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Alpha-fetoprotein-producing Pancreatic Acinar Cell Carcinoma.J
Formos Med Assoc. 2007 Aug;106(8):669-72.
A 47-year-old man
with chronic hepatitis B had progressive elevated alpha-fetoprotein of
2 years' duration. A pancreatic tail tumor, instead of liver tumor,
was detected. He underwent elective distal pancreatectomy and
splenectomy and the pathology turned out to be acinar cell carcinoma
of the pancreas. Serum level of alpha-fetoprotein returned to normal
soon after surgery. No cancer recurrence was noted after 3 years of
follow-up. Alpha-fetoprotein is commonly used as a tumor marker to
screen for hepatocellular carcinoma in high-risk patients. However,
elevated alpha-fetoprotein could occur in a much rarer disease, acinar
cell carcinoma of the pancreas.
Pancreatic
Acinar Cell Carcinoma with Excessive Alpha-Fetoprotein Expression.Pancreatology.
2007 Aug 14;7(4):370-372.
We report a
case of acinar cell carcinoma of the pancreas associated with
excessively elevated levels of serum alpha-fetoprotein (>32,000 ng/ml).
Abdominal computed tomography scan revealed a large pancreatic mass
with infiltration of the splenic artery. Because of inoperability,
palliative combination chemotherapy with gemcitabine and mitomycin C
was administered. This regimen was associated with clinical
improvement and dramatic decreases in both tumor size and serum
alpha-fetoprotein. However, the patient died 7 months later from acute
severe cardiac failure.
Acinar cell
carcinoma of the pancreas with predominant intraductal growth: report
of a case.Gastroenterol
Clin Biol. 2007 May;31(5):543-6.
Acinar cell
carcinoma (ACC) of the pancreas accounts for approximately 1% of all
exocrine pancreatic tumours. We report a rare form of ACC in a
66-year-old man. This tumour was revealed by epigastric pain and
weight loss. Abdominal computed tomography showed a hypodense,
well-demarcated, heterogeneous lesion, in the head of the pancreas,
measuring 4.2 cm in diameter. There was a marked dilatation of the
main pancreatic duct upstream, with tumour spreading within this duct.
The diagnosis of ACC was made on the fine needle aspiration cytology
performed during endoscopic ultrasound examination. On the
pancreaticoduodenectomy specimen, the dilated main pancreatic duct
(2.5 cm in diameter) was filled by an exophytic tumour. Histological
examination showed an ACC, with predominant intraductal growth (main
and accessory pancreatic ducts), with pancreatic parenchymal and
duodenal invasion. Neuroendocrine markers were negative. To our
knowledge, this is the second report of an ACC with predominant
intraductal spread. These rare forms of ACC can be confused with
intraductal papillary-mucinous neoplasms. In our report, fine needle
aspiration cytology performed during endoscopic ultrasound examination
was a valuable tool in the diagnostic assessment.
Primary acinar
cell carcinoma of the ampulla of Vater.
J Gastroenterol. 2007 Aug;42(8):694-7.
Acinar cell
carcinoma of the pancreatobiliary system is a relatively rare
malignant neoplasm arising usually in the pancreatic parenchyma. We
experienced a 68-year-old woman who presented with obstructive
jaundice due to an ampullary mass 1.0 cm in diameter, detected by
abdominal computed tomography and endoscopic examination. The patient
underwent a curative surgical operation, and histopathological
examination revealed that the tumor was confined to the ampulla of
Vater with no continuity to the pancreatic parenchyma. The tumor cells
showed acinar or tubular arrangement with eosinophilic to basophilic
granular cytoplasm, findings identical to those of acinar cell
carcinoma of the pancreas. Immunohistochemically, the tumor cells were
positive for lipase. From these findings, we concluded that the tumor
was primary acinar cell carcinoma arising in the ampulla of Vater,
probably originating from heterotopic pancreatic tissue. This is the
first reported case of primary acinar cell carcinoma in the ampulla of
Vater.
Intraductal
and papillary variants of acinar cell carcinomas: a new addition to
the challenging differential diagnosis of intraductal neoplasms.Am
J Surg Pathol. 2007 Mar;31(3):363-70.
The
recognition and differential diagnosis of pancreatic intraductal
neoplasms (IN) have gained importance in the past few years, as the
incidence of these tumors (especially intraductal papillary mucinous
neoplasms-IPMNs) have risen to >10% of pancreatic resections, and
their significance as precursors of invasive cancer is better
appreciated. Acinar cell carcinomas (ACCs) are typically solid tumors;
however, we have recently encountered 7 ACCs with either intraductal
growth and/or a papillary/papillocystic pattern that could be mistaken
for IN. The clinicopathologic features of these cases were studied.
Four patients were male and 3 female, with a mean age of 59 and mean
tumor size of 4.9 cm (as compared with 10 cm in conventional ACCs).
