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Hepatic epithelioid hemangioendothelioma
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Clinics and Research in Hepatology and Gastroenterology (2015) xxx, xxx—xxx
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Hepatic epithelioid hemangioendothelioma Athena Galletto Pregliasco a,b, Dominique Wendum c, Claire Goumard d, Lionel Arrivé a,∗ a
Sorbonne universités, UPMC — université Paris 06, faculté de médecine Pierre-et-Marie-Curie and Department of Radiology and Pathology, Saint-Antoine Hospital, Assistance publique—Hôpitaux de Paris, 184, rue de Faubourg-Saint-Antoine, 75012 Paris, France b Unit of Radiology, E.O. Ospedali Galliera, Mura della Cappuccine 14, 16128 Genova, Italy c Department of Pathology, Saint-Antoine Hospital, Paris, France d Department of Hepatobiliary Surgery, Pitié-Salpêtrière Hospital, Paris, France
A 36-year-old woman with no relevant medical or surgical history was referred to our attention for abdominal diffuse pain and mild weight loss. No risk factors such as hepatitis, alcohol abuse, oral contraceptive use, or a family history of carcinoma were reported. Liver laboratory studies were unremarkable, except for elevated gamma-glutamyl transferase (138 U/L). Viral serology tests were negative, as were serum carcinoembryonic antigen, CA 19-9, and alphafetoprotein. Abdominal ultrasonography revealed multiple round hypoechoic nodules, which produced no signal at color Doppler. Computed tomography images were obtained before and after contrast injection. The unenhanced phase showed multiple hypodense lesions. On the arterial phase (35 ), there was almost no contrast enhancement, whereas in the portal phase (90 ), only minimal peripheral enhancement was seen; the inner part of the lesion remaining unenhanced. On T1-weighted magnetic resonance (MR) images, the nodules were hypointense, with an internal area of lower signal intensity in the largest lesions. On T2-weighted MR images, all lesions showed mild signal
∗
Corresponding author. E-mail address: [email protected] (L. Arrivé).
hyperintensity, with a core of higher signal intensity (Fig. 1a). Dynamic contrast-enhanced imaging showed mild, inhomogeneous and progressive centripetal enhancement, although the center of the largest lesion remained minimally enhanced (Fig. 1b). Moreover, some nodules tended to merge together, with sub-capsular nodules causing capsular retraction (Fig. 1b). The patient underwent tumorectomy, and excisional biopsy revealed epithelioid hemangioendothelioma. This diagnosis was confirmed at pathological analysis after liver transplantation performed six months later. Macroscopic pathological analysis revealed several coalescent, gray-white hepatic lesions (Fig. 2a). Microscopic analysis revealed spindle-shaped tumor cells and epithelioid tumor cells with intracytoplasmic vacuoles, some of which contained red blood cells (Fig. 2b). Immunohistochemical staining was positive for the endothelial marker CD31 (Fig. 2c). Epithelioid hemangioendothelioma of the liver is a rare, low-grade malignant neoplasm of endothelial origin. It typically occurs in female patients aged 20—40 years. Its diagnosis can be challenging: it can be discovered accidentally or revealed by unspecific symptoms such as right upper quadrant pain, weight loss, fatigue, and jaundice. Imaging findings can be helpful in
http://dx.doi.org/10.1016/j.clinre.2015.10.002 2210-7401/© 2015 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Galletto Pregliasco A, et al. Hepatic epithelioid hemangioendothelioma. Clin Res Hepatol Gastroenterol (2015), http://dx.doi.org/10.1016/j.clinre.2015.10.002
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ARTICLE IN PRESS
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A. Galletto Pregliasco et al.
Figure 1 MR imaging: a: T2-weighted MR image showing mild signal hyperintensity of the abdominal lesions, with a core of higher signal intensity; b: injected portal phase showing inhomogeneous and progressive centripetal enhancement; the center of the largest lesion remaining minimally enhanced. Note the capsular retraction.
guiding the diagnosis; characteristic imaging features of epithelioid hemangioendothelioma include multiple hepatic masses with peripheral location, capsular retraction, nodule coalescence, target-like configuration (so-called ‘‘lollipop sign’’), and peripheral and delayed contrast enhancement [1]. Characteristic pathological features include intracytoplasmic vascular lumen formation, sinusoidal spreading, vessel obliteration, necrosis in the center of the lesion, and cellular pleomorphism. Positive immunohistochemistry for endothelial markers such as factor VIII, CD31, or CD34 confirms the diagnosis. High cellularity is the most significant factor predicting an unfavorable prognosis [2]. Several treatment strategies are available, including systemic/regional chemotherapy, radiotherapy, hormone therapy, thermoablation, liver resection, and liver transplantation [3]. The latter option is the most common treatment modality, because the vast majority of patients have multifocal lesions at the time of diagnosis [4]. The prognosis depends on whether extrahepatic involvement is present at the time of diagnosis, with survival rates at 1 and 5 years of 80% and 64%, respectively.
