title: PHACES syndrome associated with carcinoid endobronchial tumor.
abstract: PHACES syndrome consists of the constellation of manifestations including posterior fossa anomalies of the brain (most commonly Dandy-Walker malformations), hemangiomas of the face and scalp, arterial abnormalities, cardiac defects, eye anomalies and sternal defects. We present a case with a possible PHACES syndrome including sternal cleft and supraumbilical raphé, precordial skin tag, persistent left superior vena cava and subtle narrowing of the aorta with an endobronchial carcinoid tumor. All these anomalies were discovered on chest multi-detector CT. This is a unique case of PHACES syndrome associated with carcinoid tumor. Review of the literature revealed 3 cases of PHACES syndrome with glial tumor. The authors tried to find the relationship between PHACES syndrome and carcinoid tumors or gliomas, which all derive from the neural crest cells.
authors: Radiology Department, Sahloul Hospital, route ceinture, 4011, Sousse, Tunisia, dr.nmama@gmail.com.
authors: Nadia
authors: N
authors: Mama
authors: Dorra
authors: D
authors: H'mida
authors: Imen
authors: I
authors: Lahmar
authors: Mohamed Tahar
authors: MT
authors: Yacoubi
authors: Kalthoum
authors: K
authors: Tlili-Graiess
mesh_terms: D000293:Adolescent; D001017:Aortic Coarctation / Q000000981:diagnostic imaging*; D001984:Bronchial Neoplasms / Q000000981:diagnostic imaging*; D002276:Carcinoid Tumor / Q000000981:diagnostic imaging*; D005124:Eye Abnormalities / Q000000981:diagnostic imaging*; D005260:Female; D006801:Humans; D020752:Neurocutaneous Syndromes / Q000000981:diagnostic imaging*; D014057:Tomography, X-Ray Computed / Q000379:methods*