Current treatment for lymphangioleiomyomatosis

  I. Injection therapy Previously, the method of local injection of sclerotherapy for lymphangioleiomyomatosis had no obvious effect.  In recent years, the application of anti-tumor drugs for local injection therapy has achieved more satisfactory results, with complete regression and significant reduction of up to 70%. It may be that the treatment purpose is achieved by the dual action of inhibiting the growth of lymphatic vascular endothelial cells and chemical stimulants to fibrosis of the mesenchyme. Histologically, it is less effective in the more mesenchymal types such as simple and cavernous lymphangioleiomas and more effective in the mesenchymal and types such as cystic hydatid tumors. This has been shown to be true in practice.  There is a transient local swelling within 1 to 2 weeks after injection, then gradually shrinking and hardening, but there are certain side effects, with fever of about 38℃ on the same day or the next day, and occasional diarrhea and vomiting. The most serious complication is pulmonary fibrosis.  The side effects of applying OK-432 solution into the tumor cavity are local inflammatory reaction with swelling for 3 to 5 days and transient fever.  Since the injection therapy is easier and less destructive to the tissues, serious complications that may occur due to surgery can be avoided. However, it is noteworthy that the child is small and the child’s fear is strong, which affects the child’s psychology.  Surgical treatment is still the main treatment for lymphangioleiomyoma.  Surgery is not recommended when lymphangioleiomyoma is complicated by infection, and the infection must be controlled first. Intracapsular hemorrhage is not a contraindication to surgery.  The actual extent of cystic lymphangiectasia often exceeds the original estimation, and it is often difficult to completely remove the lesion during surgery. The surgery requires careful dissection of important nerves, blood vessels and other structures in the neck to prevent facial nerve palsy and damage to the lingual, recurrent laryngeal and phrenic nerves that may cause respiratory distress and hoarseness. For the remaining cyst wall, 0.5% tincture of iodine can be applied to destroy the endothelial cells to prevent recurrence.