Only 1 patient had metastasis at the time of diagnosis (as opposed to
50% in usual ACCs). In 5 cases, the tumors had nodular growth of
sheet-forming acinar cells, some of which were within ducts, as
evidenced by the polypoid nature of the process, partial ductal
lining, and presence of small tributary ducts in the walls. In 3
cases, the tumor had papillary and/or papillocystic growth, at least
focally. All cases had cystic areas. No mucin was identified. All
expressed trypsin. Markers of ductal differentiation were either
absent or focal. A minor endocrine component was present in 3. The
main histologic findings that distinguished these tumors from IPMNs
were the more sheetlike nature of the nodules (rather than villous or
arborizing papillae), cuboidal cells, overall basophilia of the
cytoplasm, prominent nucleoli, apical granules, intraluminal crystals
or pale, acidophilic secretions (enzymatic condensations), and lack of
mucin. In conclusion, some ACCs show intraductal growth or exhibit
papillary patterns, which can mimic IN, especially IPMNs. In such
cases, attention to morphologic details described above, and
immunohistochemistry are helpful. The clinical significance of this
variant is difficult to determine; however, it appears that the tumors
are relatively small and metastasis at presentation is less common
than typically seen in ACCs (1/7 vs. 50%).
Acinar
cell carcinoma of the pancreas: clinical analysis of 115 patients from
Pancreatic Cancer Registry of Japan Pancreas Society.Pancreas.
2007 Jul;35(1):42-6.
OBJECTIVES: Acinar cell carcinoma (ACC) of the pancreas is a rare
tumor, and many aspects remain unclear because no large-scale clinical
studies have been conducted. METHODS: The present study investigated
the clinical characteristics, treatment, and therapeutic outcomes of
115 patients registered in the Pancreatic Cancer Registry of the Japan
Pancreas Society, and therapeutic plans were reviewed. RESULTS:
Although ACC has been associated with advanced stage and poor
prognosis, this tumor was resectable in 76.5% of the patients, and the
5-year survival rate after resection was favorable, being 43.9%.
CONCLUSIONS: Confirming the diagnosis of ACC preoperatively is
difficult, but this diagnosis should be kept in mind while planning
surgery for ordinary pancreatic cancer. Once the diagnosis has been
confirmed, a possibility of surgical resection should be pursued to
achieve better prognosis. If ACC is unresectable or recurrent,
chemotherapy is likely to prove useful. Multidisciplinary therapy
centering on the role of surgery will need to be established.
Cytology of
pancreatic acinar cell carcinoma.
Diagn
Cytopathol. 2006 May; 34(5):367-72.
Acinar
cell carcinoma (ACC) of the pancreas is extremely uncommon and its
cytologic features have rarely been described. We describe the
cytologic features of cases we have seen, review the literature
regarding its cytologic features and discuss the pitfalls that may be
encountered and the use of immunohistochemistry for its diagnosis. We
searched our databases for all cases of histologically confirmed
pancreatic ACC which had undergone prior fine needle aspiration (FNA)
of the primary pancreatic lesion. The clinical histories, radiographic
and sonographic findings, cytologic features, original cytologic
diagnoses, and final histologic diagnoses were reviewed. Four cases of
pancreatic ACC were found that had undergone FNA prior to histologic
confirmation of the diagnoses. They were from 2 men and 2 women aged
50-75 yr. All masses were in the head of the pancreas, 2 had apparent
peri-pancreatic adenopathy and 1 had an apparent liver metastasis. On
review, all 4 had had diagnostic material on cytology samples.
Original cytologic diagnoses included "acinar cell carcinoma,"
"pancreatic endocrine tumor," "favor neuroendocrine tumor, low-grade"
and "non-diagnostic specimen." The cytologic features included small
to moderate-sized loose groups with numerous single cells, prominent
acinar formation, little anisonucleosis and prominent nucleoli. The
cytologic features showed significant overlap with those of pancreatic
endocrine tumors.
Pancreatic
acinar cell carcinoma extending into the common bile and main
pancreatic ducts.
Pathol Int. 2006 Oct;56(10):633-7.
Acinar
cell carcinoma (ACC) of the pancreas is relatively rare, accounting
for only approximately 1% of all exocrine pancreatic tumors. A
69-year-old man was found to have a mass lesion measuring
approximately 4 cm in diameter in the pancreatic head on ultrasound,
abdominal dynamic CT, and percutaneous transhepatic cholangiography.
Magnetic resonance cholangiopancreatography showed defect of the lower
common bile duct (CBD) due to obstruction by the tumor cast.
