We present a case of a 39-year-old woman with sporadic tuberous sclerosis (TSC), whose chest radiograph demonstrated bilateral diffuse nodular shadowing. A transbronchial lung biopsy specimen revealed the possibility of multiple atypical adenomatous hyperplasia (AAH), which had not been reported in TSC. Thoracoscopic lung biopsy was, therefore, performed. The specimens revealed the characteristic histological and immunohistochemical features of micronodular pneumocyte hyperplasia, which has been reported as an extremely rare pulmonary manifestation of TSC. In addition, no evidence of AAH or any other pulmonary involvements of TSC including lymphangioleiomyomatosis were detected in biopsy specimens obtained at thoracoscopy.

Keywords:
Micronodular pneumocyte hyperplasia, Atypical adenomatous hyperplasia, Tuberous sclerosis, Thoracoscopy
1.
Castro M, Shepherd CW, Gomez MR, Lie JT, Ryu JH: Pulmonary tuberous sclerosis. Chest 1995;107:189–195.
2.
Strizheva GD, Carsillo T, Kruger WD, Sullivan EJ, Ryu JH, Henske EP: The spectrum of mutations in TSC1 and TSC2 in women with tuberous sclerosis and lymphangiomyomatosis. Am J Respir Crit Care Med 2001;163:253–258.
3.
Muir TE, Leslie KO, Popper H, Kitaichi M, Gagne E, Emelin JK, Vinters HV, Colby TV: Micronodular pneumocyte hyperplasia. Am J Surg Pathol 1998;22:465–472.
4.
Popper HH, Juettner-Smolle FM, Pongratz MG: Micronodular hyperplasia of type II pneumocytes – A new lung lesion associated with tuberous sclerosis. Histopathology 1991;18:347–354.
5.
Wu K, Tazelaar HD: Pulmonary angiomyolipoma and multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis. Hum Pathol 1999;30:1266–1268.
6.
Yamanaka A, Kitaichi M, Fujimoto T, Hirai T, Hori H, Konishi F: Multifocal micronodular pneumocyte hyperplasia in a postmenopausal woman with tuberous sclerosis. Virchows Arch 2000;436:389–392.
7.
Corrin B, Liebow AA, Friedman PJ: Pulmonary lymphangioleiomyomatosis. Am J Pathol 1975;79:348–382.
8.
Flieder DB, Travis WD: Clear cell ‘sugar’ tumor of the lung: Association with lymphangioleiomyomatosis and multifocal micronodular pneumocyte hyperplasia in a patient with tuberous sclerosis. Am J Surg Pathol 1997;21:1242–1247.
9.
King TE Jr, Crausman RS: Pulmonary lymphangioleiomyomatosis; in Fishman AP (ed): Fishman’s Pulmonary Diseases and Disorders. New York, McGraw-Hill, 1982, pp 1171–1179.
10.
Logginidou H, Ao X, Russo I, Henske EP: Frequent estrogen and progesterone receptor immunoreactivity in renal angiomyolipomas from women with pulmonary lymphangioleiomyomatosis. Chest 2000;117:25–30.
© 2002 S. Karger AG, Basel
2002
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