Patients with lymphangioleiomyomatosis and lymphatic tumors, ascites or pleural effusions who are symptomatic will receive subcutaneous injections of octreotide starting at a dose of 100 micrograms per day. Doses will be gradually increased to a maximum of 800 micrograms per day, two months after enrollment, if there is no response to lower doses.

Patients with lymphangioleiomyomatosis and lymphatic tumors causing chylous effusions and abdominal pain

Octreotide

Sex: Female, Male

Region of Enrollment

Octreotide treatment will be considered successful if the patient receiving treatment for six months shows a reduction in total tumor mass/ fluid collection or reaccumulation of at least 20%.

Number of Participants With a Reduction in Total Tumor Volume of at Least 20%.

Octreotide treatment will be considered successful if the reported pain/symptom score is reduced by at least 2 levels at termination of treatment.
A simple visual numeric distress scale ranging from zero to 10 will be employed to rate the severity of individual symptoms. The best score is zero, which means absence of symptoms and the maximal is 10, meaning that the symptoms are very severe.

Number of Participants With a Reduction of Pain/Symptoms as Measured by a Simple Numeric Symptom Distress Scale (NDS) to Rate the Severity of Individual Symptoms.

One or more abnormality of the following liver function tests:
Alkaline phosphatase above 116 i.u.
SGPT above 41 i.u.
SGOT from 34 i.u.
Total bilirubin above 1.0 mg/dl

Number of Participants With Liver Function Abnormalities

Overall Study

Joel Moss

Lymphangioleiomyomatosis

Lymphangiomyomas

Pleural Effusions

Ascites

Treatment with octreotide starts at a dose of 50 micrograms(ug) twice a day which is increased to 100 ug twice a day after two weeks and to 200 ug twice a day two weeks later. After two months, if there is no response the dose shall be increased to 400 ug twice a day.

angiomyolipoma/ödəm

Treatment With Octreotide in Patients With Lymphangioleiomyomatosis

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