Giant mesenchymal hamartoma in pediatric patients: A new indication for liver transplantation

Arrunategui, Ana María; Caicedo, Luis A.; Thomas, Laura S.; Botero, Verónica; García, O Sánchez; Carrascal, Edwin; Gómez, Lorena I.; Gutiérrez, Leidys; Delgado, Alejandro; Serrano, Oscar; Villegas, Jorge I.; Echeverri, Gabriel J.

doi:10.1016/j.epsc.2017.03.012

Cited by 11 publications

(15 citation statements)

References 18 publications

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“…However, complete surgical excision with a margin of normal tissue liver is the gold standard of treatment of hepatic mesenchymal hamartomas to avoid the problems of local recurrence and long-term malignant transformation [2]. Liver transplantation is considered controversial for the treatment of this pathology; however, it is a viable therapeutic option in giant unresectable bilobed lesions [2]. In this study, radical surgical excision of the tumor was achieved without complications.…”

Section: Discussionmentioning

confidence: 85%

“…Developmental theories include exposure of otherwise normal hepatic parenchyma to regional ischemia or toxic injury during its development [6]. The term "mesenchymal hamartoma" was first used by Edmondson in 1956 who distinguished the pathology from other cystic and tumor-like lesions of the liver [2,7].…”

Section: Discussionmentioning

confidence: 99%

“…Various therapeutic strategies have been reported for the management of hepatic mesenchymal hamartomas, including enucleation for small lesions and marsupialization into the peritoneal cavity for larger lesions [10]. However, complete surgical excision with a margin of normal tissue liver is the gold standard of treatment of hepatic mesenchymal hamartomas to avoid the problems of local recurrence and long-term malignant transformation [2]. Liver transplantation is considered controversial for the treatment of this pathology; however, it is a viable therapeutic option in giant unresectable bilobed lesions [2].…”

Section: Discussionmentioning

confidence: 99%

“…Mesenchymal hamartoma of the liver is reported to be the second most common benign liver tumor in children, with 80% of these lesions diagnosed within the first 2 years of life [1,2]. The exact etiology behind the development of this pathology is still unclear and is thought to be a developmental anomaly of the portal connective tissue rather than a true neoplasm [3].…”

Section: Introductionmentioning

confidence: 99%

“…Several associated anomalies have been reported including malrotation, Beckwith-Wiedemann syndrome, biliary atresia, and congenital heart diseases [1,5]. Radical surgical excision, whenever possible, is the gold standard for treating these lesions to avoid the problems of local recurrence and possible long-term malignant transformation [2].…”

Section: Introductionmentioning

confidence: 99%

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Giant solid mesenchymal hamartoma of the liver in a neonate: case report

Hassan

2020

Ann Pediatr Surg

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Background Mesenchymal hamartoma is the second most common benign liver tumor in children, with 20% of the cases diagnosed during the neonatal period. The exact etiology in still unclear, and most investigators believe that it is a developmental anomaly rather than a true neoplasm. The presentation of these tumors is highly variable depending on the lesion’s size, ranging from small asymptomatic lesions to very large tumors with life threatening complications. Radical surgical excision, whenever possible, is the gold standard for treating these lesions to avoid the problems of local recurrence and possible malignant transformation. We present the rare occurrence of an entirely solid, giant hepatic mesenchymal hamartoma in a 3-week-old male newborn and discuss the mode of presentation, as well as the diagnostic and therapeutic approach. Case presentation A 3-week-old male newborn was referred to our institution with huge abdominal distension and respiratory distress. Imaging studies confirmed the presence of a very large solid intraabdominal mass occupying the majority of the abdominal cavity and abutting the inferior aspect of the right lobe of the liver, but did not reveal the diagnosis. At laparotomy, a huge solid mass was found attached to the right lobe of the liver. Complete excision was done, and histopathological examination confirmed the diagnosis of mesenchymal hamartoma. Conclusion Although rare, mesenchymal hamartoma of the liver can present as a neonatal surgical emergency. Emergency intervention is required in symptomatic patients. Radical surgical intervention is possible and is the treatment of choice to relieve the patient’s symptoms and avoid future complications.

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Section: Discussionmentioning

confidence: 85%