Congenital tufted angioma: A multicenter retrospective study of 30 cases

Abstract

BackgroundRecent 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.ObjectivesWe analyzed the clinicopathological characteristics, clinical course, and treatment of congenital tufted angioma (cTA) and evaluated variables that were indicative of problematic lesions.MethodsWe 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.ResultsCongenital 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.LimitationsData were collected retrospectively.ConclusionsOur 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.

Destructive (Erosive) Tufted Angioma/Kaposiform Hemangioendothelioma of the Eyelid

“…The lesion progresses slowly over months to years and the size of the lesion can be variable, though rarely it may undergo spontaneous regression [1], [2]. Congenital TA/KHE should be expected when a poorly defined congenital infiltrating vascular tumor with(out) overlying hirsutism appears over the mandibular area, where about three-fourth of all facial congenital TA/KHE develop [3].…”

Topical tacrolimus reduces the severe pain of tufted angioma: Case report

“…Lesions of TA often present as red painful patches/plaques on the neck, trunk, and extremities. The incidence of pain among all patients is approximately 20%, which often increases as the patients develop Kasabach‐Merritt Syndrome 2‐4 . Although surgical excision has been recommended for most cases as a specific treatment, medication therapy for TA with large lesion range and pain remains a clinical intricacy 1,5 .…”

“…is approximately 20%, which often increases as the patients develop Kasabach-Merritt Syndrome. [2][3][4] Although surgical excision has been recommended for most cases as a specific treatment, medication therapy for TA with large lesion range and pain remains a clinical intricacy. 1,5 In a previous study, we have reported two patients with TA showing response after topical tacrolimus application.…”

Modern classification of hemangiomas

“…Пучкова ангіома -рідкісна доброякісна судинна пухлина, яка вперше була описана у 1949 році K. Nakagawa як ангіобластома, у 1971 році A. MacMillan і R.H. Champion визначили її як прогресуючу капілярну гемангіому [36], а у 1976 році вона отримала остаточну назву «пучкова ангіома» [30]. Вона частіше виявляється протягом перших п'яти років життя (у близько 50% дітей протягом першого року) [18]. Частота вродженої пучкової ангіоми може сягати 78% від усіх випадків пучкової ангіоми [46].…”

“…Частіше пучкова ангіома локалізується на кінцівках [46,66], верхній половині тулуба та шиї [18], животі, обличчі та геніталіях [46].…”