Adult ; Aged ; Female ; Hemangiosarcoma ; diagnostic imaging ; Humans ; Liver Neoplasms ; diagnostic imaging ; Male ; Middle Aged ; Tomography, X-Ray Computed

Female ; Hemangiosarcoma ; diagnostic imaging ; Humans ; Liver Neoplasms ; diagnostic imaging ; Male ; Middle Aged ; Tomography, X-Ray Computed ; Ultrasonography

Objective: To investigate the clinical, cardiac imaging characteristics and prognosis of patients with primary cardiac angiosarcoma. Methods: The clinical data of 14 patients hospitalized with primary cardiac angiosarcoma from January 2001 to December 2017 in Peking Union Medical College Hospital were collected and analyzed. Metastatic cardiac angiosarcoma was not included in this study. Patients were followed up post discharge per telephone call or clinical visit. Results: Of the 14 patients, 8 were males and 6 were females, average age was 48 years. The main clinical symptoms were shortness of breath (8/14), hemoptysis (6/14), fever (5/14), chest pain (4/14) and cough (3/14). Imaging examinations showed that the tumors of 8 patients were located in the right heart and 6 in the pericardial cavity. Tumors in the right heart often infiltrate the atrial wall and cause pericardial effusion (7/8). Tumors in the pericardium were characterized by recurrent bloody pericardial effusion (6/6), prone to progressive constrictive pericarditis (3/6), pericardial fluid cytology was often negative (6/6). MRI showed heterogeneous high signal intensity (cauliflower aspect) on T2-weighted image and heterogeneous enhancement with a"sunray" aspect at the perfusion study. At the time of diagnosis, 8 patients developed lung or adrenal metastasis (8/14). The median survival was only 305 days. Conclusions: Primary cardiac angiosarcoma is a rare disease with non-specific clinical manifestation and poor prognosis. Imaging examinations may help diagnosis. The high invasiveness and the easy-to-metastasis feature of the tumor contribute to the poor prognosis of cardiac angiosarcoma.
Aftercare ; Female ; Heart Neoplasms/diagnostic imaging* ; Hemangiosarcoma/diagnostic imaging* ; Humans ; Male ; Middle Aged ; Patient Discharge ; Pericardial Effusion

Adult ; Coronary Artery Bypass ; Electrocardiography ; Heart Neoplasms ; diagnostic imaging ; pathology ; surgery ; Hemangiosarcoma ; diagnostic imaging ; pathology ; surgery ; Humans ; Male ; Treatment Outcome ; Ultrasonography

Actins ; metabolism ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Hemangiosarcoma ; metabolism ; pathology ; Humans ; Leiomyosarcoma ; metabolism ; pathology ; Middle Aged ; Multimodal Imaging ; Pulmonary Artery ; pathology ; Radiography ; Sarcoma ; diagnostic imaging ; metabolism ; pathology ; Soft Tissue Neoplasms ; secondary ; Vascular Neoplasms ; diagnostic imaging ; metabolism ; pathology ; Vimentin ; metabolism

We report an uncommon case of solitary, small hepatic angiosarcoma that was initially considered as a hemangioma. We present the imaging findings, with an emphasis on the initial and follow-up CT and MR findings, as well as report on the more suggestive findings of angiosarcoma than those of a hemangioma.
Contrast Media ; Diagnosis, Differential ; Gadolinium DTPA/diagnostic use ; Hemangioma/diagnosis ; Hemangiosarcoma/*diagnosis/surgery ; Humans ; Liver Neoplasms/*diagnosis/surgery ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Tomography, X-Ray Computed

OBJECTIVETo study the radiologic and pathologic features of primary intermediate hemangioendothelioma of the bone.

METHODSFive cases of primary intermediate hemangioendothelioma of bone encountered in the past three years were enrolled into the study. The clinical, radiologic, pathologic and immunohistochemical features of the tumors were reviewed.

RESULTSThe patients included 3 children with Kaposiform hemangioendothelioma and 2 elderly with retiform hemangioendothelioma. Four of the cases affected long bones and the remaining case affected the clavicle. One case showed multifocal involvement of the humerus. Radiologically, the tumors showed borderline to low-grade bony destruction, with various degrees of cortical defect. Intralesional or perilesional bone formation was demonstrated in 4 cases and radial spicules were seen in 1 case. The histopathologic features of primary intermediate hemangioendothelioma of bone were similar to those of soft tissue, except for the presence of reactive bone formation. Immunohistochemically, the tumor cells were positive for CD31 (5/5), CD34 (5/5), vimentin (5/5) and smooth muscle actin (3/5) but negative for cytokeratin and epithelial membrane antigen.

