Hemangioendothelioma is a rare vascular tumor with involvement of the liver, brain, long bones, and lung. Among the 6 histological subtypes, epithelioid hemangioendothelioma (EHE) is the most aggressive. Its occurrence in the mediastinum is quite rare, and very few cases have been documented. The reported cases in the literature have described difficulties in the preoperative diagnosis due to the unusual histological appearance of the tumor. Immunohistochemistry remains the mainstay for a definitive diagnosis. Due to its low incidence, there is no standard treatment for mediastinal EHE, but curative resection is the preferred treatment option where possible, with chemotherapy used as an adjuvant treatment or in cases of widespread inoperable disease. The present case study describes an aggressive EHE occurring in an 18-year-old woman in the anterior mediastinum.
Adolescent ; Brain ; Diagnosis ; Drug Therapy ; Female ; Hemangioendothelioma ; Hemangioendothelioma, Epithelioid* ; Humans ; Immunohistochemistry ; Incidence ; Liver ; Lung ; Mediastinum* ; Vascular Neoplasms

Hemangioendothelioma is a rare vascular tumor characterized by endothelial tumor cells and variable malignant behavior, and it's not common for this lesion to involve the bone. Although there are a few reports of cranial involvement by hemangioendothelioma, only rare cases arising in temporal bone have been published. We present the radiologic findings of a 7-year-old boy who had a high grade hemangioendothelioma involving the temporal bone with intracranial extension. Evidence of flow voids on MR images suggested a tumor of vascular origin, and the ill-defined margins, cortical destruction and intracranial extension on the CT and MR images were correlated with the tumor's high histologic grade.
Brain Neoplasms/*diagnosis/therapy ; Child ; Hemangioendothelioma/*diagnosis/therapy ; Humans ; Magnetic Resonance Imaging ; Male ; Temporal Bone/*pathology ; Tomography, X-Ray Computed

Adult ; Antineoplastic Agents ; administration & dosage ; Chemoembolization, Therapeutic ; Hemangioendothelioma, Epithelioid ; diagnosis ; therapy ; Humans ; Liver Neoplasms ; diagnosis ; therapy ; Male

Epithelioid hemangioendothelioma (EHE) is a rare tumor of vascular origin. While it can be found in any tissue, it is most often found in lung and liver and usually has an intermediate behavior. EHEs originating from pleural tissue have been less frequently described than those from other sites. Furthermore, to date, all of the cited pleural EHEs were described as highly aggressive. In the present report, we describe a rare case of pleural EHE extending to lung and bone in a 31-year-old woman. The histological diagnosis was confirmed by both conventional examination and immunohistochemistry. Her disease stabilized during the 4th course of adriamycin (45mg/m2, day 1-3), dacarbazine (300mg/m2, day 1-3) and ifosfamide (2,500mg/m2, day 1-3) with mesna, and she survived for 10 months after the diagnosis.
Adult ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Diagnosis, Differential ; Factor VIII/metabolism ; Female ; Hemangioendothelioma, Epithelioid/*diagnosis/drug therapy/metabolism ; Humans ; Immunohistochemistry ; Keratins/metabolism ; Lung Neoplasms/diagnosis ; Pleural Neoplasms/*diagnosis/drug therapy/metabolism ; Vimentin/metabolism

PURPOSE: The frequency and clinical characteristics of primary childhood hepatic tumors diagnosed in a single institution were reviewed. Method: From January 1991 to June 2000, 33 patients were diagnosed with primary hepatic tumor. Retrospective analysis of the medical records of the various primary hepatic tumors was performed. RESULTS: Among the total of 33 patients with primary hepatic tumor, twenty five (76%) were malignant and eight (24%) were benign. The mean age of patients with hepatoblastoma (HB) was 32 months (12 of 16 patients were less than 3 years), 12.8 years in hepatocellular carcinoma (HCC) (all of them were older than 11 years), 2 months in hemangioendothelioma (HE), and 3 months in hamartoma. The ratio of male to female was 1:1 in HB, and 4:1 in HCC. The presence of hepatitis B infection was detected in 80% (4/5) of HCC and in 13% (2/16) of HB. The common presenting symptoms at diagnosis were the palpable mass, abdominal pain and jaundice. Many tumors were found incidentally without any presenting symptoms. Of 33 patients, 22 had involvement at right lobe. HCC had more necrosis and hemorrhage within the tumor than HB on imaging study. HE had a distinct feature of contrast enhancement increasing from the peripheral portion of the mass to central portion. Hamartoma showed a feature with multiple cysts and septums within the mass. To confirm the diagnosis, a tissue biopsy was done in 23 patients (70%). A significantly elevated level of serum alpha-fetoprotein (AFP) is found in 88% (mean 386,000 ng/mL) of HB cases and 100% (mean 369,000 ng/mL) of HCC. The mean AFP level in HB patients was 391,000 ng/mL at diagnosis, 52 ng/mL after the preoperative chemotherapy, 8.3 ng/mL at postoperative follow-up, and 2.8 ng/mL at completion of chemotherapy. In HCC, the AFP level decreased temporarily after chemotherapy or chemo- embolization, but increasesd at recurrence in most case. Patients resectable at the time of diagnosis or after preoperative chemotherapy were 12 (75%) in HB, and 10 of them are were alive with median the follow-up of 34 months (5 mo.~8 yrs 7 mo.). All the HCC patients were unresectable or had metastatic lesion at diagnosis, and all of them were assumed dead following discharge. Conclusions: Among the primary hepatic tumors in children, malignant tumors are more frequent than benign. Age of onset, AFP level and imaging studies all play an important role in diagnosis, and biopsy is usually confirmative. HCC has an extremely poor prognosis in childhood. HB patients with complete surgical resection enjoy a relatively high long term survival.
Abdominal Pain ; Age of Onset ; alpha-Fetoproteins ; Biopsy ; Carcinoma, Hepatocellular ; Child* ; Diagnosis ; Drug Therapy ; Female ; Follow-Up Studies ; Hamartoma ; Hemangioendothelioma ; Hemorrhage ; Hepatitis B ; Hepatoblastoma ; Humans ; Jaundice ; Male ; Medical Records ; Necrosis ; Prognosis ; Recurrence ; Retrospective Studies