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Case 48 - Mesenchymal hamartoma of the liver

from Section 5 - Gastrointestinal imaging

Published online by Cambridge University Press:  05 June 2014

Aman Khurana
Affiliation:
Stanford University
Fanny Chapelin
Affiliation:
Stanford University Molecular Imaging
Heike E. Daldrup-Link
Affiliation:
Lucile Packard Children's Hospital, Stanford University
Heike E. Daldrup-Link
Affiliation:
Lucile Packard Children's Hospital, Stanford University
Beverley Newman
Affiliation:
Lucile Packard Children's Hospital, Stanford University
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Summary

Imaging description

An infant who was several weeks old presented with an incidental finding of a palpable abdominal mass on a routine clinical examination. The child did not have fever or any other clinical abnormalities. The liver was palpable 10 cm below the right costal margin. Ultrasound and CT studies demonstrated a large, multiseptated cystic lesion in the right lobe of the liver (Fig. 48.1). This was thought to be most consistent with a mesenchymal cystic hamartoma of the liver.

Importance

Mesenchymal hamartoma is a rare, benign liver neoplasm, typically found in children less than two years of age. The lesion is slightly more common in boys than in girls. It is a developmental cystic liver tumor, composed of proliferations of variably myxomatous mesenchyme and malformed bile ducts. Treatment consists of surgical resection.

Typical clinical scenario

Patients typically present with an asymptomatic abdominal mass. Rarely, complications including ascites, jaundice, and even congestive heart failure can occur. Since mesenchymal hamartomas are congenital malformations, they may be detected prenatally. The size is highly variable. The lesions may grow rapidly in the postnatal period, followed by growth stasis or even some regression. Most lesions are located within the liver parenchyma, although rare cases of pedunculated lesions have been described as well.

Type
Chapter
Information
Pearls and Pitfalls in Pediatric Imaging
Variants and Other Difficult Diagnoses
, pp. 211 - 214
Publisher: Cambridge University Press
Print publication year: 2014

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References

Begueret, H, Trouette, H, Vielh, P, et al. Hepatic undifferentiated embryonal sarcoma: malignant evolution of mesenchymal hamartoma? Study of one case with immunohistochemical and flow cytometric emphasis. J Hepatol 2001;34(1):178–9.CrossRefGoogle ScholarPubMed
Buonomo, C, Taylor, GA, Share, JC, et al. Gastrointestinal tract. In: Kirks, DR, Griscom, NT, eds. Practical Pediatric Imaging: Diagnostic Radiology of Infants and Children, 3rd edition. Philadelphia: Lippincott-Raven, 1998; 963–4.Google Scholar
Horton, KM, Bluemke, DA, Hruban, RH, et al. CT and MR imaging of benign hepatic and biliary tumors. Radiographics 1999;19(2):431–51.CrossRefGoogle Scholar

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