Neurothekeomas are very rare benign soft tissue tumors which originate from sheath of the peripheral nerves. They are commonly located on the upper extremities or the head and the neck. Pain is an unusual clinical symptom. We report a case of a sixty-nine year-old woman, who presented us with a painful nodule on the upper inner surface of the left leg. With the histopathological, histochemical and immunostaining features the lesion was diagnosed as S100 (-) / CD68 (+) myxoid hypocellular neurothekeoma. The case was presented due to the unusual clinicopathological features.
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