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Lessons from a liver hemangioma registry: subtype classification
Ann M Kulungowski1, Ahmad I Alomari, Aditya Chawla
1Department of Surgery, Children's Hospital Boston, Boston, MA 02115, USA.
Insights
This study validates a hepatic hemangioma classification, finding focal lesions distinct from multifocal and diffuse types. Early detection of infantile hemangioma may improve outcomes.
Area of Science:
- Hepatology
- Pediatric Radiology
- Vascular Anomalies
Background:
- A classification of hepatic hemangioma proposed three types: focal, multifocal, and diffuse.
- A registry was established to validate this classification scheme.
Purpose of the Study:
- To validate a proposed classification of hepatic hemangioma (focal, multifocal, diffuse).
- To analyze clinical and biological differences between hepatic hemangioma subtypes.
Main Methods:
- Review of registry records from 1995 to 2010.
- Analysis of 121 patients diagnosed with hepatic hemangioma.
Main Results:
- Focal, multifocal, and diffuse hepatic hemangiomas were identified in 119 patients.
- Multifocal and diffuse types were more common in women and associated with cutaneous hemangiomas and hypothyroidism.
- Focal lesions were detected prenatally, unlike multifocal and diffuse types.
Conclusions:
- Focal hepatic hemangiomas are biologically distinct from multifocal and diffuse types.
- Diffuse hepatic hemangioma may develop from undetected multifocal lesions.
- Screening infants with multiple cutaneous hemangiomas can enable earlier detection and treatment.
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.
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