Giant Plexiform Neurofibroma of the Pelvic Region: Unusual Presentation in an Uncommon Location (About a Case)

Abstract

Plexiform neurofibroma is a rare benign tumor of the peripheral tissue cells developed in the perineurium, generally associated with neurofibromatosis type 1 (NF1). These entities consist of multiple, twisted masses that grow along the axis of a large nerve, infiltrating and separating normal nerve fascicles. The diagnosis is essentially pathological and treatment is primarily surgical to avoid malignant degeneration. Giant neurofibromas are very difficult to manage surgically as they are extensively infiltrative and highly vascularized. This report describes a case of an advanced plexiform neurofibroma of the right lower back and pelvic region in a 25-year-old female with neurofibromatosis type 1, discovered in early childhood, with limited normal living due to the severe disfigurement as the result of progression of the disease.

Plexiform neurofibroma masquerading as a seminal vesicle mass: A case review

“…On non-enhanced CT scans, they appear as low-attenuation masses because of the lipid content, endo-neural myxoid matrix and cystic degeneration. 7 MRI is currently the preferred modality if there is a suspicion of neurofibroma. MRI can demonstrate the character and size of the neurofibroma, enable diagnosis of associated lesions and distinguish between neurofibromas and other malignant nerve sheath tumours.…”