Histopathologically, the pancreatic head tumor invaded the main
pancreatic duct (MPD) and CBD with extension into the CBD in a form of
tumor cast. The tumor cells consisted of a solid proliferation with
abundant eosinophilic cytoplasm and round nuclei in an acinar and
trabecular fashion. A 55-year-old man with upper abdominal pain and
nausea, had a cystic lesion approximately 3 cm in size in the
pancreatic tail on CT. Histopathologically, the tumor was encapsulated
by fibrous capsule and had extensive central necrosis with solid areas
in the tumor periphery, and invaded with extension into the MPD in a
form of tumor cast. The tumor cells resembled acinar cells in solid
growths. Two resected cases of ACC with unusual tumor extension into
the CBD and the MPD, respectively, are reported.
Acinar cell
carcinomas and pancreatoblastomas: related but not the same.
Pathologe. 2005 Feb;26(1):37-40.
Acinar cell
carcinomas and pancreatoblastomas are malignant tumors of the
pancreas, showing predominantly acinar differentiation characterized
by the immunohistochemical expression of pancreatic enzymes.
Histologically, they usually display acinar and/or solid patterns, but
may occasionally also exhibit cystic structures. The key feature of
pancreatoblastomas is the presence of squamoid corpuscles. Acinar cell
carcinomas predominantly occur in adults, pancreatoblastomas in
children. Both tumor types commonly show allelic losses on chromosome
11p and mutations in the APC/beta-catenin signaling pathway.
Pancreatoblastomas, in contrast to acinar cell carcinomas, are
potentially curable.
CT and MRI
features of pure acinar cell carcinoma of the pancreas in adults.AJR
Am J Roentgenol. 2005 Feb;184(2):511-9.
OBJECTIVE: We
sought to describe the CT and MRI features of pure acinar cell
carcinoma of the pancreas in adults. MATERIALS AND METHODS: Eleven
patients (six women and five men; mean age, 64 years) with acinar cell
carcinoma, documented by pathologic examination of resected specimens,
underwent CT (n=9) or MRI (n=2) examinations. Two radiologists
evaluated imaging studies and determined, by consensus, the following
data for each tumor: size, location, margination, internal density or
signal intensity, and contrast enhancement pattern. In addition, they
assessed the presence of calcification, pancreatic or bile duct
dilation, and metastases. Imaging features were correlated with gross
and microscopic pathologic features of the tumors. RESULTS: Masses
were distributed throughout the pancreas (head, n=5; body, n=2; and
tail, n=4). The mean largest dimensions were 6.0 x 5.3 cm (range, from
2 x 1.7 to 15 x 11 cm). Tumors were oval (n=5), round (n=4), or
lobular (n=2). Ten (91%) masses were well marginated; nine (82%) were
exophytic. Five (45%) masses enhanced homogeneously; the remaining
tumors contained cystic areas. All masses enhanced less than the
surrounding pancreas. Three (27%) masses contained calcifications.
Four (80%) masses invaded the duodenum. Common bile and pancreatic
duct dilatation was present in two and three patients, respectively.
One patient had metastatic liver disease at presentation. CONCLUSION:
Pure acinar cell carcinoma of the pancreas is usually an exophytic,
oval or round, well-marginated, and hypovascular mass on CT and MRI.
It typically is completely solid when small and contains cystic areas
due to necrosis when large.
Acinar cell cystadenocarcinoma of the pancreas: report of rare case
and review of the literature.Hum
Pathol. 2004 Dec;35(12):1568-71.
Most exocrine pancreatic tumors are of ductal origin, whereas acinar
cell adenocarcinomas are unusual (1% to 2% of all exocrine pancreatic
neoplasms). We recently found a cystic adenocarcinoma of the
pancreatic body whose cells had the characteristics of acinar cells,
which we term acinar cell cystadenocarcinoma. Macroscopically, this
tumor consists of a large multilocular cystic mass with a
pseudocapsule and a spongy appearance on the cut surface.
Microscopically, the cysts are lined by a single layer of cuboid/columnar
cells. The cytoplasm has the characteristics of acinar cells, with
eosinophilic granules in the apex and prominent nucleoli.
Immunohistochemically, the cells express alpha1-antitrypsin, trypsin,
and lipase in their cytoplasm, thus confirming the acinar origin of
the tumor. A review of the literature revealed only 5 other cases of
this tumor reported since its first description in 1981. Follow-up
data are available for 4 of these; all of the affected patients had
metastases at presentation or a few months later, and 2 died of the
disease, at 13 and 37 months after diagnosis. Although this variant of
adenocarcinoma of the pancreas is not prognostically different from
the classic solid type (few patients survive more than 5 years), we
believe that it is important because of its extreme rarity.
Mixed acinar-endocrine
carcinoma of the pancreas. A clinicopathological study and comparison
with acinar-cell carcinoma.Virchows
Arch. 2004 Sep;445(3):231-5.
