Primary retroperitoneal lymphangioleiomyomatosis in a postmenopausal woman: a case report and review of the literature

Abstract

Lymphangioleiomyomatosis (LAM) is a rare progressive disease of unknown etiology that typically affects women of childbearing age. It is characterized by an abnormal proliferation of smooth muscle cells causing gradual obstruction of small airways, frequently resulting in respiratory failure and death. While LAM is predominantly a lung disorder, we report a case of retroperitoneal LAM in a patient who had no evidence of pulmonary involvement. A 59-year-old female presented with postmenopausal bleeding and no other complaints. She was found to have a low attenuation retroperitoneal mass on abdominal and pelvic computed tomography (CT) scan suspicious for lymphoma. CT-guided biopsy was nondiagnostic. Laparoscopic resection of some of the enlarged retroperitoneal lymph nodes confirmed the diagnosis of LAM. This case is an atypical presentation of LAM. The disease typically presents in premenopausal women, with the initial site of involvement being the lungs and mediastinum. In rare cases, such as this of extrapulmonary LAM, patients typically present with a palpable abdominal mass, abdominal pain, or chylous ascites. As in our case, radiographic findings can mimic malignancies such as lymphoma. Laparoscopic lymph node biopsy is a valuable tool in these situations of diagnostic dilemma.

Lymphangioleiomyomatosis: A Disease Involving the Lymphatic System

“…The involvement of lymph nodes and lymphatic vessels including the thoracic duct and its tributaries, and=or extension into adjoining soft tissues beyond the connective tissue capsule of the lymph nodes and lymphatics 24,26,44,[50][51][52][53] are often associated with chyle leakage into body cavities. Chyle may leak into the pleural space (chylothorax), peritoneal cavity (chylous ascites), pericardial space (chylous pericardial effusion), urine (chylouria), 54 intestinal lumen (proteinloosing enteropathy), 55 or from the vagina.…”

Lymphangioleiomyomatosis: solitary abdominal manifestation (2009: 9b)

“…Correlated abdominopelvic abnormalities include renal angiomyolipoma (AML) (54%), enlarged abdominal lymph nodes (39%), lymphangioleiomyomas (16%), chylous ascites (10%), dilatation of the thoracic duct (9%), and hepatic AML (4%) [8]. Solitary extrapulmonary manifestation of LAM is rare [9].…”

Association of retroperitoneal lymphangioleiomyomatosis with endosalpingiosis: a case report

“…There are very few reported cases of extrapulmonary LAM without pulmonary affectation, and Kebria identified 21 cases [5]. …”