• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.27 no.4
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• pp.329-341
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• 2023

Vascular tumors of the liver are mesenchymal lesions from endothelial cells. They range from common benign lesions such as haemangioma, intermediate tumors like Kaposi sarcoma, and perivascular epithelioid cell tumor to malignant tumors such as hepatic epithelioid hemangioendothelioma and hepatic angiosarcoma in adults. Pediatric vascular tumors of the liver also include benign, locally aggressive, borderline, and malignant masses with haemangiomas being the most common benign tumors and epithelioid hemangioendothelioma being an uncommon pediatric malignancy. The list of these lesions is completed by nodular regenerative hyperplasia, solitary fibrous tumour, and hepatic small vessel neoplasms (HSVN). Some of these tumors are uncommon and rare. This review article aimed to enumerate hepatic vascular tumors along with their imaging, histopathology, molecular findings for accurate diagnosis that can result in better management.

• Archives of Plastic Surgery
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• v.43 no.3
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• pp.284-287
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• 2016

Epithelioid hemangioendothelioma (EHE) is a well-differentiated and rare vascular tumor. Systemic metastases are uncommon. Herein, we present a patient with skin metastasis of pulmonary EHE (PEH) that was treated by wide excision. A 76-year-old male was evaluated due to pulmonary thromboembolism and a solitary pulmonary nodule. A biopsy was performed and pathological examination of the mass confirmed EHE. No metastasis was observed. The patient returned to care approximately two years later due to a painful nodule in the right lower leg. A skin biopsy showed metastatic EHE from the lung. We used a safety margin of 1 cm based on clinical experience, because no prior case had been reported regarding the resection margin appropriate for primary cutaneous EHE and skin metastases of PEH. At four months after surgery, the patient recovered without complications or recurrence. Skin metastasis of PEH is extremely rare, and only two cases have been reported in the literature. In this case, we report a rare case of PEH with histologically diagnosed skin metastasis that was successfully treated by curative resection. It is expected that this case report will provide a helpful contribution to the extant data regarding PEH metastases.

• 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.

• Journal of Yeungnam Medical Science
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• v.33 no.1
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• pp.72-75
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• 2016

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare, low-to-intermediate malignant tumor of endothelial origin. Computed tomography (CT) findings of PEH demonstrate multiple small bilateral nodules; however, to the best of our knowledge, there were no reports on PEH coexisting with other malignancies. Here, we reported on a case involving PEH in a patient with colon cancer and breast cancer which was misconceived as pulmonary meta- stasis. A 63-year-old woman who suffered from constipation for 2 weeks visited our hospital. Colonoscopy showed a large mass with obstruction on hepatic flexure. The histological diagnosis was adenocarcinoma of the ascending colon. Multiple nodules in both lungs and breast were observed on a chest CT scan. A core biopsy of a breast nodule was performed and a diagnosis of invasive ductal carcinoma of the left breast was made. Pulmonary nodules observed on the chest CT scan was considered as pulmonary metastasis from colon or breast cancer. Laparoscopic right hemicolectomy was performed. At the same time, wedge resection of the lung was performed and pathological diagnosis was PEH. Radiologic features of PEH were difficult to distinguish from lung metastasis. Therefore the author reported a rare case involving PEH in a patient with primary malignancy of colon and breast.

• Tuberculosis and Respiratory Diseases
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• v.44 no.6
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• pp.1396-1402
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• 1997

Epithelioid Hemangioendothelioma(EH) is a rare vascular tumor, originating from endothelial cells. The principal locations are lung, soft tissue, bone and liver. This tumor is of borderline malignancy, relatively benign course. In the lung, the tumor is often multifocal, bilateral and frequently lead to the mistaken diagnosis of metastatic carcinoma. Although EH of the lung is relatively slow growing tumor, extensive pulmonary involvement, systemic metastasis, mainly to the liver have been documented. A 26-year-old man with EH involving the lung and liver was reported. Chest X-ray and chest CT showed multiple nodules in both lung fields and Abdominal CT multiple round low densities in liver. Transbronchial lung biopsy was performed. The patient diagnosed as EH by light microscopic finding and immunohistochemical study for Factor VIII-related antigen.

• Journal of the Korean Society of Radiology
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• v.83 no.4
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• pp.958-964
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• 2022

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor of borderline or low-grade malignancy, and its prognosis is unpredictable. Herein, we describe the case of a 47-year-old asymptomatic female with a diagnosis of multinodular PEH. During a 7-year follow-up, the nodules with large size and high ¹⁸F-fluorodeoxyglucose uptake in the initial study showed progression with increasing sizes; however, most small nodules (size < 1 cm) demonstrated spontaneous regression with peripheral rim or nodular calcification. The patient underwent surgical resection for an enlarged nodule. Of note, it is unusual for an individual to have mixed progression and regression concomitantly, which may be helpful in predicting the prognosis.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.47 no.5
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• pp.691-696
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• 1999

Epithelioid hemangioendothelioma(EH) is a rare pulmonary vascular malignancy. Clinically, EH has been considered as an indolent, generally non-aggressive tumor. We report a case of EH which was confirmed by open lung biopsy. A 34-year-old woman was admitted for further evaluation of multiple small(less than 2cm in size) nodules, incidentally detected on screening chest radiograph. The chest CT showed multiple, relatively well-marginated, variable sized nodules at both whole lung. Transbronchial lung biopsy and transthoracic needle aspiration were nondiagnostic and open lung biopsy was performed from right middle lobe of lung. On light-microscopic examination, the nodules were composed of a poorly cellular hyaline core and a more cellular peripheral zone which extended into air space in a micropolypoid fashion and obliterated blood vessels. The tumor cells at the peripheral zone had intracytoplasmic vacuoles which suggested primitive, vascular differentiation. Immuno-histochemical study revealed the cellular area which gave positive reaction to factor VIII-related antigen. She received no specific therapy after open lung biopsy and chest X-ray films had showed no change for about two years.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.40 no.3
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• pp.301-307
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• 1993

Intravascular bronchioloalveolar tumor is now recognized as a pulmonary form of hemangioendothelioma(HE). HE is an unusual tumor of adult life which is characterized by proliferation of an "epithelioid" or "spindle" endothelial cell. In the lung it usually presents as multiple bilateral slowly growing nodules less than 2 cm in diameter. The aetiology and pathogenesis of this disease are unknown. Spindle cell HE occurs at any age, but approximately one half of patient are 25 years of age or younger and males are affected twice more frequently than females. On light microscopic examination, the tumor show mild cellular atypia, nearly absent mitoses and electron-microscopic studies reveal evidence of endothelial cell differentiation. Intracytoplasmic localization of Factor VIII-related antigen is demonstrated on immunohistochemical study, which confirmed the endothelial origin of the tumor. No effective therapy is yet known for HE, but survival of this tumor can be quite long. However, one half of the patient have died, usually of progressive pulmonary insufficiency. This 19-yr-old male complained of Rt. chest pain and intermittent hemoptysis. Simple chest film and chest CT scan showed the Rt. pleural effusion, variable sized bilateral pulmonary nodules, irregular large heterogenous tumor with well enhancement and extensive necrosis in the anterior mediastinum. The mediastinal mass was biopsied and diagnosed as spindle cell HE by light microscopic finding and immunohistochemical studies.