Pathology analysis reveals that dysplastic pancreatic ductal lesions are frequent in patients with hereditary pancreatitis.
OBJECTIVE
Hereditary pancreatitis (HP) is a risk factor for pancreatic adenocarcinoma. We performed a retrospective, multicenter study to characterize and evaluate the frequency of pancreatic intraepithelial neoplasia (PanIN) and to describe the characteristics of fibrosis in pancreatic surgical specimens from patients with HP.


METHODS
Samples from partial pancreatectomies (n = 13) of patients with HP complications (n = 12; 7 males; mean age, 24 y; 1 patient underwent 2 surgeries over 16 years) were analyzed by histologic and immunohistologic analyses; patients with suspected or proven pancreatic adenocarcinoma were excluded. HP diagnosis was confirmed by analysis of PRSS1 mutations. Dysplastic lesions were described according to the PanIN classification.


RESULTS
Eleven patients were found to have the R122H mutation in PRSS1 and 1 patient was found to have the N29I mutation in PRSS1. Fifty-one PanIN lesions were observed in 10 specimens (77%): PanIN lesions 1a, 1b, 2, and 3 were observed in 8, 5, 8, and 5 specimens, respectively. The median number of PanIN lesions was 3.5 for each specimen. The density of the lesions was 2.6 per 10 cm(2). The size of lesions was greater than 0.5 mm in 55% of the samples. Two patients with PanIN-3 developed pancreatic cancer, 18 months and 44 years after surgery.


CONCLUSIONS
PanIN lesions are frequent, severe, and occur early in the course of HP. Among patients with PanINs, 50% had PanIN-3 lesions. Pancreatectomy could be considered as a prophylactic against pancreatic cancer in patients with high-grade dysplasia.
Rebours
Vinciane
V
Pôle des Maladies de l'Appareil Digestif, Service de Gastroentérologie-Pancréatologie, Hôpital Beaujon, AP-HP, Paris, Clichy, France. vinciane.rebours@bjn.aphp.fr
Lévy
Philippe
P
Mosnier
Jean-François
JF
Scoazec
Jean-Yves
JY
Soubeyrand
Marie-Sophie
MS
Fléjou
Jean-François
JF
Turlin
Bruno
B
Hammel
Pascal
P
Ruszniewski
Philippe
P
Bedossa
Pierre
P
Couvelard
Anne
A
D000293:Adolescent; D000328:Adult; D002278:Carcinoma in Situ / Q000453:epidemiology*; D021441:Carcinoma, Pancreatic Ductal / Q000453:epidemiology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D030342:Genetic Diseases, Inborn / Q000150:complications*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010180:Pancreatectomy; D010183:Pancreatic Ducts / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000453:epidemiology*; D010195:Pancreatitis / Q000150:complications*; D012189:Retrospective Studies; D014357:Trypsin / Q000235:genetics; D055815:Young Adult
C489303:PRSS1 protein, human; D014357:Trypsin