MEDIASTINAL KAPOSIFORM HEMANGIOENDOTHELIOMA: BRIEF REVIEW OF THE LITERATURE WITH THE ADDITION OF A NEW CASE

  • Prim. Dr sc Slavisa Đurićić Prim. Dr sc Slavisa Đurićić, Nauč. Sar. Služba za kliničku patologiju, Institut za zdravstvenu zaštitu majke i deteta „Dr Vukan Čupić“ Radoja Dakića 6-8, 11070 Novi Beograd tel. :+381 11 3108 231 e-mail: slavisa.djuricic@gmail.com mob. tel.: +381 65 8927260
  • Asist. dr sc. Adrijan Sarajlija Department of Metabolic Disease and Clinical Genetic, Mother and Child Health Care Institute of Serbia “Dr Vukan Cupic”, Belgrade, Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia
  • Prim. dr sc. Dragomir Djokic Department of Hematology and Oncology, Mother and Child Health Care Institute of Serbia “Dr Vukan Cupic”, Belgrade, Serbia
  • Doc. dr sc. Radoje Simic Department of Plastic and Reconstructive Surgery and Burns, Mother and Child Health Care Institute of Serbia “Dr Vukan Cupic”, Belgrade, Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia
DOI: https://doi.org/10.5937/scriptamed51-25350
Keywords: kaposiform hemangioendothelioma, Kasabach-Merritt phenomenon, mediastinum, infant

Abstract


Kaposiform hemangioendothelioma (KHE) is a rare, locally invasive, vascular tumor, which is commonly associated with Kasabach-Merritt phenomenon (KMP). We present a case of a five-month-old female infant admitted for dyspnea, stridor, and skin hematoma. Computerized tomography of the chest showed a tumor mass occupying mediastinum and most of the left hemithorax, while laboratory analysis revealed thrombocytopenia and consumption coagulopathy. Histology of tumor biopsy was characteristic of KHE with a component of tufted angioma. Corticosteroid treatment initially induced a reduction in tumor size, but progression occurred 4 weeks later and led to a fatal outcome despite additional chemotherapy. After a literature search, we found only 18 cases of mediastinal KHE published so far, with death occurring in 21% of patients. In our case, several risk factors for adverse outcome were present: onset of disease in early infancy, a large volume of the tumor, mediastinal location, KMP, and partial response to therapy.

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Published
2020/03/27
Issue
Vol. 51 No. 1 (2020)
Section
Case report
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