Hemangioendothelioma, Epithelioid ; pathology ; Humans

Hemangioendothelioma, Epithelioid ; pathology ; Humans ; Lung Neoplasms ; pathology

Hemangioendothelioma, Epithelioid ; pathology ; Humans ; Male ; Middle Aged ; Nasal Cavity ; pathology ; Nose Neoplasms ; pathology

Aged ; Ear Neoplasms ; pathology ; Ear, Middle ; pathology ; Female ; Hemangioendothelioma, Epithelioid ; pathology ; Humans

Objective: To investigate the features of contrast-enhanced ultrasound (CEUS) in hepatic epithelioid hemangioendothelioma (HEHE) in order to improve the preoperative diagnosis rate. Methods: CEUS images of 32 pathologically-proven cases of hepatic epithelioid hemangioendothelioma from January 2004 to August 2021 were collected. Lesions were analyzed to observe the features of enhancement mode, enhancement intensity, and distinct enhancement phases. Results: Among the 32 cases, one had a solitary lesion, 29 had multiple lesions, and two had diffuse-type lesions. Contrast-enhanced ultrasound revealed a total of 42 lesions in 32 cases. In terms of arterial phase enhancement, 18 lesions had overall enhancement, six lesions had uneven dendritic enhancement, 16 lesions had rim-like enhancement, and two lesions had just slight peripheral spot enhancement around the lesions. Among the three cases, there were multiple lesions that had overall enhancement and ring enhancement. In terms of the enhancement phase, 20 lesions showed "fast progression", 20 lesions showed "same progression", and two lesions showed "slow progression". During the late arterial or early portal venous phases with rapid washout, all lesions manifested as hypoechoic. With peaked enhanced intensity, 11 lesions had a lower enhancement intensity than the surrounding normal liver parenchyma; 11 lesions had the same enhancement degree as the surrounding normal liver parenchyma; and 20 lesions had a higher enhancement degree than the surrounding normal liver parenchyma. All 16 ring-enhancing lesions had marked hyperenhancement. In the typical enhancing lesions, four showed hyperenhancement, five showed low enhancement, and nine showed isoenhancement. In the dendrite-enhancing lesions, there were two isoenhancing and four hypoenhancing. Contrast-enhanced ultrasound delineated the boundaries of all lesions more clearly than two-dimensional ultrasound. Conclusion: Contrast-enhanced ultrasound has certain value in the diagnosis of hepatic epithelioid hemangioendothelioma.
Humans ; Hemangioendothelioma, Epithelioid/pathology* ; Contrast Media ; Retrospective Studies ; Liver Neoplasms/pathology* ; Portal Vein/pathology* ; Ultrasonography

Humans ; Hemangioendothelioma, Epithelioid/pathology* ; Trans-Activators ; Transcription Factors ; Basic Helix-Loop-Helix Leucine Zipper Transcription Factors ; Hemangioendothelioma

No abstract available.
Adult ; Diagnosis, Differential ; Hemangioendothelioma, Epithelioid/diagnosis/*pathology ; Humans ; Liver Neoplasms/diagnosis/*pathology ; Male ; Tumor Markers, Biological

Bone Neoplasms ; pathology ; Diagnosis, Differential ; Hemangioendothelioma ; pathology ; Hemangioendothelioma, Epithelioid ; pathology ; Hemangiosarcoma ; pathology ; Humans ; Sarcoma ; pathology ; Skin Neoplasms ; pathology ; Soft Tissue Neoplasms ; pathology ; Vascular Neoplasms ; pathology

OBJECTIVETo study the clinicopathological features of epithelioid angiosarcoma (EA) and to propose its differential diagnosis.

METHODS15 EAs were observed by means of HE staining, immunohistochemical staining (ABC method) and in part by electron microscopy.

RESULTSTwelve cases occurred in men and three in women. Clinical symptoms included a focal mass with pain, weight loss and weakness. The tumors were located in the retroperitoneum (n = 3), extremities (n = 3), femur (n = 4), thyroid (n = 2), supraobit (n = 1), soft tissue of thorax (n = 1), soft tissue of neck (n = 1). Radiographic evaluation demonstrated solid to cystic neoplasms ranging from 3 to 20 cm in diameter. Histologically, the epithelioid cells were arranged diffusely or in nests with hemorrhage and focal necrosis. Atypical tumor cells lined the irregular spaces and single or small nests of cells grouped around a lumen containing red blood cells. FVIII, CD34, CD31,CK and EMA were positive in tumor cells and SMA was positive in pericytes. Ultrastructurally, intracytoplasmic vacuoles, Weibel-Palades, intermediate filaments and basal lamina could be seen. Surgical resection was the treatment of choice, occasionally with adjuvant chemotherapy. Follow-up. Two patients are presently alive 19 months and 7 years following diagnosis. Twelve died from 2 weeks to 38 months with a median interval of 19 months after lumpectomy. One patient was lost to follow up. There were 8 cases which had metastases to lungs, bones, lymph nodes and abdominal cavity.

CONCLUSIONSEA is a high-grade sarcoma. Endothelial derivation can be confirmed by immunohistochemical staining and ultrastructural findings. The differential diagnosis of EA includes metastatic carcinoma, epithelioid hemangioendothelioma, melanoma, epithelioid sarcoma etc.

Adult ; Aged ; Diagnosis, Differential ; Epithelioid Cells ; pathology ; Female ; Hemangioendothelioma, Epithelioid ; diagnosis ; Humans ; Immunohistochemistry ; Male ; Melanoma ; diagnosis ; Middle Aged ; Sarcoma ; diagnosis

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.
Biopsy, Fine-Needle ; Diagnosis ; Endothelial Cells ; Female ; Hemangioendothelioma, Epithelioid* ; Humans ; Hyalin ; Jugular Veins* ; Lymphatic Diseases* ; Pathology ; Vascular Diseases ; Young Adult