Mesenteric Lipodystrophy: A Frequently Underdiagnosed Entity
Jason M Wells, Zhaohai Yang. Penn State Milton S. Hershey Medical Center, Hershey, PA
Background: Mesenteric lipodystrophy (ML) often has a clinical impression of mass lesion but the biopsy is frequently reported as nonspecific findings, which provides little guidance to patient management. Recently we encountered several cases and none of them was initially diagnosed correctly. This prompted us to further review the clinicopathologic features of this entity.
Design: The pathology database from the last 20 years was queried using the term 'mesenteric', 'mesenteritis', 'lipodystrophy', and 'fat necrosis'. All reports and slides were reviewed, and the clinicopathologic features were evaluated. Immunostain for IgG4 was performed on selected cases.
Results: Fifteen patients were identified, the age ranged from 26 to 81, with M:F ratio of 4:1. Twelve patients (80%) presented with mesenteric mass, and the size ranged from 2.1 to 8 cm. Microscopically, fat necrosis was seen in all cases, nodular hyalinizing fibrosis was seen in 13 cases (87%); fibrosis encasing the vessel, nerve and lymph nodes was seen in 12 (80%), 6 (40%), and 4 (27%) cases, respectively. The lesion showed infiltrative margins in all cases. The IgG4+ plasma cells varied from 0 to 80 / HPF. Only seven patients (47%) were correctly diagnosed at initial review. Five patients (33%) had a history of malignancy, and six patients (40%) had a history of abdominal surgery.
Conclusions: ML is an underdiagnosed entity. Correlation with clinical/radiological findings such as mass lesion, and attention to the pathologic features such as fat necrosis with nodular hyalinizing fibrosis and encasement of various structures is the key to a correct diagnosis and optimal patient care. IgG4+ plasma cells vary in ML thus do not appear to be helpful in the differential diagnosis.
Wednesday, March 6, 2013 9:30 AM
Poster Session V # 113, Wednesday Morning