Incidental Pelvic and Para-aortic Lymph Node Lymphangioleiomyomatosis Detected During Surgical Staging of Pelvic Cancer in Women Without Symptomatic Pulmonary Lymphangioleiomyomatosis or Tuberous Sclerosis Complex
Abstract
Extrapulmonary lymphangioleiomyomatosis (LAM) is a rare neoplasm of spindle cells exhibiting melanocytic and myoid differentiation that arises as a mass in the mediastinum, retroperitoneum, uterine wall, and/or intraperitoneal lymph nodes. Many patients also have pulmonary LAM, tuberous sclerosis complex (TSC), and/or other neoplasms of the perivascular epithelioid cell tumor family. This study reports 26 patients with clinically occult LAM involving pelvic/para-aortic lymph nodes removed from women undergoing surgical staging of a uterine (17), ovarian (5), cervical (3), or urinary bladder (1) neoplasm. None of the patients exhibited symptoms of pulmonary LAM, and the median patient age (56 y) was older than what would be expected for patients presenting with pulmonary LAM. Only 2/26 patients had TSC. Four patients also had uterine LAM. One of these 4 had uterine perivascular epithelioid cell tumor, and 1 had vaginal angiomyolipoma. In all 26 patients the lymph node LAM was grossly occult, measured 3.5 mm on average (1 to 19 mm), and involved either a single lymph node (12/26) or multiple lymph nodes (14/26). HMB45 was positive in 24/25 cases, mostly in a focal or patchy distribution. Other melanocytic markers included MiTF (1...Continue Reading
References
European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis
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