1. Cystic tumors of the pancreas
By
Ihab Samy Fayek
Lecturer of Surgical Oncology
National Cancer Institute
Cairo University
2012
2. Cystic Lesions of the Pancreas
• Congenital Pancreatic Lesions
• Inflammatory and Infectious Pancreatic Lesions
• Exocrine Pancreatic Lesions
• Endocrine Pancreatic Neoplasms
• Parapancreatic Neoplasms
3. Congenital Pancreatic Lesions
• Most congenital pancreatic cysts are multiple.
• Almost all are associated with underlying
congenital diseases that primarily affect other
organ systems.
• Solitary congenital cysts are rare.
4. Multiple Pancreatic Cysts
• Autosomal dominant polycystic kidney disease
(Extrarenal cysts are most common in the liver).
• In decreasing incidence, cysts also occur in the
pancreas, spleen, endometrium, ovaries, seminal
vesicles, epididymis, and thyroid gland.
• Pancreatic cysts vary from microscopic to several
centimeters in diameter and have an epithelial
lining of cuboidal to flat cells.
6. • Von Hippel-Lindau disease is an autosomal
dominant condition characterized by central
nervous system and retinal hemangioblastomas,
visceral cysts, pheochromocytomas, and renal cell
carcinoma.
• Pancreatic cysts are found in up to 75% of cases at
autopsy but on only approximately 50% of imaging
studies.
• In some members of families with this syndrome,
pancreatic lesions are the first or only
manifestation of the disease, with a delay of years
before the development of lesions at other sites.
8. • Cystic fibrosis :The most common pancreatic
abnormality found on imaging studies is fatty
replacement of the pancreas, but calcifications and
cysts may also be found.
• True epithelium-lined cysts probably develop as a
result of inspissated mucin that obstructs
pancreatic ducts.
• Cysts can be single or multiple;microscopic, but
infrequently cysts up to several centimeters in
diameter .
• Rarely, cysts are so numerous and large that most
of the pancreas is replaced (cystosis)
9. Solitary Pancreatic Cysts
• True cysts, most often found in infants, result from
anomalous development of pancreatic ducts and range
from microscopic to several centimeters in diameter. They
can be unilocular or multilocular, have an epithelial lining,
and contain serous fluid.
• Lymphoepithelial cyst : fewer than 40 lymphoepithelial
cysts have been reported in the literature. Most occur in
middle-aged to elderly men. Lesions vary from 1 to 17 cm,
have a squamous lining, and contain keratinous material.
11. Inflammatory and Infectious Pancreatic Lesions
Pseudocyst:
• Panceatic and parapancreatic fluid collections are most often
complications of pancreatitis resolve spontaneously
pseudocysts
• Wall consists of fibrous tissue (unlike true cysts,
lymphoepithelial cysts, and most cystic neoplasms, a
pseudocyst has no epithelial lining)
• When cysts are chronic, the cyst wall can calcify.
14. • On imaging studies, a pseudocyst may be
indistinguishable from a cystic neoplasm.
• The amylase level of pseudocyst fluid is almost
always much higher than the fluid in cystic
neoplasms, but cystic neoplasms with high levels
of amylase have been reported.
• Extrapancreatic extension or location of a
pseudocyst on imaging studies is evidence against
a cystic neoplasm.
16. Abscess:
• A serious complication of pancreatitis.
• Often suspected when gas is present in a
fluid collection.
• Pancreatic gas, however, is not
pathognomonic of an abscess.
1. A pancreatic—enteric fistula
2. A previous internal pseudocyst drainage
3. A previous drainage of the pancreas by a
pancreatico-jejunostomy can all result in
gas in the pancreatic bed.
19. Mucinous Adenocarcinoma (Colloid Carcinoma)
• An uncommon variant of adenocarcinoma.
• This neoplasm produces a large volume of mucin that
results in a cystic appearance.
• The prognosis is just as poor as for an adenocarcinoma that
has a typical appearance and histology.
• Tumoral calcification, inspissated mucin or tumor
obstructing bile ducts, and pseudomyxoma peritonei have
all been reported in association with mucinous
adenocarcinomas.
20. • Mucinous adenocarcinoma in 66-year-old woman. CT scan shows well-defined
cystic component of mass in tail of pancreas. Note liver metastasis (arrow).
21. Microcystic Adenoma (Cystadenoma, Serous Adenoma,
Glycogen-Rich Adenoma)
• Benign neoplasm, which constitutes 1-2% of all pancreatic neoplasms ; 32-
39% of all cystic neoplasms.
• Most frequent in women in the seventh decade.
• Female-to-male ratio, 2:1
• One of the pancreatic lesions found in von Hippel-Lindau disease.
