Pancreatic Cysts
Pathologic Classification, Differential Diagnosis, and Clinical Implications
Olca Basturk, MD; Ipek Coban, MD; N. Volkan Adsay, MD
● Context.—Cystic lesions of the pancreas are being recog-
nized with increasing frequency and have become a more
common finding in clinical practice because of the wide-
spread use of advanced imaging modalities and the sharp
drop in the mortality rate of pancreatic surgery. Consequent-
ly, in the past 2 decades, the nature of many cystic tumors
in this organ has been better characterized, and significant
developments have taken place in the classification and in
our understanding of pancreatic cystic lesions.
Objective.—To provide an overview of the current con-
cepts in classification, differential diagnosis, and clinical/bi-
ologic behavior of pancreatic cystic tumors.
Data Sources.—The authors’ personal experience, based
on institutional and consultation materials, combined with
an analysis of the literature.
Conclusions.—In contrast to solid tumors, most of which
are invasive ductal adenocarcinomas with dismal progno-
U ntil the end of the 1970s, the spectrum of cystic le-
sions of the pancreas was relatively narrow and con-
sisted mainly of mucinous and serous neoplasms.1–3 From
the 1980s onward, the development and widespread use
of new imaging techniques led to an increase in the num-
ber of resected cystic lesions.4 This, in turn, advanced our
knowledge of these tumors. New entities were described,
and the pathogenesis, morphology, and biology of the en-
tities already known were studied in more detail.5
This article reviews the currently available information
on the clinicopathologic features, differential diagnosis,
and biologic behavior of the pancreatic cystic lesions. The
lesions discussed below follow an order that, in the au-
thors’ experience, reflects their frequency, presumed cell
of origin, and clinical significance, as well as the published
data in the literature.5,6 Estimated relative frequency of
cystic lesions is shown in Table 1.
INJURY-RELATED AND
INFLAMMATION-RELATED CYSTS
Pseudocyst
Pseudocysts are the most common type of cystic lesions
of the pancreas,7 although they rarely come to the atten-
Accepted for publication November 4, 2008.
From the Department of Pathology, New York University, New York,
New York (Dr Basturk); and the Department of Pathology, Emory Uni-
versity, Atlanta, Georgia (Drs Coban and Adsay).
The authors have no relevant financial interest in the products or
companies described in this article.
Reprints: N. Volkan Adsay, MD, Department of Pathology, Emory
University Hospital, 1364 Clifton Rd NE, Atlanta, GA 30322 (e-mail:
volkan.adsay@emory.edu).
Arch Pathol Lab Med—Vol 133, March 2009
sis, cystic lesions of the pancreas are often either benign
or low-grade indolent neoplasia. However, those that are
mucinous, namely, intraductal papillary mucinous neo-
plasms and mucinous cystic neoplasms, constitute an im-
portant category because they have well-established malig-
nant potential, representing an adenoma-carcinoma se-
quence. Those that are nonmucinous such as serous tu-
mors, congenital cysts, lymphoepithelial cysts, and
squamoid cyst of pancreatic ducts have no malignant po-
tential. Only rare nonmucinous cystic tumors that occur
as a result of degenerative/necrotic changes in otherwise
solid neoplasia, such as cystic ductal adenocarcinomas,
cystic pancreatic endocrine neoplasia, and solid-pseudo-
papillary neoplasm, are also malignant and have variable
degrees of aggressiveness.
(Arch Pathol Lab Med. 2009;133:423–438)
tion of surgical pathologists because, often, they are man-
aged medically or by surgical drainage without resection.
They develop as a complication of alcoholic, biliary, or
traumatic acute pancreatitis,8,9 predominantly in adult
men. Those resulting from biliary disease or trauma occur
in younger patients and have an equal predilection for
both sexes.7 The lesions develop when a focus of peripan-
creatic fat necrosis is resorbed, thereby producing a de-
bris-filled space surrounded by granulation tissue that is
ultimately enclosed within a fibrous capsule. Some pseu-
docysts arise from the release of pancreatic enzymes into
an anatomic sac. There is no epithelial lining (Figure 1).
The adjacent stroma may be hypercellular and may mimic
ovarian-type stroma, characteristic of mucinous cystic neo-
plasm (MCN). Depending on the severity and duration of
the pancreatitis, the pseudocyst may resolve spontaneous-
ly, or it may achieve a size that is no longer self-resorbable
and will require surgical intervention.
The clinical diagnosis of a pseudocyst is usually
straightforward; however, on occasion, the differential di-
agnosis includes neoplastic cysts and vice versa. There are
case reports in which virtually every pancreatic neoplasm
presents as a pseudocyst.10–15 For example, solid-pseudo-
papillary neoplasms often undergo massive cystic degen-
eration, and mucinous cystic neoplasms16 have a tendency
to become infected and contain suppurative contents.
Moreover, although rare, ductal adenocarcinomas may un-
dergo central necrosis and clinically mimic pseudo-
cysts.5,17,18 Proper sampling is crucial for correct diagnosis
in such cases. Any epithelial element within the cyst wall
suggests an alternative diagnosis to pseudocyst. Cyst fluid
analysis for amylase and carcinoembryonic antigen (CEA)
Pancreatic Cysts—Basturk et al 423
 Types of Cystic Lesions in the Pancreas
Injury-related and inflammation-related cysts (30%)
Pseudocyst
Paraduodenal wall cyst
Infection-related cysts
Neoplastic cysts (60%)
Ductal lineage
Mucinous type (30%)
Intraductal papillary mucinous neoplasm
Mucinous cystic neoplasm
Intraductal oncocytic papillary neoplasm
‘‘Retention cyst,’’ ‘‘mucocele,’’ and ‘‘mucinous nonneo-
plastic cyst’’
Cystic change in ordinary ductal adenocarcinoma and
other invasive carcinomas
Serous (clear-cell) type (20%)
Serous cystadenoma
Oligocystic (macrocystic) variant of serous cystadenoma
von Hippel-Lindau syndrome–associated pancreatic
cysts
Serous cystadenocarcinoma
Not otherwise specified
Intraductal tubular carcinoma
Endocrine lineage (⬍5%)
Cystic pancreatic endocrine neoplasm
Acinar lineage (⬍1%)
Acinar cell cystadenoma (cystic acinar transformation)
Acinar cell cystadenocarcinoma
Cystic/Intraductal acinar cell carcinoma
Endothelial lineage (⬍1%)
Lymphangioma
Mesenchymal lineage (⬍1%)
Undetermined lineage (5%)
Solid-pseudopapillary neoplasm
Other
Mature cystic teratoma
Congenital cysts (⬍1%)
Duplication (enterogenous) cysts
Duodenal diverticula
Others
Miscellaneous cysts (⬍5%)
Lymphoepithelial cyst
Squamoid cyst of pancreatic ducts
Epidermoid cysts within intrapancreatic accessory spleen
Cystic hamartoma
Endometriotic cysts
Secondary tumors

are also helpful in distinguishing pseudocysts from neo-

plastic cysts that are mucinous.19 While amylase concen-
trations are consistently elevated in pseudocysts, typically
many thousands of units per liter, elevated cyst fluid CEA
levels higher than 200 ng/mL suggest a mucinous epithe-
lium-lined cyst.
