Objective: To investigate the clinical manifestations, histomorphology, and differential diagnosis of primary hepatic angiosarcoma. Methods: Nine cases of primary hepatic angiosarcoma diagnosed in the Department of Pathology, the First Affiliated Hospital of Nanjing Medical University from January 2014 to December 2021 were collected, including biopsy and surgical specimens. The histomorphology, clinical, and radiologic findings were analyzed. The relevant literature was also reviewed. Results: There were six males and three females, aged 30 to 73 years (mean 57 years). Grossly, the growth pattern of the tumor was classified as either mass formation or non-mass formation (sinusoidal). Microscopically, the mass-forming primary hepatic angiosarcoma were further subdivided into vasoformative or non-vasoformative growth patterns; and those non-vasoformative tumors had either epithelioid, spindled, or undifferentiated sarcomatoid features. Sinusoidal primary hepatic angiosarcoma on the other hand presented with markedly dilated and congested blood vessels of varying sizes, with mild to moderately atypical endothelial cells. Follow-up in all nine cases revealed 8 mortality ranging from 1 to 18 months (mean 5 months) from initial diagnosis. One patient was alive with disease within a period of 48 months. Conclusions: Primary hepatic angiosarcoma is a rare entity with a wide spectrum of histomorphology, and often misdiagnosed. It should be considered when there are dilated and congested sinusoids, with overt nuclear atypia. The overall biological behavior is aggressive, and the prognosis is worse.
Male ; Female ; Humans ; Hemangiosarcoma/diagnosis* ; Endothelial Cells/pathology* ; Liver Neoplasms/surgery* ; Prognosis ; Biopsy

Arthroplasty, Replacement, Hip ; adverse effects ; Hemangiosarcoma ; diagnosis ; Hip Joint ; pathology ; surgery ; Hip Prosthesis ; Humans

Actins ; metabolism ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Hemangioma ; metabolism ; pathology ; surgery ; Hemangiosarcoma ; pathology ; Humans ; Middle Aged ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sarcoma, Kaposi ; pathology ; Skin Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Antiporters ; metabolism ; Carcinosarcoma ; epidemiology ; metabolism ; pathology ; surgery ; ultrastructure ; Diagnosis, Differential ; Hemangiosarcoma ; pathology ; Humans ; Leiomyosarcoma ; pathology ; Mucin-1 ; metabolism ; Neoplasm Grading ; Neoplasm Staging ; Prognosis ; Rhabdomyosarcoma ; pathology ; Urinary Bladder Neoplasms ; epidemiology ; metabolism ; pathology ; surgery ; ultrastructure ; Vimentin ; metabolism

OBJECTIVETo investigate the clinicopathological characteristics, differential diagnosis, and prognosis of primary hepatic angiosarcoma, and to review the literature.

METHODSTwenty cases of primary hepatic angiosarcoma were analyzed by gross examination and light microscopy. Immunostaining was performed to detect the expression of CD34, CD31, FVIIIRAg, CK, GPC-3, Hepatocyte,vimentin, PTEN, desmin, and CD117.

RESULTSThe age of the patients ranged from 7 to 86 years. Eleven cases were male, and 9 were female. All cases showed no specific clinical manifestations and imaging results. Macroscopically, the tumors showed diffuse multi-nodular or single nodular patterns with hemorrhage. Microscopically, there were various patterns such as cavernous vascular space and epithelioid hemangioendothelioma-like appearances; however, specific pathological diagnostic features of angiosarcoma still existed in all cases. All of the cases expressed at least one of the three immunohistochemical markers: CD31, CD34 and/or FVIIIRag. Ten cases had PTEN low expression. Ki-67 proliferative index was more than 10% in all cases. None of cases expressed desmin, CD117, GPC-3 or Hepatocyte.

CONCLUSIONSPrimary hepatic angiosarcoma is a rare malignant tumor. Detailed morphological observation and using various vascular endothelial immunohistochemical markers can help to establish the diagnosis accurately.