• Can be single or multiple and can involve any part of the pancreas.
• Most are smaller than 2 cm in diameter, but larger cysts do occur.
• A minority of lesions have a central stellate scar, with or without calcification
(within a lesion consisting of cysts smaller than 2 cm diagnostic)
• Asymptomatic patient with solitary cyst < 2 cm. follow-up without tissue
confirmation.
• Cysts larger than 2 cm. fine-needle aspiration biopsy or surgery.
• Accurate diagnosis of microcystic adenomas, based on imaging studies is well
reported.
• Misdiagnosing a potentially malignant mucinous cystic tumor as a benign
microcystic adenoma is a serious problem that was reported
22. • Contains intracellular glycogen but no mucin.
• This glycogen content is a feature that is central in using percutaneous aspiration
biopsy to differentiate this neoplasm from a mucinous cystic tumor that is
characterized by mucin content.
• Synchronous microcystic adenoma and adenocarcinoma have been reported.
23. Microcystic adenoma in 76-year-old woman. CT scan shows large low-
attenuation, well-demarcated mass in head of pancreas. Note characteristic
stellate scar.
24. Microcystic adenoma in 85-year-old woman. Unenhanced CT scan shows large
hypodense mass with punctate central calcification in body of pancreas.
25. Microcystic adenoma in 85-year-old woman. CT scan after IV administration of
contrast material shows enhancing walls of cysts.
• Microcystic adenoma in 85-year-old woman. CT scan after IV administration of
contrast material shows enhancing walls of cysts.
26. Microcystic adenoma and
adenocarcinoma in 97-year-old woman
with multiple liver metastases. CT scans
show microcystic adenoma in pancreatic
head that consists of small cysts
Synchronous solid adenocarcinoma
(arrow) in tail of pancreas (the same
patient)
27. Mucinous Cystic Tumor (Cystadenoma, Cystadenocarcinoma,
Macrocystic Adenoma)
• Most common in women in the fifth and sixth decades. 10-45% of all cystic
neoplasms
• Female-to-male ratio is 9:1
• Some lesions are frankly malignant, but all are considered potentially malignant.
• Survival is related to the extent of invasion, but overall the prognosis after
surgery is better than for adenocarcinoma.
• Ten-year survival rates near 50% have been reported after resection of invasive
malignant mucinous cystic tumors.
• Most lesions are located in the pancreatic body or tail; single or multiple.
• Most, but not all cysts, are larger than 2 cm in diameter.
• Lesions with large cysts and typical peripheral curvilinear cyst wall
calcification can be confidently diagnosed, but mucinous cystic tumors have
often been mistaken for pseudocysts, and, to a lesser extent, lesions have been
diagnosed as microcystic adenomas or other cystic tumors.
• Percutaneous aspiration biopsy has been used to identify characteristic mucin,
which is not present in pseudocysts or microcystic adenomas.
28. Mucinous cystic tumor in 52-year-old woman. CT scan shows unilocular cystic
pancreatic mass that had slightly decreased in size over 3-month interval, but
patient had no history of pancreatitis, gallstones, or abdominal trauma. Pancreatic
tail was resected, and mucinous cystic tumor was diagnosed.
29. Mucinous cystic tumor in 44-year-old woman. CT scan shows cystic mass
containing faint septa and punctate peripheral calcifications.
30. Mucinous cystic tumor (cystadenocarcinoma) in 41-year-old woman. CT scan
shows irregularly thickened wall of cystic mass that contains soft-tissue nodules.
31. Mucinous cystic tumor (cystadenocarcinoma) in 72-year-old woman. CT scan shows
large cystic mass. Note irregularly thickened wall, nodular excrescences, and smaller
peripheral cysts (arrows)
32. Intraductal Papillary Mucinous Tumor (Ductectatic Cystadenoma,
Cystadenocarcinoma, Ductectatic Mucinous Tumor)
• Equal in men and women.
• Most patients present in the sixth and seventh decades. 21-33% of all cystic
neoplasms of the pancreas.
• Symptoms of abdominal pain are accompanied by an elevated serum amylase
level, so these patients are frequently thought to have pancreatitis.
• This neoplasm is a low-grade, slowly growing malignancy with a much better
prognosis than adenocarcinomas.
• The overall 5-year survival rate is approximately 60%.
• Imaging studies most often show dilatation of the main pancreatic duct and side
ducts in the pancreatic head and uncinate process. The ducts may be so dilated
that they resemble cysts.
• ERCP :1-Inspissated mucin can produce filling defects in the dilated ducts.
2-Abundant mucinous secretions issuing from a bulging papilla of Vater
33. Intraductal papillary mucinous tumor in 69-year-old woman. Neoplasm involves both main and
pancreatic head side ducts. ERCP image shows dilated main pancreatic duct and marked dilatation of
side branches in pancreatic head. The patient's clinical diagnosis was chronic pancreatitis.
