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Research ArticlePediatrics

Growth Curves of Subependymal Giant Cell Tumors in Tuberous Sclerosis Complex

J.R. Barnett, J.H. Freedman, H. Zheng, E.A. Thiele and P. Caruso
American Journal of Neuroradiology October 2021, 42 (10) 1891-1897; DOI: https://doi.org/10.3174/ajnr.A7231
J.R. Barnett
aFrom the Carol and James Herscot Center for Tuberous Sclerosis Complex (J.R.B., J.H.F., E.A.T.), Massachusetts General Hospital, Boston, Massachusetts
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  • ORCID record for J.R. Barnett
J.H. Freedman
aFrom the Carol and James Herscot Center for Tuberous Sclerosis Complex (J.R.B., J.H.F., E.A.T.), Massachusetts General Hospital, Boston, Massachusetts
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H. Zheng
bBiostatistics Center (H.Z.), Massachusetts General Hospital, Boston, Massachusetts
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E.A. Thiele
aFrom the Carol and James Herscot Center for Tuberous Sclerosis Complex (J.R.B., J.H.F., E.A.T.), Massachusetts General Hospital, Boston, Massachusetts
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P. Caruso
cPediatric Neuroimaging (P.C.), Lenox Hill Radiology & Medical Imaging Associates, New York
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  • FIG 1.
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    FIG 1.

    Serial images of patient 11's resected lesion located in the region of the right foramen of Monro (A–C) and patient 2's indeterminate lesion located in the region of left foramen of Monro (D–F). Patient 11's lesion was resected at 16 years of age. The arrows point to the lesion of interest. Cubic measurements are as follows: (AP × TV × SI), A, 9.6 mm × 8.8 mm × 10.6 mm; B, 16.1 mm × 15.9 mm × 16.3 mm; C, 21.5 mm × 16.5 mm × 19.3 mm; D, 10.4 mm × 9.2 mm × 11.2 mm; E, 10.5 mm × 9.5 mm × 11.7 mm; F, 10.4 mm × 9.5 mm × 11.9 mm.

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    FIG 2.

    A, Volume of indeterminate lesions by age at scan. Each line indicates 1 indeterminate nodule. The marked line indicates that an indeterminate nodule in patient 23 shows rapid growth. There is currently no evidence of obstructive hydrocephalus. The patient is being closely monitored. B, Volume of resected lesions by age at scan. The marked line indicates patient 4's resected lesion. Patient 4's resected lesion had an average growth of 531.3 mm3/year between 6.2 and 7.3 years of age and a period of decelerated growth between 7.3 and 8.7 and 8.7–10.4 years of age, in which the average growth rates were 204.7 mm3/year and 118.9 mm3/year, respectively. At 12.7 years of age, the patient underwent surgical resection for obstructive hydrocephalus.

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    FIG 3.

    A, Median and IQR of lesion volumes of small lesions (small), indeterminate nodules (inde), and resected lesions (resected). The median volume and IQR at the earliest scan: small lesions, 25.22 mm3 (IQR = 50.8 mm3, n = 126); indeterminate lesions, 251.9 mm3 (IQR = 319.4 mm3, n = 27), and resected lesions, 245.6 mm3 (IQR = 486.5 mm3, n = 10). The median volume and IQR at most the recent scan: small lesions, 33.18 mm3 (IQR = 61.8 mm3, n = 126); indeterminate lesions, 615.6 mm3 (IQR = 581.1 mm3, n = 27); and resected lesions, 2283.0 mm3 (IQR = 3162.0 mm3; n = 10). Indeterminate lesion volume and resected lesion volume are compared at earliest scan and most recent scan using the Mann-Whitney U test. There is no significant difference in volume at the earliest scan (P = 1.0); however, there is a significant difference at the most recent scan. The asterisk indicates P < .001. B, Median lesion volume of each lesion type by age. Each point is the median volume of ≥2 lesions at a given age. NS indicates not significant.

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    FIG 4.