Adult ; Aged ; Aged, 80 and over ; Antigens, CD34 ; metabolism ; Biomarkers, Tumor ; metabolism ; Child ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hemangioendothelioma, Epithelioid ; pathology ; Hemangiosarcoma ; diagnosis ; metabolism ; pathology ; surgery ; Humans ; Ki-67 Antigen ; metabolism ; Liver Neoplasms ; diagnosis ; metabolism ; pathology ; surgery ; Magnetic Resonance Imaging ; Male ; Middle Aged ; PTEN Phosphohydrolase ; metabolism ; Peliosis Hepatis ; pathology ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Survival Rate ; von Willebrand Factor ; metabolism

Breast Neoplasms ; pathology ; radiotherapy ; surgery ; Diagnosis, Differential ; Female ; Hemangiosarcoma ; etiology ; pathology ; surgery ; Humans ; Iatrogenic Disease ; Neoplasm Recurrence, Local ; Neoplasms, Radiation-Induced ; etiology ; pathology ; surgery ; Vascular Diseases ; etiology ; pathology ; surgery

OBJECTIVETo analyze the histopathology of, surgery for, and prognosis (survival rate, local recurrence rate, metastases rate, function) of epithelioid angiosarcoma (EA).

METHODSSeven patients diagnosed as EA were received treatment in our department from May 2001 to May 2011. According Enneking staging, 3 patients were classified as IIA, 3 for IIB, and 1 for III. Wide excision was performed in 3 patients and amputation in 4 patients. Seven patients were followed up from 3 to 20 months.

RESULTSOne patient had local recurrence 3 months after wide excision. Five patients had metastases at 4 to 20 months postoperatively. The cumulative survival rate at 0.5, 1 and 2 years postoperatively were 67%, 22% and 0. The function of 3 patients after limb salvage operation was good in 2 cases and poor in 1 case. The tumor site, surgical staging, and safe margin were crucial factors affecting survival rate.

CONCLUSIONSThe prognosis of EA in deep soft tissue was poor. Customized, multimodality treatment and safe margin in operation may increase the survival rate as well as decrease recurrence and metastasis.

Adult ; Aged ; Female ; Follow-Up Studies ; Hemangiosarcoma ; diagnosis ; surgery ; Humans ; Male ; Middle Aged ; Neoplasm Metastasis ; Neoplasm Recurrence, Local ; Prognosis ; Retrospective Studies ; Soft Tissue Neoplasms ; diagnosis ; surgery ; Survival Rate ; Treatment Outcome

Diagnosis, Differential ; Esophageal Neoplasms ; pathology ; surgery ; Granuloma, Pyogenic ; pathology ; surgery ; Hemangioma, Capillary ; pathology ; Hemangiosarcoma ; pathology ; Humans ; Male ; Middle Aged ; Sarcoma, Kaposi ; pathology

OBJECTIVETo study the clinicopathologic features, differential diagnosis and pathogenesis of sclerosing angiomatoid nodular transformation of spleen.

METHODSTen cases of sclerosing angiomatoid nodular transformation of spleen were retrieved from the archival file. Histochemical and immunohistochemical (EnVision method) studies were performed. Ultrastructural findings were also available in one of them.

RESULTSSclerosing angiomatoid nodular transformation was characterized by micronodular appearance of vascular spaces lined by plump endothelial cells with interspersed ovoid spindle cells. Immunohistochemical study showed that the endothelial cells of vessels in the angiomatoid nodules had various expressions of immunologic phenotypes and could be mainly classified into 3 types: CD34(+)/CD31(+)/CD8⁻ endothelial cells of the capillaries, CD8(+)/CD31(+)/CD34⁻ lining cells of the sinusoids and CD31(+)/CD8⁻/CD34⁻ endothelial cells of the small veins. Collagen network and dilated lymphatic sinuses were evident under transmission electron microscope.

CONCLUSIONSSclerosing angiomatoid nodular transformation of spleen is a rare benign entity. It may represent a reactive condition and bears some relationship with splenic angioma. It needs to be distinguished from borderline or malignant vascular tumors of spleen.

Adult ; Antigens, CD34 ; metabolism ; CD8 Antigens ; metabolism ; Diagnosis, Differential ; Female ; Hemangioendothelioma ; metabolism ; pathology ; Hemangiosarcoma ; metabolism ; pathology ; Histiocytoma, Benign Fibrous ; metabolism ; pathology ; surgery ; ultrastructure ; Humans ; Male ; Microscopy, Electron ; Middle Aged ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Splenic Neoplasms ; metabolism ; pathology ; surgery ; ultrastructure

Adenocarcinoma, Bronchiolo-Alveolar ; metabolism ; pathology ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Hemangioendothelioma, Epithelioid ; metabolism ; pathology ; surgery ; Hemangioma ; metabolism ; pathology ; Hemangiosarcoma ; metabolism ; pathology ; Humans ; Lung Neoplasms ; metabolism ; pathology ; surgery ; Middle Aged ; Vimentin ; metabolism