Since Peutz in 19211 recognized the hereditary nature of gastrointestinal polyposis associated with oral pigmentation and its reemphasis by Jeghers in 1949,2 the syndrome of Peutz-Jeghers has become a well-documented clinical entity. Several thorough reviews have appeared on the clinical features of this syndrome.2,4-7 It is not our intention here to reiterate these signs and symptoms. A facet still unsettled, however, is the question of whether "malignant" changes as previously described in approximately 20% of the cases8 are true malignancies or developmental anomalies as suggested by Bartholomew et al.5 A survey of the English language literature through reviews and individual case reports still fails to reveal a single, well-documented case of metastasis or death directly attributable to malignant degeneration of a polyp at any site in the Peutz-Jeghers syndrome. The following case is, to our knowledge, the first documented instance of such an occurrence.
ACHORD JL, PROCTOR HD. Malignant Degeneration and Metastasis in Peutz-Jeghers Syndrome. Arch Intern Med. 1963;111(4):498-502. doi:10.1001/archinte.1963.03620280098015