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从肝脏血管瘤登记处得到的启示:亚型分类。

Lessons from a liver hemangioma registry: subtype classification.

机构信息

Department of Surgery, Children's Hospital Boston, Boston, MA 02115, USA.

出版信息

J Pediatr Surg. 2012 Jan;47(1):165-70. doi: 10.1016/j.jpedsurg.2011.10.037.

Abstract

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.

摘要

目的

先前提出的肝血管瘤分类提出了 3 种病变类型:局灶性、多灶性和弥漫性。创建了一个注册处(www.liverhemangioma.org)来跟踪患者,以验证该分类方案。

方法

回顾了 1995 年至 2010 年期间输入的注册记录。

结果

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 例患者兼有多灶性和弥漫性特征。局灶性组性别分布均衡,而多灶性和弥漫性组女性更为常见(分别为 66.2%和 70.0%)。30%(9/30)局灶性肝血管瘤患者在产前被发现。多灶性或弥漫性肝血管瘤无法检测到,表明典型的常见皮肤婴儿血管瘤在出生后增殖。皮肤血管瘤伴发 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。弥漫性肝血管瘤患者均有甲状腺功能减退(16/16),多灶性肝血管瘤患者有 21.4%(9/42),但局灶性肝血管瘤患者无一例(0/17)。

结论

肝血管瘤登记处的分析证实,局灶性病变在生物学上与多灶性和弥漫性肝血管瘤不同。以前未检测到的多灶性肝血管瘤可能会发展为可怕的弥漫性肝血管瘤。对多发性皮肤婴儿血管瘤的婴儿进行筛查超声检查可能有助于更早发现和治疗。

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