Acinar cell carcinoma of the pancreas in a young patient with chronic pancreatitis.
BACKGROUND
Acinar cell carcinoma (ACC) is a rare malignancy of the pancreas arising from acinar cells. Unlike ductal adenocarcinoma, this tumor rarely presents with pancreatitis.


METHODS
We present a case of ACC associated with chronic calcifying pancreatitis, and a review of the literature focusing on diagnosis and management.


RESULTS
A 43-year-old man was proposed for Wirsungojejunal derivation for chronic pancreatitis. Histopathological examination of the tissue extracted revealed an ACC. Duodenopancreatectomy was performed. Six months post-operatively, the patient developed hepatic metastasis and was treated with gemcitabine as palliative chemotherapy.


CONCLUSIONS
The clinical presentation of ACC of the pancreas is not specific and the tumor can be under-diagnosed when associated with chronic pancreatitis. Data regarding course, treatment, and prognosis of this tumor are generally lacking.
Department of Pathology, Mongi Slim Hospital, 2046 Sidi Daoued La Marsa, Tunisia.
Fatima-Zahra
FZ
Kebir
Ahlem
A
Lahmar
Nafaa
N
Arfa
Saber
S
Manai
Mohamed Ali
MA
El Ouaer
Saadia
S
Bouraoui
Carole
C
Gouttalier
Sabah
S
Mezabi-Regaya
D000328:Adult; D018267:Carcinoma, Acinar Cell / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D008297:Male; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000601:surgery; D050500:Pancreatitis, Chronic / Q000150:complications*; D011379:Prognosis