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

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

Pseudomyogenic hemangioendothelioma (PHE) is a rare angiogenic tumor. Histologically, the morphological characteristics of neoplastic vessels and endothelial differentiation are not obvious, and it is easy to be confused with epithelioid sarcoma, epithelioid hemangioendothelioma and myogenic tumor. PHE usually occurs in arms and legs in young people and has a significant male predominance. The tumor has a predilection for the distal extremities and its typical manifestation is multiple center invasion of a single limb, which can involve all layers of skin and subcutaneous tissues,and is often accompanied by abvious pain. Histologically, PHE is characterized by infiltrative growth of tumor. Most tumor lesions are composed of sheets and loose fascicles of plump spindle or epithelioid cells within a background of variably prominent inflammatory infiltration, which was commonly composed of neutrophils. Some cells may resemble rhabdomyoblasts, and nuclear atypia and mitosis were rare. The tumor cells generally expressed positive cytokeratin (CK), ETS-related gene (ERG), Friend leukemia virus integration 1 (FLI1) and integrase interactor 1(INI1). In some cases, the tumor cells expressed CD31. A case of a young woman was reported in this paper, who presented with a subcutaneous mass with severe pain and was chronologically misdiagnosed with herpes zoster, low-grade malignant fibrous histiocytoma and epithelioid hemangioendothelioma. In this study, the clinical and pathological features, differential diagnosis and the latest progress in therapy of PHE were analyzed based on relevant literature.
Adolescent ; Adult ; Biomarkers, Tumor ; Child ; Diagnosis, Differential ; Diagnostic Errors ; Female ; Hemangioendothelioma, Epithelioid/pathology* ; Hemangioma ; Histiocytoma, Malignant Fibrous/diagnosis* ; Humans ; Male ; Pain ; Precancerous Conditions/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

OBJECTIVETo analyze the imaging characteristics of hepatic epithelioid hemangioendothelioma (EHE) and their correlation with histopathological findings.

METHODSCT and MRI imaging and histopathological characteristics of five patients with hepatic EHE were retrospectively reviewed and the correlation of their imaging characteristics with pathological findings was analyzed.

RESULTSA total of 92 lesions were found in the 5 patients, all presenting with multiple nodules. All the 92 lesions were located within a 2-cm zone heneath the hepatic capsule, i.e., the shortest distance from the horder of lesions to the hepatic capsule. 28 of the 92 lesions showed the "capsular retraction" sign. 36 lesions were found in three patients receiving MRI. 77.8% of the 36 lesions demonstrated the "halo" sign on a fat-suppression T2- weighted image, while 91.7% after contrast enhancement. A peripheral dark rim was found in 91.7% of the lesions on a fat-suppression T2-weighted image. In addition, 36.1% of the lesions showed slight internal or edge enhancement at the hepatohiliary phase. In the two patients receiving CT examination, 7 of 56 lesions demonstrated the "halo" sign.

CONCLUSIONSHepatic EHE may manifest as nodular lesions with predilection of peripheral subcapsular growth and nodular confluence, together with "halo" sign and " capsular retraction". The peripheral dark rim on a fat-suppression T2-weighted image and slight enhancement at the hepatobiliary phase can help to improve the accuracy of diagnosis and differential diagnosis of this hepatic tumor. MRI is superior to CT imaging to denict their intra-lesional characteristics.

Hemangioendothelioma, Epithelioid ; diagnosis ; diagnostic imaging ; pathology ; Humans ; Liver Neoplasms ; diagnosis ; diagnostic imaging ; pathology ; Magnetic Resonance Imaging ; Neoplasms, Multiple Primary ; diagnosis ; diagnostic imaging ; pathology ; Physical Examination ; Retrospective Studies ; Tomography, X-Ray Computed

Hemangioendothelioma, Epithelioid ; pathology ; Humans

OBJECTIVETo further elucidate the clinical and pathologic features of retiform hemangioendothelioma (RH) and its clinical spectrum.

METHODSEight cases of RH were reviewed. The clinicopathologic profiles, immunophenotypes and outcome data were investigated.

RESULTSAll 8 cases occurred in females with a mean age at presentation of 40 years (range, 13-69 years). Five tumors arose in the skin of the head and neck region and lower extremities, two in the long bones and one in the spleen. Clinically, the patients presented with a slowly growing cutaneous plaque or subcutaneous nodule, pain of the upper arm, and dull pain and discomfort in the left hypochondrium respectively. Grossly, the tumor appeared as a non-encapsulated gray-yellowish to tan-brown mass with a mean diameter of 2.6 cm (range, 0.8-5.0 cm). On histology, it was composed of delicate branches of elongated vessels lined by a layer of hobnail or matchstick endothelium, exhibiting a retiform pattern with close resemblance of the normal rete testis. Cords or solid nests of epithelioid cells were noted adjacent to the well-formed vessels. In three cases, dilated vascular spaces with formation of intravascular papillary tufts were observed, features overlapping with Dabska tumor. There was usually marked lymphocytic infiltration in the stroma which was also hyalinized in some cases. One case had regional lymph node metastasis. By immunohistochemistry, all cases consistently expressed endothelial markers, including CD31 (8/8), human coagulation factor VIII (5/8), CD34 (5/7) and D2-40 (1/2). Two of six cases with follow-up information (18-67 months) developed local recurrences, but distant metastasis was not identified.

CONCLUSIONSRH is a distinctive vascular tumor of adulthood characterized by retiform growth of vessels with striking hobnail endothelium. Although the tumor occurs predominantly in the skin, the long bones and the spleen can be occasionally affected. The presence of Dabska tumor-like areas in RH may suggest a morphologic continuum between these two entities, comprising the family of hobnail hemangioendothelioma. Familiarity with the characteristic features of this vascular tumor of intermediate malignancy will help in the differential diagnosis of vascular neoplasms with hobnail endothelium.

Adolescent ; Adult ; Aged ; Biomarkers, Tumor ; Bone Neoplasms ; pathology ; Diagnosis, Differential ; Epithelioid Cells ; pathology ; Female ; Hemangioendothelioma ; pathology ; Humans ; Immunohistochemistry ; Immunophenotyping ; Middle Aged ; Neoplasm Recurrence, Local ; Skin Neoplasms ; pathology ; Splenic Neoplasms ; pathology ; Young Adult

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

No abstract available.
Adult ; Antigens, CD31/metabolism ; Antigens, CD34/metabolism ; Female ; Hemangioendothelioma, Epithelioid/metabolism/*pathology/radiography ; Humans ; Immunohistochemistry ; Liver Neoplasms/metabolism/*pathology/radiography ; Magnetic Resonance Imaging ; Positron-Emission Tomography ; Tomography, X-Ray Computed ; von Willebrand Factor/metabolism

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