Plexiform neurofibromatosis of the liver and mesentery in a child

✍ Scribed by Jacqueline S. Partin; Bernard P. Lane; John C. Partin; Lawrence R. Edelstein; Cedric J. Priebe Jr.

• Publisher: John Wiley and Sons
• Year: 1990
• Tongue: English
• Weight: 759 KB
• Volume: 12
• Category: Article
• ISSN: 0270-9139
• DOI: 10.1002/hep.1840120318

✦ Synopsis

Pledform neurofibromatoeis of the liver was recognized by needle biopsy of the liver in an 11-yr-old boy who had a 2 9 history of diarrhea, intermittent abdominal pain, failure to gain weight and progreesive abdominal distention. Imaging studiee 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 hyperpleeia existed throughout the liver. The most dietal 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 time. The nontumoral reaponse was more than eimple hyperplasia because it appeared to result in fibrotic changes in the most involved areas and active breaching of the limiting plate with destruction of hepatocytea and collagen deposition throughout the liver. (HEPATOLOGY 1990,12:559-M)4.) Neurofibromatosis is an important, genetically determined group of diseases. A Consensus Development Conference on Neurofibromatosis (11, convened by the National Institutes of Health in 1987, set diagnostic criteria for two distinct forms, NF-I and NF-11. The NF-I gene has been localized to chromosome 17 and the NF-I1 defect resides on chromosome 22. Riccardi and Eichner (2) define eight classes of neurofibromatosis (NF): NF-I,