Undifferentiated Embryonal Sarcoma of the Liver is Associated with Mesenchymal Hamartoma and Multiple Chromosomal Abnormalities: A Review of Eleven Cases

2011 ◽  
Vol 14 (2) ◽  
pp. 111-116 ◽  
Author(s):  
Bahig M. Shehata ◽  
Nitika A. Gupta ◽  
Howard M. Katzenstein ◽  
Charlotte K. Steelman ◽  
Mark L. Wulkan ◽  
...  
Keyword(s):  
Chromosomal Abnormalities ◽  
Mesenchymal Hamartoma ◽  
Embryonal Sarcoma

Undifferentiated Embryonal Sarcoma of the Liver: A Concise Review

2015 ◽  
Vol 139 (2) ◽  
pp. 269-273 ◽  
Author(s):  
Juan Putra ◽  
Kim Ornvold
Keyword(s):  
Radiation Therapy ◽  
Multimodal Therapy ◽  
Chromosomal Abnormalities ◽  
Mesenchymal Hamartoma ◽  
Successful Management ◽  
Concise Review ◽  
Immunohistochemistry Staining ◽  
Hepatic Lesions ◽  
Embryonal Sarcoma ◽  
Rule Out

Undifferentiated embryonal sarcoma of the liver is an aggressive mesenchymal tumor that occurs predominantly in children. Although this entity has been described for decades, its pathogenesis is still obscure. Its association with mesenchymal hamartoma has been well described on the basis of identical chromosomal abnormalities. The clinical and radiological diagnoses are often difficult, and the diagnosis of undifferentiated embryonal sarcoma of the liver is based on its histology and immunophenotype. It is essential to recognize the characteristic histologic findings and the pattern of the immunohistochemistry staining to rule out other hepatic lesions. Multimodal therapy with surgery, chemotherapy, and radiation therapy has drastically improved the prognosis of patients with undifferentiated embryonal sarcoma of the liver. This successful management requires timely diagnosis for superior outcome.

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

2020 ◽  
Vol 7 (2) ◽  
pp. HEP19 ◽  
Author(s):  
Sebastiao N Martins-Filho ◽  
Juan Putra
Keyword(s):  
Chromosomal Aberrations ◽  
Benign Lesion ◽  
Differential Diagnoses ◽  
Mesenchymal Hamartoma ◽  
Clinicopathologic Characteristics ◽  
Radiological Features ◽  
Histologic Evaluation ◽  
Embryonal Sarcoma

This review highlights two rare entities that are predominantly seen in children: hepatic mesenchymal hamartoma (HMH) and undifferentiated embryonal sarcoma of the liver (UESL). HMH is a benign lesion predominantly seen in the first 2 years of life, while UESL is malignant and usually identified in patients between 6 and 10 years of age. UESL may arise in the background of HMH, and the association has been supported by similar chromosomal aberrations (19q13.4). The diagnosis of both lesions is primarily based on histologic evaluation, as the clinical and radiological features are not always typical. The clinicopathologic characteristics, pathogenesis, differential diagnoses and treatment for both lesions are discussed.

scholarly journals Expression of glypican-3 in undifferentiated embryonal sarcoma and mesenchymal hamartoma of the liver

2012 ◽  
Vol 43 (5) ◽  
pp. 695-701 ◽  
Author(s):  
Mary Levy ◽  
Anand Trivedi ◽  
Jun Zhang ◽  
Lili Miles ◽  
Aras N. Mattis ◽  
...  
Keyword(s):  
Mesenchymal Hamartoma ◽  
Embryonal Sarcoma

Mesenchymal hamartoma and undifferentiated embryonal sarcoma: Diagnostic keys that radiologists need to know

2021 ◽  
Vol 63 (1) ◽  
pp. 42-49
Author(s):  
M. Aineseder ◽  
M.A. Mestas Núñez ◽  
R. López Grove ◽  
M.L. Padilla ◽  
T.G. Kreindel
Keyword(s):  
Mesenchymal Hamartoma ◽  
Embryonal Sarcoma

Hepatic Undifferentiated (Embryonal) Sarcoma Arising in a Mesenchymal Hamartoma

1997 ◽  
Vol 21 (10) ◽  
pp. 1248-1254 ◽  
Author(s):  
Gregory Y. Lauwers ◽  
Larry D. Grant ◽  
William H. Donnelly ◽  
Aurelia M. Meloni ◽  
Robin M. Foss ◽  
...  
Keyword(s):  
Mesenchymal Hamartoma ◽  
Embryonal Sarcoma

Undifferentiated Embryonal Sarcoma of the Liver With Rhabdoid Morphology Mimicking Carcinoma: Expanding the Morphologic Spectrum or a Distinct Variant?