Paraduodenal Wall Cyst
A distinct form of chronic pancreatitis occurring pre-
dominantly in the periampullary region, and often asso-
ciated with cysts on the duodenal wall/adjacent pancreas,
has been reported under various names, including cystic
dystrophy of heterotopic pancreas, pancreatic hamartoma
of duodenum, paraduodenal wall cyst, myoadenomatosis,
and groove pancreatitis.7,20,21 In our experience, most pa-
424 Arch Pathol Lab Med—Vol 133, March 2009
Figure 1. Pseudocyst. Depending on the stage of pseudocyst forma-
tion, the wall of a pseudocyst consists of inflammation and granulation
tissue or variably organized fibroinflammatory tissue. By definition,
there is no epithelial lining; instead, the cyst wall merges with the cyst
contents composed of fibrin, necrotic fat cells, debris, hematoidin pig-
ment, and aggregates of histiocytes (hematoxylin-eosin, original mag-
nification ⫻4).
Figure 2. Intraductal papillary mucinous neoplasm. Dilatation of the
ducts leads to a multilocular cyst formation, with many cysts filled with
tan, friable papillary nodules.
Figure 3. Intraductal papillary mucinous neoplasm. The main duct is
filled with tall papillary fronds lined by mucin-producing cells (he-
matoxylin-eosin, original magnification ⫻4).
Pancreatic Cysts—Basturk et al
tients are men aged 40 to 50 years, and history of alcohol
abuse and cigarette smoking is characteristic.21–23 Macro-
scopically, there is mucosal thickening, marked duodenal
wall scarring, trabeculation of duodenal musculature, and
macrocystic and microcystic changes in the duodenal
wall. Presumably because they arise from the ‘‘heterotop-
ic’’ ducts on the duodenal wall or accessory ampulla, the
cysts may sometimes be partially lined by ductal epithe-
lium and partially by granulation tissue, unlike conven-
tional pseudocysts which have no association with the
ducts. The walls of the cysts are composed of markedly
inflamed fibrous tissue, with evidence of foreign-body–
type giant cell reaction, in which mucoprotein material is
engulfed, and of myofibroblastic proliferation. Luminal
and mural calcifications are common.
Infection-Related Cysts
Rarely, hydatid cysts,24–27 necrotic tuberculosis infec-
tions,28,29 and other entities can occur in the pancreas and
mimic primary cystic neoplasms.
NEOPLASTIC CYSTS
Ductal Lineage, Mucinous Type
Intraductal Papillary Mucinous Neoplasm. Intraduc-
tal papillary mucinous neoplasms (IPMNs) were thought
to be very rare; however, in recent years, better recogni-
tion of this neoplasm has led to an increase in its recog-
nized incidence.30–32 Among pancreatic resection speci-
mens, the incidence of IPMN is approximately 5%; among
cystic lesions, it is about 20%. Pathologically, IPMNs are
characterized by intraductal proliferation of neoplastic
mucinous cells, which usually form papillae and lead to
cystic dilation of the pancreatic ducts and formation of
clinically and macroscopically detectable masses30,31,33–47
(Figures 2 and 3).
Clinically, IPMNs occur slightly more frequently in men
than in women. The mean age at diagnosis is 68 years,
with a range of 25 to 94 years. Patients usually present
with nonspecific abdominal symptoms, although in some,
a history of pancreatitis is noted. Approximately 30% of
patients have tumors in other organs (particularly the co-
lorectum and stomach), some synchronous and others me-
tachronous.35,48,49 Intraductal papillary mucinous neo-
plasms have also been reported in patients with the Peutz-
Jeghers syndrome50 and with familial adenomatous pol-
yposis.51 Mucin extrusion from the ampulla of Vater
during endoscopy is virtually diagnostic for these neo-
plasms. Radiologically, a markedly distended main pan-
creatic duct, or numerous cysts representing dilated
branch ducts, can be seen. Today, the more common pre-
sentation is as an incidental small cyst in the pancreas on
a computed tomography scan obtained for other reasons.
Seventy percent of IPMNs occur in the head of the pan-
creas. The lesions may be localized, multicentric or, rarely,
the entire ductal system may be involved. By definition,
they are larger than 1 cm. Macroscopic examination of
IPMNs is imperative for documenting involvement of the
pancreatic ductal system and the distribution of the dis-
ease within the ductal system. In some cases, the IPMN
primarily involves the main pancreatic duct (main duct
type), and in others, it is mostly confined to the branch
ducts (branch duct type); the latter is predominant partic-
ularly in IPMNs that arise in younger patients and is more
likely to involve the uncinate process.44,52,53 Some authors
Arch Pathol Lab Med—Vol 133, March 2009
believe the branch duct type is a biologically distinct en-
tity, and therefore every attempt should be made during
macroscopic examination to determine the distribution of
the lesion, as discussed below42,44,48,53–55 The adjacent pan-
creatic parenchyma is usually firm and pale white because
of scarring and atrophies from chronic obstruction in main
duct–type IPMNs, whereas it is generally normal in
branch duct–type IPMNs. Associated solid or gelatinous
nodules may correspond to an invasive component.
Microscopically, papillae with 3 distinct morphologic
patterns can be seen.35,56–61 (1) In most branch duct–type
IPMNs, the papillae are lined by tall columnar cells with
basally oriented nuclei and abundant pale supranuclear
mucin, creating a pattern reminiscent of gastric foveolar
epithelium or low-grade pancreatic intraepithelial neopla-
sia (PanIN) 1A. These IPMNs are classified as gastric-fo-
veolar (Figure 4, A). Because this phenotype is also com-
mon in the nonpapillary areas of main duct–type IPMNs,
it is also referred to as null type. The pattern does appear
to be full recapitulation of gastric mucosa, with small
glandular elements at the base that express MUC6 mucin
and more papillary areas expressing MUC5AC. Scattered
goblet cells, however, are quite common and can be high-
lighted by immunolabel for MUC2. (2) Most main duct–
type IPMNs show papillae that are lined by columnar cells
with cigar-shaped nuclei and variable amounts of apical
mucin, closely resembling colonic villous adenomas and
are classified as villous-intestinal61 (Figure 4, B). They do,
in fact, show molecular characteristics of intestinal differ-
entiation, as evidenced by CDX2 and MUC2 expression,
which have tumor suppressor activity.61 The papillae are
also positive for MUC5AC. When these IPMNs are asso-
ciated with an invasive cancer, it is typically of colloid
type,61 and colloid carcinomas also express CDX2 and
MUC2, but not MUC1.59 (3) In a small proportion of
IPMNs, the papillae are more complex and are lined by
cuboidal cells, often with round nuclei containing a single
prominent eccentric nucleolus (Figure 4, C). These are re-
ferred to as pancreatobiliary. They typically do not express
CDX2 and MUC2, but may instead express MUC1 (a
‘‘marker’’ of aggressive phenotype in the pancreas) and
MUC5AC.61 Invasive carcinoma associated with this group
is usually of tubular type, with all the morphologic fea-
tures of ordinary ductal adenocarcinoma. The invasive
component also expresses MUC1, but not MUC2.59
Both villous-intestinal and pancreatobiliary types of
IPMN may transition to areas with gastric-foveolar mor-
phology; however, it is uncommon to find both intestinal-
and pancreatobiliary-type papillae within the same IPMN.
