Pathological diagnosis and treatment progress of hepatic epithelioid hemangioendothelioma

Abstract

Abstract: Hepatic epithelioid hemangioendothelioma(HEHE) is a rare vascular endothelial cell tumor, composed of epithelioid and histiocyte-like vascular endothelial cells in mucus or fibrotic stroma, and is low to moderately malignant. The etiology of HEHE is unclear. It can be characterized as single, multiple, or diffuse, and the multifocal form is most common. There is no specificity in imaging diagnosis including ultrasound, MRI, and CT. The diagnosis mainly depends on histopathology and immunohistochemical staining. Immunohistochemical antibodies include vascular endothelial markers CD31, CD34, CD10, D2-40, ERG, FLI-1, factorⅧ etc, and epithelial markers CD8/18, EMA, etc. Changes in molecular biology mainly include the WWTR1-CAMTA1 fusion gene, and a small number of YAP1-TFE3 fusion genes can be detected and diagnosed by FISH. Liver resection and liver transplantation are the preferred treatment methods for HEHE.

Key words: Epithelioid hemangioendothelioma, Liver, Pathological diagnosis, Treatment

Liang Yiting, Shi Susheng. Pathological diagnosis and treatment progress of hepatic epithelioid hemangioendothelioma[J]. Electronic Journal of Liver Tumor, 2021, 8(2): 53-56.

References

[1] WEISS S W, ENZINGER F M.Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma[J]. Cancer, 1982, 50(5): 970-981.
[2] LAU K, MASSAD M, POLLAK C, et al.Clinical patterns and outcome in epithelioid hemangioendothelioma with or without pulmonary involvement: insights from an internet registry in the study of a rare cancer[J]. Chest, 2011, 140(5): 1312-1318.
[3] ROSENBAUM E, JADEJA B, XU B, et al.Prognostic stratification of clinical and molecular epithelioid hemangioendothelioma subsets[J]. Mod Pathol, 2020, 33(4): 591-602.
[4] MEHRABI A, KASHFI A, FONOUNI H, et al.Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy[J]. Cancer, 2006, 107(9): 2108-2121.
[5] ANTONESCU C. Malignant vascular tumors--an update[J]. Mod Pathol, 2014, 27 Suppl 1: S30-38.
[6] MAMONE G, MIRAGLIA R.The “Target sign” and the “Lollipop sign” in hepatic epithelioid hemangioendothelioma[J]. Abdom Radiol (NY), 2019, 44(4): 1617-1620.
[7] GURUNG S, FU H, ZHANG W W, et al.Hepatic epithelioid hemangioendothelioma metastasized to the peritoneum, omentum and mesentery: a case report[J]. Int J Clin Exp Pathol, 2015, 8(5): 5883-5889.
[8] ZHOU L, CUI M Y, XIONG J, et al.Spectrum of appearances on CT and MRI of hepatic epithelioid hemangioendothelioma[J]. BMC Gastroenterol, 2015, 15: 69.
[9] YANG JW, LI Y, XIE K, et al.Spontaneous rupture of hepatic epithelioid hemangioendothelioma: A case report[J]. World J Gastroenterol. 2017, 23(1): 185-190.
[10] AFRIT M, NASRI M, LABIDI S, et al.Aggressive primary hepatic epithelioid hemangioendothelioma: a case report and literature review[J]. Cancer Biol Med, 2017, 14(2): 187-190.
[11] MAKHLOUF H R, ISHAK K G, GOODMAN Z D.Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases[J]. Cancer, 1999, 85(3): 562-582.
[12] REMISZEWSKI P, SZCZERBA E, KALINOWSKI P, et al.Epithelioid hemangioendothelioma of the liver as a rare indication for liver transplantation[J]. World J Gastroenterol. 2014, 20(32): 11333-11339.
[13] DEYRUP A T, TIGHIOUART M, MONTAG A G, et al.Epithelioid hemangioendothelioma of soft tissue: a proposal for risk stratification based on 49 cases[J]. Am J Surg Pathol, 2008, 32(6): 924-927.
[14] FUJII T, ZEN Y, SATO Y, et al.Podoplanin is a useful diagnostic marker for epithelioid hemangioendothelioma of the liver[J]. Mod Pathol, 2008, 21(2): 125-130.
[15] FLUCKE U, VOGELS R J C, DE SAINT AUBAIN SOMERHAUSEN N, et al. Epithelioid Hemangioendothelioma: clinicopathologic, immunhistochemical, and molecular genetic analysis of 39 cases[J]. Diagn Pathol, 2014, 9: 131.
[16] DOYLE L A, FLETCHER C D M, HORNICK J L. Nuclear Expression of CAMTA1 Distinguishes Epithelioid Hemangioendothelioma From Histologic Mimics[J]. Am J Surg Pathol, 2016, 40(1): 94-102.
[17] ERRANI C, ZHANG L, SUNG Y S, et al.A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites[J]. Genes Chromosomes Cancer, 2011, 50(8): 644-653.
[18] ANTONESCU C R, LE LOARER F, MOSQUERA J-M, et al.Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma[J]. Genes Chromosomes Cancer, 2013, 52(8): 775-784.
[19] KUO F Y, HUANG H Y, CHEN C L, et al.TFE3-rearranged hepatic epithelioid hemangioendothelioma-a case report with immunohistochemical and molecular study[J]. APMIS: 2017, 125(9): 849-853.
[20] MOGLER C, KOSCHNY R, HEILIG C E, et al.Molecular characterization of hepatic epithelioid hemangioendothelioma reveals alterations in various genes involved in DNA repair, epigenetic regulation, signaling pathways, and cell cycle control[J]. Genes Chromosomes Cancer, 2020, 59(2): 106-110.
[21] PANSE G, CHRISINGER J S, LEUNG C H, et al.Clinicopathological analysis of ATRX, DAXX and NOTCH receptor expression in angiosarcomas[J]. Histopathology, 2018, 72(2): 239-247.
[22] DEMETRIS A J, MINERVINI M, RAIKOW R B, et al.Hepatic epithelioid hemangioendothelioma: biological questions based on pattern of recurrence in an allograft and tumor immunophenotype[J]. Am J Surg Pathol, 1997, 21(3): 263-270.
[23] EMAMAULLEE J A, NOWAK K, BEACH M, et al.Hepatic Epithelioid Hemangioendothelioma Presenting as an Enlarging Vascular Lesion within the Spleen[J]. Case Rep Transplant, 2018, 2018: 3948784.
[24] GAN L U, CHANG R, JIN H, et al.Typical CT and MRI signs of hepatic epithelioid hemangioendothelioma[J]. Oncol Lett, 2016, 11(3): 1699-1706.
[25] LLUECA A, PIQUER D, MAAZOUZI Y, et al.Hepatic epithelioid hemangioendothelioma: A great mimicker[J]. International Journal of Surgery Case Reports, 2018, 53: 25-28.
[26] JUNG D H, HWANG S, HONG S M, et al.Clinicopathological Features and Prognosis of Hepatic Epithelioid Hemangioendothelioma After Liver Resection and Transplantation[J]. Ann Transplant, 2016, 21: 784-790.
[27] LAI Q, FEYS E, KARAM V, et al.Hepatic Epithelioid Hemangioendothelioma and Adult Liver Transplantation: Proposal for a Prognostic Score Based on the Analysis of the ELTR-ELITA Registry[J]. Transplantation, 2017, 101(3): 555-564.