Letter to the Editor

Pulmonary Lymphangioleio-myomatosis in Combination with Uterine Leiomyoma in a Postmenopausal Woman

Authors: Nurdan Kokturk, MD, Senay Demirtas, MD, Hatice Bakır, MD, Haluk Turktas, MD, Nalan Akyurek, MD, Cemil D. Yorgancilar, MD, Sedat Demircan, MD, Nilgun Kapucuoglu, MD

Abstract

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.2,3Even 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.

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References

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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.