The sex cord tumor with annular tubules was discovered as a distinctive entity when its unusualappearing microscopic pattern was encountered repetitively in a pathology consultation practice. The author was stimulated to describe the clinicopathological features of the tumor and assign it a specific name when a minority of the cases in his series were found to be associated with the rare Peutz-Jeghers syndrome. Both these tumors and those occurring in the absence of the syndrome have been shown to have distinctive clinicopathological features that differ from those of other tumors in the sex cord-stromal category.
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