Pulmonary lymphangioleiomyomatosis in combination with uterine leiomyoma in a postmenopausal woman.

To the Editor: Lymphangioleiomyomatosis (LAM) is a rare lung disease of unknown etiology characterized by the nonneoplastic proliferation of smooth muscle-like cells (LAM cells) that can grow to obstruct airways, lymphatics and blood vessels within the lung parenchyma, mediastinum and elsewhere. (1) It exclusively affects women at reproductive age and may be worsened by pregnancy, suggesting that the disease progression is hormone dependent. (1,2) The most common presenting symptom is slowly progressing dyspnea and recurrent pneumothoraces. (1,2) LAM occurs in association with some extrapulmonary manifestations, including renal and hepatic angiomyolipoma, enlarged abdominal lymph nodes, lymphangiomyoma, and less commonly, ascites and chylothorax. (2,3) Even though pelvic diseases, such as endosalpingitis and endometrial stromal sarcoma, have been described in patients with LAM previously, to our knowledge the relation of LAM and uterine myometrial leiomyoma has not been well described. (3,4) There seems to be a reasonable relationship between LAM and uterine leiomyoma. Both diseases are sex specific and hormone dependent. Uterine leiomyomas develop after menarche and regress after menopause. LAM is also known as a disease of reproductive age. As is described in our case, oral contraceptive use has worsened LAM. Estrogen has also been regarded as the major promoter of the growth of uterine leiomyoma. (5) This may lead to a possible link between the two diseases.

We would like to draw internists' attention to our patient who was diagnosed with both LAM and uterine leiomyoma. She was a 53-year-old nonsmoker, postmenopausal woman who had a history of asthma, hemoptysis and pneumothorax admitted to our institution in November 2003. She had complained of an exertional dyspnea, wheezing and nonproductive cough and had been diagnosed with asthma for 6 years. The patient later stated that her symptoms became aggravated during the use of exogenous estrogen for two years after menopause. Her clinical findings did not suggest tuberosclerosis.

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On physical examination, no crackles were heard on auscultation. Pulmonary function tests were as follows: FVC, 1970 mL (72%); FEVI, 930 mL (40%); FEV1/FVC, 47%; residual volume (RV), 2780 (165%); the ratio of residual volume to total lung capacity (RV/TLC), 52%. Diffusing capacity for carbon monoxide (DLCO) was reduced as 4100 mL (55%). Her chest x-ray was normal. On high resolution computed tomographic scan (HRCT), multiple parenchymal cysts with the largest diameter of 2 cm were seen without any pathologic lymph node enlargement. An abdominopelvic ultrasonography revealed multiple uterine myomas. Since the bronchoscopic biopsies were nondiagnostic, the patient underwent open lung biopsy. Microscopic findings were consistent with LAM (Fig.). Estrogen and progesterone receptors and HMB 45 were stained positive by immunohistochemistry. She later underwent hysterectomy for menometrorrhagia. Pathologic finding of hysterectomy material confirmed the presence of uterine leiomyomas.

This case demonstrates how the symptoms of LAM may mimic asthma. We would like to emphasize that LAM and uterine leiomyoma, both pathologically characterized by abnormal smooth muscle cell proliferation, may be seen in the same patient.

Nurdan Kokturk, MD

Senay Demirtas, MD

Hatice Bakir, MD

Haluk Turktas, MD

Department of Pulmonary Medicine

Gazi University School of Medicine

Ankara, Turkey

Nalan Akyurek, MD

Department of Pathology

Gazi University School of Medicine

Ankara, Turkey

Cemil D. Yorgancilar, MD

Sedat Demircan, MD

Department of Thoracic Surgery

Gazi University School of Medicine

Ankara, Turkey

Nilgun Kapucuoglu, MD

Department of Pathology

Suleyman Demirel University School of Medicine

Isparta, Turkey

References

1. NHLBI Workshop Summary. Report of workshop on lymphangioleiomyomatosis. National Heart, Lung, and Blood Institute. Am J Respir Crit Care Med 1999;159:679-683.

2. Hancock E, Osborne J. Lymphangioleiomyomatosis: a review of the literature. Respir Med 2002;96:1-6.

3. Maziak DE, Kesten S, Rappaport DC, et al. Extrathoracic angiomyolipomas in lymphangioleiomyomatosis. Eur Respir J 1996;9:402-405.

4. Kogan EA, Mikhailov OI, Sekamova SM, et al. [Combination of pulmonary lymphangioleiomyomatosis with skin leiomyomatosis and uterine leiomyoma] Arkh Patol 2001;63:39-44.

5. Pitts S, Oberstein EM, Glassberg MK. Benign metastasizing leiomyoma and lymphangioleiomyomatosis: sex-specific diseases? Clin Chest Med 2004;25:343-360.