Hemangioendothelioma News Headlines

Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival.

Hum Pathol. 2008 Jul 3;

Authors: Zhang PJ, Brooks JS, Goldblum JR, Yoder B, Seethala R, Pawel B, Gorman JH, Gorman RC, Huang JH, Acker M, Narula N

Although cardiac sarcomas are rare in comparison to their soft tissue counterparts, they are the second most common type of primary cardiac neoplasm. Of the few hundred cases reported, most has been based on autopsy series. A series of 27 cardiac sarcomas removed at surgery for curative and diagnostic intent were reviewed for clinicopathologic features with correlation to available postoperative follow-up data in 17 patients. There were 6 angiosarcomas, 6 myxofibrosarcomas, 3 malignant peripheral nerve sheath tumors, 3 leiomyosarcomas, 2 synovial sarcomas, 1 epithelioid hemangioendothelioma, 1 chondrosarcoma, 1 osteosarcoma, and 4 poorly differentiated sarcomas. There was a wide age and size range with slight female predilection. There were 20 cases that arose in the atria/pulmonary vessels, 4 in the ventricles, 1 in mitral valve, and 2 in epi/pericardium. There was a slight left predilection. The histologic grade was low in 4, moderate in 3, and high in 20 cases. Six high-grade and 1 low-grade tumors were also treated with adjuvant chemotherapy and/or radiation. In 17 patients with follow-up data, 6 of 12 patients with high-grade tumor died (4 within 5 days of the initial surgery, 1 in 21 months, and 1 in 131 months), and 1 patient with moderate-grade tumor and all 4 patients with low-grade tumor were alive without evidence of disease at the end of follow-up. Tumor grade appeared to be prognostically important in cardiac sarcoma. Long survival was achieved in patients who survived the initial surgery well.

PMID: 18602663 [PubMed - as supplied by publisher]

[Infantile hepatic hemangioendothelioma. A multifocal, bilobular and asymptomatic case with spontaneous regression.]

An Pediatr (Barc). 2008 May;68(5):507-10

Authors: Araújo AR, Maciel I, Costa JC, Vieira A, Enes C, Santos Silva E

Infantile hepatic hemangioendothelioma is a severe disease with a high mortality rate. Nevertheless these vascular lesions may experience spontaneous regression within 12 to 18 months. The decision of trying a specific treatment and the choice among the several therapeutic options remains controversial, particularly in those asymptomatic cases of multifocal, bilobular involvement. We describe a case of multifocal, bilobular and asymptomatic infantile hepatic hemangioendothelioma, untreated, and with spontaneous regression before 2 years of age.

PMID: 18447999 [PubMed - in process]

[Oropharyngeal kaposiform hemangioendothelioma.]

Acta Otorrinolaringol Esp. 2008 Apr;59(4):198-9

Authors: Maseda E, Blanco R, Ablanedo A, Iglesias E

The kaposiform hemangioendothelioma is a very infrequent tumor proceeding from the endothelial-derived spindle cells, more often found on the limbs, although peritoneal, retroperitoneal and sacrum locations are also prevailing. Head and neck are exceptional locations. The kaposiform hemangioendothelioma is almost exclusively found in children and early adolescents, and it is highly associated with the Kasabach-Merritt syndrome and lymphangiomatosis. The main treatment is the tumor surgical removal, including wide margins, plus supporting therapy when Kasabach-Merritt syndrome is linked. We report an isolated oropharyngeal kaposiform hemangioendothelioma 9-10-year old male case.

PMID: 18447980 [PubMed - in process]

Related Articles

Omental lipoblastoma.

Pathol Res Pract. 2008 Feb 12;

Authors: Koplin SA, Twohig MH, Lund DP, Hafez GR

A large intra-abdominal mass was discovered in a 6-month-old boy during a routine well-child examination. Imaging studies revealed a solid mass which appeared to arise from the left lobe of the liver, extending caudally and filling the entire pelvis. At the time of surgical excision, the mass was found to be unassociated with the liver, but was instead localized to the omentum. Histologic examination revealed adipocytes of varying stages of maturation arranged in a lobular architecture, consistent with a lipoblastoma. This unusual tumor is only the eighth reported omental lipoblastoma [J. Hicks, A. Dilley, D. Patel, J. Barrish, S. Zhu, M. Brandt, Lipoblastoma and lipoblastomatosis in infancy and childhood: histologic, ultrastructural, and cytogenetic features. Ultrastruct. Pathol. 25 (2001) 321-333; J. Harrer, G. Hammon, T. Wagner, M. Bolkenius, Lipoblastoma and lipoblastomatosis: a report of two cases and review of the literature. Eur. J. Pediatr. Surg. 11 (2001) 342-349; S. Weiss and J. Goldblum, Enzinger and Weiss's Soft Tissue Tumors, fourth ed., Mosby, St. Louis, MO, 2001, pp. 601-605, 670-686; S. Soin, S. Andronikou, R. Lisle, K. Platt, K. Lakhoo, Omental lipoblastoma in a child; diagnosis based in CT density measurements. J. Pediatr. Hematol. Oncol. 28(1) (2006) 57-58; A. Prando, S. Wallace, J.L. Marins, R.M. Pereira, E.R. de Oliveira, M. Alvarenga, Sonographic features of benign intraperitoneal lipomatous tumors in children-report of 4 cases. Pediatr. Radiol. 20(8) (1990) 571-574; C. Blank, E. Schoenmakers, P. Rogalla, E. Huys, A. Van Rijk, N. Drieschner, J. Bullerdiek, Intragenic breakpoint within RAD51L1 in a t(6;14)(p21.3;q24) of a pulmonary chondroid hamartoma. Cytogenet. Cell Genet. 95 (2001) 17-19; S. Ingraham, R. Lynch, S. Kathiresan, A. Buckler, A. Menon, hREC2, a RAD51-like gene, is disrupted by t(12;14)(q15;q24.1) in a uterine leiomyoma. Cancer Genet. Cytogenet. 115 (1999) 56-61]. Cytogenetics revealed a karyotype of 46,XY,t(8;14)(q13;q24). While lipoblastomas characteristically involve 8q, only one prior case has been reported with 14q24 as its fusion partner [M. He, K. Das, M. Blacksin, J. Benevenia, M. Hameed, A translocation involving the placental growth factor gene is identified in an epithelioid hemangioendothelioma. Cancer Genet. Cytogenet. 168 (2006) 150-154]. We report this unique case of an omental lipoblastoma with a focus on its unusual karyotype, as well as its differentiation from myxoid liposarcoma.

PMID: 18276084 [PubMed - as supplied by publisher]