Mesenchymal Neoplasms of the Liver.

Mesenchymal neoplasms of the liver can be diagnostically challenging, particularly on core needle biopsies. Here, I discuss recent updates in neoplasms that are specific to the liver (mesenchymal hamartoma, undifferentiated embryonal sarcoma, calcifying nested stromal-epithelial tumor), vascular tumors of the liver (anastomosing hemangioma, hepatic small vessel neoplasm, epithelioid hemangioendothelioma, angiosarcoma), and other tumor types that can occur primarily in the liver (PEComa/angiomyolipoma, inflammatory pseudotumor-like follicular dendritic cell sarcoma, EBV-associated smooth muscle tumor, inflammatory myofibroblastic tumor, malignant rhabdoid tumor). Lastly, I discuss metastatic sarcomas to the liver, as well as pitfalls presented by metastatic melanoma and sarcomatoid carcinoma.

• Publication year

  2023

• Venue

  Surgical Pathology Clinics

• Publication date

  2023-06-01

• Fields of study

  Medicine

• Identifiers
  DOI 10.1016/j.path.2023.04.013PMID 37536892
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Co-expression of ERG and CD31 in a subset of CIC-rearranged sarcoma: a potential diagnostic pitfall

  2022cited by this paper
• Epstein-Barr Virus-Associated Smooth Muscle Tumor and Its Correlation With CD4 Levels in a Patient With HIV Infection

  2022cited by this paper
• Antiretroviral therapy achieved metabolic complete remission of hepatic AIDS related Epstein-Barr virus-associated smooth muscle tumor

  2022cited by this paper
• The TAZ-CAMTA1 Fusion Protein Promotes Tumorigenesis via Connective Tissue Growth Factor and Ras–MAPK Signaling in Epithelioid Hemangioendothelioma

  2022cited by this paper
• Calcifying nested stromal–epithelial tumor: a clinicopathologic and molecular genetic study of eight cases highlighting metastatic potential and recurrent CTNNB1 and TERT promoter alterations

  2021cited by this paper
• WWTR1(TAZ)-CAMTA1 gene fusion is sufficient to dysregulate YAP/TAZ signaling and drive epithelioid hemangioendothelioma tumorigenesis

  2021cited by this paper
• PDGFRA Immunohistochemistry Predicts PDGFRA Mutations in Gastrointestinal Stromal Tumors

  2021cited by this paper
• Cancer registry study of malignant hepatic vascular tumors: hepatic angiosarcomas and hepatic epithelioid hemangioendotheliomas

  2021cited by this paper
• Rare variants of primary liver cancer: Fibrolamellar, combined, and sarcomatoid hepatocellular carcinomas.

  2021cited by this paper
• YAP1-TFE3-fused hemangioendothelioma: a multi-institutional clinicopathologic study of 24 genetically-confirmed cases

  2021cited by this paper
• Clinicopathologic Characterization of Epithelioid Hemangioendothelioma in a Series of 62 Cases

  2021cited by this paper
• Fibrohistiocytic Variant of Hepatic Pseudotumor

  2021cited by this paper
• Loss of expression of YAP1 C-terminus as an ancillary marker for epithelioid hemangioendothelioma variant with YAP1-TFE3 fusion and other YAP1-related vascular neoplasms

  2021cited by this paper
• Undifferentiated Embryonal Sarcoma of the Liver With Rhabdoid Morphology Mimicking Carcinoma: Expanding the Morphologic Spectrum or a Distinct Variant?

  2021cited by this paper
• TAZ-CAMTA1 and YAP-TFE3 alter the TAZ/YAP transcriptome by recruiting the ATAC histone acetyltransferase complex

  2021cited by this paper
• The genomic landscape of undifferentiated embryonal sarcoma of the liver is typified by C19MC structural rearrangement and overexpression combined with TP53 mutation or loss

  2020cited by this paper
• Hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a pathologic review

  2020cited by this paper
• In situ hybridisation for albumin RNA in paediatric liver cancers compared with common immunohistochemical markers

  2020cited by this paper
• Primary mammary angiosarcomas harbor frequent mutations in KDR and PIK3CA and show evidence of distinct pathogenesis

  2020cited by this paper
• Case series of Mesenchymal Hamartoma: a Rare Childhood Hepatic Neoplasm

  2020cited by this paper
• Aberrant keratin expression is common in primary hepatic malignant vascular tumors: A potential diagnostic pitfall.

