• Journal of the Korean Society of Radiology
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• v.83 no.6
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• pp.1373-1379
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• 2022

Epithelioid hemangioendothelioma (EHE) is a low-grade malignant vascular neoplasm that can occur anywhere in the body. EHE has a low annual incidence (0.38/10⁶) and prevalence (< 1/10⁶), and primary mediastinal EHE is exceedingly rare. We report a case of EHE in a 53-year-old female which manifested as an incidentally discovered mass in the right paratracheal region. In this report, authors describe the pathological and radiological findings of primary mediastinal EHE invading the superior vena cava in the right paratracheal area.

• Korean Journal of Head & Neck Oncology
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• v.29 no.1
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• pp.29-32
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• 2013

카포시형 혈관내피종은 주로 영유아기에 발생하는 혈관내피세포에서 유래하는 혈관종양이다. 성인에서도 발생할 수 있으나 발생률은 정확히 알려져 있지는 않다. 뼈 또는 연조직을 침범할 수 있으며 경계성 종양으로 알려져 있다. 모세혈관종 또는 카포시형 육종과 유사하게 혈관이 포함된 침습적이고 경계가 불분명한 결절을 형성하며, 보통 사지의 연조직이나 간, 폐에 발생하는 경우가 많다. 문헌상으로 두경부에 발생한 증례가 몇 편 보고되어 있으나, 그 중 익구개와에 발생한 경우를 보고한 경우는 없었다. 저자들은 5개월된 남아에서 익구개와에 발생한 카포시형 혈관내피종을 인터페론 알파와 수술적 절제로 성공적으로 치료하였기에 이를 문헌고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
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• v.37 no.12
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• pp.1025-1028
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• 2004

Epithelioid hemangioendothelioma (HE) is a very rare malignant tumor that is pathologically benign tumor originating from endothelial cell but clinically presents metastasis and recurrence. A 29-year-old asymptomatic man, preoperatively diagnosed as lung cancer in the left lower lung, underwent a lobectomy, a wedge lung resection of left upper lung, and partial resection of diaphragm. Left lower lobar lesion was confirmed as pulmonary epithelioid hemangioendothelioma, but the lesions of the left upper lung and diaphragm were remained calcified by spontaneous regression of HE. We report a case of subcutaneous metastasis that occurred two times at 10 months and 19 months after previous surgical treatment of pulmonary EH.

• Journal of Chest Surgery
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• v.30 no.6
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• pp.631-635
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• 1997

혈관에서 기원되는 종양은 혈관외피 세포로부터 발생되는 종양 혈관외피종과 glomus tumor)관 혈관내피 세포로부터 발생되는 종양(를상피 혈관내피종)의 두가지로 구분된다. 이들은 매우 드물게 발생되며 악성종양의 특성을 가지고 있는데, 폐에서 발생된 경우에는 무증상의 작은 종괴로부터 증상이 있는 커다란 종양 의 형태로 나타난다. 최근 저자들은 단일성의 유상피 혈관내피종과 혈관외피종 각각 1례를 치험하였기에 문헌고찰과 함께 보 고하는 바이다. a Tumors of vascular origin are subdivided into two groups: those composed of pericytes (hemangiopericytoma and glomus tumor), and those composed of endothelial cells(hemangioendothelioma). They are uncommon, potentially malignant tumors, and in the lung, the tumors may present as a small asymptomatic nodule or a large symptomatic lesion. Recently we experienced two cases of solitary pulmonary vascular tumors(epithelioid hemangioendothelioma and hemangiopericytoma), and reviewed them with references.

• Journal of Rhinology
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• v.25 no.2
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• pp.118-122
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• 2018

Epithelioid hemangioendothelioma is a rare vascular tumor with intermediate malignity and metastasis risk. It presents epithelioid cells with intracytoplasmic vacuoles and low mitotic activity. Its vascular nature can be confirmed by immunohistochemical studies (vimentin, CD31, CD34, and factor VIII). It is extremely rare in the nasal cavity, with only one case reported on the middle turbinate in Korea. The authors present a case of epithelioid hemangioendothelioma on the choana with a size of 2mm, which easily coult have been misdiagnosed as a blood clot.