Noninvasive IPMNs have a spectrum of cytoarchitec-
tural atypia (Figure 5). Those that have a single layer of
neoplastic cells with well-oriented, small, and uniform nu-
clei without mitoses or necrosis are classified as either an
adenoma62 or, more recently, as an IPMN with low-grade
dysplasia.32 When there is nuclear stratification, slight loss
of polarity, nuclear enlargement, and some nuclear pleo-
morphism, the lesions are classified as borderline tumor
(IPMN with moderate dysplasia). Carcinoma in situ
(IPMN with high-grade dysplasia) exhibits architectural
atypia with cribriforming, significant loss of polarity,
and severe nuclear pleomorphism. Mitoses are often
present.30,36,63,64
Until recently, approximately 30% of IPMNs resected
have had an associated invasive carcinoma, either of the
colloid65 or ordinary ductal type34,35; however, this number
Pancreatic Cysts—Basturk et al 425
Figure 4. The category of intraductal papillary mucinous neoplasms encompasses 3 morphologically distinct types of papillae. A, Gastric-foveolar
or null type. Tall columnar cells with basally located nuclei and abundant apical mucin resemble gastric-mucosa or pancreatic intraepithelial
neoplasia 1 lesions. B, Villous-intestinal type. Similar to colonic villous adenomas, with tall, fingerlike projections lined by pseudostratified,
basophilic columnar cells with cigar-shaped nuclei. A variable amount of mucin is present in the cytoplasm. C, Pancreatobiliary type. More
complex branching papillae lined by relatively cuboidal cells, some with prominent nucleoli (hematoxylin-eosin, original magnifications ⫻40).

seems to be highly population-dependent and appears to
be decreasing because of increased diagnosis of smaller
lesions. Invasion may develop both adjacent to and away
from the IPMN66 and can be invisible at gross examina-
tion. It seems that main duct–type IPMNs, and those that
are large, complex, and nodular, often prove to be carci-
nomas and require more aggressive therapy. Given the
high prevalence of cancer and the data from the literature,
it is unlikely that any combination of clinical and radio-
logic parameters will accurately discriminate between ma-
lignant and nonmalignant main duct–type IPMNs.52,67,68
Management of IPMNs is rather problematic and, there-
fore, some authors even advocate total pancreatectomy for
such cases.69 On the other hand, branch duct–type IPMNs,
and those that are small and without complex nodulari-
ties, usually prove to be IPMNs with low-grade dyspla-
sia.67 Less than 15% of these clinically innocuous-appear-
ing branch duct IPMNs prove to have carcinoma in long-
term follow-up.67 Accordingly, current consensus proto-
col67 advocates a ‘‘watchful wait’’ approach if a branch
duct IPMN is (1) less than 3 cm, (2) does not have any
complexities or mural nodules, (3) shows no changes dur-
ing follow-up, and (4) is asymptomatic.52,55,67,68 However,
this approach is applicable only if the patient can be kept
under close supervision, as outlined in the consensus pro-
tocol.
Another problematic area in the management of IPMNs
is the status of surgical resection margins.69 Our current
426 Arch Pathol Lab Med—Vol 133, March 2009
approach is mostly extrapolated from anecdotal observa-
tions and from our current view of the biology of these
tumors. If there is no or only minimal atypia (IPMN with
low-grade dysplasia) at the margin, we do not recommend
additional surgery, provided that clinically, there is no oth-
er lesion in the remaining pancreas. At the other end of
the spectrum, when there is carcinoma in situ or invasive
carcinoma at the margin, additional surgery is warranted,
if clinically feasible. Florid papillary nodules at a margin
also require careful evaluation and possibly additional
surgery, because their presence suggests the likelihood
that more tumor is present in the remaining pancreas. The
scenario that is probably most problematic is the presence
of denuded epithelium and inflammation on a frozen-sec-
tion specimen. In our opinion, this is worrisome and we
and others (G. Zamboni, MD, oral communication, May
2008) advocate extra margin evaluation for such cases.
Overall 5-year survival for patients with IPMN is higher
than 70%.32 Recent studies have shown that the prognosis
for patients with resected and carefully examined low-
grade dysplasia and borderline tumors is excellent, but
patients with carcinoma in situ may experience recurrenc-
es and metastases. The aggressive behavior of cases with
carcinoma in situ is attributed to missed foci of invasive
cancer that are either unresected because of unrecognized
multifocality or undiagnosed during pathologic exami-
nation. In some series, patients with invasive carcinoma,
Pancreatic Cysts—Basturk et al

Figure 5. Intraductal papillary mucinous neoplasms with high-grade
dysplasia (carcinoma in situ) exhibit significant architectural and nu-
clear atypia, including loss of polarity and marked pleomorphism (he-
matoxylin-eosin, original magnification ⫻20).
which may follow an aggressive clinical course, had a 5-
year survival rate as low as 36%42,54,69,70
Intraductal papillary mucinous neoplasms have several
overlapping features with mucinous cystic neoplasia, as
discussed below. These neoplasms also need to be distin-
guished from PanIN.71 The separation of IPMNs from
PanIN is based primarily on size: IPMNs are usually larg-
er (⬎1 cm) and form a macroscopically and/or radiolog-
ically detectable mass.72,73 In other words, they represent
a ‘‘mass-forming’’ type of dysplastic process, whereas
PanINs are detected microscopically/incidentally.
Mucinous Cystic Neoplasm. Mucinous cystic neo-
plasms are presumably de novo cystic tumors and have
distinctive clinicopathologic characteristics. They are seen
almost exclusively in perimenopausal female patients
(mean age, 48 years; male to female ratio ⬍1:20) and most
arise in the body or tail of the pancreas.2,74–78 Macroscop-
ically, MCNs are composed of thick-walled multilocular
cysts that can become very large (up to 35 cm). The lesions
often have a thick pseudocapsule, and the outer surface is
usually smooth and well demarcated (Figure 6). Focal cal-
cifications are sometimes present at the periphery of the
neoplasms. Unless there is fistula formation, the cysts do
not visibly communicate with the pancreatic ductal sys-
tem. The individual locules, seen grossly, are typically be-
tween 1 and 3 cm and have thick walls. Especially in larg-
er samples, the wall of the cysts may have velvety papil-
lations and even solid mural nodules that correspond to
papillary elements. The cyst contents are often mucoid,
but watery fluid, hemorrhagic fluid, or even necrotic de-
bris may also be noted.
Microscopically, the cysts are lined by tall, columnar,
Arch Pathol Lab Med—Vol 133, March 2009
mucin-producing epithelium (Figure 7) that resembles en-
docervical epithelium. Scattered goblet-type cells may be
seen. Immunohistochemically, the epithelial component of
MCNs stains for cytokeratin (CK) 7, CK8, CK18, CK19,
CEA, and MUC5AC. Only gobletlike cells express MUC2.
Scattered neuroendocrine cells are present and can be
demonstrated by immunohistochemical labeling for neu-
roendocrine markers, such as chromogranin and synap-
tophysin. On the cyst wall and septae, a distinctive ovar-
ian-type stroma composed of densely packed spindle cells
with sparse cytoplasm and uniform, elongated nuclei is
seen (Figure 7). This stroma is an entity-defining feature
of MCNs, and its presence has become a quasi-require-
ment for the diagnosis.67 It regularly expresses progester-
one receptors, and to a lesser degree, estrogen receptors.
