Value of PET in the Assessment of Patients with Neurofibromatosis Type 1
Miriam A. Bredella1,
Martin Torriani1,
Francis Hornicek2,
Hugue A. Ouellette1,
William E. Plamer1,
Ziv Williams3,
Allan J. Fischman1 and
Scott R. Plotkin4
1 Department of Radiology, Massachusetts General Hospital, 55 Fruit St., Yawkey
6E, Boston, MA 02114.
2 Department of Orthopedic Surgery, Massachusetts General Hospital, Boston,
MA.
3 Department of Neurosurgery, Massachusetts General Hospital, Boston, MA.
4 Department of Neurology, Massachusetts General Hospital, Boston, MA.

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Fig. 1A —48-year-old man with neurofibromatosis type 1 who presented
with pain in right buttock area. Coronal fat-saturated T2-weighted fast
spin-echo MR image (TR/TE, 4,000/98) shows multiple hyperintense soft-tissue
masses (arrows) that are consistent with multiple nerve sheath
tumors.
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Fig. 1B —48-year-old man with neurofibromatosis type 1 who presented
with pain in right buttock area. Coronal whole-body FDG PET image shows mild
FDG uptake in multiple nerve sheath tumors (black arrows); dominant
mass (white arrow) along right sciatic nerve shows intense FDG uptake
that is suspicious for malignant peripheral nerve sheath tumor (MPNST).
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Fig. 1C —48-year-old man with neurofibromatosis type 1 who presented
with pain in right buttock area. Axial FDG PET image corresponding to B
shows markedly increased FDG uptake along right sciatic nerve (standardized
uptake value = 5.1) (arrows). MPNST was confirmed at surgery and
patient underwent right hemipelvectomy.
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Fig. 2A —20-year-old man with neurofibromatosis type 1 who presented
with enlarging calf mass. Sagittal fat-saturated T2-weighted fast spin-echo MR
image (TR/TE, 4,000/98) shows large plexiform neurofibroma along tibial nerve
(arrows).
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Fig. 2B —20-year-old man with neurofibromatosis type 1 who presented
with enlarging calf mass. Coronal FDG PET image shows increased FDG uptake in
large plexiform neurofibroma with two foci of increased uptake (standardized
uptake value = 4.1) (arrows) that are suspicious for malignant
transformation. Mass was resected and foci of malignant transformation were
found.
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Fig. 3 —False-negative FDG PET study in 38-year-old man with
neurofibromatosis type 1 who presented with enlarging abdominal mass. Axial
FDG PET image shows large area of photopenia with surrounding mildly increased
FDG uptake (standardized uptake value = 1.8) (arrows). At surgery,
poorly differentiated carcinoma was found.
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Fig. 4A —53-year-old man with neurofibromatosis type 1. Coronal fused
FDG PET/CT image depicts mass (arrow) in left iliopsoas muscle that
shows increased FDG uptake (standardized uptake value = 4.2).
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Fig. 4B —53-year-old man with neurofibromatosis type 1. Coronal
carbon-11 (11C) methionine PET image, obtained after A,
shows no abnormal activity in left iliopsoas muscle. Biopsy and long-term
follow-up showed no evidence of malignancy. Note lack of radiotracer uptake in
heart on 11C methionine PET. Physiologic uptake within several
bowel loops (arrows) is noted.
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Fig. 5C —35-year-old woman with neurofibromatosis type 1. Coronal FDG
PET image shows increased FDG uptake (standardized uptake value = 4.1)
(arrow) that is suspicious for malignant transformation.
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Fig. 5D —35-year-old woman with neurofibromatosis type 1. Coronal
fused carbon-11 methionine PET/CT image shows no abnormal radiotracer uptake
in left lung apex, and biopsy and follow-up were negative for malignancy.
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Fig. 6A —Gastrointestinal stromal tumor (GIST) in 36-year-old man with
neurofibromatosis type 1. Contrast-enhanced axial CT image shows mass in small
bowel (arrows) that was not appreciated on this CT scan prior to PET
imaging.
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Fig. 6B —Gastrointestinal stromal tumor (GIST) in 36-year-old man with
neurofibromatosis type 1. Axial fused FDG PET/CT image shows increased FDG
uptake within mass (arrow), and surgical resection showed GIST.
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Fig. 7 —Mean standardized uptake value (SUV) of benign and malignant
lesions in patients with neurofibromatosis type 1. Box plot of maximum SUV
(SUVmax) for benign and malignant lesions shows there is a
statistically significant difference between absolute SUV values of benign and
those of malignant lesions (p < 0.001, Student's t test).
Middle horizontal lines in boxes correspond to mean SUV, and upper and lower
horizontal lines correspond to mean ± SEM (standard error of the
mean).
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Copyright © 2007 by the American Roentgen Ray Society.