Faculty Collaboration Database

Retroperitoneal lymphangioleiomyoma with lymph node involvement: A pathologic-radiologic correlation of a rare form of myomelanocytic tumor. Ann Diagn Pathol 2017 Apr;27:69-73

Date

03/23/2017

Pubmed ID

28325364

DOI

10.1016/j.anndiagpath.2017.01.008

Scopus ID

2-s2.0-85011915343 (requires institutional sign-in at Scopus site)   4 Citations

Abstract

Lymphangioleiomyomatosis (LAM) is a rare and slowly progressive disorder that usually arises in the lung, affects exclusively women in their childbearing years, and typically presents with progressive dyspnea on exertion and pneumothorax. Infrequently, extra-pulmonary LAM can occur in the retroperitoneum, uterine wall, mediastinum and intraperitoneal lymph nodes. Histologically, LAM is characterized by a proliferation of perivascular epithelioid cells (PEC) that express markers for both melanocytes and smooth muscle cells. We report a case of a peripancreatic retroperitoneal mass that was incidentally discovered on magnetic resonance image (MRI) scan of a 38-year-old female. The morphologic findings and the immunohistochemical staining were consistent with a lymphangioleiomyoma. The radiologic and pathologic correlation along with differential diagnosis of this rare entity is discussed.

Author List

Abdelkader A, Lam CA, Shahir KS, Christians K, Suster SM

Author

Kathleen K. Christians MD Professor in the Surgery department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adult
Biomarkers, Tumor
Diagnosis, Differential
Epithelioid Cells
Female
Humans
Immunohistochemistry
Lung Neoplasms
Lymph Nodes
Lymphangioleiomyomatosis
Lymphangiomyoma
Retroperitoneal Neoplasms