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CASE REPORT  
Year : 2021  |  Volume : 64  |  Issue : 5  |  Page : 143-145
Epithelioid hemangioma of liver—The first reported case


1 Department of Surgery, Moritz Kaposi General Hospital, Kaposvár, 20-32. Tallián Gy. Street, Kaposvár 7400; Doctoral School of Clinical Medicine of University of Debrecen, Debrecen, 1. Egyetem Square, Debrecen 4032, Hungary
2 Department of Surgery, Moritz Kaposi General Hospital, Kaposvár, 20-32. Tallián Gy. Street, Kaposvár 7400, Hungary
3 Department of Pathology, Moritz Kaposi General Hospital, Kaposvár, 20-32. Tallián Gy. Street, Kaposvár 7400, Hungary
4 Department of Surgery, Borsod-Abaúj-Zemplén County Hospital and University Teaching Hospital, Miskolc, 72-76. Szentpéteri Gate, Miskolc 3526, Hungary

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Date of Submission17-Feb-2020
Date of Decision10-May-2020
Date of Acceptance28-Oct-2020
Date of Web Publication7-Jun-2021
 

   Abstract 


Epithelioid hemangioma, otherwise known as angiolymphoid hyperplasia with eosinophilia, is a rare benign vasoproliferative disease with an unknown etiology. We report the case of a 42-year-old man with routine bloods test showing mildly elevated serum bilirubin level. CT scan revealed a lesion in the left liver lobe not typical of FNH. PET/CT scan and a dynamic liver MRI were consistent with a malignant mass. Surgical resection was performed. Histopathology of the 45 mm mass reported well-defined, slightly lobular proliferations of capillary-sized vessels around several central muscular vessels. Although the endothelial cells revealed a “hobnail” appearance, none of them showed pleomorphism or mitotic activity. Endothelial cells showed reactivity for the endothelial markers (CD34, CD31) and smooth muscle was detected in the blood vessel walls via immunohistochemistry. Despite its benign nature, epithelioid hemangioma of the liver can lead to a diagnostic and therapeutic dilemma due to the malignant looking features on imaging modalities.

Keywords: Angiolymphoid hyperplasia with eosinophilia, epithelioid hemangioma, liver

How to cite this article:
Biro A, Ternyik L, Czoma V, Hegedűs G, Bálint I, Tóth D, Káposztás Z. Epithelioid hemangioma of liver—The first reported case. Indian J Pathol Microbiol 2021;64, Suppl S1:143-5

How to cite this URL:
Biro A, Ternyik L, Czoma V, Hegedűs G, Bálint I, Tóth D, Káposztás Z. Epithelioid hemangioma of liver—The first reported case. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 Oct 25];64, Suppl S1:143-5. Available from: https://www.ijpmonline.org/text.asp?2021/64/5/143/317904





   Introduction Top


Epithelioid hemangioma (EH), otherwise known as angiolymphoid hyperplasia with eosinophilia (AILH), is a rare benign vasoproliferative disease with unknown etiology. The first mentioning is from Wells and Whimstar in 1961 who described it as a late stage of Kimura disease. Now we know that they are separate entities.[1],[2] Buder et al. reported that EH and Kimura disease might represent two extreme variants of the same condition.[3] EH commonly presents in middle-aged adults with female predominancy, mainly with cutaneous or subcutaneous nodules in the head and neck region.[4] This is the first reported EH case in the liver.


   Case History Top


We report the case of a 42-year-old man without any significant previous medical history or symptoms. Routine blood tests showed a mildly elevated serum bilirubin level (24 umol/l). An abdominal ultrasound scan showed features of focal nodular hyperplasia (FNH). A CT scan revealed a lesion in the left liver lobe, which was not typical of FNH. A contrast-enhanced ultrasound scan was than performed to further characterize the mass. It proved the malignant dynamics of the solitary liver lesion, which seemed to be typical for hepatocellular carcinoma. A PET/CT scan and a dynamic liver MRI were consistent with a malignant mass formation in segment three. Tumor markers, namely CEA, CA19-9, and AFP were normal. Surgical resection of segment three and segment four was performed. Macroscopic observation showed a mass measuring 45 mm in the resected liver segments. Histopathology reported well- defined, slightly lobular proliferations of capillary-sized vessels around several central muscular vessels. The blood vessels were lined with large eosinophilic vacuolated endothelial cells with a vesicular nucleus, one or more nucleoli, abundant cytoplasm, and a polygonal shape, resembling histiocytes or epithelial cells. Although the endothelial cells revealed a “hobnail” appearance, none of them showed pleomorphism or mitotic activity. The lesion had a fibrous background and was accompanied by lymphoid aggregates, eosinophils, histiocytes, and scattered plasma cells. Lymphoid nodules having no clear germinal center were detected, but no epithelioid cells, multinucleated giant cells or granulomas were seen. Immunohistochemistry showed a predominance of T-lymphocytes with scattered B-cells occasionally forming lymphoid follicles. Endothelial cells showed reactivity for the endothelial markers (CD34, CD31) and smooth muscle was detected in the blood vessel wall with smooth muscle actin immunohistochemistry [Figure 1] and [Figure 2].
Figure 1: Proliferations of vessels surrounded by lymphocytes, eosinophils and histiocytes within the fibrous stroma (hematoxylin-eosin, original magnification x40)

