Research Article

How Effective Are Noninvasive Tests for Diagnosing Malignant Peripheral Nerve Sheath Tumors in Patients with Neurofibromatosis Type 1? Diagnosing MPNST in NF1 Patients

Table 2

Sensitivity, specificity, positive predictive values, and negative predictive values, along with their associated confidence intervals, for potential predictors of malignancy. These included tumor characteristics (depth relative to the fascia, and size > 5 cm in diameter), MRI findings (any necrosis, or necrosis >25% by volume), PET findings (SUVmax > 5, SUV peak > 4.5, and TLR > 3), and clinical findings (pain, enlarging, and nerve symptoms). Combinations of imaging findings (SUVpeak > or < 4.5 on PET and any or >25% necrosis on MRI) and clinical findings (1 of 3, 2 of 3, or 3 of 3 symptoms) are also included.

SensitivitySpecificityPPVNPVAccuracy

Tumor characteristicsDeep100.0 (88.0–100.0)20.7 (8.7–40.3)61.0 (47.4–73.2)100.0 (51.7–100.0)64.6
Size > 5 cm68.0 (46.4–84.2)53.5 (34.2–72.0)56.7 (37.7–74.0)65.2 (42.8–82.8)60.4

MRI findingsNecrosis (any)87.5 (60.4–97.8)76.1 (52.4–90.9)51.4 (34.7–67.8)73.7 (32.2–65.3)81.1
Necrosis (>25%)75.0 (47.4–91.7)95.2 (74.1–99.8)92.3 (62.1–100)83.3 (61.8–94.5)86.5

PET findingsSUVmax > 589.3 (70.6–97.2)73.3 (50.8–87.0)75.8 (57.4–88.2)88.0 (67.7–96.8)78.0
SUVpeak > 4.594.7 (71.9–99.7)70.8 (48.8–86.6)72.0 (50.4–87.1)94.4 (70.6–99.7)78.0
TLR > 391.7 (71.5–98.5)73.9 (51.3–88.9078.6 (58.5–91.0)89.5 (65.5–98.2)82.1
Visual score94.1 (69.2–99.7)70.8 (48.7–86.6)69.6 (47.0–86.0)94.4 (70.6–99.7)80.5

Combined PET/MRI findingsSUVpeak > 4.5 (PET) or any necrosis (MRI)95.5 (75.1–100)66.7 (47.1–82.1)67.7 (48.5–82.7)95.2 (74.1–100)78.8
SUVpeak > 4.5 (PET) and any necrosis (MRI)56.2 (30.6–79.2)93.9 (78.4–98.9)81.8 (47.8–96.8)81.6 (65.1–91.7)81.6
SUVpeak > 4.5 (PET) or necrosis >25% (MRI)72.2 (46.5–90.3)50.0 (26.0–74.0)60.1 (36.7–78.5)64 (42.8–81.2)61.1
SUVpeak > 4.5 (PET) and necrosis >25% (MRI)61.5 (31.6–86.14)100 (81.5–100)100 (59.8–100)78.3 (64.4–87.6)83.9

Clinical findingsPain85.2 (65.4–95.1)28.1 (14.4–47.0)50 (35.1–64.9)69.2 (38.9–89.6)54.2
Growth85.2 (65.4–95.1)25.0 (12.1–43.8)48.9 (34.3–63.7)66.7 (35.4–88.7)52.5
Nerve symptoms44.8 (26.9–64.0)75.0 (57.5–87.3)59.1 (36.7–78.5)62.3 (46.7–76.6)61.0

Combined clinical findings1 of 3 findings92.9 (75.0–98.8)9.68 (2.53–26.9)48.1 (34.5–62.0)60.0 (17.0–92.7)49.1
2 of 3 findings89.3 (70.6–97.2)43.8 (26.8–62.1)58.1 (42.2–72.6)82.3 (55.8–95.3)65.0
3 of 3 findings32.1 (16.6–5.24)78.1 (60.0–90.1)56.2 (30.6–79.2)43.2 (28.7–58.9)56.7