Join us in Philadelphia from Sept. 25-27th for our "Together We Will" Weekend: - Register today!

The Desmoid Tumor Research Foundation

February 2024

Multifocal Desmoid-Type Fibromatosis: Case Series and Potential Relationship to Neuronal Spread

Author Information

Keith M Skubitz 1, Shelly Marette 2, Paari Murugan 3, Bevan Yueh 4, Denis R Clohisy 5

1Medicine, University of Minnesota, Minneapolis, USA. 2Radiology, University of Minnesota, Minneapolis, USA. 3Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, USA. 4Otolaryngology, University of Minnesota, Minneapolis, USA. 5Orthopaedic Surgery, University of Minnesota, Minneapolis, USA.

Publication

Cureus

Summary

Multifocal desmoid-type fibromatosis (DTF) is very rare and usually regional. We report three cases that initially appeared to be multifocal, but subsequent detailed imaging revealed unsuspected tracking along nerves in two cases. This neural spread is reminiscent of neuromuscular choristoma (NMC), a rare developmental lesion in which mature skeletal muscle cells, or rarely smooth muscle cells, infiltrate and enlarge peripheral nerves. NMC is frequently associated with DTF. These two cases suggest that DTF spread along nerves and appeared as distinct multifocal lesions while actually being contiguous. The third case was felt to represent true multifocal tumor development, possibly due to tumor seeding at the time of chest surgery. The relationship of DTF to NMC is discussed.

View Abstract