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. 2021 Dec 8;50(12):1341-1345.
doi: 10.3760/cma.j.cn112151-20210602-00402.

[Clinicopathological features of verrucous hemangioma]

[Article in Chinese]
Affiliations

[Clinicopathological features of verrucous hemangioma]

[Article in Chinese]
P H Fan et al. Zhonghua Bing Li Xue Za Zhi. .

Abstract

Objective: To investigate the clinicopathological features, and differential diagnosis of verrucous hemangioma (VH). Methods: Twenty-eight VH cases diagnosed from 2005 to 2020 in Henan Provincial People's Hospital, Zhengzhou, China were analyzed retrospectively. Immunohistochemical studies were used to detect diagnostic markers. The mutation status of PIK3CA (exons 9 and 20) was detected using fluorescence PCR. Results: There were 13 males and 15 females in 28 cases, with the male to female ratio of 1.0∶1.2. There were 25 patients under the age of 18 years. The age range was from 10 months to 56 years (mean, 9.7 years; median, 4.5 years). There were 17 cases occurred in the lower extremities, 7 in the upper extremities and 4 in the trunk. All 28 cases were irregular red patches on the skin, which grew slowly. Some of them were thickened with uneven surface, which was light pink or red-white. Skin lesions of the 7 cases ranged from dark red and reddish brown, with a rough and hard surface. Satellite foci were present. Microscopically, 28 cases had a wide range of pathological features. Dilated, malformed vessels were observed from dermal papilla to deep soft tissue. Among them, the dermal papillary layer was mainly composed of many proliferating and expanding thin-walled capillaries and cavernous blood vessels. Thin-walled small vessels were found in the dermal reticular layer and subcutaneous fascia layer, with no obvious endothelial cell proliferation, occasional papillary hyperplasia, and lobular distribution of the malformed vessels in the fascia layer mixed with the fibroadipose tissue. There was epidermal papillary hyperplasia with hyperkeratosis and parakeratosis, lengthening and mutual fusion of epithelial horns. Immunohistochemistry showed that CD31, CD34, ERG and WT-1 were diffusely and strongly positive. The expression of GLUT-1 was present in superficial dermal vascular endothelial cells, but undetectable in the deep layer. The PIK3CA tests of 13 cases showed that no somatic mutations were found in exons 9 and 20. Twenty-five patients were followed up for 5 months to 10 years. Seven patients underwent multiple surgical resections and plastic surgeries due to the large size, and 8 patients had recurrence. Conclusions: VH is a rare congenital vascular malformation and more commonly occurs in infants and children. It tends to appear in limbs, especially lower limbs and distal limbs. Its morphology and immunophenotype are characteristic and should be distinguished from other vascular malformations and the resolution phase of infant hemangiomas. In about one third of the cases, postoperative recurrence may occur and long-term follow-up is often required.

目的: 探讨疣状血管瘤(verrucous hemangioma,VH)临床病理学特点、诊断及鉴别诊断。 方法: 回顾性分析河南省人民医院病理科2005—2020年VH病例28例,进一步分析其临床及组织学特征,采用免疫组织化学EnVision法检测相关指标表达情况,荧光聚合酶链反应(PCR)法检测PIK3CA基因突变情况(第9、20号外显子),并随访患者。 结果: 28例VH男性13例,女性15例,男女比例1.0∶1.2,年龄10个月至56岁(其中<18岁25例),平均年龄9.7岁,中位年龄4.5岁。17例发生于下肢,7例发生于上肢,4例发生于躯干。28例皮损呈不规则红色斑片,缓慢生长,部分可见红斑疣状增厚,表面凹凸不平,呈浅粉色或红白色。7例病变范围广,皮损上沿至臀部,向下至足背及脚趾,呈暗红色至红褐色,表面粗糙坚硬,条带状分布并形成铠甲样外观,周围可见卫星灶。镜下见28例病变累及范围较广,自真皮乳头层至深部软组织均可见不同程度扩张畸形血管;其中,真皮乳头层主要由大量增生和扩张的薄壁毛细血管和海绵状血管组成;真皮网状层及皮下筋膜层可见散在分布薄壁小血管,内皮细胞增生不明显,偶见乳头状增生,筋膜层内畸形血管与宿主脂肪纤维组织混杂呈小叶状分布;表皮疣状增生,上皮角延长并相互融合。免疫组织化学显示血管内皮细胞均弥漫强表达CD31、CD34、ERG、WT-1,GLUT-1真皮浅层血管内皮细胞阳性表达,深部绝大部分阴性。13例行PIK3CA基因检测,结果显示第9、20号外显子均未发现体细胞突变。获随访25例,随访时间5个月至10年,7例由于面积较大行多次手术切除及整形修复,8例复发。 结论: VH是一种极少见的先天性静脉畸形,婴幼儿及儿童多见,大多出生时即存在。好发于四肢,尤其是下肢及肢体远端。形态学及免疫表型均具有特征性,并需要与其他脉管畸形及消退期婴幼儿血管瘤相鉴别。少数病例易术后多次复发,需长期随诊。.

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