lymphatic system
Tumours
Lymphangioma, lymphangiosarcoma and lymphomas: tumours of the lymphatic system
Lymphangiomas, lymphangiosarcoma and lymphomas are three different types of cancer of the lymphatic system.
The former is rare and only occur in the first two years of children’s lives, in the form of swellings under the skin made up of dilated lymphatic vessels. Lymphangiosarcoma, on the other hand, is associated with chronic lymphatic stasis. It is extremely malignant and more particularly it is secondary to mastectomy, the total removal of the breast for cancer, which can occur even after many years, as a complication of secondary lymphedema (Stewart-Treves syndrome) Tumours affecting lymphocytes (cells found in the lymph nodes, spleen, bone marrow, and thymus) are lymphomas, which come in two categories:
Hodgkin’s lymphoma
(it is rare and in most cases, it presents with an increasein the volume of the cervical lymph nodes, weight loss, itching, fever and night sweats);
Non-Hodgkin’s lymphoma
(there are several types and arriving at a unique classification is not easy).
The causes of tumours in the lymphatic system
The nature of Lymphangiomas is controversial and, consequently, the search for the causes has not brought definitive results. However, its development has been ascertained following a blockage of the lymphatic system in the phases of growth of the fetus (but the symptomsgenerally become visible only after the birth of the newborn).
The pathogenesis of Lymphangiosarcoma and Lymphoma is also not certain. The first is related to lymphedema in the legs or arms for a long time, caused by problems in the lymphatic system; while it has been noted that non-Hodgkin’s lymphoma occurs as a result of the body’s production of an excessive amount of abnormal lymphocytes which, due to acquired genetic mutations, replicate and accumulate in the organsof the lymphatic system. As for Hodgkin’s lymphoma, however, it is impossible to recognize specific risk factors.
Treatment of tumours of the lymphatic system
For lymphangiomas, three different therapeutic strategies are traditionally recognized: clinical observation (for infants without respiratory symptoms or important blemishes), surgery (for infants with severe respiratory symptoms, or facing micro-cystic lesions, of doubtful histological nature, or resistant to non-surgical therapies) and non-surgical interventional treatments (sclerotherapy, thermoablation and laser therapy).
Depending on the severity class of lymphangiosarcoma, different treatments are available. The therapeutic possibilities, although conditioned by the high degree of malignancy of this tumor, range from surgery (for forms in localized phase), to radiotherapy (for forms in infiltrating and metastasizing evolution), up to chemotherapy (to reduce the extent of metastases and the widespread systematic recurrence of metastases).
The treatment of lymphomas combines chemotherapy, immunotherapy, radiation therapy and, when necessary, also stem cell transplantation. The treatment is carried out by the specialist ono-hematologist, taking into consideration the type of lymphoma, the age and health conditions of the patient and the possible comorbidity, i.e. the presence of other concomitant diseases.
