Facial "glomangiomas": large facial venous malformations with glomus cells.
BACKGROUND
"Glomangiomas" are benign cutaneous vascular lesions consisting of convoluted, abnormally formed venous channels lined by cuboidal and oval epithelioid, alpha-actin-positive, glomus cells. Three different clinical variants of glomangioma have been recognized: solitary, multiple, and nodular, or plaquelike. Inheritable forms are common.


OBJECTIVE
We describe in 7 patients (2 of them having a familial glomangiomatosis) the rare facial location of glomangiomas to differentiate this type from common facial venous malformation (VM).


METHODS
We analyzed clinical data (photographs), course, investigations (computed tomographic scans in 4 patients, magnetic resonance imaging in 6, arteriography in 2, direct puncture phlebography in 4, and pathologic examinations in all 7), and outcome with treatment.


RESULTS
Lesions were soft, composed of multiple nodules, confluent and plaquelike, deep blue or blue-to-purple, sometimes sagging, one-sided in a cheek, extending to the lips in 5 patients, to the chin in 4, and to the lower eyelid in 4. They were poorly compressible, a finding different from common facial VMs. In a young man extensive back involvement was associated. Among radiologic investigations, only magnetic resonance imaging after gadolinium enhancement offered some differential features with common VMs. However, histopathologic examination clarified the differential diagnosis: although the large tortuous venous channels were reminiscent of capillary-venous malformation, in many vessels the walls contained one or several rows of glomus cells.


CONCLUSIONS
Multiple plaquelike facial "glomangiomas" mimic a common venous malformation because of their blue hue. However, with experience, one can clinically recognize them, and their pathologic aspect is distinctive. Management should differ slightly from that for common facial VM because sclerotherapy has proven to be less effective. Therefore surgical treatment is the only helpful therapeutic option.
Mounayer
C
C
Department of Neuroradiology, Hôpital Lariboisière, Paris, France.
Wassef
M
M
Enjolras
O
O
Boukobza
M
M
Mulliken
J B
JB
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005153:Facial Neoplasms / Q000175:diagnosis / Q000473:pathology*; D005260:Female; D005918:Glomus Tumor / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D006801:Humans; D008297:Male; D012867:Skin / Q000098:blood supply* / Q000473:pathology; D012878:Skin Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D014680:Veins / Q000002:abnormalities*