  2020cited by this paper
• Dedifferentiated and Undifferentiated Melanomas

  2020cited by this paper
• GNA11 joins GNAQ and GNA14 as a recurrently mutated gene in anastomosing hemangioma

  2019cited by this paper
• WW Domain-Containing Proteins YAP and TAZ in the Hippo Pathway as Key Regulators in Stemness Maintenance, Tissue Homeostasis, and Tumorigenesis

  2019cited by this paper
• Angiosarcoma of the Liver: Clinicopathologic Features and Morphologic Patterns

  2019cited by this paper
• Nested Stromal-Epithelial Tumor of the Liver: A Review

  2019cited by this paper
• Anastomosing hemangioma of the liver: a case series

  2019cited by this paper
• Malignant Rhabdoid Tumor, an Aggressive Tumor Often Misclassified as Small Cell Variant of Hepatoblastoma

  2019cited by this paper
• Hepatic YAP1-TFE3 Rearranged Epithelioid Hemangioendothelioma

  2019cited by this paper
• Mesenchymal Hamartoma of the Liver and DICER 1 Syndrome

  2019cited by this paper
• Mesenchymal Hamartoma of the Liver and DICER1 Syndrome.

  2019cited by this paper
• Prognostic stratification of clinical and molecular epithelioid hemangioendothelioma subsets

  2019cited by this paper
• Sarcomatoid carcinoma presenting as cancers of unknown primary: a clinicopathological portrait

  2019cited by this paper
• Mesenchymal Hamartoma of the Liver and DICER1 Syndrome

  2019cited by this paper
• Calcifying Nested Stromal-Epithelial Tumor of the Liver: An Update and Literature Review.

  2018cited by this paper
• Frequent GNAQ and GNA14 Mutations in Hepatic Small Vessel Neoplasm

  2018cited by this paper
• Hepatic Angiosarcoma: A Multi-institutional, International Experience with 44 Cases

  2018cited by this paper
• Recurrent GNA14 mutations in anastomosing haemangiomas

  2018cited by this paper
• TFE3‐rearranged hepatic epithelioid hemangioendothelioma—a case report with immunohistochemical and molecular study

  2017cited by this paper
• Epithelioid haemangioendothelioma

  2017cited by this paper
• Loss of H3K27 trimethylation is not suitable for distinguishing malignant peripheral nerve sheath tumor from melanoma: a study of 387 cases including mimicking lesions

  2017cited by this paper
• Calcifying nested stromal-epithelial tumor (CNSET) of the liver in Beckwith-Wiedemann syndrome.

  2017cited by this paper
• Recurrent GNAQ mutations in anastomosing hemangiomas

  2017cited by this paper
• Hepatic small vessel neoplasm, a rare infiltrative vascular neoplasm of uncertain malignant potential.

  2016cited by this paper
• A single‐center experience with undifferentiated embryonal sarcoma of the liver

  2016cited by this paper
• Undifferentiated Embryonal Sarcoma of the Liver (UESL): A Single-Center Experience and Review of the Literature

  2016cited by this paper
• Nuclear Expression of CAMTA1 Distinguishes Epithelioid Hemangioendothelioma From Histologic Mimics

  2016cited by this paper
• Cushing syndrome as presenting symptom of calcifying nested stromal–epithelial tumor of the liver in an adolescent boy: a case report

  2016cited by this paper
• Recurrent CIC Gene Abnormalities in Angiosarcomas: A Molecular Study of 120 Cases With Concurrent Investigation of PLCG1, KDR, MYC, and FLT4 Gene Alterations

  2016cited by this paper
• Mechanism of action of a WWTR1(TAZ)-CAMTA1 fusion oncoprotein

  2016cited by this paper
• Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics

  2016cited by this paper
• Expression of Programmed Cell Death 1 Ligands (PD-L1 and PD-L2) in Histiocytic and Dendritic Cell Disorders

  2016cited by this paper
• Prevalence and clinical outcome of hepatic haemangioma with specific reference to the risk of rupture: A large retrospective cross-sectional study.

