Retroperitoneal lymphangioleiomyomatosis –⁠ a case reports

Czech version

Authors: E. Lajtman ¹; Miloš Mlynček ¹; M. Matejka ¹; A. Sládeček ²; I. Langová ²
Authors‘ workplace: Gynekologicko-pôrodnícka klinika FN a Univerzita Konštantína Filozofa, Nitra, prednosta prof. MUDr. M. Mlynček, CSc. ¹; Ústav patológie FN, Nitra, primár MUDr. A. Sládeček ²
Published in: Ceska Gynekol 2013; 78(2): 206-210

Overview

Lymphangioleiomyomatosis (LAM) is a rare progressive disease affecting women of childbearing age. The disease is characterised by an abnormal proliferation of immature smooth muscle cells predominantly in the lung. It gradually leads to respiratory failure, and it frequently result in death. Extrapulmonary LAM typically presents with abdominal mass, abdominal pain and chylous ascites. In the case reports we describe two cases of premenopausal females with extrapulmonary LAM. In both cases they occur in pelvic location in the obturator fossa and around the external iliac artery. After surgical procedures patients were primary treated with progesterone. Sirolimus was second-line drugs.

Keywords:
lymphangioleiomyomatosis –⁠ chylous ascites –⁠ sirolimus

Sources

1. Blei, F. Medical Management. In Lee, B.-B., Bergan, J., Rockson, SG. Lymphedema. London: Springer-Verlag, 2011, p. 449–453.

2. Bonetti, F., Chiodera, P., Pea, M., et al. Transbronchial biopsy in lymphangioleiomyomatosis of the lung: HMB 45 for diagnosis. Am J Surg Pathol, 1993, 17, p. 1092–1102.

3. Bonetti, F., Pea, M., Martignoni, G., et al. Clear cell (Sugar) tumor of the lung is a lesion strictly related to angiomyolipoma: the concept of a family of lesion characterized by the presence of the perivascular epithelioid cells (PEC). Pathology, 1994, 26, p. 230–236.

4. Cornog, JL., Enterline, HT. Lymphangiomyoma, a benign lesion of chyliferous lymphatic synonymous with lymphangiopericytoma. Cancer, 1966, 19, p. 1909–1930.

5. Eliasson, AH., Phillips, YY., Tenholder, MF. Treatment of lymhangioleiomyomatosis. A meta-analysis. Chest, 1989, 96, p. 1352–1355.

6. Henske, E. Safety study of sirolimus and hydrochloroquine in women with lymphangioleiomyomatosis [on line]. [cit. 2012-10-01].Dostupný na WWW: http://clinicaltrialsfeeds.org/clinical-trials/show/NCT01687179>

7. Hohman, DW., Noghrehkar, D., Ratnayake, S. Lymphagio-leiomyomatosis: a review. Eur J Intern Med, 2008, 19, p. 319–324.

8. Kebria, M., Black, D., Bonelli, C., et al. Primary retroperitoneal lymphangioleiomyomatosis in a postmenopausal woman: a case report and review of the literature. Int J Gynecol Cancer, 2007, 2007, 17, p. 528–532.

9. Kelly, J., Moss, J. Lymphangioleiomyomatosis. Am J Med Sci, 2001, 321, p. 17–25.

10. Matsui, K., Tatsuguchi, A., Valencia, J., et al. Expora-pulmonary lymphangioleiomyomatosis (LAM): clinicopathologic features in 22 cases. Hum Pathol, 2000, 31, p. 1242–1248.

11. McCormack, FX., Yoshikazu, Y., Moss, J., et al. Multicenter international lymphangioleiomyomatosis efficaccy and safety of sirolimus (MILES) trial. N Engl J Med, 2011, 364, p. 1595–1606.

12. Nebeský, T., Pokojová, E., Dvořák, K. Lymfangioleio-myomatóza. Čes Radiol, 2004, 58, 1, s. 27–29.

13. Rapamune. SPC [on line]. [cit. 2012-10-01]. Dostupný na WWW:http://sukl.sk/buxus/generate_page.php?page_id=368&lie_id=34341

14. Sullivan, EJ. Lymphangioleiomyomatosis: a review. Chest, 2006, 114, 6, p 1689–1703.

15. Svendsen, TL., Viskum, K., Hansborg, N., et al. Pulmonary lymphagioleiomyomatosis: a case of progesterone receptor positive lymphangioleiomyomatosis treated with medroxyprogesterone, oophorectomy and tamoxifen. Br J Dis Chest, 1984, 78, p. 264–271.

16. Taviera-DaSilva, AM., Hathaway, O., Stylianou, M.,Moss, J. Changer in lung funkction and chylous effusion in patients with lymphangioleiomyomatosis treated with sirolimus. Ann Intern Med, 2011, 154, p. 797–805.

17. Taviera-DaSilva, AM., Moss, J. Progress in the treatment of lymfangioleiomyomatosis: From bench to bedside. Rev Port Pneumol, 2012, 18, p. 142–144.

18. Taviera-DaSilva, AM., Steagall, W., Moss, J. Lymphangio-leiomyomatosis. Cancer, Control, 2006, 13, 4, p 276–285.

19. Toshiaki, O., Takhiro, O., Kentaro, M., et al. Sirolimus ameliorated post lung transplant chylothorax in lymphangioleiomyomatosis. Ann Thorac Surg, 2008, 86, p. e7–e8.

20. Žurková, M., Kolek, V., Vašáková, M., Loštáková, V. Lymfangioleiomyomatóza –⁠ standard diagnózy, léčby a sledování [on line]. [cit. 2012-09-28]. Dostupný na WWW: http://pneumologie.cz/odborne/doporučenie-postupy.php