OVERVIEW
OVERVIEW
Lymphangioleiomyosarcoma is a systemic disease of atypical proliferation of smooth muscle of undetermined etiology resulting in progressive pulmonary cystic degeneration, systemic lymphatic systemic lesions, and lung tumors.
Insured or not
Yes
Department
Medical Oncology, Respiratory Medicine
Clinical symptoms
Dyspnea, fatigue,, cough, etc.
Hazard
Can cause respiratory failure, leading to death.
Examination
Lung function test, X-ray film, CT, etc.
Diagnosis
Diagnosis is based on medical history, clinical manifestations such as dyspnea and celiac chest, combined with imaging tests.
Treatment principle
There is no effective treatment yet, hormone therapy or surgery can be used.
Curability
Cannot be cured.
Dietary advice
Give a light, soft, easy-to-digest diet, avoid spicy and stimulating foods, avoid smoking and alcohol.
Etiology
Epidemiology
The incidence of the disease is almost exclusively in women of childbearing age.
Etiology
The cause is unknown and may be related to abnormal cellular enzyme function and estrogen levels.
Symptoms and Diagnosis
Typical symptoms
1. slowly progressive dyspnea. 2. recurrent, which may be unilateral or bilateral. 3. dry cough, and less commonly chest pain, celiac leakage from the chest, blood in the sputum, and wheezing. 4. extrapulmonary symptoms include, celiac pericardial effusion, celiac ascites.
Other symptoms
Uterine tumors can be found in some patients or, pulmonary function changes: often obstructive ventilation dysfunction and diffusion dysfunction.
Diagnostic basis
Clinical recurrence of pneumothorax or celiac chest, chronic progressive dyspnea, may have a small amount of hemoptysis. Examination reveals hypoxemia, obstructive or mixed ventilation dysfunction, and diffuse cystic changes in both lungs on imaging.
Treatment
Treatment guidelines
Relief of symptoms by hormonal therapy, removal of ovaries, lung transplantation, etc.
Surgery
Oophorectomy combined with progesterone therapy is a relatively effective treatment. Lung transplantation may be considered if this method of treatment is ineffective or if lung function is severely impaired.
Prognosis
The prognosis is poor, with most patients developing progressive pulmonary dysfunction 1 to 2 years after diagnosis and eventually dying of respiratory failure.
Nursing care
Daily care
1. pay attention to rest, regular life, ensure enough sleep, avoid exertion. 2. keep indoor ventilation, avoid entering crowded public places to prevent respiratory infections. 3. do a good job of psychological care, maintain an optimistic state of mind, avoid emotional excitement. 4. follow the doctor’s instructions, take medication on time, and consult a doctor when discomfort occurs. 5. follow the doctor’s instructions, take medication on time, and consult a doctor when discomfort occurs. 6. take care of the patient’s health.
Diet
Give light, soft, easy to digest diet, avoid spicy stimulating food, avoid smoking and alcohol.