Marta Feito-Rodríguez1, Alba Sánchez-Orta1, Raúl De Lucas1, Juan Carlos López-Gutiérrez2, Elena Ruiz-Bravo3, Eulalia Baselga4, Ana M Victoria5, Angela Hernández-Martín6, Minia Campos-Domínguez7, Beatriz Berenguer Fröhner8, Gloria Garnacho-Saucedo9, Angel Vera Casaño10, Asunción Vicente Villa11, José Bernabeu-Wittel12, Heinz Kutzner13, Luis Requena14. 1. Department of Dermatology, Hospital Universitario La Paz, Madrid, Spain. 2. Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain. 3. Department of Pathology, Hospital Universitario La Paz, Madrid, Spain. 4. Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. 5. Department of Dermatology, Consorcio Hospital General Universitario de Valencia, Valencia, Spain. 6. Department of Dermatology, Hospital Infantil Niño Jesús, Madrid, Spain. 7. Department of Dermatology, Hospital Universitario Gregorio Marañón, Madrid, Spain. 8. Department of Pediatric Surgery, Hospital Universitario Gregorio Marañón, Madrid, Spain. 9. Department of Dermatology, Hospital Reina Sofía, Córdoba, Spain. 10. Department of Dermatology, Hospital Materno-Infantil, Complejo Hospitalario Carlos Haya, Málaga, Spain. 11. Department of Dermatology, Hospital Sant Joan de Deu, Barcelona, Spain. 12. Department of Dermatology, Hospital Universitario Virgen del Rocío, Sevilla, Spain. 13. Dermatopathology Laboratory, Friedrichshafen, Germany. 14. Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain.
Abstract
BACKGROUND: Recent reports indicate that tufted angioma is a rare vascular neoplasm that manifests more frequently at birth than previously thought. Few studies specifically address congenital presentation. OBJECTIVES: We analyzed the clinicopathological characteristics, clinical course, and treatment of congenital tufted angioma (cTA) and evaluated variables that were indicative of problematic lesions. METHODS: We performed an observational retrospective study of 30 patients with cTA in 9 Spanish hospitals over a 14-year period. Histopathology and immunohistochemistry studies were performed. RESULTS: Congenital tufted angioma mainly affected the limbs (56.67%), followed by the face and/or neck (23.33%). Almost three-quarters of facial cTA were located over the mandibular area. Immunohistochemically, proliferating cells expressed markers of endothelial cells, with some clusters of cells, especially at the periphery of the aggregates, showing positivity for podoplanin. As no associated complications were observed in 66.67% of cases, no treatment was started. LIMITATIONS: Data were collected retrospectively. CONCLUSIONS: Our findings emphasize the clinical features and course of cTA. The possibility of cTA should be considered when a poorly defined congenital infiltrative vascular tumor with(out) overlying hirsutism appears over the mandibular area. Location on the face and/or neck requires a more comprehensive workup, since potentially severe complications often appear early.
BACKGROUND: Recent reports indicate that tufted angioma is a rare vascular neoplasm that manifests more frequently at birth than previously thought. Few studies specifically address congenital presentation. OBJECTIVES: We analyzed the clinicopathological characteristics, clinical course, and treatment of congenital tufted angioma (cTA) and evaluated variables that were indicative of problematic lesions. METHODS: We performed an observational retrospective study of 30 patients with cTA in 9 Spanish hospitals over a 14-year period. Histopathology and immunohistochemistry studies were performed. RESULTS:Congenital tufted angioma mainly affected the limbs (56.67%), followed by the face and/or neck (23.33%). Almost three-quarters of facial cTA were located over the mandibular area. Immunohistochemically, proliferating cells expressed markers of endothelial cells, with some clusters of cells, especially at the periphery of the aggregates, showing positivity for podoplanin. As no associated complications were observed in 66.67% of cases, no treatment was started. LIMITATIONS: Data were collected retrospectively. CONCLUSIONS: Our findings emphasize the clinical features and course of cTA. The possibility of cTA should be considered when a poorly defined congenital infiltrative vascular tumor with(out) overlying hirsutism appears over the mandibular area. Location on the face and/or neck requires a more comprehensive workup, since potentially severe complications often appear early.