Korean J Thorac Cardiovasc Surg 2015;48:294-297 ISSN: 2233-601X (Print)
□ Case Report □
http://dx.doi.org/10.5090/kjtcs.2015.48.4.294
ISSN: 2093-6516 (Online)
Epithelioid Hemangioendothelioma Arising from Interanl Jugular Vein Mimicking Cervical Metastatic Lymphadenopathy Ju Sik Yun, M.D., Seung Ku Kang, M.D., Sang Hyung Kim, M.D., Kook Joo Na, M.D., Sang Yun Song, M.D.
Epithelioid hemangioendothelioma (EHE) is a relatively rare and moderately aggressive type of vascular tumor. We describe a case of EHE that presented in a 24-year-old woman as a palpable nodule in the left cervical area. Radiological examinations and fine-needle aspiration cytology led to a preliminary diagnosis of metastatic lymphadenopathy with an unknown primary site. However, during surgery, we determined that the nodule was an intravascular tumor arising from the left internal jugular vein. The histopathological examination revealed cords of epithelioid endothelial cells distributed in a hyaline stroma, compatible with a diagnosis of EHE. Key words: 1. Vascular disease 2. Pathology 3. Epithelioid hemangioendothelioma
tomography (PET)/CT, was performed on the assumption that
CASE REPORT
the nodule reflected lymph node metastasis from an unknown A 24-year-old woman was referred to Chonnam National
primary site. Blood tests and tumor marker levels were
University Hwasun Hospital with a palpable nodule in the
normal. On PET/CT, the maximum standardized uptake value
left side of her neck. A nodule had appeared on the site
was 7.7 in the left supraclavicular area (Fig. 1C). However,
where she had undergone a left thyroidectomy for nodular
no detectable primary site was found. We then planned the
hyperplasia two years previously. On physical examination,
surgical excision of the tumor in order to make a definitive
the nodule was found to be hard and painful when pressure
histological diagnosis, in accordance with the patient's desire
was applied, and intermittent swelling of the left upper limb
to remove the tumor. The neck was extended in the supine
was noted, leading to suspicion of a chronically occluded left
position, and a left collar incision was performed. Contrary to
subclavian vein. A chest computed tomography (CT) scan
our expectations, a tumor was found that arose from the in-
showed a 3-cm mass-like lesion in the left supraclavicular
ternal jugular vein. The incision was extended medially and
area with suspected internal jugular vein invasion (Fig. 1A,
laterally in order to define the tumor boundaries and adjacent
B). The diagnosis of the nodule as suspicious for metastatic
vasculature. The intraluminal mass extended inferiorly into
carcinoma was made by sonography-guided fine needle aspi-
the innominate vein and proximal subclavian vein. After the
ration cytology. Further testing, including positron emission
identification of a chronic innominate vein occlusion, the tu-
Department of Thoracic and Cardiovascular Surgery, Chonnam National University Hwasun Hospital, Chonnam National University Medical School Received: August 28, 2014, Revised: November 15, 2014, Accepted: November 18, 2014, Published online: August 5, 2015 Corresponding author: Sang Yun Song, Lung and Esophageal Cancer Clinic, Department of Thoracic and Cardiovascular Surgery, Chonnam National University Hwasun Hospital, 322 Seoyang-ro, Hwasun 519-763, Korea (Tel) 82-61-379-7663 (Fax) 82-61-379-7665 (E-mail)
[email protected] C The Korean Society for Thoracic and Cardiovascular Surgery. 2015. All right reserved. CC This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
− 294 −
Epithelioid Hemangioendothelioma
Fig. 1. Chest computed tomography scan shows a 3-cm mass (arrow) in the left supraclavicular area with a markedly narrowed left internal jugular vein. (A) Coronal view. (B) Axial view. (C) A positron emission tomography scan shows increased uptake (maximum standardized uptake value of 7.7) in the left supraclavicular area. mor was resected en bloc with the involved veins following
HE), and composite HE [1]. Each of these neoplasms shows
ligation of the innominate, left internal jugular, and left sub-
characteristic histopathological features. According to the cur-
clavian veins. Unfortunately, no photographs were taken dur-
rent World Health Organization histological classification
ing or after the operation. The patient’s postoperative course
published in 2002, EHE is defined as ‘an angiocentric vas-
was uneventful and no swelling of the left upper limb was
cular tumor with metastatic potential, composed of epithelioid
observed.
endothelial cells arranged in short cords and nests set in a
Histopathologically, the tumor was found to be composed
distinctive myxohyaline stroma.’ EHE develops in either su-
of cords and nests of spindled epithelioid cells with intra-
perficial or deep soft tissues such as the skin, liver, lung, or
cytoplasmic vacuoles in a hyalinized stroma. Immunohisto-
the extremities. Although EHE usually originates from a ves-
chemical staining for vimentin, CD31, and CD34 was pos-
sel, it is relatively rare for primary vascular EHE to originate
itive (Fig. 2). The surgical margins were free from tumoral
from a large vein or artery, is relatively rare [2]. A few re-
invasion. These histopathological and immunohistochemical
cent reports have described intravascular EHEs arising from
findings were compatible with the diagnosis of an epithelioid
large vessels such as the inferior vena cava [2] and azygos
hemangioendothelioma (EHE).
vein [3].