Moreover, some MCNs are reported to be associated with
ovarian thecomas,79 further suggesting a hormone influ-
ence in the pathogenesis of these neoplasms. Cells of the
stromal component also stain for ␣-inhibin and calretinin.80
Just as with IPMNs, MCNs also exhibit characteristics
of an adenoma-carcinoma sequence. Those without atypia
are classified as MCNs with low-grade dysplasia32 or mu-
cinous cystadenomas.62 In such cases, the cysts are lined
by flat, mucin-producing, cuboidal to columnar epitheli-
um with basally oriented uniform nuclei (Figure 7). These
are typically devoid of papillae and no mitoses are seen.
Those MCNs with mild to moderate architectural and cy-
tologic atypia are classified as MCNs with moderate dys-
plasia or borderline tumor. The epithelium may be several
cells thick and often forms papillae. The columnar nature
of the cells is maintained, with no significant pleomor-
phism or nuclear irregularities. The nuclei vary slightly in
size and shape; nucleoli may be present. Occasional mi-
toses may be seen. Those MCNs that exhibit prominent
papillary proliferations that form intraluminal polypoid
masses with cribriform architecture and severe cytologic
atypia are classified as MCNs with high-grade dyspla-
sia63,81 or carcinoma in situ63,81 (Figure 8). Significantly in-
creased mitoses are characteristic. Not surprisingly, the in-
cidence of carcinoma in situ increases with the size and
complexity of the lesion. It should be remembered, how-
ever, that foci of carcinoma in situ can be very patchy or
focal, not visible grossly, often with an abrupt transition
between histologically bland epithelium and epithelium
with severe atypia. The neoplasm is classified based on
the highest, rather than the average, degree of atypia.75,76,78,82
Less than one-fifth of all MCNs are associated with in-
vasive carcinoma.82,83 The invasive component can be very
focal, and numerous sections may be required to properly
evaluate these neoplasms. Grossly papillary or nodular ar-
eas should be sampled first, as these areas are most likely
to harbor a carcinoma component. If an invasive carcino-
ma is not identified in this initial sampling, then the entire
neoplasm should be submitted for histologic examination.
The invasive carcinomas that arise in association with
MCNs are usually of tubular/ductal type. Additionally,
some MCNs may be associated with undifferentiated car-
cinoma with osteoclast-like giant cells,84,85 adenosquamous
carcinoma,86 choriocarcinoma, or even high-grade sarco-
ma.87 Pure mucinous (colloid) carcinoma is exceedingly
uncommon with MCNs.88 If an invasive carcinoma is pres-
ent,63,81 it is preferable to report these entities as ‘‘invasive
carcinoma of 㛮㛮㛮 type, 㛮㛮㛮 cm, arising in association with
MCNs (㛮㛮㛮 cm).’’
Recent studies indicate that grade does accurately pre-
Pancreatic Cysts—Basturk et al 427
Figure 6. Mucinous cystic neoplasm typically forms a thick-walled cyst in the tail of pancreas.
Figure 7. Mucinous cystic neoplasm with low-grade dysplasia (mucinous cystadenoma). The cells have tall mucin-laden cytoplasm with basally
located bland-appearing nuclei. Note the underlying ovarian-like cellular stroma (hematoxylin-eosin, original magnification ⫻10).
Figure 8. Mucinous cystic neoplasm with high-grade dysplasia (carcinoma in situ) exhibits prominent papillary proliferations that form intraluminal
polypoid masses with cribriform architecture and loss of nuclear polarity. The nuclei show significant pleomorphism, prominent nucleoli and
increased mitoses (hematoxylin-eosin, original magnification ⫻20).
Figure 9. Intraductal oncocytic papillary neoplasms are characterized by complex branching papillae, oncocytic cells and intraepithelial lumina
that often contain mucin (hematoxylin-eosin, original magnifications ⫻4 and ⫻40 [inset]).
Figure 10. Large-duct variant of ductal adenocarcinoma is composed of irregularly distributed invasive glandular elements that are larger than
those of ordinary ductal adenocarcinoma. Some may show abortive papilla formation (hematoxylin-eosin, original magnification ⫻2).
Figure 11. Serous cystadenoma is characterized by a well-demarcated tumor composed of innumerable small cysts creating a spongelike or
honeycomb appearance. A stellate scar is commonly present.
428 Arch Pathol Lab Med—Vol 133, March 2009 Pancreatic Cysts—Basturk et al
dict outcome,75,76,78,82 but this statement holds true only for
cases that can be graded properly. Patients with complete-
ly resected MCNs, in whom the presence of even a minute
in situ or invasive carcinoma has been effectively excluded
by through examination and extensive sampling, are al-
most always cured. However, those with invasive carci-
noma often have a relatively aggressive clinical course
with recurrences and metastasis. Some tumors may have
more indolent behavior, presumably because of the small
size (early stage) of the invasive carcinoma, which is
brought to clinical attention with a large MCN. In fact, in
their analysis of 56 MCNs, Zamboni et al78 have reported
that the extent of invasion is the most significant prog-
nostic factor. Patients with in situ carcinoma may, on oc-
casion, also experience recurrences and metastases, pre-
sumably due to undocumented foci of invasive carcinoma.
Although MCNs and IPMNs have some features that
overlap, the two can be distinguished by clinical, gross,
and microscopic findings.67 While IPMNs are seen pre-
dominantly in the head of the pancreas and in older men
(60–70 years),34,78,89 most MCNs occur in the tail of the pan-
creas in patients who are perimenopausal women. Intra-
ductal papillary mucinous neoplasms involve the pancre-
atic ducts, whereas MCNs typically do not communicate
with the ductal system. Most importantly, the presence of
ovarian stroma is diagnostic for MCNs and has become a
requirement for the diagnosis of this tumor type.67 The
only instance in which the requirement for the presence
of an ovarian stroma may perhaps be waived is when a
tumor, with all the clinicopathologic characteristics of a
classical MCN, occurs in an older woman. Typically, how-
ever, even in these cases, the tumor would give the im-
pression that an atrophied and hyalinized ovarian-like
stroma forms a band around the cyst.
Intraductal Oncocytic Papillary Neoplasm. Intraduc-
tal oncocytic papillary neoplasm (IOPN)90 is regarded as
a special subtype of IPMN,74 although recent molecular
findings suggest that it may be different from IPMN. The
IOPNs typically lack the KRAS2 mutations, which are seen
in 70% of IPMNs.91,92 Regardless, many of the attributes
discussed above for IPMNs also apply to IOPNs. The fea-
tures that characterize and delineate IOPNs from other
IPMNs are the following.
The unilocular or multilocular lesions exhibit cystic di-
latation of the pancreatic ducts, many of which contain
large, tan, and friable nodular proliferations. Microscopi-
cally, they are characterized by very tall and complex ar-
borizing papillae93 (Figure 9). One distinctive feature that
appears to be relatively specific for these neoplasms is the
presence of intraepithelial lumina, which are round,
punched-out spaces within the epithelium that often give
the proliferation a cribriform architecture (Figure 9). These
intraepithelial lumina often contain mucin. The cells of
IOPNs are oncocytic, because of an abundance of mito-
chondria, and the nuclei contain single, prominent, and
eccentric nucleoli (Figure 9). Scattered goblet cells may be
identified. In contrast to other IPMN types, labeling of
IOPNs for MUC6 is usually positive,94 whereas MUC5AC
and MUC2 are largely restricted to goblet cells, or are
present only focally.
