Epithelioid Hemangioendothelioma, Multiple Focal Nodular Hyperplasias, and Cavernous Hemangiomas of the Liver

1999 ◽  
Vol 123 (9) ◽  
pp. 846-849 ◽  
Author(s):  
Marie-Pierre Bralet ◽  
Benoit Terris ◽  
Valérie Vilgrain ◽  
Laurence Brégeaud ◽  
Georges Molas ◽  
...  
Keyword(s):  
Young Woman ◽  
Focal Nodular Hyperplasia ◽  
Vascular Supply ◽  
Epithelioid Hemangioendothelioma ◽  
Unusual Association ◽  
Vascular Neoplasms ◽  
Nodular Hyperplasia ◽  
Cavernous Hemangiomas ◽  
True Neoplasm

Abstract Malignant vascular neoplasms of the liver are uncommon. We report the case of a young woman who developed an epithelioid hemangioendothelioma of the liver associated with multiple focal nodular hyperplasias and hepatic cavernous hemangiomas. Such an unusual association is probably not fortuitous and could support the theory that focal nodular hyperplasia is a reaction to an abnormal vascular supply rather than a true neoplasm.

scholarly journals HepaticYAP1-TFE3Rearranged Epithelioid Hemangioendothelioma

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Mira M. Lotfalla ◽  
Andrew L. Folpe ◽  
Karen J. Fritchie ◽  
Patricia T. Greipp ◽  
Gretchen G. Galliano ◽  
...  
Keyword(s):  
Fusion Gene ◽  
Vascular Tumor ◽  
Epithelioid Hemangioendothelioma ◽  
Low Grade ◽  
Diagnostic Pitfall ◽  
Vascular Neoplasms ◽  
Nodular Hyperplasia ◽  
Genetic Features ◽  
Diagnostic Confusion

Epithelioid hemangioendothelioma (EHE) is an uncommon low-grade malignant vascular tumor that may arise in soft tissue/bone or visceral sites such as the liver and lung. As this tumor exhibits epithelioid morphology, it frequently causes diagnostic confusion with other epithelioid vascular neoplasms as well as carcinoma. While 90% of classic EHE are driven by aWWTR1-CAMTA1fusion gene, a histologically distinctive subset of EHE has been recently shown to harbor a different fusion gene,YAP1-TFE3. This variant is likely underrecognized given its rarity and only recent description. Notably, EHE frequently involves the liver but only one case of hepaticYAP1-TFE3rearranged EHE has been reported to date. We present the second case ofYAP1-TFE3rearranged EHE affecting a 65-year-old woman and presenting as multiple liver masses, with characterization of the fusion gene at the transcriptomic and genomic levels. There are several educational points noted from this case.YAP1-TFE3rearranged EHE shows distinctly vasoformative foci, unlike classic EHE and mimicking angiosarcoma or epithelioid hemangioma. The tumors cells show a histiocytoid appearance with voluminous cytoplasm, similar to otherTFE3-rearranged tumors. Finally, in the liver, this tumor may in part mimic focal nodular hyperplasia of the liver which is an underrecognized diagnostic pitfall. This report highlights the key diagnostic and genetic features of this newly recognized variant of hepatic EHE to aid pathologists in appropriately classifying these tumors.

scholarly journals Focal nodular hyperplasia of the liver: an unusual association with diabetes mellitus in a child and review of literature

2010 ◽  
Vol 36 (1) ◽  
pp. 41 ◽  
Author(s):  
Piero Farruggia ◽  
Rita Alaggio ◽  
Francesca Cardella ◽  
Serena Tropia ◽  
Antonino Trizzino ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Focal Nodular Hyperplasia ◽  
Review Of Literature ◽  
Unusual Association ◽  
Nodular Hyperplasia

Focal nodular hyperplasia of the liver: Direct evidence of circulatory disturbances

2000 ◽  
Vol 15 (11) ◽  
pp. 1344-1347 ◽  
Author(s):  
Hideki Kumagai ◽  
Tomoyuki Masuda ◽  
Hiroki Oikawa ◽  
Kaoru Endo ◽  
Mikiya Endo ◽  
...  
Keyword(s):  
Focal Nodular Hyperplasia ◽  
Direct Evidence ◽  
Nodular Hyperplasia ◽  
Circulatory Disturbances

FOCAL NODULAR HYPERPLASIA WITH CALCIFICATION AND OSSIFICATION

Pathology ◽  
2001 ◽  
Vol 33 (3) ◽  
pp. 396-398
Author(s):  
Gerald Langman ◽  
Pauline De La M Hall
Keyword(s):  
Focal Nodular Hyperplasia ◽  
Nodular Hyperplasia

ANGIOSONOGRAPHIC PATTERN OF FOCAL NODULAR HYPERPLASIA: EVALUATION IN 65 LESIONS

2005 ◽  
Vol 26 (S 1) ◽  
Author(s):  
P Tittoto ◽  
E Accogli ◽  
A Domanico ◽  
L Riccardi ◽  
M Pompili ◽  
...  
Keyword(s):  
Focal Nodular Hyperplasia ◽  
Nodular Hyperplasia

Focal Nodular Hyperplasia of the Liver: Triphasic Helical CT Findings

2003 ◽  
Vol 49 (3) ◽  
pp. 181
Author(s):  
Jin Wook Lee ◽  
Won Jae Lee ◽  
Jae Min Cho ◽  
Min Hee Lee ◽  
Hyo K Lim ◽  
...  
Keyword(s):  
Focal Nodular Hyperplasia ◽  
Helical Ct ◽  
Ct Findings ◽  
Nodular Hyperplasia
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