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Utility of [18F]2-Fluoro-2-Deoxyglucose-PET in Sporadic and Tuberous Sclerosis-Associated Lymphangioleiomyomatosis

Lisa R. Young, MD; David N. Franz, MD; Preeti Nagarkatte, MD; Christopher D. M. Fletcher, MD; Kathryn A. Wikenheiser-Brokamp, MD, PhD; Matthew D. Galsky, MD; Thomas C. Corbridge, MD, FCCP; Anna P. Lam, MD; Michael J. Gelfand, MD; Francis X. McCormack, MD, FCCP
Author and Funding Information

Affiliations: From the Department of Pulmonary, Critical Care, and Sleep Medicine (Drs. Young and McCormack), University of Cincinnati College of Medicine, Cincinnati, OH; the Departments of Pulmonary Medicine (Dr. Young), Neurology (Dr. Franz), Pathology & Laboratory Medicine and Pulmonary Biology (Dr. Wikenheiser-Brokamp), and Radiology (Dr. Gelfand), Cincinnati Children's Hospital Medical Center, Cincinnati, OH; Northeast Nephrology (Dr. Nagarkatte), Joliet, IL; the Department of Pathology (Dr. Fletcher), Brigham and Women's Hospital, Boston, MA; Comprehensive Cancer Centers of Nevada (Dr. Galsky), Las Vegas, NV; and the Department of Pulmonary and Critical Care (Drs. Corbridge and Lam), Northwestern University, Chicago, IL.

Correspondence to: Francis X. McCormack, MD, Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati, 231 Albert Sabin Way, ML 0564, Cincinnati, OH 45267; e-mail: Frank.McCormack@uc.edu


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.xhtml).


© 2009 American College of Chest Physicians


Chest. 2009;136(3):926-933. doi:10.1378/chest.09-0336
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Mutations in tuberous sclerosis complex (TSC) genes are associated with dysregulated mammalian target of rapamycin (mTOR)/Akt signaling and unusual neoplasms called perivascular epithelioid cell tumors (PEComas), including angiomyolipomas (AMLs) and lymphangioleiomyomatosis (LAM). Tools that quantify metabolic activity and total body burden of AML and LAM cells would be valuable for the assessment of disease progression and the response to therapy in patients with TSC and LAM. Our hypothesis was that constitutive activation of mTOR in LAM and AML cells would result in increased glucose uptake of [18F]2-fluoro-2-deoxyglucose (FDG) on PET scanning, as has been suggested by a single prior case report. After institutional review board approval, FDG-PET scanning was performed in six LAM patients. Six additional LAM patients underwent FDG-PET scanning for clinical evaluation of suspected malignancy. Pleural uptake related to prior therapy was identified in four individuals with a remote history of talc pleurodesis. Focal increased uptake was observed in a supraclavicular lymph node in a patient with Hodgkin lymphoma and in a lung nodule in a patient with a biopsy-documented primary lung adenocarcinoma. In one TSC-LAM patient with a biopsy-documented malignant uterine PEComa, robust uptake was noted in metastatic nodules in the lung but not in the LAM-involved lung parenchyma or the patient's massive abdominal lymphangioleiomyomas. No abnormal uptake was identified in the AMLs or LAM lesions in any patients. This pilot study suggests that FDG-PET scans are negative in patients with benign PEComas and therefore are not likely to be useful for estimating the burden of disease in patients with TSC or LAM, but that FDG-PET scans can be used to identify or exclude other neoplasms in these patients.

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