Clinicopathological study of hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a single center study from Iran

Parham Habibzadeh,Ali Bahador,Mohaddese Ansari Asl,Hamid Reza Foroutan,Mohammad Hossein Anbardar

doi:10.1186/s13000-021-01117-z

Parham Habibzadeh, Ali Bahador + Show 3 more

Open Access

https://doi.org/10.1186/s13000-021-01117-z

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Journal: Diagnostic Pathology	Publication Date: Jun 24, 2021
Citations: 7	License type: open-access

Affiliation: Shiraz University of Medical Sciences

Abstract

BackgroundUndifferentiated embryonal sarcoma of liver (UESL) and hepatic mesenchymal hamartoma (HMH) are two rare entities which mainly affect the pediatric population. The aim of this investigation was to provide a comprehensive overview of the clinicopathologic characteristics of the patients diagnosed with these two conditions in a tertiary referral center in Iran.MethodsIn this retrospective study patients diagnosed with UESL or HMH between 2012 and 2020 were studied. A comprehensive histopathologic evaluation of the cases along with immunohistochemistry evaluation using a panel of antibodies was conducted. Furthermore, clinical, paraclinical, and treatment data and follow up information was collected.ResultsA total of 16 patients (8 UESL, 8 HMH) were studied in this investigation. Patients with UESL had a significantly (p = 0.002) higher age at diagnosis compared with those with HMH. Histologically, UESL cases were characterized by anaplastic cells with eosinophilic cytoplasm and bizarre nuclei and frequent atypical mitosis and spindling in a myxoid stroma while disordered arrangement of hepatic parenchyma, bile ducts, and primitive mesenchyme was seen in HMH. Furthermore, small round cells and extramedullary hematopoiesis were seen in 2 UESL and 3 HMH cases, respectively. Concurrent HMH was also seen in two UESL cases. Immunohistochemistry panel showed positive staining for Vimentin, Glypican-3, Desmin, CD56, CD10, and BCL2 in UESL cases and immunoreactivity for Vimentin, HepPar 1, Glypican-3, SMA, CD56, BCL2, and CD34 in various components of HMH.ConclusionsIn this study, the clinicopathologic features of UESL and HMH cases are presented. We also evaluated the utility of an immunohistochemistry panel in the diagnosis of these two rare entities and suggested novel markers. Our study corroborated the findings of previous investigations and expanded the clinicopathologic features of these two rare entities with diagnostic and potential therapeutic implications.

Highlights

Undifferentiated embryonal sarcoma of liver (UESL) and hepatic mesenchymal hamartoma (HMH) are two rare entities which mainly affect the pediatric population
Undifferentiated embryonal sarcoma of the liver (UESL) and hepatic mesenchymal hamartoma (HMH) are two rare pathologic entities that are primarily seen in the pediatric population [1]
The clinicopathologic characteristics and demographic data of the patients with UESL and HMH are presented in Tables 1 and 2, respectively

Summary

Introduction

Undifferentiated embryonal sarcoma of liver (UESL) and hepatic mesenchymal hamartoma (HMH) are two rare entities which mainly affect the pediatric population. The aim of this investigation was to provide a comprehensive overview of the clinicopathologic characteristics of the patients diagnosed with these two conditions in a tertiary referral center in Iran. Undifferentiated embryonal sarcoma of the liver (UESL) and hepatic mesenchymal hamartoma (HMH) are two rare pathologic entities that are primarily seen in the pediatric population [1]. UESL usually arises from the right hepatic lobe with tumor size varying from 10 cm to 30 cm [5]. Abdominal distention or mass is the most common clinical presentation of HMH which is usually seen as a multi-loculated cyst with a varying solid component on radiologic studies [10]

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Conclusion