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Figure 2: Proliferation of capillary-sized vessel with “hobnailed” endothelial cells (hematoxylin-eosin, original magnification x100)

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   Discussion Top


EH is a rare benign vasculoproliferative disorder, which is considered to be a reactive disorder induced by various stimuli (e.g., pregnancy, trauma, vascular malformations such as arterio-venous shunt) The patient in this case report had a hemangioma and a benign cyst in the liver. Despite its benign nature EH can lead to a diagnostic and therapeutic dilemma due to the malignant looking features on imaging modalities. Systemic eosinophilia may also be seen, but not required to confirm the diagnosis. Although recurrence is frequent, treatment for EH is usually by surgical resection. Histologically it demonstrates proliferations of capillary-sized vessels with large eosinophilic vacuolated endothelial cells within a fibrous background, typically accompanied by lymphoid aggregates with eosinophils, plasma cells, and histiocytes. The predominance of vascular structures and inflammatory background depends on the age of the lesion. The vascular component is typically seen in the early stages, whereas lymphocytes may predominate in older lesions. It is difficult to distinguish from Kimura disease, which is more common in Asian patients and presents as deep nodules or subcutaneous lesions that typically affect regional lymph nodes and is combined with eosinophilia and an elevated serum IgE-level.[5] Other potential pathologies that should also be considered include Kaposi sarcoma, epithelioid angiosarcoma, epithelioid hemangioendothelioma, hemangioma, and bacillary angiomatosis.[6] Angiosarcoma presents as infiltrative, anastomosing vascular channels with tumor cells having nuclear atypia, hyperchromatism, mitotic activity and scarce eosinophils in the inflammatory infiltrate. Epithelioid hemangioendothelioma is a semimalignant tumor with characteristic zonal growth. The periphery shows sinusoidal proliferation with tufting of tumor cells and the midzone has sclerotic or myxochondroid stroma. The round- shaped epithelioid cells have mild or moderately atypical nuclei with prominent nucleoli and focal vacuolized cytoplasm containing red blood cells. The tumor often has infiltrative margins. Hemangiomas do not show inflammatory infiltration and enlarged polygonal endothelial cells. Bacillary angiomatosis also presents as lobular proliferation of capillaries with ectatic vessels lined by prominent endothelial cells. The presence of neutrophils in the infiltrate is a characteristic sign.

Here we presented a case of a 42-year-old man with a left liver lobe lesion of unknown origin. Histopathology and immunohistochemistry revealed epithelioid hemangioma. In case of an unknown liver mass, EH should be kept in mind, especially when AFP levels are high. As it has malignant looking features on imaging modalities, it can lead to diagnostic and therapeutic dilemma which gives the importance of this unique case.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgement

The authors state that they have received no payment in the preparation of this paper.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Wells GC, Whimster IW. Subcutaneous angiolymphoid hyperplasia with eosinophilia. Br J Dermatol 1969;81:1-14.  Back to cited text no. 1
    
2.
Googe PB, Harris NL, Mihm MC. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: Two distinct histopathological entities. J Cutan Pathol 1987;14:263-271.  Back to cited text no. 2
    
3.
Buder K, Ruppert S, Trautmann A, Bröcker EB, Goebeler M, Kerstan A. Angiolymphoid hyperplasia with eosinophilia and Kimura's disease - a clinical and histopathological comparison. J Dtsch Dermatol Ges 2014;12:224-8.  Back to cited text no. 3
    
4.
Grimwood R, Swinehart JM, Aeling JL. Angiolymphoid hyperplasia with eosinophilia. Arch Dermatol 1979;115:205-7.  Back to cited text no. 4
    
5.
Briggs PL. Kimura disease is not angiolymphoid hyperplasia with eosinophilia: Clinical and pathological correlation with literature review and definition of diagnostic criteria. An Bras Dermatol 2006;2:167-73.  Back to cited text no. 5
    
6.
Guo R, Gavino AC. Angiolymphoid hyperplasia with eosinophilia. Arch Pathol Lab Med 2015;139:683-6.  Back to cited text no. 6
    

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Correspondence Address:
Adrienn Biro
Department of Surgery, Moritz Kaposi General Hospital, Kaposvár, 20-32 Tallián Gy. Street, Kaposvár 7400
Hungary
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_148_20

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