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A case of hyperkeratosis follicularis et parafollicularis in cutem penetrans Kyrle's disease in a 41-year-old woman is described. It was a recurrence of a lesion surgically removed and originally diagnosed as invasive squamous cell carcinoma. Kyrle's disease is a rare dermatosis and should not be confused with neoplastic conditions i.e. squamous cell carcinoma. Pathologists should be aware of such conditions in order to avoid misdiagnosis, which can imply over treatment for the patient.

Here we describe a case of gastrointestinal autonomic nerve tumor (GANT) arosen in a patient with von Recklinghausen's disease. To the best of our knowledge, only four other similar cases have been previously reported in the literature. Histologically, the tumour was composed of spindle shaped cells arranged in short bundles. The neoplastic cells had pleomorphic nuclei and numerous mitoses. Immunohistochemical investigation displayed evidence of neural differentiation. Neoplastic cells were diffusely anti-synaptophysin, anti-neuron specific enolase and anti-vimentin positive. The clinicopathological features of the present case are described and discussed together with those of the other previously reported cases.