  2016cited by this paper
• Follicular dendritic cell sarcoma: clinicopathologic study of 15 cases with emphasis on novel expression of MDM2, somatostatin receptor 2A, and PD-L1.

  2016cited by this paper
• Primary embryonal rhabdomyosarcoma of the liver

  2016cited by this paper
• Anastomosing Hemangiomas Arising in Unusual Locations: A Clinicopathologic Study of 17 Soft Tissue Cases Showing a Predilection for the Paraspinal Region

  2016cited by this paper
• Epithelioid hemangioendotheliomas with TFE3 gene translocations are compossible with CAMTA1 gene rearrangements

  2016cited by this paper
• Sox10—A Marker for Not Only Schwannian and Melanocytic Neoplasms But Also Myoepithelial Cell Tumors of Soft Tissue: A Systematic Analysis of 5134 Tumors

  2015cited by this paper
• CAMTA1 is a useful immunohistochemical marker for diagnosing epithelioid haemangioendothelioma

  2015cited by this paper
• Thoracic Epithelioid Malignant Vascular Tumors: A Clinicopathologic Study of 52 Cases With Emphasis on Pathologic Grading and Molecular Studies of WWTR1-CAMTA1 Fusions

  2015cited by this paper
• Methylation Status of the Chromosome Arm 19q MicroRNA Cluster in Sporadic and Androgenetic-Biparental Mosaicism–Associated Hepatic Mesenchymal Hamartoma

  2015cited by this paper
• Molecular Characterization of Inflammatory Myofibroblastic Tumors With Frequent ALK and ROS1 Gene Fusions and Rare Novel RET Rearrangement

  2015cited by this paper
• Array CGH analysis identifies two distinct subgroups of primary angiosarcoma of bone

  2015cited by this paper
• Metastatic Malignant Melanoma With Complete Loss of Differentiation Markers (Undifferentiated/Dedifferentiated Melanoma): Analysis of 14 Patients Emphasizing Phenotypic Plasticity and the Value of Molecular Testing as Surrogate Diagnostic Marker

  2015cited by this paper
• Targeted massively parallel sequencing of angiosarcomas reveals frequent activation of the mitogen activated protein kinase pathway

  2015cited by this paper
• Characterization of the 12q amplicons in lipomatous soft tissue tumors by multiplex ligation-dependent probe amplification-based copy number analysis.

  2015cited by this paper
• Towards an international pediatric liver tumor consensus classification: proceedings of the Los Angeles COG liver tumors symposium

  2014cited by this paper
• Malignant vascular tumors—an update

  2014cited by this paper
• Epstein-Barr virus-associated smooth muscle tumors in AIDS patients: a largest case (series).

  2014cited by this paper
• STAT6 is amplified in a subset of dedifferentiated liposarcoma

  2014cited by this paper
• Recurrent PTPRB and PLCG1 mutations in angiosarcoma

  2014cited by this paper
• Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics

  2014cited by this paper
• Epithelioid Hemangioendothelioma: clinicopathologic, immunhistochemical, and molecular genetic analysis of 39 cases

  2014cited by this paper
• The utility of ERG, CD31 and CD34 in the cytological diagnosis of angiosarcoma: an analysis of 25 cases

  2014cited by this paper
• Novel YAP1‐TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma

  2013cited by this paper
• Nested Stromal Epithelial Tumor of the Liver in Beckwith-Wiedemann Syndrome

  2013cited by this paper
• Whole exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors

  2013cited by this paper
• Characterization of translocations in mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver.

  2013cited by this paper
• Treatment of undifferentiated embryonal sarcoma of the liver in children--single center experience.

