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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.


Figure 1
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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.

 

Figure 2
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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).

 

Figure 3
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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.

 

Figure 4
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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).

 

Figure 5
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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.

 

Figure 6
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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.

 

Figure 7
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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).

 

Figure 8
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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.

 

Figure 9
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Fig. 5A 35-year-old woman with neurofibromatosis type 1. Sagittal fat-saturated T2-weighted fast spinecho MR image (TR/TE, 4,000/98) shows multiple nerve sheath tumors (arrows).

 

Figure 10
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Fig. 5B 35-year-old woman with neurofibromatosis type 1. Axial fat-saturated T2-weighted fast spin-echo MR image (4,000/98) shows dominant left apical mass (arrows).

 

Figure 11
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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.

 

Figure 12
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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.

 

Figure 13
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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.

 

Figure 14
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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.

 

Figure 15
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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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