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

Primary aortic angiosarcoma is very rare, and preoperative diagnosis is challenging with resultant poor prognosis. Angiosarcoma may mimic an infected aneurysm or a mural thrombus. Clinical suspicion of angiosarcoma is vital for an early diagnosis and proper surgical treatment, especially in cases with atypical rapid growth of an aortic abdominal aneurysm with a thrombotic mass. Herein, we report a case of angiosarcoma in the abdominal aorta mimicking an infected aneurysm and present computed tomography and positron emission tomography findings.
Aneurysm ; Aneurysm, Infected ; Aorta ; Aorta, Abdominal ; Diagnosis ; Early Diagnosis ; Hemangiosarcoma ; Positron-Emission Tomography ; Prognosis ; Thrombosis

Angiosarcomas are rare, but aggressive malignancies. Reports of brain metastasis are uncommon. In this report, we describe the clinical features of a 62 year-old man who presented with hemoptysis and right-sided weakness. Chest computed tomography showed multiple ill-defined part solid nodules in both lungs and brain magnetic resonance imaging showed multiple hemorrhagic bran masses. The patient underwent whole body proton emission tomography to identify biopsy site and showed multiple bone lesions. A right acetabulum biopsy in confirmed the diagnosis of angiosarcoma.
Acetabulum ; Biopsy ; Brain ; Diagnosis ; Hemangiosarcoma ; Hemoptysis ; Humans ; Lung ; Magnetic Resonance Imaging ; Neoplasm Metastasis ; Protons ; Thorax

Although the exact etiology of cutaneous angiosarcoma remains unclear, MYC gene amplification has been recently discovered as a new pathogenesis. MYC is a proto-oncogene, and aberration of MYC signaling in malignancies is associated with tumor metastasis, recurrence, and mortality. Moreover, upregulation of the miRNA polycistron, miR-17-92 cluster, were confirmed in both cutaneous angiosarcoma and multiple myeloma with MYC amplification. The correlation between MYC and miRNA expression is predictable as the coincident aberrant phenotype in two diseases. Moreover, the exploiting MYC dependency may be an attractive disease-specific strategy for the diagnosis and treatment of patients who are unaware of the causes of cutaneous angiosarcoma. Herein, a rare case of cutaneous angiosarcoma of the foot, which is also the first case of cutaneous angiosarcoma accompanied by multiple myeloma, has been described.
Diagnosis ; Foot ; Genes, myc ; Hemangiosarcoma ; Humans ; MicroRNAs ; Mortality ; Multiple Myeloma ; Neoplasm Metastasis ; Phenotype ; Proto-Oncogenes ; Recurrence ; Up-Regulation

Intravascular papillary endothelial hyperplasia or Masson's hemangioma is a rare vascular tumor. The reactive proliferation of endothelial cells in this disease mimics other benign or malignant vascular proliferation such as angiosarcoma or Kaposi's sarcoma. It is important to make an accurate distinction to avoid confusion with these malignant tumors. This would facilitate a proper diagnosis, which is essential so that the patient is not subjected to unnecessarily aggressive or inappropriate treatment.
Diagnosis ; Endothelial Cells ; Hemangioma ; Hemangiosarcoma ; Humans ; Hyperplasia ; Sarcoma, Kaposi

We present image findings, especially rare MRI of a primary breast angiosarcoma with its histopathology, and also analyze the relevant medical literature reports in terms of the MRI findings. As our patient had unique features of a primary breast angiosarcoma, this case could be very helpful for future diagnosis of this rare breast malignancy by MRI.
Breast ; Diagnosis ; Hemangiosarcoma ; Humans ; Magnetic Resonance Imaging

