Lymphangioma in Patients with Pulmonary Lymphangioleiomyomatosis: Results of Sirolimus TreatmentElżbieta Radzikowska1*, Katarzyna Błasińska-Przerwa2, Paulina Skrońska3, Elżbieta Wiatr1, Tomasz Świtaj4, Agnieszka Skoczylas5 and Kazimierz Roszkowski-Śliż1
- *Corresponding Author:
- Radzikowska E
Department of Lung Diseases
National Tuberculosis and Lung Diseases Research Institute
Płocka 26, St. 01-138
Fax: + 48 224312408
E-mail: [email protected]
Received date: August 21, 2016; Accepted date: September 26, 2016; Published date: September 28, 2016
Citation: Radzikowska E, Błasińska-Przerwa K, Skrońska P, Wiatr E, Świtaj T, et al. (2016) Lymphangioma in Patients with Pulmonary Lymphangioleiomyomatosis: Results of Sirolimus Treatment. J Cancer Sci Ther 8: 233-239. doi: 10.4172/1948- 5956.1000419
Copyright: © 2016 Radzikowska E, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Objective: Lymphangioleiomyomatosis (LAM) is a rare disease caused by abnormal proliferation of smooth muscle-like cells mainly in lungs and axial lymph nodes, that belongs to the group of PEComa tumours. Chylous pleural and abdominal effusions, lymhangioleiomiomas, angiomyolipomas are noticed in disease course. Sirolimus has been approved for LAM treatment, and also it has been reported to decrease the size of angiomyolipomas, lymphangiooedma, pleural and peritoneal chylous effusion. The aim of the study was to assess the significance of sirolimus therapy in patients with LAM and lymphangioma in retroperitoneal space. Method: Fourteen women with confirmed diagnosis of LAM (13 with sporadic LAM, and one TSC/LAM) and presence of lymphangioma in abdominal cavity were retrospectively reviewed. Seven patients displayed chyloperitoneum and chylothorax. Mean time of sirolimus exposure was 19 ± 12.99 months. Results: After ten months of treatment a complete regression of chylothorax and chyloperitoneum occurred in 13 patients, and partial regression occurred in one patient. Treatment resulted in decrease of lymphangioma volume (1603.85 ± 2437.56 cm3 vs. 198.01 ± 315.43 cm3; p=0.00026), stabilisation of pulmonary function parameters such as (FVC, FEV1, TLC, DLCO), improvement of distance in 6MWT (514.46 ± 101.72 m vs. 526.79 ± 139.33 m; p=0.002), blood oxygenation (68.93 ± 13.6 mmHg vs. 78.23 ± 11.14 mmHg; p=0.04), and decrease of serum VEGF-D concentration (2591 ± 1085 pg/mL vs. 1274 ± 708 pg/mL; p=0.003). Conclusion: Sirolimus has proved to be an effective treatment of lymphangioma, chylous pleural and peritoneal effusions in LAM patients.