Octreotide Acetate Uptake In Tuberous Sclerosis Complex With Lymphangioleiomyomatosis

摘要

Lymphangioleiomyomatosis (LAM) is a rare progressive lung disease of reproductive age women. It occurs as an isolated disorder (sporadic LAM) or in an association with tuberous sclerosis complex (TSC). Coexisting renal angiomyolipomas occur in TSC and LAM, and there is an association between TSC and neuroendocrine tumors. Commonly used therapies aimed at decreasing endogenous estrogen in LAM have not been verified by randomized trial. Case Report An octreoScan was performed to detect tumors/tissue containing somatostatin receptors (such as neuroendocrine tumors) in a 29 year-old woman with TSC, end-stage renal disease secondary to angiomyolipomas (figure 1), LAM lung (figure 2), and “potato-like” cerebral lesions. Whole body images were obtained 24 hours post IV injection of In-111 pentatreotide 5.5 mCi demonstrating increased uptake of the radio pharmaceutical diffusely in the lung and kidney (figure 3).