Stewart-Treves syndrome (STS) is a rare cutaneous angiosarcoma that develops in chronic lymphedema. The majority of STS is described in the upper extremity after aggressive locoregional therapy for breast cancer and is rarely reported in lower extremities. A 68-year-old woman presented with a 3-month history of multiple purpuric tumorous plaques and nodules on the right posterior thigh. She had a history of radical hysterectomy with lymph node dissection and postoperative radiotherapy due to uterine cervical cancer 16 years ago. She received right total hip replacement surgery due to hip joint avascular necrosis 14 years ago. She had suffered from chronic leg edema, especially on the right side. Skin biopsy on the right posterior thigh showed irregular vascular channels lined by atypical endothelial cells. Special stains showed positivity for CD31, CD34, factor VIII, and D2~40, which are pan-vascular or lymphatic markers. She showed a pelvic mass and pelvic bone metastasis on radiologic staging work-up. She refused all treatment, including surgery, radiotherapy, and chemotherapy, except for pain control. She died 2 months after diagnosis of this highly malignant tumor. The lymphedema on both lower extremities after uterine cervical cancer treatment was aggravated especially on the right lower extremity after right total hip replacement surgery. Increased weight of the right lower extremity resulted in 4 episodes of recurrent hip dislocation. We contend that these multiple factors (uterine cervical cancer treatment, total hip replacement surgery on the right side, and recurrent hip dislocations) attributed to development of Stewart-Treves syndrome. We herein report a case of Stewart-Treves syndrome of the lower extremity following chronic leg lymphedema after uterine cervical cancer treatment and hip surgery.
Aged ; Arthroplasty, Replacement, Hip* ; Biopsy ; Breast Neoplasms ; Coloring Agents ; Diagnosis ; Drug Therapy ; Edema ; Endothelial Cells ; Factor VIII ; Female ; Hemangiosarcoma ; Hip ; Hip Dislocation ; Hip Joint ; Humans ; Hysterectomy* ; Leg ; Lower Extremity* ; Lymph Node Excision ; Lymphedema ; Necrosis ; Neoplasm Metastasis ; Pelvic Bones ; Radiotherapy ; Skin ; Thigh ; Upper Extremity ; Uterine Cervical Neoplasms

Primary angiosarcoma of the breast is extremely rare and is observed in 0.0005% to 0.05% of primary breast tumor cases. The diagnosis of this tumor is difficult due to its undefined characteristics. Radiologic findings are often nonspecific and appear completely normal in one-third of patients with primary angiosarcomas. The prognosis is usually poor, and the treatment choices include mastectomy or wide excision. Radiotherapy and chemotherapy produce varying results. We report a patient with primary angiosarcoma of the breast to further our understanding of the characteristics of this tumor and facilitate the correct diagnosis of breast angiosarcoma.
Breast Neoplasms ; Breast* ; Diagnosis ; Drug Therapy ; Hemangiosarcoma* ; Humans ; Lymphangiosarcoma ; Mastectomy ; Prognosis ; Radiotherapy ; Sarcoma

Indeterminate dendritic cell tumor (IDCT) is a proliferation of CD1a+, S100+ and langerin- histiocytes with a generally benign course. Here, we describe a case of a 90-year-old male who developed skin lesions on his scalp mimicking angiosarcoma and lymphadenopathy. He died six months after the onset of skin lesions despite of months' radiotherapy. Pathological examination ruled out scalp angiosarcoma and showed a high Ki-67 index. The appearance of skin lesions and lymphadenopathy led to challenges in diagnosis and the development of a treatment plan.
Aged, 80 and over ; Dendritic Cells* ; Diagnosis ; Hemangiosarcoma* ; Histiocytes ; Humans ; Lymphatic Diseases ; Male ; Radiotherapy ; Scalp* ; Skin

Angiosarcoma is a very rare form of neoplasm derived from soft tissue. It is reported even more rarely in hepatobiliary system. Because of its nonspecific symptoms and difficulty in diagnosis, angiosarcoma is often presented in a progressed state and often results in poor prognosis. To our best knowledge, there has been no report regarding angiosarcoma in common bile duct worldwide. We report a case of 77-year-old-woman presented with right upper abdominal pain diagnosed as angiosarcoma in common bile duct.
Abdominal Pain ; Common Bile Duct* ; Diagnosis ; Hemangiosarcoma* ; Prognosis