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 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.
del Pozo
Jesús
J
Unit of Pediatric Vascular Anomalies, Department of Dermatology, CHU A Coruña, Spain. jesus.del.pozo.losada@sergas.es
López-Gutiérrez
Juan Carlos
JC
Gómez-Tellado
Manuel
M
Nistal
Manuel
M
Soler
Rafaela
R
Sacristán
Felipe
F
Tovar
J A
JA
D034841:Abdominal Cavity / Q000601:surgery; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001241:Aspirin / Q000627:therapeutic use; D004176:Dipyridamole / Q000627:therapeutic use; D006390:Hemangioendothelioma / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery; D006801:Humans; D007223:Infant; D007372:Interferons / Q000627:therapeutic use; D059885:Kasabach-Merritt Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery; D042601:Lymphatic Vessels / Q000002:abnormalities* / Q000187:drug effects / Q000473:pathology / Q000601:surgery; D008297:Male; D012514:Sarcoma, Kaposi / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery; D013921:Thrombocytopenia / Q000175:diagnosis / Q000188:drug therapy; D016896:Treatment Outcome; D019043:Vascular Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery; D014750:Vincristine / Q000627:therapeutic use
D000970:Antineoplastic Agents; D014750:Vincristine; D004176:Dipyridamole; D007372:Interferons; D001241:Aspirin