Most IOPNs qualify for classification as carcinomas in
situ based on the exuberance of papillary elements, the
large nuclei, prominent nucleoli, and mitotic activity; how-
ever, the relationship between this cytoarchitectural com-
plexity and the potential for malignancy has yet to be fully
Arch Pathol Lab Med—Vol 133, March 2009
documented. In our experience, despite their highly pro-
liferative nature and large size (with a mean of 6 cm),
invasive carcinomas arising from IOPNs are typically un-
common, and such cases may have a long protracted clin-
ical course. In other organs, such as the kidney, oncocytic
change is associated with a biologic behavior different
from that of nononcocytic neoplasms. Whether this obser-
vation applies also to the pancreas remains to be seen.
‘‘Retention Cyst,’’ ‘‘Mucocele,’’ and ‘‘Mucinous Non-
neoplastic Cyst.’’ In general, most cysts lined by mucin-
ous epithelium in the pancreas are considered neoplastic.
This has become especially true since the inclusion of the
entity formerly called mucinous metaplasia or mucinous hy-
pertrophy into the PanIN category as PanIN-1A.72 However,
obstruction and fibrosis may lead to cystic dilatation of
the upstream ducts7 (ie, a retention cyst, or if mucus filled,
a mucocele).95,96 Most patients with this type of cyst are
asymptomatic adults and the cysts are detected inciden-
tally. The lesions are usually single and unilocular with a
smooth and glistening lining. Microscopically, they have
a simple epithelial lining. Most authors restrict the term
retention cyst to unilocular cysts lined by cuboidal cells
lacking significant apical mucin,7 but controversy exists
when these cysts exhibit columnar mucinous lining. Some
authors classify such cases as mucinous nonneoplastic
cysts,97,98 whereas others prefer to include them in the cat-
egory of IPMNs with low-grade dysplasia. In other words,
there are no specific criteria that distinguish these innoc-
uous mucin-lined cysts from IPMNs, or for small cysts,
from PanINs, except the presence of papillae, which allows
an unequivocal diagnosis of branch duct IPMN. The au-
thors’ current approach is to classify a grossly detectable
cystic lesion, larger than 1 cm and lined by mucinous ep-
ithelium, as an IPMN; however, it is quite possible that
this approach will change as more facts about these lesions
are elucidated. Since retention cysts may result from duc-
tal obstruction, it is important to ensure that they are not
caused by a tumor, such as a small invasive carcinoma
located proximal to the cyst.
Cystic Change in Ordinary Ductal Adenocarcinoma
and Other Invasive Carcinomas. Rarely, conventional
infiltrating ductal adenocarcinomas of the pancreas may
undergo cystic change.5,17,18 In our experience, this occurs
in less than 1% of cases.17 In some pancreatic cancers, a
large, radiologically detectable cyst may form because of
central necrosis. Such cases can be misdiagnosed preop-
eratively as ‘‘pseudocysts.’’ 99 In other cases, infiltrating
ductal adenocarcinomas can obstruct the pancreatic duct
and lead to cystic dilatation of the upstream duct. A large
duct (microcystic) variant of ductal adenocarcinoma100–102
also exists, with infiltrating tubular units that are larger
than those of ordinary ductal adenocarcinomas (Figure
10). This variant mimics noninvasive pancreatic tumors
characterized by cystic and papillary patterns such as
MCNs or IPMNs. It may be distinguished from the latter
group of neoplasms by the smaller size of its cysts, irreg-
ularity of the duct contours, clustering of the ducts, pres-
ence of intraluminal neutrophils and granular debris, de-
gree of cytologic pleomorphism, and myxoid quality of
the stroma. Clinically it behaves like ordinary grade 1 duc-
tal adenocarcinoma.
Other invasive malignant neoplasms of the pancreas,
such as undifferentiated carcinoma with osteoclast-like gi-
ant cells13 or squamous cell carcinomas,103 occasionally
present as cystic masses.
Pancreatic Cysts—Basturk et al 429
Figure 12. Serous cystadenoma. Numerous, tightly packed small cysts lined by low cuboidal tumor cells with clear cytoplasm, and small, round
uniform nuclei that have dense, uniform chromatin (hematoxylin-eosin, original magnification ⫻20).
Figure 13. Oligocystic variant of serous cystadenoma showing a large unilocular cavity.
Figure 14. Intraductal tubular carcinoma with nodular and smooth-contoured growth resembling intraductal papillary mucinous neoplasm. Inset
shows tightly packed small tubular glands lined by cuboidal cells with round to oval atypical nuclei (hematoxylin-eosin, original magnifications
⫻2 and ⫻40 [inset]).

430 Arch Pathol Lab Med—Vol 133, March 2009 Pancreatic Cysts—Basturk et al
 Ductal Lineage, Serous (Clear Cell) Type
Serous Cystadenoma. Serous cystadenoma (SCA) is a
benign neoplasm composed of uniform glycogen-rich ep-
ithelial cells that form innumerable small cysts containing
serous fluid.1,104 The mean age of affected patients is 61
years. Up to one-third of the patients with SCA are
asymptomatic and the neoplasms are discovered inciden-
tally. Almost two-thirds of SCAs occur in the body-tail
region of the pancreas and are seen predominantly in fe-
male patients (female to male ratio, 3:1). The lesions usu-
ally present as relatively large masses measuring up to 25
cm. Macroscopic appearance is very distinctive and read-
ily diagnostic for this tumor type. Their cut surface shows
numerous, tightly packed, small, thin-walled cysts
(spongelike or honeycomb appearance) arranged around
a central stellate scar, which may contain calcifications
(Figure 11). The cysts usually do not communicate with
the pancreatic duct system.
Microscopically, the single layer of cuboidal or flattened
cells lining the small cysts have well-defined cytoplasmic
borders, pale to clear cytoplasm, and small, round uni-
form nuclei with dense, homogeneous chromatin and in-
conspicuous nucleoli (Figure 12). The nuclear contours are
very smooth. Atypia and mitosis are absent. The tumor
cells are intimately admixed with prominent capillary net-
work akin to other clear-cell tumors also associated with
von Hippel-Lindau (VHL) syndrome (renal cell carcino-
ma, capillary hemangioblastoma, etc).105 The central stel-
late scar and the stroma separating the cysts are composed
of acellular collagenous connective tissue. Special stains
highlight the abundant intraepithelial glycogen with ab-
sence of mucin. Serous cystadenomas are presumed to
arise from the centroacinar cell/intercalated duct sys-
tem106 and express cytokeratins (AE1/AE3, CAM 5.2, CK7,
CK8, CK18, CK19), ␣-inhibin, and MUC6. Characteristi-
cally, there is no immunoreactivity to CEA.107 They do not
harbor the molecular genetic alterations that are charac-
teristic of mucinous-type ductal neoplasia of the pancre-
as.108,109 Instead, VHL gene alterations (loss of heterozy-
gosity at chromosome 3p25 and a VHL-gene germline mu-
tation) are detected in 40% of cases.106,110 GLUT-1, a mol-
ecule involved in glycogen metabolism, is also expressed
consistently.111
Currently, with the advances in radiologic methods, it
is more feasible to recognize SCAs preoperatively, and de-
ciding which patients are candidates for surgery becomes
an issue. Some authors recommend nonoperative tumor
management with clinical follow-up for patients with
asymptomatic and small (⬍4 cm) tumors and reserve the
option of resection for symptomatic patients, larger tu-
mors (⬎4 cm), or for tumors that show rapid growth rate
(doubling time of a few months) in clinical follow-up.112
Oligocystic (Macrocystic) Variant of Serous Cystade-
noma. A rare oligocystic (macrocystic) variant of SCA is
composed of fewer but larger loculi and lacks the central
stellate scar (Figure 13).106,113 This variant occurs predom-
←
Figure 15. Pancreatic endocrine neoplasm with extensive cystic and hemorrhagic degeneration.
