A case of retroperitoneal lymphangioleiomyomatosis misdiagnosed as lymphangioma without pulmonary symptom

초록

Lymphangioleiomyomatosis (LAM) is a rare, progressive disease of unknown etiology that generally affects women of childbearing age. It is characterized by gradual obstruction of small airways, resulting in the formation of thin-walled pulmonary cysts, leading to dyspnea, pneumothorax, respiratory failure and death. LAM is predominantly a lung disorder. However, extrapulmonary involvement such as retroperitoneal mass is rare but can occur. However, LAM showed only retroperitoneal abnormalities are very rare. We report a case of retroperitoneal LAM in a patient without any pulmonary symptom initially misdiagnosed as retroperitoneal lymphangioma. A 36-year-old female patient was admitted to our hospital with lower abdominal discomfort two years ago. Abdominal computed tomography revealed cystic masses located adjacent to the duodenum and retroperitoneal spaces of the pelvis. We considered retroperitoneal lymphangioma and performed laparoscopic cyst marsupialization. Histopathologic examination revealed endometriosis. Patient received hormone(GnRH agonist) therapy at gynecology. Abdominal CT was performed during the follow ? up period. There were cystic masses at the site where the previous procedure was performed. Cystic mass excision was planned through a laparotomy. A cystic mass excision was performed through the laparotomy. After surgery, the patient recovered without any complications. On histologic examination, SMA and HMB45 positive findings were confirmed as lymphangioleiomyomatosis. Many small cystic lung lesions were identified in chest CT after surgery. After operation, GnRH agonist and sirolimus are used for the treatment. The cystic mass in the retroperitoneum is difficult to determine the scope of operation because preoperative histologic examination is difficult to confirm. In this case, open-cystic mass excision was performed after recurrence. After resection, lymphangioleiomyomatosis was confirm