Lessons from a liver hemangioma registry: subtype classification

Lessons from a liver hemangioma registry: subtype classification
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DOI:
10.1016/j.jpedsurg.2011.10.037
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发表时间:
2012-01-01
影响因子:
2.4
通讯作者:
Fishman, Steven J.
Fishman, Steven J.
中科院分区:
医学3区
文献类型:
--
作者:
Kulungowski, Ann M.;Alomari, Ahmad I.;Fishman, Steven J.

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目的:以前提出的肝血管瘤的分类假设3种类型的病变:局灶性,多灶性和弥漫性。创建了一个注册表(www.liverhemangioma.org)来跟踪患者以验证此分类schema.Methods:1995年至2010年期间输入的注册表记录进行了回顾。结果:在121例肝血管瘤患者中,119例为局灶性(n = 33),多灶性(n = 68)和弥漫性(n = 18)。2例患者具有多灶性和弥漫性特征。病灶组性别分布均衡,而多灶性和弥漫性在女性中更常见(分别为66.2%和70.0%)。30%(9/30)的局灶性肝血管瘤患者在产前检出。在多灶性或弥漫性肝血管瘤中无法检测到,表明出生后增殖是常见皮肤婴儿血管瘤的典型特征。77.4%(48/62)的多灶性肝血管瘤、53.3%(8/15)的弥漫性肝血管瘤和15.3%(4/26)的局灶性肝血管瘤伴发皮肤血管瘤。弥漫性肝血管瘤16例(16/16),多灶性肝血管瘤21.4%(9/42),局灶性肝血管瘤0例(0/17)。弥漫性肝血管瘤可能是由以前未发现的多灶性肝血管瘤演变而来。超声筛查婴儿多发性皮肤血管瘤可使早期检测和治疗。(C)2012 Elsevier Inc. All rights reserved.
Purpose: A previously proposed classification of hepatic hemangioma postulated 3 types of lesions: focal, multifocal, and diffuse. A registry (www.liverhemangioma.org) was created to track patients to validate this classification scheme.Methods: Registry records entered between 1995 and 2010 were reviewed.Results: Of 121 patients with hepatic hemangioma, 119 were focal (n = 33), multifocal (n = 68), and diffuse (n = 18). Two patients shared features of multifocal and diffuse. The focal group had a balanced sex distribution, whereas multifocal and diffuse were more common in women (66.2% and 70.0%, respectively). Hepatic hemangioma was detected prenatally in 30% (9/30) of patients with focal disease. Detection was not possible in multifocal or diffuse hepatic hemangioma, indicating postnatal proliferation typical of common cutaneous infantile hemangioma. Cutaneous hemangiomas accompanied 77.4% (48/62) of multifocal hepatic hemangioma, 53.3% (8/15) of diffuse hepatic hemangioma, and 15.3% (4/26) of focal hepatic hemangioma. Hypothyroidism was documented in all (16/16) patients with diffuse hepatic hemangioma, 21.4% (9/42) with multifocal hepatic hemangioma, but in no patients with focal hepatic hemangioma (0/17).Conclusions: Analysis of the Liver Hemangioma Registry confirms that focal lesions are biologically distinct from multifocal and diffuse hepatic hemangioma. Feared diffuse hepatic hemangioma may evolve from previously undetected multifocal hepatic hemangioma. Screening ultrasounds in infants with multiple cutaneous infantile hemangioma may allow for earlier detection and therapy. (C) 2012 Elsevier Inc. All rights reserved.