Perivascular epithelioid cell tumor of the heart in a child
| Autor: | Lixin Wei, Huaiyin Shi, Yan-hong Tai |
|---|---|
| Rok vydání: | 2010 |
| Předmět: |
Pathology
medicine.medical_specialty business.industry Perivascular Epithelioid Cell Neoplasms Cardiovascular Surgical Procedures Disease free Solid mass General Medicine Immunohistochemistry Perivascular Epithelioid Cell Pathology and Forensic Medicine Heart Neoplasms Smooth muscle Pediatrics Perinatology and Child Health Eosinophilic Medicine Humans Female business Child Epithelioid cell |
| Zdroj: | Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society. 13(5) |
| ISSN: | 1093-5266 |
| Popis: | We report a case of cardiac perivascular epithelioid cell tumor (PEComa) that occurred in a 10-year-old girl. The patient presented with cardiac murmur and increasing dyspnea. A solid mass was detected in the left atrioventricular groove by sonography and chest computed tomography. Histologic examination revealed a tumor composed of spindle and epithelioid cells, exhibiting either a clear or a slightly eosinophilic cytoplasm and a bland nucleus. Intranuclear pseudoinclusions and calcifications could be found occasionally. Immunohistochemically, tumor cells were positive for HMB45, melan-A, and smooth muscle actin. We regard this case as a PEComa of uncertain malignant potential. The patient remains disease free at her 18-month follow up. To the best of our knowledge, this is the 3rd report of cardiac PEComa and the 1st report of cardiac PEComa affecting a child. |
| Databáze: | OpenAIRE |
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