2021 ◽  
pp. 109352662110189
Author(s):  
David J Papke ◽  
Adam S Fisch ◽  
Sarangarajan Ranganathan ◽  
Allison O’Neill ◽  
Micheál Breen ◽  
...  
Keyword(s):  
Tp53 Mutation ◽  
Mesenchymal Hamartoma ◽  
High Grade ◽  
Micro Rnas ◽  
Cytogenetic Testing ◽  
Poorly Differentiated ◽  
Chromosome 19Q13 ◽  
Rhabdoid Features ◽  
Embryonal Sarcoma ◽  
Specific Cluster

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare aggressive neoplasm that occurs predominantly in children. Like mesenchymal hamartoma of the liver (MHL), UESL harbors recurrent rearrangements involving 19q13.3 and 19q13.4, a region of the genome that contains a primate-specific cluster of micro-RNAs. Here, we present a case of a high-grade neoplasm that arose in the left hepatic lobe of a 5-year-old male and gave rise to widespread lymph node, visceral, and soft tissue metastases. The tumor was composed of sheets, tubules, and papillae of epithelioid cells with rhabdoid morphology. INI1 and BRG1 expression were retained. Tumor cells diffusely expressed epithelial markers, including multiple keratins. While the morphologic and immunophenotypic features were suggestive of poorly differentiated carcinoma with rhabdoid features, the tumor was found to harbor the t(11;19)(q13;q13.3) translocation characteristic of UESL, as well as a TP53 mutation. Given the clinical presentation, imaging, clinical course, the tumor was classified as UESL with unusual, carcinoma-like histopathologic features. In the context of an unclassified high-grade hepatic tumor in a young child, molecular or cytogenetic testing for chromosome 19q13 alterations should be considered.

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

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Parham Habibzadeh ◽  
Mohaddese Ansari Asl ◽  
Hamid Reza Foroutan ◽  
Ali Bahador ◽  
Mohammad Hossein Anbardar
Keyword(s):  
Pediatric Population ◽  
Mesenchymal Hamartoma ◽  
Positive Staining ◽  
Clinicopathologic Features ◽  
Comprehensive Overview ◽  
Clinicopathologic Characteristics ◽  
Tertiary Referral Center ◽  
Therapeutic Implications ◽  
Eosinophilic Cytoplasm ◽  
Embryonal Sarcoma

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.

Third Report of t(19q)(13.4) in Mesenchymal Hamartoma of Liver with Comments on Link to Embryonal Sarcoma

1998 ◽  
Vol 1 (5) ◽  
pp. 438-442 ◽  
Author(s):  
Kevin E. Bove ◽  
Ruth I. Blough ◽  
Shirley Soukup
Keyword(s):  
Developmental Disorders ◽  
Chromosome Abnormality ◽  
Post Treatment ◽  
Mesenchymal Hamartoma ◽  
Low Grade ◽  
Liver Lesions ◽  
Balanced Translocation ◽  
The Third ◽  
Karyotype Abnormality ◽  
Embryonal Sarcoma

We report the third known case of mesenchymal hamartoma of the liver (MHL) with a balanced translocation involving a common breakpoint, 19q13.4. A common clonal chromosome abnormality appears to characterize an important subset of MHL, some of which may be low-grade neoplasms. We found no consistent karyotype abnormality in a post-treatment sample of embryonal sarcoma of the liver (ESL). Reports of coexistent MHL and ESL in two patients and detection of 19q abnormalities in two ESLs appear to support Stocker's hypothesis of a histogenetic link between these two rare liver lesions. More data are needed to clarify this relationship. It is possible that MHLs are etiologically heterogenous and may be developmental disorders, disruptions, or neoplasms.

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