(Left) This 28-year-old man has known Peutz-Jeghers syndrome (PJS). A film from a small bowel follow-through shows some of the hundreds of small polyps , presumably hamartomas, throughout his bowel.
(Right) Axial CT section in the same patient shows a large mass causing partial obstruction of the duodenum. The mass proved to be a metastasis from a testicular nonseminomatous germ cell tumor.
(Left) Another CT section from the same patient shows 2 segmental, nonobstructing intussusceptions , probably due to the hamartomatous polyps.
(Right) Another CT section in the same patient shows some of the innumerable small bowel polyps . As illustrated by this case, some patients with PJS may develop malignant tumors, not just of the bowel, but also of the breast, pancreas, or reproductive tract.
TERMINOLOGY
Abbreviations
• Peutz-Jeghers syndrome (PJS)
• Multiple hamartoma (Cowden) syndrome (MHS)
• Juvenile polyposis (JP)
• Cronkhite-Canada syndrome (CCS)
Synonyms
Definitions
• Spectrum of hereditary and nonhereditary polyposis syndromes characterized by gastrointestinal (GI) tract polyps and other associated lesions
IMAGING
General Features
• Best diagnostic clue
PJS: Cluster of small filling defects in small bowel (SB) with intussusception
• Location
PJS: Jejunum and ileum > duodenum > colon > stomach
, presumably hamartomas, throughout his bowel.
causing partial obstruction of the duodenum. The mass proved to be a metastasis from a testicular nonseminomatous germ cell tumor.
, probably due to the hamartomatous polyps.
. As illustrated by this case, some patients with PJS may develop malignant tumors, not just of the bowel, but also of the breast, pancreas, or reproductive tract.
