OVERVIEW
Mesenteric lymphangiosarcoma is a malignant tumor arising from the endothelium of mesenteric lymphatic vessels. It is extremely rare and most often occurs secondary to chronic lymphedema.
Etiology
The etiology of the tumor is unclear. Currently, it is believed that trauma, surgery, radiation therapy and other lymphatic system damage, tumor invasion or metastasis to the lymphatic system, parasites such as filarial lymphatic obstruction and congenital developmental disorders causing lymphatic reflux obstruction, etc., can trigger chronic lymphedema. Long-term chronic lymphedema can cause tissue malnutrition, as well as long-term stimulation of lymphatic fluid stagnation and spillage, leading to excessive proliferation and malignant transformation of endothelial cells in lymphatic vessels and inducing the formation of lymphangiosarcoma.
Symptoms
Often due to chronic lymphatic reflux obstruction there is low back pain and discomfort, abdominal distension, diarrhea, nausea, vomiting, celiac ascites, hypoproteinemia and so on. Late stage of the disease can be metastasized to the lungs and bones via lymphatic and bloodstream. Abdomen can be palpable mass, hard, positive tenderness, bowel sounds are weakened. The abdominal wall and lower limbs may have different degrees of indurated edema. If secondary infection, intracapsular hemorrhage, collapse, the tumor rapidly increases in size, local pressure pain is obvious, accompanied by fever, anemia, shock and other symptoms. Weight loss is obvious in the late stage.
Examination
1. Ultrasonography and CT examination can show the echogenicity of cystic mass, which may be separated and encapsulated, with no enhancement after enhancement, thickening of intestinal wall and dilatation of intestinal tubes, which can initially clarify the morphology, location, size and relationship with surrounding tissues and organs.
2. Lymphatic radionuclide imaging and lymphangiography can further define the extent of lesion involvement.
Diagnosis
Most patients have a history of surgery, trauma or radiation therapy. Diagnosis is made on the basis of clinical manifestations and the results of ultrasound, CT, lymphatic radionuclide imaging and lymphangiography, and histopathologic examination is often needed to confirm the diagnosis.
Treatment
Surgery is the main treatment, and the tumor often shows infiltrative growth, which makes local resection difficult, and joint resection of affected mesentery, part of intestinal canal, ureter, bladder, etc. is often needed.