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Eur Respir J 2008; 32:522-523
Copyright ©ERS Journals Ltd 2008

Silence of the LAM

C. S. H. Ng, S. Wan, I. Y. P. Wan and M. J. Underwood

The Chinese University of Hong Kong, Dept of Surgery, Prince of Wales Hospital, Shatin, NT, Hong Kong, China.

To the Editors:

We read with interest the article by Warth et al. 1 on mediastinal angiomyolipoma and tuberous sclerosis. Apart from mediastinal angiomyolipoma, lymphangioleiomyomatosis (LAM) can occur as part of the genetic condition tuberous sclerosis 2. Since the first report by Cornog and Eterline 3, LAM has been increasingly studied for the pathological processes involved in atypical smooth muscle LAM cell proliferation. However, it has been difficult to gain complete understanding of the natural history of LAM due to its rarity. LAM tends to progress slowly and ultimately leads to respiratory failure. Nevertheless, the clinical course of patients with LAM can vary, with 10 yr survival between 10–60% 2. Some patients survive for 20 yrs following presentation 2. LAM may remain silent until spontaneous pneumothorax occurs, which is the most frequently presenting symptom 4. The indications for pleurodesis in this condition are not well established. In our experience, surgical pleurodesis is usually recommended for patients with recurrent or spontaneous bilateral pneumothoraces, but higher rates of recurrence can be expected for this group of patients 4. In addition, pleural symphysis following surgical pleurodesis may make future lung transplantation impossible. Multiple diffuse blebs may be seen intraoperatively (fig. 1Go), alerting the clinician towards this condition.


Figure 1
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  		Fig. 1— Intraoperative video-assisted thoracic surgery view of lymphangioleiomyomatosis on the surface of lung.

 
Classically, a computed tomography (CT) scan shows multiple thin-walled cysts distributed throughout both lungs, although unilateral lung involvement has been described 5. A CT scan can also help to detect other unsuspecting pathology 6. Pathological examination is required for confirmation of LAM, which can be obtained by transbronchial, percutaneous or surgical biopsy. We advocate video-assisted thoracic surgery biopsy with excision of any bleb and culprit lesion, particularly in the case of associated pneumothorax. The lung is characterised by cystic changes associated with proliferation of atypical smooth muscle cells (LAM cells), which can involve bronchioles, vessels and airspaces explaining, in part, the occurrence of pneumothorax, chylothorax and haemoptysis in these patients. (fig. 2Go) Positive HMB-45 immunostaining and the presence of oestrogen and progesterone receptors are also characteristics of LAM. Despite reports of various therapeutic regimens, none offer a consistently effective response. Corticosteroids and cytotoxic agents usually provide little benefit. Medical hormonal therapy (progesterone, tamoxifen, luteinising hormone-releasing hormone agonist) and surgical hormonal therapy (oophorectomy, ovarian radiofrequency ablation) have been used with variable responses 7.


Figure 2
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  		Fig. 2— Histopathology section showing blood-filled cystic spaces with fascicular spindle cells (lymphangioleiomyomatosis cells) and vascular congestion. Interstitial proliferation (vascular and bronchial) and sub-pleural proliferation is also seen.

 
For end-stage lymphangioleiomyomatosis, lung transplantation can be an effective treatment option, but recurrences in the transplanted lung may occur following lymphangioleiomyomatosis cell migration 7.

Statement of interest

None declared.

REFERENCES

  1. Warth A, Herpel E, Schmähl A, et al. Mediastinal angiomyolipomas in a male patient affected by tuberous sclerosis. Eur Respir J 2008;31:678–680.[Abstract/Free Full Text]
  2. Johnson SR, Whale CI, Hubbard RB, Lewis SA, Tattersfield AE. Survival and disease progression in UK patients with lymphangioleiomyomatosis. Thorax 2004; 59; 800–803.
  3. Cornog JL Jr, Enterline HT. Lymphangiomyoma, a benign lesion of chyliferous lymphatics synonymous with lymphangiopericytoma. Cancer 1966;19:1909–1930.[CrossRef][Web of Science][Medline] [Order article via Infotrieve]
  4. Taylor JR, Ryu J, Colby TV, Raffin TA. Lymphangioleiomyomatosis: clinical course in 32 patients. N Engl J Med 1990;323:1254–1260.[Web of Science][Medline] [Order article via Infotrieve]
  5. Avila NA, Chen CC, Chu SC, et al. Pulmonary lymphangioleiomyomatosis: correlation of ventilation-perfusion scintigraphy, chest radiography, and CT with pulmonary function tests. Radiology 2000;214:441–446.[Abstract/Free Full Text]
  6. Ng CS, Wan S, Lee TW, Yim AP. Cystic lesions of the lung: a forgotten menace. Eur Respir J 2005;26:748–749.[Free Full Text]
  7. Taveira-Da Silva AM, Stylianou MP, Hedin CJ, Hathaway O, Moss J. Decline in lung function in patients with lymphangioleiomyomatosis treated with or without progesterone. Chest 2004;126:1867–1874.[CrossRef][Web of Science][Medline] [Order article via Infotrieve]




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