Figure 16. Cystic and intraductal acinar cell carcinoma forms papillary nodules that are punctuated by glandular and microcystic areas. The
cysts vary in size and configuration, and some contain intraluminal pale, acidophilic, amorphous material that is characteristic of enzymatic acinar
products (hematoxylin-eosin, original magnification ⫻10).
Figure 17. Solid-pseudopapillary neoplasm characterized by marked hemorrhage and cystic degeneration.
Arch Pathol Lab Med—Vol 133, March 2009
inantly in the head of the pancreas, where it may obstruct
the common bile duct and cause jaundice.114 It shows no
evidence of sex predilection. The epithelial lining of these
cysts may become denuded, and it may be difficult to dis-
tinguish oligocystic SCAs from mucinous neoplasms or
pseudocysts unless the lesion is extensively sampled.
VHL-Associated Pancreatic Cysts. Pancreatic involve-
ment is seen in 50% to 80% of patients with VHL syn-
drome. The pancreatic cysts are virtually indistinguish-
able from those of SCAs when taken out of context, but
they affect the pancreas diffusely or in a patchy fashion
rather than forming a distinct, well-demarcated tumor.
Gene alterations associated with VHL are present in these
lesions.
Serous Cystadenocarcinoma. Although extremely
rare, serous cystic neoplasms of the pancreas that involve
lymph nodes and the liver have been reported104,115–122 and
form the basis for their designation as serous cystadeno-
carcinomas. Most are microscopically identical to SCAs,
and no morphologic findings, other than tumorigenic be-
havior, distinguish these malignant variants from their be-
nign counterparts.
Ductal Lineage, Not Otherwise Specified
Intraductal Tubular Carcinoma. Intraductal tubular
carcinoma is a distinct clinicopathologic entity that has yet
to be fully characterized.123–125 By its intraductal nature, it
resembles IPMNs and may occasionally have a similar
cystic component. Microscopically, the lesions are com-
posed of intraductal nodules of tightly packed, small tu-
bular glands and solid areas lined by predominantly cu-
boidal cells with modest amounts of cytoplasm, which do
not contain any apparent mucin. The nuclei are round to
oval and atypical. Mitotic figures are readily identifiable
(Figure 14). Necrosis has been reported in some cases, fo-
cally showing comedo pattern.125 Immunohistochemically,
all the tumors are positive for CK7 and CK19, most ex-
press MUC1 and MUC6, and all are negative for CK20,
CDX2, MUC2 and MUC5AC.
Approximately one-third of intraductal tubular carci-
nomas have an associated, typically grossly invisible, in-
vasive adenocarcinoma.125 Therefore, careful sampling and
evaluation is warranted. Limited data indicate that if there
is no associated invasive carcinoma despite the histologic
indicators of a high-grade malignancy, overall outcome is
relatively favorable.
Endocrine Lineage
Cystic Pancreatic Endocrine Neoplasm. A mild cystic
change is not uncommon in pancreatic endocrine neo-
plasms, particularly in the larger tumors, but marked cys-
tic alteration is rarely seen.126–139 Although 25% of patients
with these neoplasms have hereditary multiple endocrine
neoplasia syndrome, most tumors are clinically nonfunc-
tioning. They exhibit either a unilocular cyst (Figure 15)
or a multilocular microcystic pattern. The cysts are lined
Pancreatic Cysts—Basturk et al 431
by a ragged cuff of well-preserved neoplastic endocrine Mesenchymal Lineage
cells and filled with a clear serosanguineous fluid instead
Schwannomas in the pancreas and retroperitoneum
of necrotic debris. The solid areas of the tumors reveal
tend to be cystic and are termed multicystic schwanno-
characteristic cytomorphologic features of a well-differ-
mas.148–150 They can be mistaken for MCN with denuded
entiated pancreatic endocrine neoplasm, including round,
epithelium. Some sarcomas,151 especially gastrointestinal
monotonous cells with a moderate amount of cytoplasm
stromal tumors, and other nonepithelial tumors of this re-
and distinctive nuclear chromatin pattern. Although the
gion, such as paragangliomas, also present as a cyst.5,152
tumors are large, malignant behavior is not as high as
expected, which raises the question of whether the cystic
Undetermined Lineage
component leads to an overestimation of size without con-
tributing to the effective tumor volume. Solid-Pseudopapillary Neoplasm. Solid-pseudopap-
illary neoplasm (SPN) is a distinctive tumor type in the
Acinar Lineage pancreas that often presents as a cystic mass, and for this
reason was previously referred to as solid and cystic,153,154
Acinar Cell Cystadenoma (Cystic Acinar Transforma-
solid and papillary,155 cystic and papillary, and papillary cys-
tion). This uncommon phenomenon is seen in adults
tic.156,157 It is now known that the cavities of SPNs are not
with a mean age of 47 years, and the consensus is that it
‘‘true’’ cysts, but rather represent a necrotic/degenerative
is a benign process.7,140,141 The lesion is usually discovered
process.47,158
incidentally but may produce a clinically detectable uni-
Most SPNs occur in women in their twenties or thir-
locular or multilocular cystic mass ranging from 1.5 to 10
ties158 (mean age, 28 years; male to female ratio, 1:20). The
cm in greatest diameter. Multicentricity is common, and
lesions are relatively evenly distributed throughout the
some lesions diffusely involve the pancreas. Microscopi-
gland and are often large (with a mean diameter of 9 to
cally, the cysts are lined by 1 to several layers of cytolog-
10 cm).4 Some appear grossly encapsulated. The cut sur-
ically bland acinar cells with round, basally oriented nu-
face typically shows variable appearance depending on
clei and granular, eosinophilic apical cytoplasm. The cy-
the degree of hemorrhage and necrosis. Some have a
toplasmic zymogen granules are positive for periodic
bloody appearance with only scattered, beige-tan solid tu-
acid–Schiff and resistant to diastase digestion. Immuno-
mor foci (Figure 17); others may be solid, fleshy tumors
histochemical labeling for markers of acinar differentiation
throughout. Small streaks extending to the adjacent pan-
(trypsin, chymotrypsin, and lipase) is also positive. Inter-
creas are common. Often, the cellular areas show a deli-
estingly, the tumor cells express CK7, while normal acinar
cate microvasculature that forms pseudorosettes, creating
cells are negative for this marker.
