising within a pre‐existing combined vascular malformation.
doi_str_mv 10.1111/j.1525-1470.2010.01089.x
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This patient demonstrates the Kasabach‐Merritt phenomenon with kaposiform hemangioendothelioma arising within a pre‐existing combined vascular malformation.</abstract><cop>Oxford, UK</cop><pub>Blackwell Publishing Ltd</pub><pmid>20403115</pmid><doi>10.1111/j.1525-1470.2010.01089.x</doi><tpages>5</tpages></addata></record>
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ispartof Pediatric dermatology, 2011-07, Vol.28 (4), p.439-443
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source MEDLINE; Access via Wiley Online Library
subjects Abdominal Cavity - surgery
Antineoplastic Agents - therapeutic use
Antineoplastic Combined Chemotherapy Protocols - therapeutic use
Aspirin - therapeutic use
Biological and medical sciences
Blood and lymphatic vessels
Cardiology. Vascular system
Dermatology
Dipyridamole - therapeutic use
Diseases of the lymphatic vessels
Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous
Hemangioendothelioma - diagnosis
Hemangioendothelioma - drug therapy
Hemangioendothelioma - surgery
Hereditary diseases of the skin. Congenital diseases of the skin. Haemangioma of the skin, of mucosae and of soft tissue
Humans
Infant
Interferons - therapeutic use
Kasabach-Merritt Syndrome - diagnosis
Kasabach-Merritt Syndrome - drug therapy
Kasabach-Merritt Syndrome - surgery
Lymphatic Vessels - abnormalities
Lymphatic Vessels - drug effects
Lymphatic Vessels - pathology
Lymphatic Vessels - surgery
Male
Medical sciences
Sarcoma, Kaposi - diagnosis
Sarcoma, Kaposi - drug therapy
Sarcoma, Kaposi - surgery
Thrombocytopenia - diagnosis
Thrombocytopenia - drug therapy
Treatment Outcome
Vascular Neoplasms - diagnosis
Vascular Neoplasms - drug therapy
Vascular Neoplasms - surgery
Vincristine - therapeutic use
title Capillary‐Lymphatic Malformation, Kaposiform Hemangioendothelioma and Delayed Kasabach‐Merritt Phenomenon
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