Figure 2 Pathological analysis: a: gross appearance of two gray-white hepatic lesions; b: the lesions showed spindleshaped tumor cells and epithelioid tumor cells with vacuoles, some of which contained red blood cells (arrows) (hematoxylin and eosin, × 200); c: tumor cells were positive for the endothelial marker, CD31 (CD31 immunostaining, × 200).
Disclosure of interest The authors declare that they have no competing interest.
References [1] Azzam RI, Alshak NS, Pham HP. AIRP best cases in radiologicpathologic correlation: hepatic epithelioid hemangioendothelioma. Radiographics 2012;32(3):789—94. [2] Thin LW, Wong DD, De Boer BW, Ferguson JM, Adams L, Macquillan G, et al. Hepatic epithelioid haemangioendothelioma:
Please cite this article in press as: Galletto Pregliasco A, et al. Hepatic epithelioid hemangioendothelioma. Clin Res Hepatol Gastroenterol (2015), http://dx.doi.org/10.1016/j.clinre.2015.10.002
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ARTICLE IN PRESS
Hepatic epithelioid hemangioendothelioma challenges in diagnosis and management. Intern Med J 2010;40(10):710—5. [3] Wang LR, Zhou JM, Zhao YM, He HW, Chai ZT, Wang M, et al. Clinical experience with primary hepatic epithelioid hemangioendothelioma: retrospective study of 33 patients. World J Surg 2012;36(11):2677—83.
3 [4] Remiszewski P, Szczerba E, Kalinowski P, Gierej B, Dudek K, Grodzicki M, et al. Epithelioid hemangioendothelioma of the liver as a rare indication for liver transplantation. World J Gastroenterol 2014;20(32):11333—9.
Please cite this article in press as: Galletto Pregliasco A, et al. Hepatic epithelioid hemangioendothelioma. Clin Res Hepatol Gastroenterol (2015), http://dx.doi.org/10.1016/j.clinre.2015.10.002
ARTICLE IN PRESS
CLINRE-820; No. of Pages 3
Clinics and Research in Hepatology and Gastroenterology (2015) xxx, xxx—xxx
Available online at
ScienceDirect www.sciencedirect.com
IMAGE OF THE MONTH
Hepatic epithelioid hemangioendothelioma Athena Galletto Pregliasco a,b, Dominique Wendum c, Claire Goumard d, Lionel Arrivé a,∗ a
Sorbonne universités, UPMC — université Paris 06, faculté de médecine Pierre-et-Marie-Curie and Department of Radiology and Pathology, Saint-Antoine Hospital, Assistance publique—Hôpitaux de Paris, 184, rue de Faubourg-Saint-Antoine, 75012 Paris, France b Unit of Radiology, E.O. Ospedali Galliera, Mura della Cappuccine 14, 16128 Genova, Italy c Department of Pathology, Saint-Antoine Hospital, Paris, France d Department of Hepatobiliary Surgery, Pitié-Salpêtrière Hospital, Paris, France
A 36-year-old woman with no relevant medical or surgical history was referred to our attention for abdominal diffuse pain and mild weight loss. No risk factors such as hepatitis, alcohol abuse, oral contraceptive use, or a family history of carcinoma were reported. Liver laboratory studies were unremarkable, except for elevated gamma-glutamyl transferase (138 U/L). Viral serology tests were negative, as were serum carcinoembryonic antigen, CA 19-9, and alphafetoprotein. Abdominal ultrasonography revealed multiple round hypoechoic nodules, which produced no signal at color Doppler. Computed tomography images were obtained before and after contrast injection. The unenhanced phase showed multiple hypodense lesions. On the arterial phase (35 ), there was almost no contrast enhancement, whereas in the portal phase (90 ), only minimal peripheral enhancement was seen; the inner part of the lesion remaining unenhanced. On T1-weighted magnetic resonance (MR) images, the nodules were hypointense, with an internal area of lower signal intensity in the largest lesions. On T2-weighted MR images, all lesions showed mild signal
∗
Corresponding author. E-mail address: [email protected] (L. Arrivé).
