A novel mechanism for the formation and propagation of neural tumors and lesions through neural highways

Abstract

By recognizing anatomic and radiologic patterns of rare and often misdiagnosed peripheral nerve tumors/lesions, we have defined mechanisms for the propagation of neural diseases. The novel concept of the nervous system serving as a complex system of "highways" driving the neural and perineural spread of these lesions is described in three examples: Intraneural dissection of joint fluid in intraneural ganglion cysts, perineural spread of cancer cells, and dissemination of unknown concentrations of neurotrophic/inhibitory factors for growth in hamartomas/choristomas of nerve. Further mapping of these pathways to identify the natural history of diseases, the spectrum of disease evolution, the role of genetic mutations, and how these neural pathways interface with the lymphatic, vascular, and cerebrospinal systems may lead to advances in targeted treatments.

Unrecognized neuromuscular choristoma with recurrent desmoid-type fibromatosis and Marjolin ulcer: expanding the spectrum of neuromuscular choristoma sequelae within the nerve territory? Illustrative case

“…We have previously recommended not biopsying suspected cases of NMC given the pathognomonic clinicoradiologic features (and the potential for inducing DTF with biopsy or surgery). 3 (2) The finding of nerve-territory NMC-DTFs, 4 , 5 whether unicentric or multicentric, has been established in patients, with the NMC-DTFs developing either spontaneously or after iatrogenic injury (e.g., biopsy, resection of the NMC 6 , 7 ). (3) To our knowledge, this is the first case of NMC and NMC-DTF associated with an invasive squamous cell carcinoma/Marjolin ulcer within the nerve territory.…”

Correction to: Lumbosacral plexopathy caused by the perineural spread of pelvic malignancies: clinical aspects and imaging patterns

“…The neural highways [2] underlying neoplastic LSP originate at the affected pelvic organ (or staple line) where perineural invasion transitions to perineural spread. Perineural spread continues via pelvic autonomic nerves (i.e., inferior hypogastric plexus) to the lumbosacral plexus and can extend proximally to spinal nerves, dura, and, even to the other limb [3] and/or distally to the sciatic nerve.…”