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. 2010 Mar;254(3):851-7.
doi: 10.1148/radiol.09090227.

CT of sclerotic bone lesions: imaging features differentiating tuberous sclerosis complex with lymphangioleiomyomatosis from sporadic lymphangioleiomymatosis

Nilo A Avila  1 , Andrew J Dwyer, Antoinette Rabel, Thomas Darling, Chien-Hui Hong, Joel Moss
Affiliations

CT of sclerotic bone lesions: imaging features differentiating tuberous sclerosis complex with lymphangioleiomyomatosis from sporadic lymphangioleiomymatosis

Nilo A Avila et al. Radiology. 2010 Mar.

Abstract

Purpose: To determine if sclerotic bone lesions evident at body computed tomography (CT) are of value as a diagnostic criterion of tuberous sclerosis complex (TSC) and in the differentiation of TSC with lymphangioleiomyomatosis (LAM) from sporadic LAM.

Materials and methods: Informed consent was signed by all patients in this HIPAA-compliant study approved by the institutional review board. Retrospective analysis was performed of the body CT studies of 472 patients: 365 with sporadic LAM, 82 with TSC/LAM, and 25 with TSC. The images were reviewed by using a picture archiving and communication system workstation with bone settings (window width, 1500 HU; window level, 300 HU) and fit-to-screen option. CT image characteristics assessed included shape, size, and distribution of sclerotic bone lesions with subsequent calculation of differences in the frequency of these lesions.

Results: Most commonly the sclerotic bone lesions were round, measured 0.3 cm (range, 0.2-3.2), and were distributed throughout the spine. The frequencies differed among the three patient groups Four or more sclerotic bone lesions were detected in all 25 (100%) of those with TSC, with a sensitivity of .89 (72 of 82) and specificity of .97 (355 of 367) in the differentiation of sporadic LAM from TSC/LAM (P < .01).

Conclusion: The number of sclerotic bone lesions at body CT is of potential value in the diagnosis of TSC and in the differentiation of patients with sporadic LAM from those with TSC/LAM. (c) RSNA, 2010.

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Figures

Figure 1:
Figure 1:
CT scan in a 47-year-old woman with TSC/LAM. Axial section at the T4 level viewed with bone windows demonstrates multiple sclerotic round bone lesions (arrows) in the vertebral body, right transverse process, and left rib.
Figure 2:
Figure 2:
CT scan in a 38-year-old woman with LAM. Axial section through the pelvis viewed with bone windows shows single flame-shaped lesion in the right iliac bone (arrow).
Figure 3:
Figure 3:
CT scan in a 43-year-old woman with TSC/LAM. Axial section at the T8 level demonstrates rectangular-shaped SBLs conforming to the shape of the bones but not expanding them in the pedicle (white arrow), transverse process (arrowhead), and right rib (black arrow). Note three coalescing round lesions in the anterior aspect of the vertebral body.
Figure 4:
Figure 4:
Graph depicts cumulative probabilities of the numbers of SBLs in 364 patients with LAM, 82 patients with TSC/LAM, and 25 patients with TSC.
Figure 5:
Figure 5:
Empirical ROC curve demonstrates data of patients with TSC/LAM from those with sporadic LAM in terms of the number of SBLs seen at CT of the chest, abdomen, and pelvis. Area under the curve of 0.96 indicates high discriminating ability. The criterion of four or more SBLs in a patient (arrow) has sensitivity of 0.89 and specificity of 0.97 for TSC/LAM versus LAM. FPR = false-positive rate, TPR = true-positive rate.
Figure 6:
Figure 6:
Empirical ROC curve demonstrates data of patients with TSC from those with sporadic LAM in terms of number of SBLs seen at CT of the chest, abdomen, and pelvis. Area under the curve of 0.99 indicates high discriminating ability. The criterion of four or more SBLs in a patient (arrow) has sensitivity of 1.0 and specificity of 0.97 for TSC versus LAM. FPR = false-positive rate, TPR = true-positive rate.
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References

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