A single‐center experience with undifferentiated embryonal sarcoma of the liver

Mathias, Melissa; Ambati, Srikanth R.; Chou, Alexander J.; Slotkin, Emily K.; Wexler, Leonard H.; Meyers, Paul A.; Magnan, Heather

doi:10.1002/pbc.26154

Cited by 18 publications

(20 citation statements)

References 19 publications

(44 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…3 Mathias et al report upto a median of eight years of survival after multimodal management. 16 Similarly, May et al reported successful treatment of these patients using a chemotherapy regimen of vincristine, cyclophosphamide and actinomycin-D to encounter any residual tumor cells after surgical resection. 33 For unresectable cases, neoadjuvant chemotherapy may be used.…”

Section: Discussionmentioning

confidence: 98%

“…14,15 A retrospective study conducted by Mathias et al, shows a 100% event-free (EFS) and 100% overall survival (OS) with no recurrence or secondary malignancy over a median of eight years of follow-up. 16 However, the mortality ensues from recurrent or metastatic disease. 17,18 In the upcoming section, we discuss an interesting case of undifferentiated embryonal sarcoma of the liver in a seven-year-old male child.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Undifferentiated embryonal sarcoma of liver in a 9-year-old: case report

2020

View full text Add to dashboard Cite

<p>The embryonal sarcoma of the liver is an extremely malignant, aggressive and infrequent condition of the liver, predominantly affecting children in the age groups of four to ten. The disease manifests with fever, abdominal pain or mass, nausea and laboratory findings suggestive of liver cell injury. The diagnosis can be made using tumor markers, imaging, immunohistochemistry and histopathology. The multimodal management protocol focuses the use of surgical resection, chemotherapy and radiation to significantly improved survival rates and quality of life in these patients.</p>

show abstract

Section: Discussionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

Undifferentiated embryonal sarcoma of liver in a 9-year-old: case report

2020

View full text Add to dashboard Cite

show abstract

“…In the original report of 31 patients with UESL by Stocker and Ishak, only six patients were alive without any evidence of the disease [ 7 ]. Over the past couple of years, mounting evidence suggests that radical surgical resection of the tumor supplemented by adjuvant chemotherapy leads to an overall improvement in the survival of these patients [ 19 ]. Herein, we report encouraging results of following this protocol in patients with UESL.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2021

View full text Add to dashboard Cite

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.

show abstract

“…Classically, it presents in children from the age of six to ten and shows no gender predilection. It is considered to be the third most common malignant hepatic tumor in childhood, after hepatoblastoma and hepatocellular carcinoma, accounting for approximately 9–15% of all pediatric liver tumors, with an incidence of one case per million people per year [ 30 , 158 , 159 , 160 , 161 , 162 , 163 ].…”

Section: Malignant Tumors In School-aged Children and Adolescentsmentioning

confidence: 99%

Pediatric Primary Hepatic Tumors: Diagnostic Considerations

2021

View full text Add to dashboard Cite

The liver is the third most common site of abdominal tumors in children. This review article aims to summarize current evidence surrounding identification and diagnosis of primary hepatic tumors in the pediatric population based upon clinical presentation, epidemiology, and risk factors as well as classical imaging, histopathological, and molecular diagnostic findings. Readers will be able to recognize the features and distinguish between benign and malignant hepatic tumors within different age groups.

show abstract

A single‐center experience with undifferentiated embryonal sarcoma of the liver

Cited by 18 publications

References 19 publications

Undifferentiated embryonal sarcoma of liver in a 9-year-old: case report

Undifferentiated embryonal sarcoma of liver in a 9-year-old: case report

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

Pediatric Primary Hepatic Tumors: Diagnostic Considerations

Contact Info

Product

Resources

About