goserelin and Puerperal-Disorders

Immediately after delivery a 17-year-old Turkish primipara developed edema, supraclavicular lymphoma and pleural effusion. CT-scans showed massive abdominal and mediastinal lymphoma. Lymphangioleiomyomatosis (LAM) was diagnosed by supraclavicular and retroperitoneal biopsy and progesterone receptors were documented in the tumor. There was no evidence of pulmonary involvement. 5 months' treatment with the LHRH-analogue goserelin showed neither clinical improvement nor regression of LAM. Irradiation of the ovaries and the abdomen with 30.2 Gy was followed by amenorrhea without immediate tumor remission. A follow-up examination 1 year later revealed clinical improvement and CT scans showed 50% abdominal and mediastinal regression of LAM. 5 1/2 years after diagnosis the asymptomatic patient is still in partial remission. Successful treatment of LAM is rare. We describe a case in which a major tumour reduction was documented by CT scanning.

Topics: Adolescent; Combined Modality Therapy; Female; Goserelin; Humans; Lymphangioleiomyomatosis; Pregnancy; Puerperal Disorders; Radiotherapy Dosage; Tomography, X-Ray Computed

Lymphangioleiomyomatosis is a rare tumour of the lymphatic system, affecting mostly women of childbearing age. Due to its rare occurrence, diagnosis is mostly made after thorough examination therefore time for an early treatment is lost. Diagnostically important are, on the one hand, the histological findings with smooth muscle cells invading the lymphatic system, and on the other hand thin-walled cysts in CT scanning of the lung. CA-125 seems to be a suitable screening parameter for LAM, which may be additionally of prognostic value. Sensitivity and specificity of CA-125 require further observation.

Topics: Adolescent; Antigens, Tumor-Associated, Carbohydrate; Biomarkers, Tumor; Combined Modality Therapy; Female; Follow-Up Studies; Goserelin; Humans; Lymphangiomyoma; Puerperal Disorders; Radiotherapy Dosage; Retroperitoneal Neoplasms; Tomography, X-Ray Computed