Mediastinal and Neck Kaposiform Hemangioendothelioma: Report of Three Cases

Authors: O'Regan, Grainne M.¹; Irvine, Alan D.¹; Yao, Nana²; O'Marcaigh, Aengus³; Sheridan-Pereira, Margaret⁴; Phelan, Eithne⁵; McDermott, Michael B.⁶; Twomey, Anne²; Russell, John⁷; Watson, Rosemarie¹

Source: Pediatric Dermatology, Volume 26, Number 3, May/June 2009 , pp. 331-337(7)

Publisher: Wiley-Blackwell

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Abstract:

Kaposiform hemangioendothelioma is an aggressive vascular tumor, named for its striking histologic resemblance to Kaposi sarcoma and locally invasive growth. Mortality is high, and ranges from 10% to 24% for all kaposiform hemangioendothelioma lesions, with a significantly higher mortality for deep soft-tissue or visceral lesions occurring in infants less than 6 months. Mediastinal and neck kaposiform hemangioendothelioma in particular merit special discussion, as involvement of these critical anatomic locations results in significant site-specific therapeutic challenges due to invasion of vital structures, inherent delays in establishing histopathologic confirmation, and difficulties in monitoring disease status. We report our experience with three cases of mediastinal and neck kaposiform hemangioendothelioma, emphasizing the unique diagnostic and management challenges, variable response to treatment and outcome of this anatomic variant of kaposiform hemangioendothelioma.

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1525-1470.2009.00913.x

Affiliations: 1: Departments of Paediatric Dermatology 2: Neonatology, National Maternity Hospital, Dublin, Ireland 3: Departments of Paediatric Haematology 4: Neonatology 5: Paediatric Radiology 6: Histopathology 7: Paediatric Otolaryngology, Our Lady's Children's Hospital, Dublin, Ireland