Neurofibromatosis type 1

Case contributed by Dr Dalia Ibrahim

Presentation

History withheld.

Patient Data

Age: 25 years
Gender: Male
Modality: CT

Large confluent masses of low attenuation are seen within the superior, middle and posterior mediastinum.

Diffuse abdominal and retroperitoneal plexiform neurofibromatosis with large confluent hypodense masses almost totally occupying the abdominal and pelvic cavities. They are seen within the celiac region, extending to the periportal region, para-aortic region, small bowel mesentery, greater omentum, and retrocrural space.

They also encase the patent splanchnic branches without narrowing, displacing and compressing the adjacent organs and bowel loops without invasion.

Multiple diffuse and scattered subcutaneous and muscular nodules and masses (plexiform neurofibromas).

Multiple low-density well-defined hypodense neurofibromas are seen along lumbosacral spinal canal, in the neural foramina and paravertebral regions bilaterally, along the sacral plexus in the pelvis on both sides and along the course of the nerves in both thighs with large right anterior thigh mass. 

Case Discussion

The patient is known to have neurofibromatosis 1 and presented complaining of increased abdominal girth and recurrent attacks of intestinal obstruction.

This is a case of huge abdominal and retroperitoneal plexiform neurofibromas in a known case of neurofibromatosis type 1 (NF1).

He underwent abdominal surgical intervention and biopsy proved neurofibromas.

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Case Information

rID: 49538
Case created: 25th Nov 2016
Last edited: 21st Feb 2017
Inclusion in quiz mode: Included

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