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

Heart Atria ; Heart Neoplasms/surgery* ; Hemangiosarcoma/surgery* ; Humans

Primary hepatic angiosarcoma (PHA) is a rare disease with high malignancy. Due to the low incidence, atypical clinical symptoms and signs, it is often misdiagnosed, which results in missing timely treatment, and subsequently influencing the therapeutic effect and prognosis. A case of hepatic angiosarcoma with acute abdominal pain as the first symptom was reported in First Hospital affiliated to Jilin University in Match, 2017. Computed Tomography (CT) examination for this patient was misdiagnosed as a ruptured hemangioma of the liver, and emergency interventional embolization for hemostasis was performed. Blood routine was monitored several times after the operation, which indicated active bleeding. We performed laparotomy and excised the tumor, which was pathologically confirmed as hepatic angiosarcoma. Immunohistochemistry showed that tumor cells expressed CD31 and CD34. After the operation, the family members gave up treatment due to economic limitations and other reasons, and the patient died on the second day after discharge.
Embolization, Therapeutic ; Fatal Outcome ; Hemangiosarcoma ; surgery ; Humans ; Liver Neoplasms ; surgery ; Rupture, Spontaneous ; surgery

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

A 29-year-old pregnant woman with recurrent pericardial effusion and a cardiac tumor, diagnosed as an angiosarcoma, was treated with surgical resection of the tumor followed by radiotherapy. Immediately after completion of radiotherapy, she developed bilateral breast masses, which were also confirmed as angiosarcomas. We thought this might be the first case of bilateral angiosarcoma of the breast metastasizing to heart mimicking a primary cardiac angiosarcoma, although we could not conclude with certainty that angiosarcoma of the heart was not the primary site.
Adult ; Biopsy ; Breast Neoplasms/*pathology ; Cardiac Surgical Procedures ; Female ; Heart Neoplasms/complications/*pathology/surgery ; Hemangiosarcoma/*pathology ; Humans ; Magnetic Resonance Imaging ; Pericardial Effusion/etiology ; Positron-Emission Tomography ; Pregnancy ; Radiotherapy, Adjuvant ; Tomography, X-Ray Computed

OBJECTIVETo study the disease spectrum, clinical and pathologic features of primary cardiac neoplasms at a single medical institution during a period of eight years.

METHODSThe clinical and pathologic features of 81 cases of primary cardiac neoplasms encountered at the Affiliated Hospital of Medical College, Qingdao University from January, 2004 to December, 2011, either for cardiovascular surgery or as pathology consultation cases, were retrospectively reviewed. Immunohistochemical study was carried out in selected examples.

RESULTSAmongst the 81 cases studied, 73 cases (90.1%) were benign and 8 cases (9.9%) were malignant. Cardiac myxomas accounted for 97.3% (71/73) of all the benign cases. Six of the 8 malignant cases represented soft tissue sarcomas. The commonest presenting symptom was dyspnea. The histologic subtypes included myxoma (number = 71), angiosarcoma (number = 2), malignant fibrous histiocytoma (number = 2), hemangioma (number = 1), liposarcoma (number = 1), lymphoma (number = 1), myxofibrosarcoma (number = 1) and papillary fibroelastoma (number = 1).

CONCLUSIONSWhile the majority of primary cardiac neoplasms are myxomas, other tumor types do exist. Accurate histologic diagnosis and timely surgery are crucial in patient management.

Adolescent ; Adult ; Aged ; Child ; Female ; Fibroma ; metabolism ; pathology ; surgery ; Fibrosarcoma ; metabolism ; pathology ; surgery ; Follow-Up Studies ; Heart Neoplasms ; metabolism ; pathology ; surgery ; Hemangioma ; metabolism ; pathology ; surgery ; Hemangiosarcoma ; metabolism ; pathology ; surgery ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Liposarcoma ; metabolism ; pathology ; surgery ; Lymphoma ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Myxoma ; metabolism ; pathology ; surgery ; Sarcoma ; metabolism ; pathology ; surgery ; Treatment Outcome ; Vimentin ; metabolism ; Young Adult

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