Plexiform neurofibromatosis of the liver and mesentery in a child
| Autor: | Bernard P. Lane, Lawrence Edelstein, Cedric J. Priebe, John C. Partin, Jacqueline S. Partin |
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| Rok vydání: | 1990 |
| Předmět: |
Male
Pathology medicine.medical_specialty Neurofibromatosis 1 medicine.medical_treatment Plexiform neurofibroma Laparotomy Biopsy medicine Humans Neurofibroma Mesentery Neurofibromatosis Child Peritoneal Neoplasms Hyperplasia Hepatology medicine.diagnostic_test business.industry Biopsy Needle Liver Neoplasms Anatomy medicine.disease Microscopy Electron Hilum (anatomy) medicine.anatomical_structure Liver Lymphatic Metastasis business |
| Zdroj: | Hepatology. 12:559-564 |
| ISSN: | 1527-3350 0270-9139 |
| Popis: | Plexiform neurofibromatosis of the liver was recognized by needle biopsy of the liver in an 11-yr-old boy who had a 2-yr history of diarrhea, intermittent abdominal pain, failure to gain weight and progressive abdominal distention. Imaging studies demonstrated a large retroperitoneal mass; a laparotomy was performed. At surgery, the mesentery was greatly thickened by neurofibromas, and plexiform neurofibroma extended through the hilum of the liver. Light and electron microscopy demonstrated that in addition to the direct involvement by tumor, neural hyperplasia existed throughout the liver. The most distal ramifications of the portal spaces were filled with Schwann cells, bundles of unmyelinated nerves and perineurium-surrounded nerves containing myelinated and unmyelinated fibers. The ultrastructural findings were consistent with stimulation of proliferation of all the portal neural elements and tumoral tissue. The nontumoral response was more than simple hyperplasia because it appeared to result in fibrotic changes in the most involved areas and active breaching of the limiting plate with destruction of hepatocytes and collagen deposition throughout the liver. |
| Databáze: | OpenAIRE |
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