Mesenchymal hamartoma of the liver demonstrating peripheral calcification in a 12-year-old boy

Steiner, Michel; Giles, Henry

doi:10.1007/s00247-008-0942-6

Cited by 9 publications

(5 citation statements)

References 7 publications

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“…Although extremely rare, there are a few example of intratumoural calcification with MH. Calcification is more often seen in other hepatic tumours than in MH [8]. A solid variant of MH is occasionally seen but it is usually smaller in size [9].…”

Section: Discussionmentioning

confidence: 99%

Cystic hepatic mesenchymal hamartoma: the role of radiology in diagnosis and perioperative management

Gopinathan

Fortier²,

Low³

2011

BJR

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ABSTRACT. A 13-month-old male presented with large hepatic mass that was confirmed diagnostically to be a cystic hepatic mesenchymal hamartoma. The sieve-like appearance of the solid components of the mass, as seen at ultrasonography, and findings of dynamic post-contrast MRI are highlighted here. Beyond diagnostic imaging, we extended our role in patient management through ultrasound-guided intra-operative aspiration of fluid from the cystic components of the tumour to reduce its volume and thereby facilitate surgical resection. Mesenchymal hamartoma (MH) of the liver is a rare, benign developmental malformation composed of a mixture of loose mesenchymal tissue, bile ducts, connective tissue and hepatocytes along with cysts formed either from degenerative areas of mesenchyme or from dilated bile ducts and lymphatics [1,2]. They are predominantly cystic, with the stromal predominant type rarely seen [1]. This pathology was variably labelled as lymphangioma of the liver, bile duct fibroadenoma, cavernous lymphangiomatoid tumour, giant lymphangioma and pseudocystic mesenchymal tumour before being unified under the single term ''mesenchymal hamartoma''. This term conveys that they are not true neoplasms.We present a case that demonstrates an approach from clinical presentation to treatment of MH with an emphasis on dynamic post-contrast MRI and ultrasound appearance. We also highlight the scope for radiologists to expand their role in peri-operative patient management, through the example of the simple but pro-active step of ultrasound-guided intra-operative aspiration of fluid from the massive cystic components of the mass that facilitated surgical excision in this patient. Case reportA 13-month-old male presented with abdominal distension, shortness of breath and loss of appetite for the past 2 months. On examination, non-tender abdominal mass was noted. Besides elevated serum alphafetoprotein (830 IU; normal 0-10) and gamma-glutamyl transpeptidase (127 U l 21 ; normal ,18 U l 21 ), the laboratory reports were unremarkable. There were no signs of sepsis. The patient underwent an ultrasound of the abdomen on the day of admission and an MRI of the abdomen on the following day. A 1-month-old CT scan, performed elsewhere, was reviewed.Ultrasound revealed a well-defined, multicystic mass involving the right lobe of liver. The cysts were anechoic and variable in size (largest 12 cm). Thin (1-2 mm) intervening septae and peripheral solid components were noted. The solid areas were isoechoic to the liver but demonstrated a sieve-like appearance constituted by multiple tiny (1-4 mm) anechoic areas with posterior enhancement and intervening iso-to hyperechoic tissue (Figure 1). Colour Doppler interrogation showed minimal vascularity. The visualised portal and hepatic veins in the vicinity were displaced. There was no biliary dilatation or evidence of portal hypertension. The review of postcontrast CT images showed a circumscribed hypodense mass with minimally enhancing septations and peripheral solid components but no inte...

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Section: Discussionmentioning

confidence: 99%

Cystic hepatic mesenchymal hamartoma: the role of radiology in diagnosis and perioperative management

Gopinathan

Fortier²,

Low³

2011

BJR

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“…Tumor calcification also occurs in bile duct adenoma (Maeda et al 2006). A distinct type of peripheral calcification occurs in mesenchymal hamartoma of the liver (Steiner and Giles 2008). Tumor calcification, often punctate, is regarded as rather typical for fibrolamellar hepatocellular carcinoma and occurs more frequently than calcification in ordinary hepatocellular carcinoma.…”

Section: Calcified Tumors In the Livermentioning

confidence: 99%

Hepatic and Perihepatic Involvement in Pneumokonioses and Other Mineral-Induced Diseases

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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“…Los tumores hepáticos en general constituyen entre el 1 y 4% de los tumores sólidos en niños (0.5 a 2%), siendo más frecuentes las lesiones metastásicas hepáticas procedentes de Neuroblastomas, tumor de Wilms y Linfomas; los tumores hepáticos son raros en la infancia y tienen una incidencia de 0.5 a 2 por millón cada año [1,3,10].…”

Section: Introductionunclassified

“…Se lo define como una malformación benigna compuesta por tejidos alterados de células normalmente presentes en la constitución del órgano [4,10,15].…”

Section: Introductionunclassified

“…El Hamartoma hepático se presenta en un 75% de los casos en el lóbulo derecho y su tamaño es variable (promedio de 1300 a 1900 gramos); el Hamartoma hepático tiene una clínica de presentación como una masa abdominal no dolorosa, de progreso rápido que se acompaña de otras manifestaciones cuando compromete a estructuras vitales del órgano; siendo una lesión solitaria casi siempre [10,16]. El diagnostico se puede complementar utilizando técnicas de imagen como el ultrasonido, tomografía axial computada y resonancia magnética; generalmente será definido como una masa hepática multiloculada, lo que invita a considerar un diagnóstico diferencial con quiste mesentérico y quiste de colédoco [12].…”

Section: Introductionunclassified

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Caso Clínico: Hamartoma Hepático Gigante.

Astudillo¹,

Dávila

Bravo³

2015

Rev Med HJCA

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Mesenchymal hamartoma of the liver demonstrating peripheral calcification in a 12-year-old boy

Cited by 9 publications

References 7 publications

Cystic hepatic mesenchymal hamartoma: the role of radiology in diagnosis and perioperative management

Cystic hepatic mesenchymal hamartoma: the role of radiology in diagnosis and perioperative management

Hepatic and Perihepatic Involvement in Pneumokonioses and Other Mineral-Induced Diseases

Caso Clínico: Hamartoma Hepático Gigante.

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