    A, Median growth velocity between scans for each lesion type: small lesions, 0.45 mm3/year (IQR = 3.2 mm3/year, n = 789); indeterminate lesions, 21.95 mm3/year (IQR = 71.6 mm3/year; n = 167); resected lesions, 216.1 mm3/year (IQR = 487.7 mm3/year; n = 38). A Mann-Whitney U test with a Bonferroni correction was used to compare the velocity of growth between lesion types. The triple asterisks indicate a significant difference in velocity comparing resected lesions and indeterminate lesions (P <.001), resected lesions and small lesions (P <.001), and indeterminate lesions and small lesions (P <.001). B, Median acceleration of growth of all lesion types: small lesions, 0.00 mm3/year2 (IQR = 3.4 mm3/year2, n = 673); indeterminate lesions, −0.18 mm3/year2 (IQR = 70.63 mm3/year2, n = 141); resected lesions, 77.01 mm3/year2 (IQR = 257.7 mm3/year2; n = 30). A Mann-Whitney U test with a Bonferroni correction was used to compare the acceleration of growth among lesion types. The double asterisks indicate significant differences between resected lesions and indeterminate lesions (P = .01) and resected lesions and small lesions (P = .004). There is not a significant difference in the rate of acceleration between indeterminate lesions and small lesions (P = 1.0).

Tables

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    Table 1:

    Median growth velocitiesa

    0–5 Years of Age6–10 Years of Age11–15 Years of Age16–20 Years of Age
    Resected lesions333.2 mm3/year
    58.1–608.23b
    n = 2
    351. mm3/year
    IQR = 344.5
    n = 4
    460.3 mm3/year
    IQR = 647.7
    n = 5
    292.0 mm3/year
    NA
    n = 1
    Indeterminate lesions32.5 mm3/year
    IQR = 58.1 mm3/year
    n = 11
    18.8 mm3/year
    IQR = 37.0 mm3/year
    n = 16
    15.8 mm3/year
    IQR = 43.9 mm3/year
    n = 13
    15.7 mm3/year
    IQR = 103.6 mm3/year
    n = 13
    Small lesions0.6 mm3/year
    IQR = 4.2 mm3/year
    n = 42
    0.3 mm3/year
    IQR = 1.6 mm3/year
    n = 59
    0.3 mm3/year
    IQR = 3.0 mm3/year
    n = 39
    0.6 mm3/year
    IQR = 3.1 mm3/year
    n = 32
    • Note:—NA indicates not applicable.

    • ↵a Listed are the median growth and IQR for each lesion type for each age period.

    • ↵b Range and not IQR are reported for resected lesions at 0–5 years of age.

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    Table 2:

    Growth velocity comparisonsa

    0-5 Years of Age6-10 Years of Age11-15 Years of Age16-20 Years of Age
    Resected vs indeP = .62P = .007bP = .006bNA
    Resected vs smallP = .01bP < .001bP < .001bNA
    Resected vs inde + smallP = .03bP < .001bP < .001bNA
    Inde vs smallP < .001bP < .001bP = .053P = .034b
    • Note:—Inde indicates indeterminate lesions; small, small lesions; resected, resected lesions; NA, not applicable.

    • ↵a P-values calculated using Mann-Whitney U test and Bonferroni correction.

    • ↵b Statistical significance.

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    Table 3:

    Percentage of lesions with growth rates > 4.2 mm3/yeara

    Baseline to Most Recent Scan0–5 Years of Age6–10 Years of Age11–15 Years of Age16–20 Years of Age
    Small lesions14%21%12%10%16%
    Indeterminate lesions96%100%81%62%69%
    Resected lesions100%100%b100%100%100%c
    All lesions34%41%35%30%33%
    • ↵a 4.2 mm3 was chosen to reflect an increase in growth of 2 mm in the AP, TV, and SI dimensions using an ellipsoid volume formula because a 2-mm increase would be an appreciable measurement on MR imaging.

    • ↵b Of the 3 patients with symptomatic SGCTs in this age group, only 2/3 MRIs were considered diagnostic.

    • ↵c Only 1 lesion was available for measurement.

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American Journal of Neuroradiology: 42 (10)
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Cite this article
J.R. Barnett, J.H. Freedman, H. Zheng, E.A. Thiele, P. Caruso
Growth Curves of Subependymal Giant Cell Tumors in Tuberous Sclerosis Complex
American Journal of Neuroradiology Oct 2021, 42 (10) 1891-1897; DOI: 10.3174/ajnr.A7231

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Growth Curves of Subependymal Giant Cell Tumors in Tuberous Sclerosis Complex
J.R. Barnett, J.H. Freedman, H. Zheng, E.A. Thiele, P. Caruso
American Journal of Neuroradiology Oct 2021, 42 (10) 1891-1897; DOI: 10.3174/ajnr.A7231
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