• Journal of Korean Neurosurgical Society
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• v.42 no.2
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• pp.129-131
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• 2007

Intracranial epithelioid hemangioendothelioma is extremely rare. We report a case of intracranial epithelioid hemangioendothelioma which developed in a 55-year-old man who presented with dysarthria for two weeks. The brain computed tomography scan and magnetic resonance image showed masses which had fat component at the left frontal convexity and at left posterior parietal area. Excisional biopsy at the left frontal convexity confirmed epithelioid hemangioendothelioma which is immunopositive for CD31, supporting endothelial differentiation, and negative for CD68, SMA and HMB-45.

• Journal of Korean Neurosurgical Society
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• v.30 no.5
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• pp.627-632
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• 2001

Hemangioendotheliomas are vascular neoplasms characterized by histologic appearance that are intermediate between hemangiomas and angiosarcomas first described by Weiss and Enzinger in 1982. They are classified into at least 3 subgroups, including epithelioid hemangioendothelioma, spindle cell hemangioendothelioma, and malignant endovascular angioendothelioma, and have been reported principally in soft tissues of the extremities, lung, liver, and bone. The cases involving the central nervous system reported are very rare. We report an intraspinal epithelioid hemangioendothelioma occurring in a 52-year-old man. To our knowledge, it is first case of spinal hemangioendothelioma in Korea.

• The Korean Journal of Cytopathology
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• v.14 no.1
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• pp.36-41
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• 2003

Epithelioid hemangioendothelioma is a rare vascular tumor of borderline malignancy which is characterized by the presence of "epithelioid" or "histiocytoid" endothelial cells. Superficial and deep tumors have been recognized in the extremities, head, neck, chest, and mediastinum of adult patients. It may also occur as a primary tumor of liver, bone, and other visceral organs. Few effusion cytologic findings of epithelioid hemangioendothelioma have been reported. We report a case of composite epithelioid hemangioendothelioma with focal epithelioid angiosarcomatous areas of the iliac bone and adjacent soft tissue in a 38-year-old female, which, during its metastatic course, was presented as a pleural effusion. The effusion was cellular with epithelioid cells presenting both singly and in clusters. The tumor cells were round to ovoid shewing cytoplasmic vacuolization, variability in cell size, and prominent nucleoli. The effusion smears and cell block sections revealed strong positive staining for CD31 and vimentin, weak positive for CD34 and Factor VIII-related antigen, and negative for cytokeratin, CEA, and calretinin. The cytologic findings in this case were similar to that of metastatic adenocarcinoma or malignant mesothelioma. Therefore, immunocytochemical staining in smear and cell block is a helpful tool to differentiate malignant 'epithelioid' cells in effusion.

• Journal of Chest Surgery
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• v.37 no.11
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• pp.937-941
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• 2004

Epithelioid hemangioendothelioma, originating from the vascular endothelium, is a very rare and low-grade malignancy. World-wide, about 50 cases of pulmonary epithelioid hemangioendothelioma have been reported. This is more common in female and is usually shown as multiple nodules in both lung fields. A 41-year-old male, who had suffered from right pleuritic chest pain for 3 months, was initially diagnosed as adenocarcinoma under bronchofiberscopic biopsy. At that time, the stage of tumor according to the TNM staging was llla. He received bronchoscopic biopsy again during follow-up period and it was diagnosed as hamartoma. After surgery, the final diagnosis was pulmonary epithelioid hemangioendothelioma.

• Journal of Chest Surgery
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• v.51 no.6
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• pp.419-422
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• 2018

Hemangioendothelioma is a rare vascular tumor with involvement of the liver, brain, long bones, and lung. Among the 6 histological subtypes, epithelioid hemangioendothelioma (EHE) is the most aggressive. Its occurrence in the mediastinum is quite rare, and very few cases have been documented. The reported cases in the literature have described difficulties in the preoperative diagnosis due to the unusual histological appearance of the tumor. Immunohistochemistry remains the mainstay for a definitive diagnosis. Due to its low incidence, there is no standard treatment for mediastinal EHE, but curative resection is the preferred treatment option where possible, with chemotherapy used as an adjuvant treatment or in cases of widespread inoperable disease. The present case study describes an aggressive EHE occurring in an 18-year-old woman in the anterior mediastinum.

• 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.

• 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 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.

• 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.

• Journal of Chest Surgery
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• v.54 no.6
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• pp.524-527
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• 2021

Herein, we describe the case of a 20-year-old woman who presented with dysphagia of 2 months' duration associated with vomiting, moderate abdominal pain, decreased oral intake, and significant weight loss. During the past 3 years, the patient experienced intermittent mild abdominal pain with infrequent vomiting. Endoscopy at Jordan University Hospital showed a mass in the esophagus, and endoscopic biopsies were performed. The preliminary histopathological report excluded malignancy. Two days after endoscopy, the patient presented to the emergency department complaining of severely worsening pain and total dysphagia. The pain persisted despite intravenous paracetamol administration, which was concerning for esophageal perforation; therefore, an urgent surgical intervention was performed. The mass was removed surgically, along with a para-esophageal lymph node. The final histopathological results of the endoscopic and resected specimens supported the diagnosis of pseudomyogenic hemangioendothelioma (PMHE). This is the first case reporting esophageal involvement of PMHE.