Pulmonary lymphangioleiomyomatosis (PLAM) is a rare, diffuse progressive interstitial lung disease that affects females of childbearing age and is characterized by diffuse proliferation of abnormal smooth muscle fibers predominantly developing in the lung and leading to cystic destruction. A prepubertal 13-year-old girl with PLAM associated with renal and hepatic angiomyolipomas who was treated by nephroureterectomy and thoracoscopic pleurodesis is presented. To the best of our knowledge, this is the first reported case of PLAM associated with renal and hepatic angiomyolipomas at the prepubertal age. After evaluating the clinicopathologic features of this rare entity, the authors conclude that PLAM should be considered in the differential diagnosis of cystic pulmonary pathologies in children, particularly teenagers. The most important clinical aid is to bear this rare entity in mind when a child presents with renal and/or hepatic angiomyolipomas. Thoracoscopic pleurodesis is the most effective treatment modality for recurrent pneumothoraces.

Keywords:
Pulmonary lymphangioleiomyomatosis, Child angiomyolipoma
1.
King TE, Crausman RS: Pulmonary lymphangioleiomyomatosis. Interstitial and inflammatory lung diseases; in Fishman AP, Elias JA, Fishman JA, et al (eds): Fishman’s Pulmonary Disease and Disorders. New York, McGraw-Hill, 1998, vol 1, pp 1171–1178.
2.
Johnson S: Lymphangioleiomyomatosis: Clinical features, management and basic mechanisms. Thorax 1999;54:254–264.
3.
Logginidou H, Ao X, Russo I, et al: Frequent estrogen and progesterone receptor immunoreactivity in renal angiomyolipomas from women with pulmonary lymphangioleiomyomatosis. Chest 2000;117:25–30.
4.
Hancock E, Osborne J: Lymphangioleiomyomatosis: A review of the literature. Respir Med 2002;96:1–6.
5.
Hancock E, Tomkins S, Sampson J, et al: Lymphangioleiomyomatosis and tuberose sclerosis. Respir Med 2002;96:7–13.
6.
Maziak DE, Kesten S, Rappaport DC, et al: Extrathoracic angiomyolipomas in lymphangioleiomyomatosis. Eur Respir J 1996;9:402–405.
7.
Chu SC, Horiba K, Usuki J, et al: Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis. Chest 1999;115:1041–1052.
8.
Avila NA, Kelly JA, Chu SC, et al: Lymphangioleiomyomatosis: Abdominopelvic CT and US findings. Radiology 2000;216:147–153.
9.
Hsu TH, O’Hara J, Mehta A, et al: Nephron-sparing nephrectomy for giant renal angiomyolipoma associated with lymphangioleiomyomatosis. Urology 2002;56:138–141.
10.
Rappaport DC, Weisbrod GL, Herman SJ, et al: Pulmonary lymphangiomatosis: High-resolution CT findings in four case. AJR Am J Roentgenol 1989;152:961–964.
11.
Sherrier RH, Chiles C, Roggli V: Pulmonary lymphangioleiomyomatosis. AJR Am J Roentgenol 1989;153:937–940.
12.
Templeton PA, McLoud TC, Muller NL, et al: Pulmonary lymphangioleiomyomatosis: CT and pathologic findings. J Comput Assist Tomogr 1989;13:54–57.
13.
Johnson SR, Tattersfield AE: Clinical experience of lymphangioleiomyomatosis in the UK. Thorax 2000;55:1052–1057.
14.
Boelher A, Speich R, Russi EW, et al: Lung transplantation for lymphangioleiomyomatosis. N Engl J Med 1996;335:988–1007.
15.
Stucki A, Tamm M, Gugger M, Bolliger CT: Characteristic lesions on CT scan and progressive dyspnea. Respiration 1998;65:495–498.
16.
Taylor JR, Ryu J, Colby TV, et al: Lymphangioleiomyomatosis: Clinical course in 32 patients. New Engl J Med 1990;323:1254–1260.
© 2005 S. Karger AG, Basel
2005
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.