Retroperitoneal kaposiform hemangioendothelioma with tufted angioma-like features in an infant with Kasabach-Merritt syndrome
Autor: | Lidija Dokmanovic, Ivan Boricic, Dimitrije Brasanac, Dragana Janic, Nada Jovanovic |
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Rok vydání: | 2003 |
Předmět: |
Tufted angioma
Pathology medicine.medical_specialty Kasabach–Merritt syndrome Sigmoid mesocolon Pathology and Forensic Medicine Hemangioma 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine Colon Sigmoid Proliferating Cell Nuclear Antigen Colostomy Biomarkers Tumor medicine Humans Retroperitoneal Neoplasms Sarcoma Kaposi business.industry Infant Sigmoid colon Syndrome General Medicine medicine.disease Combined Modality Therapy Immunohistochemistry Thrombocytopenia 3. Good health Ki-67 Antigen Treatment Outcome medicine.anatomical_structure Proto-Oncogene Proteins c-bcl-2 Kaposiform Hemangioendothelioma 030220 oncology & carcinogenesis Hemangioendothelioma Prednisone Sarcoma business Venous malformation |
Zdroj: | Pathology International. 53:627-631 |
ISSN: | 1440-1827 1320-5463 |
DOI: | 10.1046/j.1440-1827.2003.01518.x |
Popis: | Kasabach-Merritt syndrome denotes profound thrombocytopenia and coagulopathy in an infant with a vascular tumor. A retroperitoneal vascular tumor with an unusual combination of histopathological features is reported, and compared with vascular lesions described in the reported cases of Kasabach-Merritt syndrome in the literature. A large retroperitoneal tumor that had expanded through the sigmoid mesocolon into the sigmoid colon wall was resected from an 8-month-old infant with fully developed Kasabach-Merritt syndrome. Histological examination revealed a combination of venous (cavernous) malformation, kaposiform hemangioendothelioma and tufted angioma-like areas. Cellular tumor components (especially tufted angioma-like parts) infiltrated the wall of the sigmoid colon to the submucosal level. Immunohistochemical staining with antibodies to the Ki-67 antigen and proliferating cell nuclear antigen showed a low proliferative activity, whereas the antiapoptotic bcl-2 protein was expressed diffusely in tumor cells. This is the first reported case of a vascular tumor with tufted angioma-like elements found in the retroperitoneum, and the first reported in combination with kaposiform hemangioendothelioma and venous malformation in the same lesion. Considering the immunohistochemical results and overlapping histological features, it may be considered that tufted angioma and kaposiform hemangioendothelioma represent different growth patterns or stages in the development of a single type of hemangioma. |
Databáze: | OpenAIRE |
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