Cutaneous intravascular epithelioid hemangioma. A clinicopathological and molecular study of 21 cases.
Cutaneous intravascular epithelioid hemangioma. A clinicopathological and molecular study of 21 cases.
复制标题
DOI：
10.1038/s41379-020-0505-4
复制
发表时间：
2020-08
期刊：
影响因子：
--
通讯作者：
Calonje E
中科院分区：
文献类型：
--
作者：
Luzar B;Ieremia E;Antonescu CR;Zhang L;Calonje E
单纯血管内生长的上皮样血管瘤(EH)是例外的。在这里，我们报告了一系列21例血管内EHS，通过模仿上皮样形态的恶性血管肿瘤，代表了一个潜在的严重的诊断陷阱。男性12例，女性4例，年龄11~71岁(平均40.2岁)，好发于四肢(13/21，61.9%)，其次为头颈部(8/21，38.1%)。病变大小从2 mm到30 mm不等(平均13 mm)。最常见的表现是结节生长缓慢。大多数肿瘤为单发(13/16，81.2%)，但有3例(3/16，18.8%)发生一次以上血管内EH。治疗包括完全手术切除，一般是治愈的。对10例患者发生的13个病变进行了随访(4~72个月，平均27.3个月)。未观察到其他肿瘤复发或发展。所有21个病变均发生在皮下静脉。血管内上皮样内皮细胞的增殖有两种形态：1)小叶毛细血管瘤样增殖，形成不同的开放血管腔；2)固体增殖，一般缺乏开放的血管空间。9个病灶以小叶毛细血管瘤样病变为主，8个病灶为混合小叶血管瘤样实性病变，4个血管内EHS为单纯实性病变。上皮样内皮细胞有丝分裂活性为0~7个/10高倍视野，平均2.1个/10个高倍视野。有6个病灶有丝分裂活跃，每10hpf有5个或以上有丝分裂(6/21，28.5%)。有丝分裂的数量通常在生长稳健的区域更为突出。未见不典型的有丝分裂。未见瘤内坏死。细胞学异型性较轻(20/21例)。免疫组化CD31阳性14例，ERG阳性5例。免疫组织化学检测Fos均为阴性(6/6)，其中1/6(6/6)的皮损内皮细胞FosB核阳性。对8例进行FISH分析，检测Fos和FosB基因重排。所有病例FosB重排均为阴性，1例Fos基因断裂阳性。总而言之，EH的血管内生长与不良生物学行为无关。内皮细胞的固体血管内增殖可以模拟具有上皮样形态的恶性血管肿瘤。然而，血管内EHS表现为轻度的细胞学异型性和低有丝分裂活性，缺乏不典型的有丝分裂，明显的核不典型，多层或肿瘤坏死。最后，Fos基因很少重排，在EHS的这一亚型中没有FosB基因异常，这表明与大多数经典的EHS有潜在的不同的发病机制。
Pure intravascular growth of epithelioid hemangioma (EH) is exceptional. Herein, we report a series of 21 intravascular EHs, representing a potential serious diagnostic pitfall by mimicking malignant vascular neoplsms with epithelioid morphology. The tumors developed in 12 males and 4 females, aged from 11 to 71 years (mean age 40.2 yrs) with a predilection for the extremities (13 of 21, 61.9%), followed by the head and neck (8 of 21, 38.1%). Lesions ranged in size from 2 to 30 mm (mean size 13 mm). The most common presenting feature was a slowly growing nodule. Most neoplasms were solitary (13 of 16 patients, 81.2%) but three patients developed more than one intravascular EH (3 of 16, 18.8%). Treatment consisted of complete surgical excision and was generally curative. Follow-up was available for 13 lesions that had developed in 10 patients (range 4 to 72 months, mean 27.3 months). No recurrences or development of additional tumors were observed. All 21 lesions developed in subcutaneous veins. Two morphological patterns of intravascular epithelioid endothelial cell proliferation were observed: 1) a lobular capillary hemangioma-like proliferation with variable formation of open vascular lumina and 2) a solid proliferation generally lacking open vascular spaces. A lobular capillary hemangioma-like pattern was the sole pattern in 9 lesions, a mixed lobular hemangioma-like pattern and solid pattern in 8 and a pure solid pattern in 4 intravascular EHs. Mitotic activity in epithelioid endothelial cells ranged from 0 to 7 mitoses per 10 high-power field (mean 2.1 mitoses per 10 HPFs). Six lesions displayed brisk mitotic activity of 5 or more mitoses per 10 HPF (6 of 21, 28.5%). The number of mitoses was usually more prominent in areas with solid growth. Atypical mitoses were not observed. No intratumoral necroses were seen. Cytological atypia was mild (20 out of 21 cases). By immunohistochemistry, all tumors were positive for CD31 (14 out of 14) and ERG (5 out of 5). While all tested cases were FOS negative by immunohistochemistry (6 out of 6), one out of six cases (case 6) displayed FOSB nuclear positivity in about 30% of the lesional endothelial cells. Eight cases were analysed by FISH for the presence of FOS and FOSB gene rearrangements. While all cases were negative for FOSB rearrangements, a single case proved positive for FOS gene break-apart. In conclusion, intravascular growth of EH is not associated with adverse biological behavior. Solid intravascular proliferations of endothelial cells can mimic a malignant vascular tumor with epithelioid morphology. Nevertheless, intravascular EHs display mild cytological atypia coupled with low mitotic activity, and a lack of atypical mitoses, pronounced nuclear atypia, multilayering or tumor necrosis. Finally, the FOS gene is infrequently rearranged, and there are no FOSB gene abnormalities in this subset of EHs, suggesting a potential distinct pathogenesis than most classic EHs.
登录
查看更多内容
影响因子：
3.7
作者：
Antonescu, Cristina R.;Chen, Hsiao-Wei;Fletcher, Christopher D.
通讯作者：
Fletcher, Christopher D.
影响因子：
3.2
作者：
Guinovart, R. M.;Bassas-Vila, J.;Ferrandiz, C.
通讯作者：
Ferrandiz, C.
影响因子：
1.2
作者：
Machado, Isidro;Chong, Agustin;Llombart-Bosch, Antonio
通讯作者：
Llombart-Bosch, Antonio
DOI：
10.4103/0970-9290.100439
发表时间：
2012-03-01
期刊：
Indian journal of dental research : official publication of Indian Society for Dental Research
影响因子：
--
作者：
Aggarwal, Anshul;Keluskar, Vaishali
通讯作者：
Keluskar, Vaishali
影响因子：
5.6
作者：
Hung, Yin P.;Fletcher, Christopher D. M.;Hornick, Jason L.
通讯作者：
Hornick, Jason L.