CONCLUSIONSPrimary intermediate hemangioendothelioma of bone is a distinct entity and similar histologic classification applies as in its soft tissue counterparts. Comparison of the biologic behavior requires long-term follow-up studies.

Actins ; metabolism ; Antigens, CD34 ; metabolism ; Bone Neoplasms ; diagnostic imaging ; metabolism ; pathology ; Child ; Clavicle ; pathology ; Diagnosis, Differential ; Female ; Femur ; pathology ; Hemangioendothelioma ; diagnostic imaging ; metabolism ; pathology ; Hemangiosarcoma ; pathology ; Humans ; Humerus ; pathology ; Immunohistochemistry ; Infant ; Kasabach-Merritt Syndrome ; diagnostic imaging ; metabolism ; pathology ; Male ; Middle Aged ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Radiography ; Sarcoma, Kaposi ; diagnostic imaging ; metabolism ; pathology ; Vimentin ; metabolism

Angiosarcoma is an uncommon malignancy, which spread out from the endothelial cells of vessels. Scalp angiosarcoma with cervical lymph node metastasis is particularly rare. This article describes a rare case of angiosarcoma of the scalp, presenting as neck inflammation. Imaging procedures such as computed tomography (CT), magnetic resonance image (MRI) and ultrasonography (US) were not sufficient to diagnose this case. A needle biopsy provided an effective and accurate diagnosis of cervical lymph node metastasis. Additional observation and physical examination was required to diagnose the origin of the primary cancerous lesion. Once the angiosarcoma diagnosis was confirmed histologically, sequential weekly and monthly docetaxel (DTX) treatment was effective in preventing reoccurrence. Nonetheless, the optimization of angiosarcoma treatment remains a future goal. Although patients generally describe pain and swelling at the primary lesion site, this patient complained only of painful neck inflammation, without any indication of pain or swelling of the scalp. A revised diagnostic protocol should note that cervical lymph node metastasis of unknown primary origin may result from angiosarcoma of the scalp.
Aged ; Antineoplastic Agents ; therapeutic use ; Cranial Irradiation ; Head and Neck Neoplasms ; diagnostic imaging ; pathology ; therapy ; Hemangiosarcoma ; diagnostic imaging ; pathology ; therapy ; Humans ; Lymphatic Metastasis ; Male ; Neck ; pathology ; Positron-Emission Tomography ; Radiography ; Scalp ; pathology ; Skin Neoplasms ; diagnostic imaging ; pathology ; therapy ; Taxoids ; therapeutic use ; Ultrasonography

OBJECTIVETo evaluate the pathologic diagnosis of hepatic epithelioid hemangioendothelioma (EH) in needle biopsy specimens.

METHODSFive cases of hepatic EH diagnosed in needle biopsies encountered during the period from 1999 to 2010 in Beijing Cancer Hospital were retrospectively reviewed. The specimens were formalin-fixed, paraffin-embedded and stained with hematoxylin and eosin. Immunohistochemical study was also carried out.

RESULTSAll the 5 patients were females. The age ranged from 23 to 47 years (mean = 39 years). The tumors in 4 patients were multiple and diagnosed as "metastasis" on ultrasound examination. The blood test results in all of the 5 patients were normal. Histologically, the tumor cells had an epithelioid appearance and were arranged in cords, solid nests or isolation, amongst a myxoid or hyaline matrix. The tumor cells contained scattered intracytoplasmic vacuoles which sometimes harbored red blood cells. There was no evidence of significant cellular pleomorphism, high mitotic activity and necrosis. Immunohistochemically, all of the 5 cases were positive for at least two endothelial markers (CD31, CD34 and factor VIII-related antigen). Smooth muscle actin was expressed in 1 case.

CONCLUSIONSThe diagnosis of hepatic EH can be established in needle biopsy specimens. The histologic pattern, when coupled with immunohistochemical findings, is useful in arriving at the correct diagnosis.

Actins ; metabolism ; Adult ; Antigens, CD34 ; metabolism ; Biopsy, Needle ; Carcinoma, Signet Ring Cell ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Hemangioendothelioma, Epithelioid ; diagnostic imaging ; metabolism ; pathology ; Hemangiosarcoma ; metabolism ; pathology ; Humans ; Liver Neoplasms ; diagnostic imaging ; metabolism ; pathology ; Melanoma ; metabolism ; pathology ; secondary ; Middle Aged ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Radiography ; Retrospective Studies ; Young Adult ; von Willebrand Factor ; metabolism