The patient was referred to the hematology department for
EHE occurs in nearly all age groups, with no gender
adjuvant radiotherapy in order to ensure for better local con-
predilection. Most EHEs are asymptomatic, although edema or
trol, and received a total dose of 60 Gy. Over the course of
thrombophlebitis can develop due to their vascular origin [4].
18 months of follow-up, she has remained in good condition,
In most cases that have been described, the diagnosis was
with no evidence of local recurrence or distant metastasis.
based on postoperative histological findings, because no specific radiological features led to the suspicion of EHE. In our case, the tumor was first suspected to be metastatic lympha-
DISCUSSION
denopathy with an unknown primary site based on preHemangioendothelioma (HE) refers to a group of vascular
operative radiological findings and fine-needle aspiration cy-
neoplasms involving the skin and soft tissues, and is sub-
tology, whereas intraoperative findings indicated that the tu-
classified into six categories: papillary intralymphatic angioen-
mor originated in the left internal jugular vein. A definitive
dothelioma (Dabska tumor), retiform HE, kaposiform HE, ep-
diagnosis of EHE must be based on pathological findings.
ithelioid HE, pseudomyogenic HE (epithelioid sarcoma-like
Typical histological findings include nests and cords of epi-
− 295 −
Ju Sik Yun, et al
Fig. 2. Histopathological and immunohistochemical findings. (A) Cords and nests of spindled epithelioid cells with intracytoplasmic vacuoles were observed in a hyalinized stroma (H&E, ×100). The tumor cells were immunopositive for (B) vimentin (×200), (C) CD31 (×200), and (D) CD34 (×200). thelioid endothelial cells distributed in a myxohyaline stroma
The overall five-year disease-specific survival rate was 81%,
and the presence of intracytoplasmic lumina (vacuoles). The
and the metastasis rate was 22%. They suggested that EHE
tumor cells can be confirmed by immunohistological studies,
can be classified into two risk groups, with markedly different
in which CD31, CD 34, FLI1, and vimentin are the most
clinical courses depending on mitotic activity and tumor size.
commonly used markers [5]. Surgical resection is the preferred
In summary, we have reported a case of epithelioid HE
treatment modality, and chemotherapy or radiotherapy can be
arising from the left internal jugular vein that was pre-
deployed as adjuvant therapies for highly aggressive EHE [6].
operatively misdiagnosed as metastatic lymphadenopathy with
EHE is clinically intermediate between hemangioma and
an unknown primary site.
conventional angiosarcoma. Although the majority of EHEs show an indolent clinical course, metastasis (20%–30%) and
CONFLICT OF INTEREST
death (10%–20%) have been reported in the literature [7]. Deyrup et al. [8] described the histological and clinical features of 49 cases of EHE that originated in the soft tissue.
No potential conflict of interest relevant to this article was reported.
− 296 −
Epithelioid Hemangioendothelioma
REFERENCES 1. Requena L, Kutzner H. Hemangioendothelioma. Semin Diagn Pathol 2013;30:29-44. 2. Scordi-Bello IA, Snyder A, Schwartz M, Fallon JT. Intravascular epithelioid hemangioendothelioma of the inferior vena cava: case report of an unusual and unpredictable vascular tumor. Cardiovasc Pathol 2009;18:243-6. 3. De Palma A, Pagliarulo V, Ardo N, Loizzi D. Surgical treatment of a rare case of epithelioid hemangioendothelioma of the azygos vein. Interact Cardiovasc Thorac Surg 2012;14: 91-3. 4. Campos J, Otero E, Dominguez MJ, Gonzalez-Quintela A. Epithelioid hemangioendothelioma in the posterior mediastinum. Eur J Intern Med 2007;18:331-2. 5. Chirieac LR, Rice DC, Raymond AK. Epithelioid he-
mangioendothelioma in a patient with unusual involvement of the rib and intercostal lymph nodes. J Thorac Cardiovasc Surg 2006;132:1488-9. 6. Bagan P, Hassan M, Le Pimpec Barthes F, et al. Prognostic factors and surgical indications of pulmonary epithelioid hemangioendothelioma: a review of the literature. Ann Thorac Surg 2006;82:2010-3. 7. Weiss SW, Bridge JA. Epithelioid hemangioendothelioma. In: Fletcher CD, Unni K, Mertens F, editors. World Health Organization classification of tumours, in pathology and genetics: tumors of soft tissue and bone. Lyon: IARC Publishing Group; 2002. p. 173-5. 8. Deyrup AT, Tighiouart M, Montag AG, Weiss SW. Epithelioid hemangioendothelioma of soft tissue: a proposal for risk stratification based on 49 cases. Am J Surg Pathol 2008;32:924-7.
− 297 −
