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Published ahead of print on April 12, 2007, doi:10.1164/rccm.200610-1408CR

Am. J. Respir. Crit. Care Med., Volume 176, Number 1, July 2007, 96-98

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Submitted on October 3, 2006
Accepted on April 11, 2007

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 Cooke6, Walter F Grigioni6, and Antonia D'Errico-Grigioni6*

1 Unit of Lung Physiopathology, Azienda Ospedaliera S. Orsola-Malpighi and University of Bologna, Bologna, Italy, 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, 5 Institute of Thoracic Surgery, Azienda Ospedaliera S. Orsola-Malpighi and University of Bologna, Bologna, Italy, 6 Unit of Pathologic Anatomy, F Addarii Institute of Oncology, Azienda Ospedaliera S. Orsola-Malpighi and University of Bologna, Bologna, Italy

* To whom correspondence should be addressed. 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: Describe an unequivocal case of pulmonary LAM occurring in a man with no clinical or genotypic evidence of TSC. Case report: At high-resolution CT, 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. 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. Conclusion: This report 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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