Embryonal (Undifferentiated) Sarcoma of the Liver With Peripheral Angiosarcoma Differentiation Arising in a Mesenchymal Hamartoma in an Adult Patient

Abstract

Embryonal (undifferentiated) sarcoma of the liver (ESL) is a rare malignant neoplasm composed of undifferentiated sarcomatous tissue. We are presenting a case of a 74-year-old woman diagnosed with an ESL arising from a mesenchymal hamartoma of the liver (MHL). Both lesions occur typically in childhood, with only rare reported cases in adults. Histologically, the mass consisted primarily of loose myxoid stroma admixed with bland spindle cells and extensive, cystic (lymphangioma-like) degeneration. However, also present peripherally were markedly atypical cells (including multi-nucleated forms) and hyaline globules. Additionally, atypical cells with a sinusoid tectorial growth pattern were identified, which were positive for CD31, CD34 and Factor VIII. The tumor cells of ESL are classically described as being negative for tissue-specific immunohistochemical markers. However our case demonstrated focal positivity for vascular markers CD31, CD34 and Factor VIII and this along with the sinusoidal tectorial growth pattern, mimicked an angiosarcoma.

Hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a pathologic review

“…Reports of UESL and angiosarcoma arising in HMH have been well documented [30][31][32][33][34][35]. Clinical and histological evidence has strongly suggested that UES can develop within a pre-existing HMH.…”

Ongewone presentatie van een mesenchymaal ooglidhamartoma en een overzicht van het voorkomen van perioculaire hamartomen bij de hond

“…Although mesenchymal hamartomas have a benign histological appearance, malignant transformation of nasal chondromesenchymal hamartoma (Li et al, 2013) and mesenchymal hamartoma of the liver (Ramanujam et al, 1999;Tucker et al, 2012) has been described. These cases emphasize the need for the complete removal of mesenchymal hamartoma and the need for long-term follow-up.…”

Undifferentiated Embryonal Sarcoma in Adult Liver

“…Although common in children between 6 and 10 years old, UES is very rare in adults over 60-years-old; for adults aged >60 years, only 14 UES cases have been reported in the world until now 3,4,5,6,7. UES was known to have no gender predominance in children, but there was female predominance (62.5%) in UES patients over the age of 30 worldwide 9.…”

“…This rare presentation is especially true for adults aged >60 years with only 14 cases of UES reported in this population until now 3,4,5,6,7. In Korea, eight cases of UES in adults have been reported: five cases were in women and four cases were in people older than 60 years 5,6,7,8.…”