Retroperitoneal lymphangioleiomyoma with lymph node involvement: A pathologic-radiologic correlation of a rare form of myomelanocytic tumor.
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.
Department of Pathology, Medical College of Wisconsin, Milwaukee, WI, USA.
Amrou
A
Abdelkader
Department of Radiology, Medical College of Wisconsin, Milwaukee, WI, USA.
Cesar A
CA
Lam
Department of Radiology, Medical College of Wisconsin, Milwaukee, WI, USA.
Kaushik S
KS
Shahir
Division of Surgical Oncology, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI, USA.
Kathleen
K
Christians
Department of Pathology, Medical College of Wisconsin, Milwaukee, WI, USA. Electronic address: ssuster@mcw.edu.
Saul M
SM
Suster
D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis*; D003937:Diagnosis, Differential; D015622:Epithelioid Cells / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology*; D008198:Lymph Nodes / Q000473:pathology*; D018192:Lymphangioleiomyomatosis / Q000000981:diagnostic imaging / Q000473:pathology*; D008203:Lymphangiomyoma / Q000000981:diagnostic imaging / Q000473:pathology*; D012186:Retroperitoneal Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology*
D014408:Biomarkers, Tumor
Extrapulmonary clear cell tumor; Lymphangioleiomyoma; Lymphangioleiomyomatosis; PEComa; Retroperitoneum