• Tuberculosis and Respiratory Diseases
• /
• 제51권5호
• /
• pp.466-473
• /
• 2001

Pulmonary epithelioid hemangioendothelioma (PEH), originally termed an intravacular bronchioloalveolar tumor, is a rare pulmonary neoplasm with a vascular origin and slow rate of malignancy. It affects various organs such as the liver, the central nervous system, lung, etc. Clinically, pulmonary epithelioid hemangioendothelioma has been considered to be a borderline malignancy, a generally indolent and nonaggressive tumor that displaces the pulmonary parenchyma over a number of years by slowly enlarging the tumor nodule. The clinical course of PEH is known to be usually benign. Here we report an unusual case of PEH that was highly malignant and was eventually fatal. The PEH was confirmed by microscopic analysis and immunohistochemical staining of CD31+(a membrane receptor and a sensitive and specific marker for vascular lesions) from an open lung biopsy specimen.

• Clinical and Experimental Pediatrics
• /
• 제57권11호
• /
• pp.500-504
• /
• 2014

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor of intermediate malignancy with resemblance to Kaposi sarcoma. It occurs predominantly in pediatric age groups as a cutaneous lesion with focal infiltration into the adjacent soft tissue and bone. Although visceral involvement is very uncommon, several cases with bone, retroperitoneal, or mediastinal involvement have been described. KHE has been reported to occasionally occur in unusual sites such as the thymus, tonsils, larynx, paranasal sinuses, deltoid muscle, spleen, uterine cervix, thoracic spine, and even the breast. Multifocal KHE is an extremely rare entity with few reports available in the literature, none of which describes pulmonary involvement. Herein, we report a unique case of multifocal KHE in a 13-year-old boy presenting with a huge soft tissue mass in the upper extremity complicated by bilateral pulmonary nodules that developed into large, necrotic tumor masses.

• Archives of Plastic Surgery
• /
• 제45권6호
• /
• pp.583-587
• /
• 2018

Kaposiform hemangioendothelioma (KHE) is a very rare, locally aggressive vascular neoplasm. It occurs mostly in children and is rarely observed in adults. It typically originates on the skin, later affecting the deep soft tissue of the extremities, head or neck, and retroperitoneum by infiltrative growth. It is locally aggressive, does not regress spontaneously, and tends to metastasize locally as well as to the regional lymph nodes. In this article, we report a case of adult-onset KHE with neurofibromatosis type 1. The patient presented to our department with a 2-month history of a painful ulceration in her left popliteal area. Since KHE had not previously been reported in patients with neurofibromatosis, the diagnosis was difficult due to the similarity of the skin manifestation to neurofibromatosis-associated lesions. We share our experience of diagnosing and treating this rare case of adult-onset KHE.

• Clinical and Experimental Pediatrics
• /
• 제48권6호
• /
• pp.660-664
• /
• 2005

저자들은 신생아 시기에 심부전 증세를 보인 간내 혈관내피종 환아에서 내과적 치료와 간동맥 색전술 후 호전을 보이지 않아 간좌엽 절제술을 시행하여 치료한 1례를 경험하였기에 보고하는 바이다.

• Journal of Chest Surgery
• /
• 제48권4호
• /
• pp.294-297
• /
• 2015

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 intra-vascular 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.

• 대한치과의사협회지
• /
• 제11권6호
• /
• pp.379-381
• /
• 1973

The authors have observed a case o hemangioendothelioma occurred on the left palate and buccal mucosa of a 40 year old woman. The results are as follows. 1) A blue-red round mass, 1.0cm in diameter, appeared on the palate and a blueviolet oval mass, 1.2cm in diamcter, on he buccal mucosa, and both of them showed a tendency to bleed after even slight trauma. 2) Microscopically the lesion was composed predominantly of masses of endothelial cells proliferating in and about the undifferentiated vascular channels. 3) well-vascularized tissue was made up of vascular spaces, showing infilitration of small round cells.

• 한국간담췌외과학회지
• /
• 제27권4호
• /
• pp.329-341
• /
• 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
• /
• 제43권3호
• /
• pp.284-287
• /
• 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
• /
• 제58권2호
• /
• pp.179-184
• /
• 2005

폐의 유상피 혈관 내피종이 진단 당시 흉막과 간을 동시에 침범한 경우는 드물다. 저자 등은 폐에 결절이 있고 동측에 다량의 흉막 삼출액이 동반되어 있으면서 간에 다발성 결절을 보인 26세의 젊은 여자환자에서 개흉 폐생검으로 진단된 유상피 혈관내피종 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Journal of Yeungnam Medical Science
• /
• 제33권1호
• /
• pp.72-75
• /
• 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.