Unusual lymphangioleiomyomatosis presenting as a pure perivascular growth pattern

• Published in: Clinical Surgical Oncology Vol. 4; no. 3; p. 100089
• Main Authors: Magpantay, Maria Angela D., Astroth, Corine, Garcia, Alfred, Ren, Dong, Eng, Oliver, O'Leary, Michael P.
• Format: Journal Article
• Language: English
• Published: Elsevier B.V 01.09.2025
• Subjects: Lymphangioleiomyomatosis; Perivascular growth pattern; Retroperitoneum; Retroperitoneum; Perivascular growth pattern; Lymphangioleiomyomatosis
• ISSN: 2773-160X; 2773-160X
• DOI: 10.1016/j.cson.2025.100089

Lymphangioleiomyomatosis (LAM) is a rare disease mainly affecting women of child-bearing age that primarily involves lung parenchyma smooth muscles with resulting cystic changes. This disease can present symptomatically as shortness of breath or as a recurrent pneumothorax. Rare instances of extrapulmonary manifestations involving the kidneys and other visceral organs have been reported. Herein, we describe an unusual case of LAM in a patient with a retroperitoneal mass showing pure perivascular growth pattern. We report a case of a 41-year-old female who presented with abdominal pain and was found to have a left para-aortic mass. This was at the aortic bifurcation and concerning for a paraganglioma; however, laboratory analysis excluded a functional mass. The patient was lost to follow-up due to complications from the COVID-19 pandemic and established care 4 years later, at which time biopsy revealed the diagnosis of LAM. She ultimately underwent surgical resection. This is a unique presentation of non-pulmonary, para-aortic LAM. This case reviews the relevant literature and presents a methodical histologic work up of this unusual mass.