Benign Hepatic Mesenchymal Hamartoma (HMH) – A Case Report

Abstract

A one-year-old male child presented with progressive enlargement of abdomen and loss of appetite which he had suffered from, since 4 months. Physical examination showed a palpable mass in right hypochondrium. Ultrasonography and MRI showed a solid mass arising from right lobe of liver. The resected mass was well encapsulated, yellowish white in colour and it measured 20 x 18 x 17cm. Cut surface showed a Nutmeg like appearance. Histology showed a well-defined, fibrous capsule with numerous ducts which were lined by flat to low cuboidal epithelium and which were surrounded by concentric layers of fibrous tissue which showed myxoid changes at places. Intervening area showed normal appearing hepatocytes with clear cell changes, dilated congested sinusoids, along with extramedullary haematopoiesis. Based on distinctive morphological features, a histological diagnosis of Benign Hepatic Mesenchymal Hamartoma of liver was made. IHC was not done, as no specific marker has been described for making a differential diagnosis. Benign Hepatic Mesenchymal Hamrtoma (HMH) of liver is an extremely rare tumour of childhood and therefore, the case was considered worth documentation.

A diagnostic pitfall in hepatic mesenchymal hamartoma mimicking hepatoblastoma: A case report

“…The mesenchymal components were composed mainly of architecturally abnormal biliary structures. [4][5][6][7] Therefore, both CK7 and CK19 were positive in most cases which indicates biliary epithelium in the cystic component. [8][9][10] Usually, there was limited immunoreactivity to CD34 and CD31 markers for hepatic mesenchymal hamartomas.…”

Mesenchymal Hamartoma of the Liver: Case Report and Review of the Literature

“…Karaciğerin mezenkimal hamartomu, malforme safra kanalları ve miksomatöz mezenkimin proliferasyonu ile karakterize benign bir karaciğer tümörüdür (7) . Kitlede kistik yapılar baskındır, arada solid yapılar da içerir ve gerçek bir kapsülü yoktur.…”

“…Mezenkimal hamartoma özgü bir laboratuvar bulgusu yoktur. Karaciğer fonksiyon testleri, AFP ve βhCG yüksek bulunabilir (6,7) . AFP tümör belirteci olarak kullanılabilir, tümör içerisindeki prolifere hepatositlerden salındığı düşünülmektedir (7) .…”

“…Karaciğerin mezenkimal hamartomu, genellikle 2 yaşından küçük çocuklarda görülen nadir bir kistik karaciğer tümördür. İlk defa 1903'de Maresch tarafından tanımlanmış ve 1956'da Edmondson "mezenkimal hamartom" şeklinde adlandırmıştır (2,7) . Tümör safra yolları, mezenkim ve karaciğer parankiminden oluşur; kistik, solid veya mikst tip olmak üzere üç farklı tipi vardır (6) .…”

Surgery is the preferred treatment for bile duct hamartomas

“…Whether BDH has malignant potential remains controversial. BDH was previously considered as a benign liver tumor that should be followed regularly (11,(24)(25)(26). However, an increasing number of cases indicated that BDH may transform to cholangiocarcinoma and hepatocellular carcinoma (3,4,10,27-29).…”