an ependymoma-like appearance (Figure 18), or they may
Acinar Cell Cystadenocarcinoma. The cystic form of
be accompanied by hyalinized or myxoid stroma. This dis-
acinar cell carcinoma (ACC) is well documented but is
tinctive pattern is created by the differential dyscohesion
extremely uncommon; only a handful of cases have been
of cells away form the vasculature, apparently related to
documented in the literature.142–146 The lesions are large
the recently documented alterations in cell adhesion mol-
(mean size, 24 cm), circumscribed, and diffusely cystic,
ecules such as E-cadherin and ␤-catenin.159–161 Clusters of
with individual locules ranging from a few millimeters to
uniform tumor cells usually have eosinophilic cytoplasm.
several centimeters. Microscopically, the cysts are lined by
Cytoplasmic vacuoles and foamy macrophages are also
single or several layers of neoplastic acinar cells, some-
common. Some cells may contain periodic acid-Schiff–pos-
times forming minute lumina within the epithelial lining.
itive and diastase-resistant intracytoplasmic eosinophilic
There is nuclear atypia with prominent single nucleoli.
hyaline globules. The nuclei are round to oval and uniform
Solid nests of neoplastic cells, areas of necrosis, and easily
(Figure 18) and have finely stippled chromatin and fre-
identifiable mitotic figures support a malignant diagnosis.
quent longitudinal grooves. The overall appearance of
Special stains and immunohistochemical markers can be
SPNs closely resembles that of pancreatic endocrine neo-
used to document the presence of acinar differentiation.
plasia, but unlike endocrine tumors, the nests in SPNs
Cystic/Intraductal Acinar Cell Carcinoma. Acinar cell
tend to be less distinct. There are irregular clusters of cells,
carcinomas are typically solid tumors; however, some
nuclei are more ovoid, and the chromatin pattern is more
ACCs show prominent intraductal growth and/or papil-
fine and diffuse rather than having a salt-and-pepper ap-
locystic patterns, which brings in the differential diagnosis
pearance.
of intraductal neoplasia147 (Figure 16), and may also have
Solid-pseudopapillary neoplasm is one of very few neo-
a cystic component. The correlation of macroscopic and
plasms for which the direction of differentiation of the
microscopic findings, histochemical and immunohisto-
neoplastic cells has yet to be established. No evidence ex-
chemical features can be helpful for distinguishing these
ists for ductal, acinar, or frank endocrine differentia-
tumors from intraductal neoplasms, especially IPMNs.
tion.47,158 Immunohistochemically, the neoplastic cells dif-
fusely and strongly express vimentin, ␣1-antitrypsin,
Endothelial Lineage
CD56, and neuron-specific enolase; meanwhile, expression
Lymphangioma. Lymphangiomas may also present as of epithelial markers (AE1/AE3, CAM 5.2) can be focal or
pancreatic and peripancreatic cystic masses that may mea- weak. Cells are commonly positive for synaptophysin and
sure as much as 25 cm.6 Histologically, the lesions are neuron-specific enolase; however, staining for chromo-
lined by endothelial cells, as demonstrated by immuno- granin, the most specific endocrine marker, is typically
histochemical labeling for endothelial markers (CD31, negative or only very focal. Recently, CD10 expression162
CD34, or D2-40). Labeling for epithelial markers (cytoker- was found to be almost uniformly present, and c-kit
atins) is consistently negative. The stroma may contain (CD117)163 and FLI-1164 expressions were detected in many
smooth muscle cells, aggregates of mature lymphocytes, SPNs. Moreover, the neoplastic cells consistently express
and foamy histiocytes. progesterone receptors and also the beta form of estrogen
432 Arch Pathol Lab Med—Vol 133, March 2009 Pancreatic Cysts—Basturk et al

Figure 18. Solid-pseudopapillary neoplasm. Prominent pseudopapil-
lary growth pattern is present (hematoxylin-eosin, original magnifica-
tion ⫻10).
Figure 19. Nuclear and cytoplasmic ␤-catenin staining in solid-pseu-
dopapillary neoplasm (original magnification ⫻40).
receptors,165 suggesting a role for hormones in the evolu-
tion of these neoplasms.
The only known, generally consistent genetic aberration
is a mutation in exon 3 of the ␤-catenin gene, which results
in nuclear ␤-catenin staining166,167 (Figure 19). The expres-
sion of CD56, progesterone receptor, and FLI-1, all located
on chromosome 11q, points to the fact that chromosome
11q may be involved in a translocation or harbor a mu-
tation that results in the expression of some or all of these
proteins in SPNs.164,168
Yet another peculiar aspect of SPNs is their clinical be-
havior.158 More than 80% of SPNs are cured by surgical
resection. Ten percent to 15% of affected patients may
have metastases to the liver or peritoneum, rarely to the
lymph nodes, but even patients with metastases are often
cured by surgical resection. There do not appear to be any
reliable histopathologic criteria to distinguish SPNs that
can metastasize from those that do not.169 Necrosis, mitotic
activity, perineurial invasion, and invasion to adjacent
pancreas have all failed to prove any correlation with met-
astatic behavior. However, recently, 2 cases of anaplastic
Arch Pathol Lab Med—Vol 133, March 2009
transformation with aggressive clinical course were re-
ported.170
Other
Mature Cystic Teratoma. Mature cystic teratomas are
exceedingly rare neoplasms in the pancreas and, as in oth-
er sites, are recognized by the presence of mature tissue
elements from three germlines.171 Those reported in the
pancreas are predominantly monodermal teratomas with
only ectodermal derivatives and are referred to as der-
moid cysts.172 They are usually seen in younger patients
(in their second or third decade of life) and are difficult
to distinguish from lymphoepithelial cysts. The presence
of sebaceous glands or hair follicles is more typical for
dermoid cysts. Thorough macroscopic and microscopic
examinations are warranted for areas of solid, fleshy, or
necrotic tissue to assess the presence of a carcinoma aris-
ing within the teratoma.
CONGENITAL CYSTS
Duplication (Enterogenous) Cysts
Very rarely, congenital cysts of foregut derivation may
also occur adjacent to the pancreas.173–177 They may cause
pancreatitis and often present in childhood. Most are
found in the head of the pancreas and some communicate
with the pancreatic ducts. They are lined by a variety of
epithelia including squamous, gastric, small intestinal, re-
spiratory (bronchogenic), or simple ciliated epithelium.178–180
The wall of the cyst contains bundles of smooth muscles.
We have also seen 2 examples with ciliated epithelium and
one of these had an associated high-grade papillary ade-
nocarcinoma with pancreatobiliary-type features.
Duodenal Diverticula
Duodenal diverticula are outpouchings of the duode-
num, and the lumen of the cyst (as well as mucosa) is
contiguous with the duodenal lumen. These rare lesions
may coexist with other anatomic abnormalities such as
choledochocele, annular pancreas, or polysplenia syn-
drome.181 Most are found at or near the ampulla of Vater
and extend distally into the lumen of the duodenum. The
common bile duct and pancreatic duct usually empty into
the diverticulum, and then a second orifice in the diver-
ticulum allows drainage of the duct contents into the in-
testine.182 Patients may have complications such as inter-
mittent duodenal obstruction, bleeding, abdominal pain,
or occasionally pancreatitis.182 In fact, some complications
may result from a paraduodenal wall cyst associated with
paraduodenal pancreatitis, as discussed above. Duodenal
diverticula should be distinguished from foregut cysts,
which do not communicate with the duodenal lumen.