hyperintensity, with a core of higher signal intensity (Fig. 1a). Dynamic contrast-enhanced imaging showed mild, inhomogeneous and progressive centripetal enhancement, although the center of the largest lesion remained minimally enhanced (Fig. 1b). Moreover, some nodules tended to merge together, with sub-capsular nodules causing capsular retraction (Fig. 1b). The patient underwent tumorectomy, and excisional biopsy revealed epithelioid hemangioendothelioma. This diagnosis was confirmed at pathological analysis after liver transplantation performed six months later. Macroscopic pathological analysis revealed several coalescent, gray-white hepatic lesions (Fig. 2a). Microscopic analysis revealed spindle-shaped tumor cells and epithelioid tumor cells with intracytoplasmic vacuoles, some of which contained red blood cells (Fig. 2b). Immunohistochemical staining was positive for the endothelial marker CD31 (Fig. 2c). Epithelioid hemangioendothelioma of the liver is a rare, low-grade malignant neoplasm of endothelial origin. It typically occurs in female patients aged 20—40 years. Its diagnosis can be challenging: it can be discovered accidentally or revealed by unspecific symptoms such as right upper quadrant pain, weight loss, fatigue, and jaundice. Imaging findings can be helpful in
http://dx.doi.org/10.1016/j.clinre.2015.10.002 2210-7401/© 2015 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Galletto Pregliasco A, et al. Hepatic epithelioid hemangioendothelioma. Clin Res Hepatol Gastroenterol (2015), http://dx.doi.org/10.1016/j.clinre.2015.10.002
+Model CLINRE-820; No. of Pages 3
ARTICLE IN PRESS
2
A. Galletto Pregliasco et al.
Figure 1 MR imaging: a: T2-weighted MR image showing mild signal hyperintensity of the abdominal lesions, with a core of higher signal intensity; b: injected portal phase showing inhomogeneous and progressive centripetal enhancement; the center of the largest lesion remaining minimally enhanced. Note the capsular retraction.
guiding the diagnosis; characteristic imaging features of epithelioid hemangioendothelioma include multiple hepatic masses with peripheral location, capsular retraction, nodule coalescence, target-like configuration (so-called ‘‘lollipop sign’’), and peripheral and delayed contrast enhancement [1]. Characteristic pathological features include intracytoplasmic vascular lumen formation, sinusoidal spreading, vessel obliteration, necrosis in the center of the lesion, and cellular pleomorphism. Positive immunohistochemistry for endothelial markers such as factor VIII, CD31, or CD34 confirms the diagnosis. High cellularity is the most significant factor predicting an unfavorable prognosis [2]. Several treatment strategies are available, including systemic/regional chemotherapy, radiotherapy, hormone therapy, thermoablation, liver resection, and liver transplantation [3]. The latter option is the most common treatment modality, because the vast majority of patients have multifocal lesions at the time of diagnosis [4]. The prognosis depends on whether extrahepatic involvement is present at the time of diagnosis, with survival rates at 1 and 5 years of 80% and 64%, respectively.
Figure 2 Pathological analysis: a: gross appearance of two gray-white hepatic lesions; b: the lesions showed spindleshaped tumor cells and epithelioid tumor cells with vacuoles, some of which contained red blood cells (arrows) (hematoxylin and eosin, × 200); c: tumor cells were positive for the endothelial marker, CD31 (CD31 immunostaining, × 200).
Disclosure of interest The authors declare that they have no competing interest.
References [1] Azzam RI, Alshak NS, Pham HP. AIRP best cases in radiologicpathologic correlation: hepatic epithelioid hemangioendothelioma. Radiographics 2012;32(3):789—94. [2] Thin LW, Wong DD, De Boer BW, Ferguson JM, Adams L, Macquillan G, et al. Hepatic epithelioid haemangioendothelioma:
Please cite this article in press as: Galletto Pregliasco A, et al. Hepatic epithelioid hemangioendothelioma. Clin Res Hepatol Gastroenterol (2015), http://dx.doi.org/10.1016/j.clinre.2015.10.002
+Model CLINRE-820; No. of Pages 3
ARTICLE IN PRESS
Hepatic epithelioid hemangioendothelioma challenges in diagnosis and management. Intern Med J 2010;40(10):710—5. [3] Wang LR, Zhou JM, Zhao YM, He HW, Chai ZT, Wang M, et al. Clinical experience with primary hepatic epithelioid hemangioendothelioma: retrospective study of 33 patients. World J Surg 2012;36(11):2677—83.
3 [4] Remiszewski P, Szczerba E, Kalinowski P, Gierej B, Dudek K, Grodzicki M, et al. Epithelioid hemangioendothelioma of the liver as a rare indication for liver transplantation. World J Gastroenterol 2014;20(32):11333—9.
Please cite this article in press as: Galletto Pregliasco A, et al. Hepatic epithelioid hemangioendothelioma. Clin Res Hepatol Gastroenterol (2015), http://dx.doi.org/10.1016/j.clinre.2015.10.002