ABSTRACT
Objective:
To investigate the clinicopathological features of glomuvenous malformation (GVM).
Methods:
Thirty-one cases of GVM diagnosed at the Henan Provincial People's
Hospital from January 2011 to December 2021 were collected. Their clinical and pathological features were analyzed. The expression of relevant markers was examined using
immunohistochemistry. The
patients were also followed up.
Results:
There were 16
males and 15
females in this study, with an average age of 11 years (range, 1-52 years). The locations of the
disease included 13 cases in the
limbs (8 cases in the
upper limbs, 5 cases in the
lower limbs), 9 cases in the trunks, and 9 cases in the
foot (
toes or subungual area). Twenty-seven of the cases were solitary and 4 were multifocal. The lesions were characterized by blue-
purple papules or plaques on the
skin surface, which grew slowly. The lumps became larger and appeared to be conspicuous. Microscopically, GVM mainly involved the
dermis and
subcutaneous tissue, with an overall ill-defined border. There were scattered or clustered irregular dilated
vein-like lumens, with thin walls and various sizes. A single or multiple layers of relatively
uniform cubic/glomus
cells were present at the abnormal wall, with scattered small nests of the glomus
cells. The
endothelial cells in the wall of abnormal lumen were flat or absent.
Immunohistochemistry showed that glomus
cells strongly expressed SMA, h-
caldesmon, and
collagen IV. Malformed
vascular endothelial cells expressed CD31, CD34 and ERG. No postoperative
recurrence was found in the 12 cases.
Conclusions:
GVM is an uncommon type of simple venous malformation in the superficial soft
tissue and different from the classical
glomus tumor. Morphologically, one or more layers of glomus
cells grow around the dilated venous malformation-like lumen, which can be combined with common venous malformations.