• Tuberculosis and Respiratory Diseases
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• v.53 no.1
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• pp.56-65
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• 2002

A pulmonary epithelioid hemangioendothelioma (PEH) is a rare tumor with a vascular origin. A PEH can arise in many organ systems, such as the lung, liver, bone and soft tissues. It is a borderline malignancy but the clinical course is usually benign. In this report, we describe three cases of PEH. Case 1, a 61-year-old man, had nonspecific chest discomfort and the chest X-ray showed a solitary lung nodule. This nodule was diagnosed by an open lung biopsy. The pathologic findings including abundant necrosis, mitosis and hyperchromatic and pleomorphic nuclei, indicated a malignancy. The electron microscopic study showed Weibel-Palade bodies and the immunohistochemical stain for CD31 showed a positive reaction in the tumor cells, and linear staining along the vascular lumina. Case 2, a 34-year-old man, was admitted for an evaluation of multiple small nodules, incidentally detected a screening chest X-ray. The nodules were diagnosed by a immunohistochemical stain for the factor VIII-related antigen. Case 3, a 34-year-old woman, complained of left pleuritic chest pain. A simple chest film and the chest CT scan revealed multiple bilateral nodules and a left pleural effusion. An immunohistochemical stain for the factor VIII-related antigen was used to diagnose the nodules. Forth-one months after the diagnosis, she died of pulmonary insufficiency.

• Neonatal Medicine
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• v.17 no.1
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• pp.136-140
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• 2010

Galactosemia is a group of inherited enzyme deficiencies characterized by increase in the blood galactose levels. This condition may be associated with deficiencies of galactose-1-phosphate uridyl transferase, galactokinase, or uridine diphosphate galactose-4-epimerase. However, the elevated galactose identified by neonatal screening tests has several other possible etiologies, including hepatic hemangioendothelioma, hepatic hemangioma, and patent ductus venosus with hypoplasia of the portal vein. We report a 13-day-old Korean male with hepatic hemangioendothelioma, which was incidentally detected during the evaluation for suspected galactosemia. Laboratory studies revealed that mildly elevated levels of galactose, galactose-1-phosphate and alpha- fetoprotein, at the time of admission, were graduallydecreased to the normal range over the 6 months of observation. Ultrasonography showed a well-defined heterogeneous hypoechoic mass in the liver, and magnetic resonance imaging study showed multiple enhanced mass lesions, which was compatible with the diagnosis of a hepatic hemangioendothelioma. Thus, hepatic imaging, especially ultrasonography, should be performed if neonatal screening suggests galactosemia.

• Tuberculosis and Respiratory Diseases
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• v.58 no.2
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• pp.179-184
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• 2005

Epithelioid hemangioendothelioma (EH) is a rare borderline malignant tumor which originating from vascular endothelial cells and occurs in many organs such as soft tissues, lung, liver and bone. But, pulmonary EH which simultaneously involves pleura and liver is very rare. In the present report, we describe an uncommon case of EH involving the lung, pleura and liver in a 26-year-old woman. Chest CT showed single nodule in right upper lobe with large amount of pleural effusion at ipsilateral side and abdominal CT multiple round low densities in liver. The EH was confirmed by microscopic analysis and immunohistochemical staining of CD34+ from open lung biopsy specimen.

• Clinical and Experimental Pediatrics
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• v.48 no.6
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• pp.660-664
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• 2005

Infantile hemangioendothelioma(IHE) of the liver is the most common vascular tumor in infants before the age of 6 months. It is a histologically benign tumor with potentially life-threatening complications. The clinical manifestations are variable, ranging from asymptomatic forms to intractable high-output heart failure. In addition, abdominal mass, intraperitoneal hemorrhage due to rupture of mass, respiratory distress, hematologic abnormalities and jaundice can occur. Diagnostic work-up is through doppler ultrasound sonography, computed tomography scan, magnetic resonance imaging and angiography. Treatment consists of medical treatment, interventional therapy, surgical resection and liver transplantation. We experienced symptomatic IHE in a premature neonate who presented with high output heart failure and respiratory distress. Initial medical treatment and steroid therapy failed to improve his condition. Coil embolization of left hepatic artery resulted in improvement of respiratory symptoms. However, a left lobectomy was performed because the mass size was not decreased with development of collateral vessels. The infant was well, after a successful discharge from the hospital.

• Neonatal Medicine
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• v.15 no.2
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• pp.183-189
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• 2008

Infantile hepatic hemangioendotheliomas (IHHEs) are benign vascular tumors, but can be associated with the life-threatening complications, such as congestive heart failure, disseminated intravascular coagulation, and massive bleeding. Various therapeutic options have been developed and the treatment response depends on the patient's clinical status and the nature of the lesion. In the case of a symptomatic IHHE, a non-invasive and precise diagnosis should be performed promptly before the therapeutic method is chosen. Additionally, it should be kept in mind that the residual lesions have malignant potential. We report a case of a congenital giant IHHE that was successfully reduced in size by interferon-$\alpha$ and completely removed by surgical tumor resection with a hepatic lobectomy.