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Ganglion cell-containing tumours of the central nervous system (CNS) include gangliogliomas and gangliocytomas which are among the least common tumours of the CNS.1–3 Ganglion cell-containing tumours of the pituitary gland are extremely rare, and consist of well-differentiated mature ganglion cells within a neuropil-rich background.4–6 There have also been two reported cases of ganglion cell-containing cutaneous tumours, reported as ‘cutaneous ganglion cell choristoma’ 7 and ‘ganglioneuromatous tumour of the skin’.8 Ganglion cells are not normal components of the peripheral nerve, and ganglion cell-containing tumours of the peripheral nerve have not been previously described. We report a ganglion cell-containing benign tumour, corresponding to ganglion cell choristoma, in the peripheral nerve in a 52-year-old man who presented with a deep radial neuropathy.
A 52-year-old, right-handed man was evaluated at our institution for left hand weakness of 3 months duration. He first noticed inability to extend his left index finger after waking up from sleep. Subsequently, he noticed weakness in extending all his fingers, and tightness in them. The hand weakness was severe enough to cause functional impairment. There was no associated pain or paresthesias, and no history of preceding trauma. His past medical history was unremarkable.
Neurological examination revealed atrophy of the extensor compartment of the left forearm. Severe weakness of finger extension (Figure 1A), and mild wrist extension and supination was noted on the left side. He had preserved elbow flexion from biceps and brachioradialis muscles. No sensory deficit was noted on clinical evaluation. Deep tendon reflexes, including the left triceps jerk, were preserved. The remainder of the neurological examination was normal. A mass lesion could not …
Contributors All the authors have contributed to this manuscript.
Competing interests None.
Patient consent Obtained.
Ethics approval Mayo Clinic IRB.
Provenance and peer review Not commissioned; internally peer reviewed.