APSA Paper
Hepatic hemangiomas: subtype classification and development of a clinical practice algorithm and registry

https://doi.org/10.1016/j.jpedsurg.2006.09.041Get rights and content

Abstract

Purpose

Hepatic hemangiomas, though histologically benign, may be associated with significant morbidity and mortality in afflicted infants. The literature presents much confusion regarding the natural history and treatment options for hepatic hemangiomas. Clinical manifestations range from asymptomatic self-limiting lesions to congestive heart failure associated with high-volume vascular shunting to fulminant hepatic failure with hypothyroidism, abdominal compartment syndrome, and death. There has been little rationale to choose among observation, corticosteroid, other pharmacologic agents, arterial embolization, hepatic artery ligation, resection, or liver transplantation for any given patient.

Methods

We analyzed several recent retrospective radiologic analyses and pathologic studies to determine whether hepatic hemangiomas could be categorized, allowing prediction of their natural history and rational choice of therapies based upon their clinical presentation and radiographic appearance.

Results

We propose that hepatic hemangiomas do not represent a single entity but, rather, 3 principle categories of lesions: focal, multifocal, and diffuse. Because these 2 categories represent different anatomical and physiologic variants, so, too, may they respond differently to previously anecdotally applied treatment regimens. With input from international multidisciplinary authorities on hemangiomas, we developed and proposed a clinical practice algorithm for the evaluation and management of hepatic hemangiomas. Toward that end, we propose a plan to institute a web-based international hepatic hemangioma registry. Participants in the registry could obtain no-cost centralized review of imaging studies (and histology if available) and guidance regarding the management algorithm from an established multidisciplinary team. In exchange, the registry will facilitate the acquisition of systematic clinical and imaging information.

Conclusion

Longitudinal observation of response to more directed treatment protocols may contribute greatly to the understanding of these potentially fatal tumors.

Section snippets

Purpose

Infantile hemangioma (IH) is the most common pediatric tumor, affecting 4% to 5% of white infants. It is a benign endothelial cell neoplasm that exhibits rapid postnatal growth followed by slow involution during childhood. Nevertheless, approximately 10% to 20% of IHs cause vision-threatening, life-threatening, or disfiguring complications [1]. The life cycle evolves in 3 stages: (1) a proliferating phase of rapid growth, beginning shortly after birth and lasting approximately 9 to 12 months;

Methods

We performed a Pubmed search with keywords hepatic hemangioma, liver hemangioma, visceral hemangioma, and hemangioma screening, limiting the inquiry to publications over the past decade (1995-2005) that correctly identified hepatic hemangiomas. These publications, chiefly composed of retrospective radiographic, dematologic, and histopathologic analyses as well as case reports, were critically reviewed to elucidate patterns of physiologic and radiographic presentation that might allow more

Focal lesions

This type can be identified on magnetic resonance imaging (MRI) as a well-defined, solitary, spherical tumor that is hypointense relative to liver on T1-weighted sequences and hyperintense on T2-weighted sequences (Fig. 1). The typical tumor demonstrates centripetal enhancement on gadolinium sequences. Areas of central necrosis, thrombosis, or intralesional hemorrhage are heterogeneously enhanced. The solid nonthrombosed (noninvoluted) areas of the lesion exhibit intense homogeneous

Conclusion

Infantile hepatic hemangiomas represent a clinically diverse spectrum of benign hepatic tumors. A novel systematic classification scheme encompassing clinical presentation, radiographic appearance, pathologic features, physiologic behavior, and natural (untreated) and treated history is presented along with a proposed therapeutic algorithm. A registry for prospective data collection has been established with the goal of validating and refining current understanding and management of hepatic

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  • Cited by (0)

    Presented at the 37th Annual Meeting of the American Pediatric Surgical Association, May 20–24, 2006, Hilton Head, SC.

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