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

Abstract

Background 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. Methods In 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. Results A 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. Conclusions In 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.

Case Report of Undifferentiated Hepatic Embryonal Sarcoma with Mesenchymal Hamartoma: A Rare Entity

“…Most cases of UES are positive for vimentin, α-1 antitrypsin, and CD68. Study by Habibzadeh et al 12 reveals two markers Bcl-2 and CD34 show strong immunoreactivity in different components of the mesenchymal hamartoma case found in association with UES. The development of UES after incomplete excision of mesenchymal hamartoma reported in the literature corroborates this hypothesis.…”

Undifferentiated embryonal sarcoma of the liver with epithelioid features in an adult patient

“…As such, some areas may contain relatively uniform undifferentiated cells, while other areas may contain extensive pleomorphic cells. Considerable necrotic areas can be found in most specimens, and mitosis is common [11] …”

New insights in gastrointestinal “pediatric” neoplasms in adult patients: pancreatoblastoma, hepatoblastoma and embryonal sarcoma of the liver. A practical approach by GIPPI-GIPAD Groups

“…Although ESL is a malignant tumor with an aggressive biological behavior characterized by metastatic spread to the lungs and peritoneum. The prognosis of patients treated with surgical resection (partial hepatectomy) and adjuvant chemotherapy is generally favorable (5-year overall survival > 70%) 1 , 55 , 56 , 74-77 . Tumor size > 15 cm and extrahepatic dissemination at the diagnosis are negative prognostic indicators 74-77 .…”

“…Tumor size > 15 cm and extrahepatic dissemination at the diagnosis are negative prognostic indicators 74-77 . Neoadjuvant chemotherapy followed by surgery is reserved to the patients with unresectable tumors, while liver transplantation is a possibility for patients who are resistant to neoadjuvant chemotherapy 74-77 .…”

“…The prognosis of patients treated with surgical resection (partial hepatectomy) and adjuvant chemotherapy is generally favorable (5-year overall survival > 70%) 1 , 55 , 56 , 74-77 . Tumor size > 15 cm and extrahepatic dissemination at the diagnosis are negative prognostic indicators 74-77 . Neoadjuvant chemotherapy followed by surgery is reserved to the patients with unresectable tumors, while liver transplantation is a possibility for patients who are resistant to neoadjuvant chemotherapy 74-77 .…”