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Peutz-Jeghers syndrome with osseous metaplasia of the intestinal polyps
T Narita1, H Ohnuma, S Yokoyama
1Department of Pathology, Mutsu General Hospital, Japan.
Insights
A rare case of Peutz-Jeghers syndrome (PJS) featured osseous metaplasia in hamartomatous polyps. This finding, observed in the small intestine, is novel for PJS and may aid diagnosis.
Area of Science:
- Gastroenterology
- Pathology
- Genetics
Background:
- Peutz-Jeghers syndrome (PJS) is a rare genetic disorder characterized by hamartomatous polyps and mucocutaneous pigmentation.
- PJS significantly increases the risk of various cancers, necessitating vigilant monitoring.
Observation:
- A 40-year-old male with diagnosed PJS presented with intestinal obstruction.
- Surgical resection revealed 15 hamartomatous polyps in the jejunum, characteristic of PJS.
- Three of these PJS polyps exhibited osseous metaplasia, a finding previously unreported in this condition.
Findings:
- Histological examination confirmed mature bone formation and calcification within the PJS polyps.
- The osseous metaplasia occurred in proximity to hyperplastic glands in the submucosa or muscularis propria.
- No malignant transformation was identified in the affected polyps.
Implications:
- Osseous metaplasia is an exceptionally rare finding in benign polyps.
- This case represents the first documented instance of osseous metaplasia within hamartomatous polyps in Peutz-Jeghers syndrome.
- Recognizing this association could potentially assist in the clinical and pathological diagnosis of PJS-related polyps.
Abstract:
A case of Peutz-Jeghers syndrome (PJS) with osseous metaplasia in three of 15 hamartomatous polyps of the small intestine is reported. At 35 years of age, the patient was diagnosed as having PJS by cutaneous pigmentation around the mouth and polyposis of the stomach, duodenum and intestine. Fifty-two polys of the large intestine were resected, which were characteristic of those of PJS. Three of them showed adenomatous and carcinomatous changes, but there was no osseous metaplasia in any of the resected polyps. At age 40, he had surgery under the diagnosis of intestinal obstruction. There were 15 polyps in the resected jejunum. These polyps were also characteristic of those of PJS. Additionally, three of these polyps were accompanied by osseous metaplasia. Histologically, mature bone formation and calcification were found close to the hyperplastic glands in the submucosa or in the propriate muscle. Malignant transformation was not observed. Osseous metaplasia is extremely rare in benign polyps, and it has not been reported in hamartomatous polyps of PJS to date. The knowledge of this association may be helpful in the clinical diagnosis of this benign lesion in PJS.