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Published on: July 13, 2016
Peutz-Jeghers syndrome: quantitative study on enterochromaffin cells in hamartomatous intestine polyps
Miljan Krstić1, Vuka Katić2, Slavica Stojnev2
1Department of Pathology, Faculty of Medicine, University of Nis, Nis, Serbia. krstic.miljan@gmail.com
Insights
Peutz-Jeghers (PJ) syndrome polyps show significantly increased enterochromaffin (EC) cells. This marked hyperplasia of EC cells in PJ polyps may cause functional disorders in affected patients.
Area of Science:
- Gastroenterology
- Oncology
- Cell Biology
Background:
- Peutz-Jeghers (PJ) syndrome is a rare autosomal disorder.
- Characterized by intestinal polyps, pigmentation, and increased cancer risk.
- Clinical issues may relate to serotonin secretion from enterochromaffin (EC) cells.
Purpose of the Study:
- Quantitatively assess the EC cell population in PJ polyps.
- Investigate EC cells in hamartomatous intestinal polyps.
- Address the lack of prior data on EC cells in PJ polyps.
Main Methods:
- Collected 21 PJ polyps over 34 years.
- Used HE, AB-PAS, Van Gieson, Fontana-Masson, FIF, and Grimelius staining.
- Performed immunohistochemistry for chromogranin A, serotonin, Ki-67, desmin, vimentin, and cytokeratin.
Main Results:
- Observed significant EC cell hyperplasia in PJ polyps.
- Demonstrated a higher EC cell content in PJ polyps versus normal ileal mucosa.
- Confirmed diagnosis and ruled out differential diagnoses using markers.
Conclusions:
- Marked EC cell hyperplasia is present in PJ polyps.
- This hyperplasia may significantly contribute to functional disorders in PJ syndrome.
- Highlights the role of EC cells in PJ syndrome pathophysiology.
Introduction:
Peutz-Jeghers (PJ) syndrome is a rare familial disorder with the autosomal transmission characterized by multiple intestinal polyps, mucocutaneous pigmentation and increased incidence of various malignancies. Some clinical manifestations of PJ syndrome may be associated with the serotonin secretion from the enterochromaffin cells (EC).
Objective:
Since no data have been reported so far regarding EC cells in PJ polyps, the aim of our study was to quantitatively investigate EC population in hamartomatous intestinal polyps in patients with the PJ syndrome.
Methods:
The samples of surgically removed PJ polyps from family members with the PJ syndrome were collected during 34-year follow-up period. Formalin-fixed paraffin-embedded specimens of twenty-one PJ polyps were stained with HE, AB-PAS, Van Gieson, Fontana-Masson, FIF and Grimelius. For immunohistochemical analysis, the following antibodies were used: chromogranin A, serotonin, Ki-67, desmin, vimentin and cytokeratin in order to eliminate differential diagnostic possibilities and to confirm diagnosis of PJ polyps.
Results:
Strong EC cell hyperplasia was observed within the tissue of the investigated polyps. Statistical analysis demonstrated significantly higher content of EC cells in PJ polyps than in the normal ileal mucosa.
Conclusion:
Marked hyperplasia of EC cells within the PJ polyps may be the most important contributor to functional disorders in patients with the PJ syndrome.
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