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Published ahead of print on April 12, 2007, doi:10.1164/rccm.200610-1408CR
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American Journal of Respiratory and Critical Care Medicine Vol 176. pp. 96-98, (2007)
© 2007 American Thoracic Society
doi: 10.1164/rccm.200610-1408CR

Case Report

Pulmonary Lymphangioleiomyomatosis in a Karyotypically Normal Man without Tuberous Sclerosis Complex

Mario Schiavina1, Valerio Di Scioscio2, Paola Contini1, Alberto Cavazza3, Andrea Fabiani1, Marco Barberis4, Alessandro Bini5, Annalisa Altimari6, Robin M. T. Cooke6, Walter F. Grigioni6 and Antonia D'Errico-Grigioni6

1 Unit of Lung Physiopathology, and 2 Unit of Radiology, Azienda Ospedaliera S. Orsola-Malpighi and University of Bologna, Bologna, Italy; 3 Unit of Pathologic Anatomy, S. Maria Nuova Hospital, Reggio Emilia, Italy; 4 Department of Genetics, Biology, and Biochemistry, University of Turin, Turin, Italy; and 5 Institute of Thoracic Surgery, and 6 Unit of Pathologic Anatomy, F. Addarii Institute of Oncology, Azienda Ospedaliera S. Orsola-Malpighi and University of Bologna, Bologna, Italy

Correspondence and requests for reprints should be addressed to Antonia D'Errico-Grigioni, M.D., Istituto di Oncologia F. Addarii, Viale Ercolani 4/2, 40138 Bologna, Italy. E-mail: derrico{at}aosp.bo.it

Rationale: The three previously reported cases of conclusively documented pulmonary lymphangioleiomyomatosis (LAM) in men were associated with definite or probable tuberous sclerosis complex (TSC).

Objectives: To describe an unequivocal case of pulmonary LAM occurring in a man with no clinical or genotypic evidence of TSC.

Methods: At high-resolution computed tomography, a 37-year-old phenotypically and karyotypically normal man with left pneumothorax and massive pulmonary collapse had widespread thin-walled cysts throughout both lungs. Histological diagnosis of LAM was performed on biopsy material, and immunohistochemically confirmed with the HMB-45 monoclonal antibody.

Measurements and Main Results: Remarkably, the HMB-45–positive cells lining the cysts also showed strong reactivity for estrogen and progesterone receptor proteins. TSC was clinically excluded, and TSC1 and TSC2 germline mutations were not detected at DNA analysis.

Conclusions: This article indicates that occurrence of LAM may be possible in a chromosomally normal man unaffected by TSC. On diagnostic grounds, the possibility of LAM should be borne in mind when diffuse cystic lung disease occurs in a man, even in the absence of signs of TSC.

Key Words: smooth muscle cells • histopathology • sex distribution • rare diseases




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Proc. Am. Thorac. Soc. Am. J. Respir. Cell Mol. Biol.
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