Krebswissenschaft und -therapie

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.

Purpose: The aim of this clinical study was to compare breast-conserving therapy (BCT) and radical mastectomy (RM) in the treatment of early breast cancer. Materials and methods: From March 1986 to the end of 1989, 117 women with a breast tumor of up to 2 cm in size were included in this study; 66 women had a quadrantectomy, while 51 had a radical mastectomy. Full axillary dissection was performed in all patients, while adjuvant chemotherapy with cyclophosphamide, methotrexate and fluorouracil was conducted in node-positive patients. Radiation to the remaining breast parenchyma consisted of external-beam therapy, with or without regional irradiation, without boost to the tumor bed. Results: The follow-up of patients in the two treatment groups was limited to 240 months. The mean period of observation (SD±) was 201.34 months (54.12 ± 226.5) for the patients with breast-conserving therapy (BCT) and 195.84 months (35.1 ± 226.5) for the patients with radical mastectomy. No significant difference in overall survival (p=0.5410), distant metastases (Chi-Square tests, p=0.117) and contralateral breast cancers (Chi- Square Tests, p=0.809) between the two groups was established. The average survival for patients in both study groups was also statistically insignificant (211.4 months with 95% Confidence Interval 197.9*224.9 in the quadrantectomy group and 204.0 months with 95% Confidence Interval 188.0*219.9 in the radical mastectomy group, Log Rank, p=0.342). Conclusion: Our data support the view that breast-conserving surgery and irradiation is equivalent to mastectomy in terms of long-term survival for patients with stage I breast cancer.