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

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

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

Epithelioid hemangioendothelioma is a neoplasm of vascular origin with a low-to-intermediate malignant potential and is one of the rare sarcomas arising from the liver. Its etiology is unknown and its clinical outcome is unpredictable. There is no generally accepted therapeutic strategy because of its rarity and the variable natural course between hemangioma and angiosarcoma. We report a case of a 64-year old woman who underwent hepatic resection due to epithelioid hemangioendothelioma in the right lobe that progressed to extrahepatic metastases of the bone, pleura, and peritoneum 22 months later. However, after resection there was no primary hepatic recurrence.
Bone Neoplasms/diagnosis/pathology/secondary ; Female ; Hemangioendothelioma, Epithelioid/*diagnosis/pathology/*secondary ; Hepatectomy ; Humans ; Liver Neoplasms/*diagnosis/pathology ; Lung Neoplasms/diagnosis/pathology/secondary ; Middle Aged ; Tomography, X-Ray Computed

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

Epithelioid hemangioendothelioma is a rare vascular origin tumor which usually occurs in soft tissues, liver, and lung. It usually affects adult women and presents as multiple hepatic nodules with mainly peripheral distribution. It is difficult to diagnose and treat because of non-specific clinical manifestations and findings on the imaging study. Moreover, pathological misdiagnosis is common. We report a case of this rare tumor that was detected incidentally. Final diagnosis was based on histological evidence. A 52-years old man suffered from right upper quadrant abdominal pain for 3 months, and was initially misdiagnosed as a metastatic carcinoma. Physical examination revealed superior cervical lymphadenopathy with mild hepatomegaly. Finally, hepatic epithelioid hemangioendothelioma was diagnosed on the basis of positive immunohistochemical staining for factor VIII, CD34, and VEGF. Our case highlights the importance of a histological diagnosis to avoid misdiagnosis.
Antigens, CD34/analysis/immunology ; Carcinoma/secondary ; Diagnosis, Differential ; Factor VIII/analysis/immunology ; Hemangioendothelioma, Epithelioid/*diagnosis/pathology ; Humans ; Immunohistochemistry ; Liver Neoplasms/*diagnosis/pathology ; Male ; Middle Aged ; Positron-Emission Tomography

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare tumor that occurs among young women and typically presents as bilateral multiple nodules. In the present report, we describe an uncommon case of PEH presented as a single cavitary nodule in a 33-yr-old asymptomatic man. This is the first case of PEH presented as a single cavitary nodule in the English literature. Three years of the follow-up without treatment was performed. Overall histologic findings were accord with conventional PEH, but some atypical features such as, increased mitotic activity (mean; two per ten high power fields), necrosis, spindling, and pleural and vascular invasion were recognized. Immunohistochemically, the tumor cells were positive for CD34. This report may contribute to the data on clinical findings and natural history of this rare tumor.
Adult ; Antigens, CD34/biosynthesis ; Hemangioendothelioma, Epithelioid/*diagnosis/pathology ; Human ; Immunohistochemistry ; Lung Neoplasms/*diagnosis/pathology ; Male ; Mitosis ; Necrosis ; Time Factors ; Tomography, X-Ray Computed

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*

Primary hepatic epithelioid hemangioendothelioma is a rare neoplasm of endothelial origin. The clinical manifestations are nonspecific, ranging from complete absence of symptoms to hepatic failure and death. Spontaneous rupture of a hepatic epithelioid hemangioendothelioma is an extremely rare presentation. We present a case of primary hepatic epithelioid hemangioendothelioma in a 65-year-old male patient with alcoholic liver cirrhosis. He was hospitalized due to epigastric pain and multiple liver masses on abdominal ultrasound. Dynamic liver CT imaging revealed multiple peripheral nodular enhanced mass lesions with delayed centripetal enhancement, and the adjacent collection of high-attenuation fluid along the liver capsule. Abdominal tapping revealed blood in the peritoneal cavity. Primary hepatic epithelioid hemangioendothelioma with spontaneous rupture was finally diagnosed based on a histopathologic examination revealing positive immunohistochemical staining for CD34.
Antigens, CD34/metabolism ; Bone Neoplasms/diagnosis/secondary ; Diagnosis, Differential ; Hemangioendothelioma, Epithelioid/*diagnosis/pathology/ultrasonography ; Humans ; Liver Cirrhosis, Alcoholic/complications/diagnosis ; Liver Neoplasms/*diagnosis/pathology/ultrasonography ; Male ; Pancreatic Neoplasms/diagnosis/secondary ; Rupture, Spontaneous ; Tomography, X-Ray Computed

Angiofibroma ; metabolism ; pathology ; Biomarkers, Tumor ; metabolism ; Diagnosis, Differential ; Epithelioid Cells ; Extremities ; Fibroma ; metabolism ; pathology ; Fingers ; Hemangioblastoma ; metabolism ; pathology ; Hemangioendothelioma ; metabolism ; pathology ; Humans ; Rhabdomyosarcoma ; metabolism ; pathology ; Sclerosis ; Soft Tissue Neoplasms ; classification ; metabolism ; pathology