PMC8524238_01
Computed tomography images of multifocal micronodular pneumocyte hyperplasia (MMPH) and renal angiomyolipoma (AML). (A) (B) MMPH images at the age of 18 and before initiation of treatment with everolimus. (C) AML image at the age of 28 and before initiation of everolimus. (D) (E) MMPH and (F) AML at the age of 28, 6 months after initiation of treatment with everolimus. (G) (H) MMPH and (I) AML at the age of 30, 2 years after initiation of treatment with everolimus. Representative lesions were shown with arrow heads (MMPH) and arrows (AML).
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An 18-year-old woman, known to have sporadic TSC, was referred to out department because of multiple ground-glass opacities revealed following CT of her chest (Fig. 1A and B). At the age of 28 years, given progressing AML (Fig. 1C) and intractable epilepsy, the patient started receiving a mTOR inhibitor, 5 mg/day of everolimus. CT, performed 6 months after starting everolimus, revealed that the lung lesions were less intense than they had been on previous evaluation (Fig. 1D and E). Everolimus also shrunk the AML lesions (Fig. 1F) and facial angiofibroma.
The histological findings of transbronchial lung biopsy representing multifocal micronodular pneumocyte hyperplasia (hematoxylin and eosin staining, original maginification x 20). A demarcated nodular lesion comprised of alveolar lining of enlarged cuboidal cells. These cells have abundant pale to eosinophilic cytoplasms and round to oval-shaped nuclei, reminiscent of type II pneumocyte proliferation.
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Pathological findings of a transbronchial lung biopsy from the right middle lobe revealed enlarged cuboidal cells lining the collapsed alveolar septa (Fig. 2).