  2013cited by this paper
• Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing

  2013cited by this paper
• Anastomosing Hemangioma of the Liver and Gastrointestinal Tract: An Unusual Variant Histologically Mimicking Angiosarcoma

  2013cited by this paper
• Sox10 and S100 in the Diagnosis of Soft-tissue Neoplasms

  2012cited by this paper
• The dark side of a success story: microRNAs of the C19MC cluster in human tumours

  2012cited by this paper
• Connective tissue growth factor in tumor pathogenesis

  2012cited by this paper
• β-Catenin mutations in 2 nested stromal epithelial tumors of the liver--a neoplasia with defective mesenchymal-epithelial transition.

  2012cited by this paper
• Diagnostic Utility of p16, CDK4, and MDM2 as an Immunohistochemical Panel in Distinguishing Well-differentiated and Dedifferentiated Liposarcomas From Other Adipocytic Tumors

  2012cited by this paper
• In hepatocellular carcinoma miR‐519d is up‐regulated by p53 and DNA hypomethylation and targets CDKN1A/p21, PTEN, AKT3 and TIMP2

  2012cited by this paper
• High level of Ets‐related gene expression has high specificity for prostate cancer: a tissue microarray study of 11 483 cancers

  2012cited by this paper
• Nested Stromal Epithelial Tumor of the Liver—Liver Transplantation and Follow-Up

  2011cited by this paper
• Rhabdoid tumors of the liver: Rare, aggressive, and poorly responsive to standard cytotoxic chemotherapy

  2011cited by this paper
• Identification of a Disease-Defining Gene Fusion in Epithelioid Hemangioendothelioma

  2011cited by this paper
• Clinical patterns and outcome in epithelioid hemangioendothelioma with or without pulmonary involvement: insights from an internet registry in the study of a rare cancer.

  2011cited by this paper
• Undifferentiated Embryonal Sarcoma of the Liver is Associated with Mesenchymal Hamartoma and Multiple Chromosomal Abnormalities: A Review of Eleven Cases

  2011cited by this paper
• Consistent MYC and FLT4 gene amplification in radiation‐induced angiosarcoma but not in other radiation‐associated atypical vascular lesions

  2011cited by this paper
• The final steps of integrin activation: the end game

  2010cited by this paper
• The primate-specific microRNA gene cluster (C19MC) is imprinted in the placenta.

  2010cited by this paper
• Arginase-1: A New Immunohistochemical Marker of Hepatocytes and Hepatocellular Neoplasms

  2010cited by this paper
• MYC high level gene amplification is a distinctive feature of angiosarcomas after irradiation or chronic lymphedema.

  2010cited by this paper
• A Distinctive Subset of PEComas Harbors TFE3 Gene Fusions

  2010cited by this paper
• Anastomosing Hemangioma of the Genitourinary Tract: A Lesion Mimicking Angiosarcoma

  2009cited by this paper

• Use of Indocyanine Green in Fluorescence-Guided Resection of Mesenchymal Hamartoma of the Liver: A Report of Two Cases

  2025cites this paper
• Calcifying Nested Stromal-Epithelial Tumor (CNSET) of the Liver: A Case Report With Its Molecular Profile

  2025cites this paper
• Massive undifferentiated embryonal sarcoma: An unusual primary hepatic malignancy

  2025cites this paper
• A Practical Approach to the Diagnosis of Liver Metastases from Cancer of Unknown Primary: Application of Immunohistochemistry and an Update.

  2025cites this paper
• Risk prediction criteria for the primary hepatic perivascular epithelioid cell tumour family, including angiomyolipoma: analysis of 132 cases with a literature review

  2024cites this paper
• Undifferentiated Embryonal Sarcoma of Liver- A Rare Case Entity

  2024cites this paper
• Tumor-like conditions that mimic liver tumors

  2024cites this paper
• Aggressive Variant of Hepatic Epstein–Barr Virus-Associated Inflammatory Pseudotumor-like Follicular Dendritic Cell Sarcoma with PD-L1 and SSTR2a Expression

  2023influential citation
• Update on the Pathology of Pediatric Liver Tumors: A Pictorial Review

  2023cites this paper