We compared
the clinicopathological features of acinar-cell carcinomas (ACCs) with
those of mixed acinar-endocrine carcinomas (MAECs). Specimens from 37
patients with ACC and 6 patients with MAEC were examined
histologically and immunohistochemically. The mean age of ACC and MAEC
patients was similar (61.3 years versus 58.4 years), but the sex ratio
differed (ACC, 29 males and 8 females; MAEC, 2 males and 4 females).
The size of the tumor was large in both cases (ACC, 13.8 cm in
diameter; MAEC, 8.2 cm). Immunohistochemically, more than half of the
tumor cells in all tumors, whether ACC or MAEC, stained for trypsin.
In 20 of the 37 ACCs (54%), scattered endocrine cells (SECs) were
found, which stained positively for synaptophysin (SYN) and/or
chromogranin A (CGA). Interestingly, there was also a difference in
the sex ratio between ACC patients without SECs (16 males and 1
female) and ACC patients with SECs (13 males and 7 females). In MAECs,
the cells staining for SYN were more common than those staining for
CGA and made up more than one-third of the neoplastic-cell population.
In all but one case (in which the endocrine component was arranged in
islet-like cell clusters), the endocrine cells were intimately mixed
with trypsin-positive tumor cells. The endocrine cells only rarely
expressed one of the known pancreatic or gastrointestinal hormones.
Both ACCs and MAECs had a high proliferation rate and lacked p53
overexpression or progesterone and estrogen receptors. This study
revealed that ACCS and MAECs share most clinicopathological features
and, therefore, may form a single tumor entity, though they differ in
the number of endocrine cells. The frequent identification of
endocrine cells in these tumors suggests the existence of a
pluripotent cell of origin that is capable of differentiating into
acinar and endocrine cells.
Acinar
cell carcinoma of the pancreas eroding the pylorus and duodenal bulb.J
Hepatobiliary Pancreat Surg.
2004;11(4):276-9.
A 74-year-old
woman presented at the National Defense Medical College Hospital in
April 2001 with a chief complaint of upper abdominal pain. She had
been diagnosed as having adenocarcinoma on the basis of results of
examination of a biopsy specimen taken from an ulcer of the duodenal
bulb at a local hospital. On admission, she showed no jaundice, but a
hard mass, about 10 cm in diameter, was palpated in the right upper
quadrant. Laboratory data showed high levels of serum carcinoembryonic
antigen (CEA) and carbohydrate antigen (CA) 19-9. Abdominal computed
tomography (CT) and angiography demonstrated a giant enhanced mass in
a pattern of eccentric gradation extending to the pylorus, duodenal
bulb, and pancreatic head. She underwent pancreatoduodenectomy with
combined resection of the transverse colon. The histologic diagnosis
was acinar cell carcinoma (ACC), originating in the pancreatic head
and extending to the stomach, duodenum, and transverse colon, without
any lymph node involvement. In most reported cases of ACC, the
preoperative diagnosis was a pancreatic mass or endocrine tumor of the
pancreas. The correct diagnosis in those cases was made by
postoperative or postmortem pathological examination. If criteria for
detecting the slight differences between ACC and endocrine tumors on
some images were to be established, the diagnostic skill for ACC would
improve dramatically.
Clinical
characteristics and outcomes from an institutional series of acinar
cell carcinoma of the pancreas and related tumors. J
Clin Oncol. 2002 Dec 15;20(24):4673-8.
PURPOSE:
Acinar cell carcinoma is a rare tumor of the exocrine pancreas.
Clinical features such as prognostic information, survival, and
treatment outcomes are unknown. We present the largest retrospective
review to date. PATIENTS AND METHODS: Thirty-nine patients with
pathologically confirmed acinar neoplasms of the pancreas were
identified between August 1981 and January 2001. Demographic data,
tumor characteristics, and treatment information were obtained by
chart review. Survival probabilities were estimated by using the
Kaplan-Meier method and compared using the log-rank test. RESULTS: The
median survival for all patients was 19 months. On the basis of a
univariate analysis, the patients' stage of disease correlated
significantly with survival. The median survival of patients with
localized disease was 38 months, versus 14 months for those presenting
with metastases (P = 0.03). Patients who could be treated with surgery
as first-line therapy had a longer survival time (36 months) compared
with those who did not have surgery (14 months). Two of 18 patients
who received chemotherapy and three of eight patients who received
radiation had a major response. CONCLUSION: The survival curves
suggest a more aggressive cancer than pancreatic endocrine neoplasms
but one that is less aggressive than ductal adenocarcinoma of the
pancreas. Those patients who present with localized disease have a
much better prognosis than those who present with metastases. There is
a high recurrence rate after complete surgical resection, suggesting
that micrometastases are present even in localized disease and that
adjuvant therapies may be indicated. Chemotherapy and radiation afford
disappointing results, however, and novel therapies are needed.
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