Others
Patients with polycystic kidney disease,183 both adult
and infantile types, and with medullary cystic kidneys184
may have cystic lesions in the pancreas. Cystic fibrosis
may lead to the cystic dilatation of the pancreatic ducts,
generally not clinically detectable, by causing intraluminal
impaction.185,186
Cystic transformation of the pancreas has also been de-
scribed in a variety of congenital syndromes187 including
Ivemark syndrome, trisomy 13 or 15, Meckel-Gruber syn-
drome, Elejalde syndrome, glutaric aciduria, chondrodys-
plasia, short-rib polydactyly syndrome (Jeune syndrome
Pancreatic Cysts—Basturk et al 433
and Saldino-Noonan type),188 and others with no specific
name.189
Familial fibrocystic pancreatic atrophy is an interesting
phenomenon described in a family with pancreatic cancer
from Seattle, Washington, and is characterized by a lobu-
locentric pancreatic atrophy associated with fibrocystic
(microcystic) changes and endocrine cell proliferation.190
Other families with pancreatic cancer may also show sim-
ilar cystic changes.191

MISCELLANEOUS CYSTS
Lymphoepithelial Cyst
Lymphoepithelial cysts are multilocular (60%) or uni-
locular (40%) cystic lesions mostly seen in older adults
(mean age, 56 years) with a male predominance (male to
female ratio, 4:1).172,192 They can be seen in any component
of the pancreas and often project into the peripancreatic
tissues. The cyst content may vary from serous to cheesy/
caseous appearing. If the cyst content is removed, the lin-
ing of the cysts is observed to be smooth or finely gran-
ular. The cyst wall and trabeculae are usually 1 to 3 mm
thick.
Microscopically, the cysts are lined by well-differenti-
ated stratified squamous epithelium (Figure 20), usually
with keratinization. In some areas, the lining may appear
more transitional, and in others, appear flat, cuboidal, and
focally denuded. Rarely, there are scattered sebaceous and
mucinous gobletlike cells (Figure 20). A recent report in-
dicates that goblet cells may be more common than pre-
viously appreciated.193 Extensive sebaceous or mucinous
elements are more suggestive of a teratoma. The squa-
mous epithelium is surrounded by a band of dense lym-
phoid tissue (Figure 20) composed of mature T lympho-
cytes. Reactive germinal center formation is common. In
some examples, the lymphoid tissue has a thin capsule
and a subcapsular sinus, suggesting that the process may
have arisen in a peripancreatic lymph node. Epithelioid
granulomas, collections of foamy histiocytes, cholesterol
clefts, and fat necrosis may be present. The adjacent pan-
creas is usually unremarkable. Lymphoepithelial cysts of
the pancreas do not appear to be associated with condi-
tions related to lymphoepithelial cysts of the parotid gland
or head and neck region, such as autoimmune disorders,
human immunodeficiency virus infection, lymphoma, or Figure 20. Lymphoepithelial cyst. The cyst is lined by squamous-type
carcinoma, and their origin is uncertain. epithelium with scattered goblet cells surrounded by a distinct band of
lymphoid tissue (hematoxylin-eosin, original magnification ⫻20).
Squamoid Cyst of Pancreatic Ducts Figure 21. Epidermoid cyst within intrapancreatic accessory spleen.
The cyst has a thin squamous epithelial lining surrounded by splenic
Squamoid cysts of pancreatic ducts are relatively small, red and white pulps (hematoxylin-eosin, original magnification ⫻4).
unilocular cysts with a median size of 1.5 cm; however,
some are large, may have high CEA levels, and thus un-
dergo resection with the clinical impression of being an
IPMN. The lesions have variable lining ranging from at- the squamous/transitional nature of the epithelial lining
tenuated, flat, nonstratified squamous to transitional to (p63 nuclear expression, which is not otherwise detected
mucosal-type stratified squamous epithelium without cor- in other components of the pancreas),195 and also suggests
nified layer or parakeratosis. They typically contain dis- a relationship to the centroacinar/intercalated duct sys-
tinctive muco-proteinaceous acidophilic acinar secretions tem (MUC1 and MUC6 expression).
that form concretions, thus confirming communication
with the acinar system. The wall of the cyst is composed Epidermoid Cysts Within Intrapancreatic
of a thin band of fibrous tissue devoid of any lymphoid Accessory Spleen
tissue. Neither acute nor chronic inflammation is a feature These cysts are extremely rare. All the reported cases
of this lesion.194 Clinical, macroscopic, and microscopic have occurred in the tail of the pancreas,172,196–198 where
findings indicate that squamoid cysts of pancreatic ducts accessory spleens are not too uncommon. They are seen
represent cystic dilatation of the native ducts rather than in adults (mean age, 37 years), and occur equally in men
a de novo cyst formation. The immunophenotype confirms and women. The lesions can be unilocular or multilocular
434 Arch Pathol Lab Med—Vol 133, March 2009 Pancreatic Cysts—Basturk et al
and are lined by attenuated keratinizing squamous cells,
surrounded by unremarkable splenic tissue (Figure 21).
Cystic Hamartoma
The haphazard distribution of the abnormal pancreatic
elements in chronic pancreatitis may be reminiscent of
hamartoma. Therefore, many of the cases reported in the
literature as hamartomas of the pancreas appear to rep-
resent subsets of chronic pancreatitis; however, rare le-
sions exist that would qualify as true hamartomas in the
pancreas, some of which are cystic.199,200 These form a
sharply delimited lesion and are composed of haphazard-
ly distributed cystic ductal elements lined by cuboidal to
flattened epithelium, surrounded by well-differentiated
acini embedded in fibro-inflammatory stroma. Scattered
ill-formed clusters of endocrine cells composed predomi-
nantly of pancreatic-polypeptide–producing cells are also
present.
Endometriotic Cysts
Endometriotic cysts,201,202 some associated with massive
hemorrhage,203 have been rarely reported in the pancreas.
As in other locations, they occur in females of reproduc-
tive age and are lined by endometrial epithelium and are
accompanied by endometrial stroma.
Secondary Tumors
Rarely, metastatic neoplasms can exhibit cystic
change.152,204 We have seen examples of metastatic ovarian
and renal cell carcinomas in the pancreas that presented
as cystic masses.
CONCLUSIONS
In contrast to solid tumors of the pancreas, most of
which are invasive ductal adenocarcinomas with dismal
prognosis, most of the cystic neoplasia in this organ are
either benign tumors or low-grade malignancies with rel-
atively indolent behavior. However, their differential di-
agnosis can be quite challenging. It is important to rec-
ognize the mucinous lesions because they often exhibit an
adenoma-carcinoma sequence. Therefore, careful macro-
scopic examination and thorough sampling of these le-
sions are mandated, not only to rule out carcinoma, but
also for proper subclassification of these tumors. For ex-
ample, gross examination and dissection play an impor-
tant role in verifying the radiologic impression that a giv-
en IPMN falls into the branch or main duct type. Along
the same lines, it is important to examine a cystic lesion
thoroughly before it can be diagnosed as a ‘‘pseudocyst’’
because SPNs and even ordinary ductal adenocarcinomas
or endocrine neoplasia may present as a pseudocyst.
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