Clinical Trial: Transformation of Plexiform Neurofibromas to Malignant Peripheral Nerve Sheath Tumors in Neurofibromatosis Type 1

Study Status: Recruiting
Recruit Status: Recruiting
Study Type: Interventional

Official Title: Transformation of Plexiform Neurofibromas to Malignant Peripheral Nerve Sheath Tumors in Neurofibromatosis Type 1: Clinical, Histopathologic, and Genomic Analysis

Brief Summary:

Background:<TAB>

- Many people with neurofibromatosis type 1 (NF1) get tumors of the nervous system. Finding malignant tumors early is important for removing them. Researchers want to find ways of doing this with scans and genetic testing.

Objectives:

- To learn more about neurofibromatosis type 1.

Eligibility:

- People age 10 and older with NF1 who have a benign tumor or have had a malignant one.

Design:

Participants will be screened in another study with medical history, physical exam, and urine and blood tests. They will have a magnetic resonance imaging (MRI) scan.
MRI: Participants will lie on a table that slides into a metal cylinder. They will be in the scanner for 60 90 minutes, lying still for 15 minutes at a time. Participants will get earplugs for the loud sounds. They will get a contrast agent (dye) through a thin plastic tube (catheter) inserted in an arm vein.
As part of their regular care, participants will have:
FDG-PET/CT scan. They will get radioactive glucose (sugar) through a catheter in an arm vein.
[18F]-FLT-PET/CT scan. This is like the FDG scan but with a different radioactive chemical.
Biopsy. A piece of tumor tissue is removed with a needle. A piece of tissue from a previous biopsy may also be studied.
Participants may have genetic testing. Blood will be taken. It will be tested along with biopsy samples. Res