BACKGROUND/AIMS: Hepatic angiosarcoma, a rare and aggressive liver malignancy, is difficult to diagnose because of a lack of specific clinical features. The clinical and radiological features of patients with histologically confirmed hepatic angiosarcoma were examined. METHODS: Among 2,336 patients diagnosed with primary hepatic carcinoma at Keimyung University Dongsan Medical Center (Daegu, Korea) between May 2002 and February 2012, eight (0.03%) with histologically confirmed primary hepatic angiosarcoma were included. The patterns of disease diagnosis, tumor characteristics, treatment responses, and prognoses were reviewed retrospectively. RESULTS: Median age was 66 years-old (range, 41-80 years). Four patients were male. Five patients were compulsive drinkers. All patients had no HBsAg and anti-HCV. Initial radiologic diagnoses revealed primary hepatic angiosarcoma (n=2), hepatocellular carcinoma (n=2), hemangioma (n=2), and hepatic metastatic carcinoma (n=2). Definitive diagnoses were made by percutaneous needle biopsies in seven patients and surgical resection in one patient. At the time of the initial diagnosis, extrahepatic metastases were detected in three patients (37.5%). Metastatic sites included the spleen and lung, pericardium, and bone, in one patient each. Two patients underwent conservative treatments. The remaining patients underwent surgical resection (n=1), transcatheter arterial chemoembolization (n=1), and systemic chemotherapy (n=4). The median survival period was 214 days (range, 21-431 days). CONCLUSIONS: Hepatic angiosarcoma is a highly progressive disease with a poor prognosis. Detailed studies including histological examinations are essential to facilitate early diagnosis of the disease.
Adult ; Aged ; Aged, 80 and over ; Antineoplastic Agents/therapeutic use ; Embolization, Therapeutic ; Female ; Hemangiosarcoma/*diagnosis/pathology/therapy ; Humans ; Immunohistochemistry ; Liver Neoplasms/*diagnosis/pathology/therapy ; Male ; Middle Aged ; Neoplasm Metastasis ; Prognosis ; Retrospective Studies ; Survival Rate ; Tomography, X-Ray Computed

OBJECTIVETo study the clinicopathologic characteristics of sclerosing rhabdomyosarcoma (SRMS) and its distinction from embryonal rhabdomyosarcoma (ERMS) and alveolar rhabdomyosarcoma (ARMS).

METHODSThe clinical, histologic and immunohistochemical features of 4 cases of SRMS were studied. The literature was reviewed.

RESULTSAll the 4 cases occurred in adults. The age of patients ranged from 20 to 54 years (mean = 41.5 years). The male-to-female ratio was 1:1. The tumor was located in the left wrist, right thigh, right face and right cheek respectively and the tumor size varied from 2.5 cm to 10 cm in dimension (mean = 5.7 cm). Histologically, SRMS was characterized by the presence of large amounts of heavily hyalinized matrix, mimicking osteoid or chondroid tissue. The tumor cells were composed predominantly of primitive small round cells which were arranged in diverse growth patterns, including fascicular, cord-like, single-file, trabecular, microalveolar and pseudovascular structures. A few rhabdomyoblasts were identified in 1 case. A second spindle cell component was focally found in 2 cases, resembling spindle cell rhabdomyosarcoma or peripheral nerve sheath tumor. Immunohistochemically, all cases showed diffuse staining for Myo D1 and focal staining for desmin. The staining for myogenin was often negative. Three of the cases also expressed muscle-specific actin and 2 cases were positive for alpha-smooth muscle actin. They were all negative for h-caldesmon, S-100 protein, CD31, CD34, AE1/AE3 and anaplastic lymphoma kinase protein.

CONCLUSIONSSRMS differs from ERMS and ARMS morphologically. Recent cytogenetic studies however suggest a histogenetic relationship with ERMS. Familiarity with its morphologic features and immunophenotype may help to distinguish this peculiar variant of rhabdomyosarcoma from a variety of lesions with abundant sclerosing matrix.

Actins ; metabolism ; Adult ; Chondrosarcoma ; pathology ; Combined Modality Therapy ; Desmin ; metabolism ; Diagnosis, Differential ; Facial Neoplasms ; metabolism ; pathology ; therapy ; Female ; Follow-Up Studies ; Hemangiosarcoma ; pathology ; Humans ; Male ; Middle Aged ; MyoD Protein ; metabolism ; Osteosarcoma ; pathology ; Rhabdomyosarcoma ; classification ; metabolism ; pathology ; therapy ; Rhabdomyosarcoma, Alveolar ; classification ; pathology ; Rhabdomyosarcoma, Embryonal ; classification ; pathology ; Sclerosis ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; therapy ; Vimentin ; metabolism ; Young Adult