Multifocal rapidly involuting congenital hemangioma: A link to chorangioma

Abstract

Common infantile hemangioma is intriguing because of its variable presentation, rapid postnatal growth and slow regression in childhood. Interest in this tumor has increased with the recognition that it can be associated with structural anomalies in the craniofacial and ventral-caudal regions. The phenotype has expanded by characterization of rare vascular tumors that arise in the fetus and manifest at birth as rapidly involuting congenital hemangioma (RICH) or non-involuting congenital hemangioma (NICH). We describe a boy born with three RICH on the abdominal wall; one extended into the base of the umbilical cord. Two weeks later a small, infantile hemangioma arose on his neck. This patient stimulated a review of what is known about placental vascular tumors and their possible relationship to fetal and infantile hemangiomas. We suggest that chorangioma and umbilical cord hemangioma are clinically and histopathologically similar to cutaneous and hepatic RICH. These placental vascular tumors can also occur in conjunction with solitary and multiple infantile hemangiomas.

Rapid Involuting Congenital Hemangioma in the Setting of PHACE Association

“…The cause of diverging behaviors has not been discovered, although the idea of somatic mutations leading to varying phenotypes has been introduced. 18,19 Mulliken and colleagues 18 introduced the idea of a unified theory of origin for congenital and common hemangiomas; application of this hypothesis, in the case of our patient, allows a plausible explanation for the development of the rare RICH as the hemangioma within a polymalformative syndrome such as PHACE association.…”

Health care of children with facial hemangioma in the treatment of beta-blockers

“…Mogu biti vidljivi odmah po rođenju, ali većina se javlja tokom prva dva meseca života.Tokom prva tri meseca razvoja, hemangiomi porastu do 80% svoje maksimalne veličine. Tada nastupa period stagnacije i od šestog meseca života pa do 18 meseci, odvija se zadnja faza rasta (7). Od tada mogu da involuiraju do 60 %, a do 9 god.…”

“…Celokupan proces je obeležen fazama proliferacije i involucije (8). Facijalni hemangiom (FACE syndrome) predstavlja jedan od najtežih oblika i manifestuje se lokalizacijom u predelu zadnje lobanjske jame, abnormalnostima oka, uha, usta i usne šupljine, kao i neurovaskularnim sindromom koji se karakteriše velikim segmentom hemangioma na glavi i vratu zajedno sa urođenim anomalijama mozga, srca, očiju i grudnog koša (1,3,7,8).…”

“…Tokom trećeg trimestra fetalnog razvoja, nezrele endotelne ćelije koegzistiraju sa nezrelim pericitima, koji održavaju svoju proliferativnu sposobnost za ograničen period tokom postnatalnog života. Angiogeni peptidi kao što su beta-fibroblastni faktor rasta, faktor rasta vaskularnog endotela, koji proliferišu nuklearnim antigenom ćelija, indikuju proliferaciju ovih nezrelih ćelija, što dovodi do razvoja hemangioma (3,7,8).…”

“…Najčešće su: krvarenje, ulceracija, bol, opstrukcija disajnog puta (supra i subglotični hemangiom), gubitak vida, astigmatizam, strabizam (periorbitalni Infantilni hemangiom), gubitak sluha, trombocitopenija, anemija, unutrašnje krvarenje, oštećenje CNS-a i ožiljci. Teške kliničke manifestacije su primećene kod 10 % sa hemangiomom i zahtevaju bolničko lečenje, dok je samo 1% ove dece životno ugroženo i to pretežno zbog opstrukcije disajnih puteva, iskrvarenja ili infekcije ulcerisanog hemangioma koji je doveo do poremećaja opšteg stanja organizma (3,7,8,9).…”