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We describe a peripheral primitive neuroectodermal tumour (PPNET) arising from the cervical paravertebral region of a 34 year old woman, who presented with Horner’s syndrome and a cervical radiculopathy. PPNETs are rare malignant small round cell tumours. This appears to be the first documented case of localised PPNET with Horner’s syndrome at initial presentation.
Case report
A 34 year old woman presented with acute left scapula pain, numbness of her left forearm, a left upper lid ptosis, and left hemifacial anhydrosis. Her symptoms disappeared spontaneously within a fortnight, but returned 2 months later with greater intensity. Examination then revealed wasting of the small muscles of her left hand with reduced power in the distribution of C8 and T1; there was loss of light touch and pinprick in the C8 dermatome. The presence of left 1 mm upper lid ptosis, miosis, hemifacial anhydrosis, and 1 mm lower lid (“upside down”) ptosis was highly suggestive of a preganglionic left Horner’s syndrome (Fig 1A). Magnetic resonance imaging …