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Mindy X.
Wang et al.
of the Department of Radiology, University of Texas Anderson Cancer Center, reviewed the genetics and molecular pathogenesis, clinical and pathological features, imaging findings, and multidisciplinary management and monitoring of NF1 and NF2, published in the July-August 2022 issue
of Radiographics.
—Excerpted from the article chapter
【Ref: Wang MX, et al.
Radiographics.
2022 Jul-Aug; 42(4):1123-1144.
doi: 10.
1148/rg.
210235.
Epub 2022 Jun 24.
】
Research background
Neurofibromatosis type 1 (NF1) and neurofibromatosis type 2 (NF2) are autosomal dominant diseases, but the diagnostic criteria and imaging findings are different between the two
.
Mindy X.
Wang et al.
of the Department of Radiology, University of Texas Anderson Cancer Center, reviewed the genetics and molecular pathogenesis, clinical and pathological features, imaging findings, and multidisciplinary management and monitoring of NF1 and NF2, published in the July-August 2022 issue
of Radiographics.
Research results
NF1 is the most common and is considered a diagnostic feature
with major clinical manifestations including café au lait spots, axillary or inguinal freckles, neurofibroma or plexiform neurofibromas, optic gliomas, Lisch nodules, and bone lesions such as sphenoid dysplasia.
However, there are many other manifestations, such as concomitant low-grade gliomas, interstitial lung disease, etc
.
Common focal area (FASI) intensity signals on MRI imaging, occurring in 70% of NF1 cases, FASI in MRI-T2-weighted imaging and FLAIR imaging, showing focal or diffuse hyperintensity in the basal ganglia, cerebellum, or brainstem; T1-weighted imaging, on the other hand, is usually equisignal or mildly hyperintensive, with no reinforcement and no mass effect
.
MRS sequences showed no decrease in N-acetylaspartate levels, unlike
gliomas.
Gliomas are distinguished from FASI by the presence of mass effects, T1 and T2 sequence characteristics, and potentiation benefits over
time.
Gliomas in MRS present with decreased or absent N-acetylaspartate, increased choline, and decreased creatine levels, which help to differentiate
.
Heterogeneous signal intensity on MRI-T1-weighted images suggests malignancy
.
Four imaging features on MRI can help distinguish malignancy from benign neurofibromas: mass lesion size, peripheral enhancement effect, lesional edema, and intratumor cystic changes
.
Various NF1-related intraperitoneal tumors can be classified by their cellular origin: neurogenic tumors, mesenchymal cell Cajal tumors, neuroendocrine tumors, and embryonic tumors
.
Typical manifestations of NF2 include features of vestibular schwannomas, multiple meningiomas, and spinal ependymomas (Table 1, 2; Figure 1).
Table 1.
NF1 diagnostic criteria
Table 2.
NF2 diagnostic criteria
Figure 1.
Diagnostic points for NF1 and NF2
Conclusion of the study
In summary, the main manifestations of NF1 include café au lait spots, freckles, neurofibromas, optic gliomas, Lisch nodules, and various bone lesions
.
NF2 typically presents with features
of vestibular schwannomas, multiple meningiomas, and spinal ependymomas.
Radiologists play a vital role
in the diagnosis and follow-up of NF1 and NF2.
