Kashif Siddique, Zeeshan Shabeer Sipra.
Periportal Plexiform Neurofibromatosis; a rare intra-abdominal manifestation of NF1..
J Pak Med Assoc Jan ;70(3):557-8.

Periportal Plexiform Neurofibromatosis (PPNF) is a rare visceral manifestation of Neurofibromatosis Type 1 (NF1) or Von Recklinghausen disease. Neurofibromas are the most common lesions in NF1. We present a case of a young female diagnosed with NF1 who initially presented with hard abdominal mass. Contrast enhanced CT revealed the unusual lintrahepatic periportal plexiform neurofibromatosis in addition to a typical large retroperitoneal lumbar neurofibroma.

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