nodal lymphangioleiomyomatosis

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.

Clinically occult lymph node LAM can be detected during surgical staging of pelvic cancer and is not commonly associated with pulmonary LAM or TSC, although these patients should still be formally evaluated for both of these diseases.

References

 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. Rabban JT, Firetag B, Sangoi AR, Post MD, Zaloudek CJ. Am J Surg Pathol. 2015 Mar 16. PMID: 25786086