34. Intraductal papillary mucinous tumor in 69-year-old woman. Neoplasm involves both main
and pancreatic head side ducts. CT scan shows cystlike dilated branches of side ducts
(arrows) in pancreatic head.
35. Intraductal papillary mucinous tumor in 57-year-old man. Neoplasm is isolated to main
pancreatic duct. CT scan shows dilated main duct (arrows) in pancreatic body and tail.
36. Solid Papillary Epithelial Neoplasm (Solid Pseudopapillary
Tumor, Papillary Cystic Tumor)
• 1% of pancreatic neoplasms and < 10% of all pancreatic cystic neoplasms.
• High incidence in young women (mean, 24 years old) is especially noteworthy.
• Low-grade malignancy, and resection is usually curative.
• Distinctive clinical features include eosinophilia and polyarthralgia.
• Solid?, but they often present as thick-walled cysts on CT and sonography.
• CT will show peripheral or central stippled calcification in some lesions.
• Tumor vascularity is moderate.
37. Solid papillary epithelial neoplasm in 13-year-old girl. CT scan 1 day after blunt
abdominal trauma shows sharply defined water-density mass deforming pancreatic
head. Before trauma and this serendipitous discovery, patient was asymptomatic.
39. Giant Cell Tumor
• This rare neoplasm has two histologic types: pleomorphic
and osteoclastic.
• The pleomorphic type is highly malignant and has a poor
prognosis, similar to that of adenocarcinoma.
• The osteoclastic type, which has the same histology as
giant cell tumors of the bone, has a better prognosis.
• Most lesions are large and highly vascular.
• A cystic appearance is the result of central necrosis.
40. Giant cell tumor, osteoclastic type, in 66-year-
old woman. Unenhanced T1-weighted spin-
echo MR image shows lobulated, low-signal-
intensity mass (arrows) protruding from tail
of pancreas.
Giant cell tumor, osteoclastic type, in 66-
year-old woman. Unenhanced T2-
weighted inversion-recovery MR image
shows high-signal-intensity cystic
component of lesion.
41. Endocrine Pancreatic Neoplasms
• Insulinomas, glucagonomas, gastrinomas, and nonfunctioning endocrine tumors
can all present as cystic lesions.
• Isolated or associated with the multiple endocrine neoplasia syndrome type 1
(MEN 1), which is characterized by the triad of parathyroid, pituitary, and
pancreatic lesions (3P’s).
• Solitary, multiple, or diffuse; different histologic types may occur in the same
patient.
• Gastrinomas, however, are an uncommon pancreatic neoplasm in MEN 1
because most gastrinomas are located in the duodenum rather than in the
pancreas.
42. Insulinoma
• Insulinoma is the most common functioning islet cell tumor.
• The clinical triad leading to the diagnosis is fasting hypoglycemia,
symptoms of hypoglycemia, and immediate relief of symptoms after the
administration of IV glucose.
• Symptoms and signs are caused by both hypoglycemia and the
secondary release of catecholamines and include palpitations,
headache, confusion, pallor, sweating, slurred speech, and coma.
• Despite this striking list of symptoms, the clinical presentation is often
insidious.
• Fasting hypoglycemia with no decrease in insulin level is confirmatory.
• Approximately 10% of insulinomas are multiple, 10% are malignant,
and 10% of patients have hyperplasia rather than neoplasia.
• Most insulinomas are small and hypervascular. Some contain
calcification.
• Malignant tumors tend to be large. Cystic lesions are rare.
43. Cystic insulinomas in 36-year-old woman with multiple endocrine neoplasia type 1. CT
scan shows small cystic masses (arrows) in body and tail of pancreas. Patient presented
with primary hyperparathyroidism.
44. Gastrinoma
• Gastrinoma is the second most common functioning islet cell tumor.
• Most are sporadic and associated with the Zollinger-Ellison syndrome.
• The initial manifestations are most often in young adults who have peptic ulcer
disease that suggests the diagnosis when ulcers are recurrent, intractable,
multiple, or in unusual locations.
• Diarrhea due to the large volume of hydrochloric acid and a direct effect of
gastrin on the small bowel is common.
• The diagnosis is confirmed by increased serum gastrin concentrations.
• Gastinomas are frequently multiple, extrapancreatic, difficult to locate, and
malignant.
• Hepatic metastases are associated with a poor prognosis, whereas patients with
metastases isolated to lymph nodes often have long-term survival.
• The tumor size is variable, but pancreatic lesions average 3-4 cm. The lesions
are often hypervascular, so they may be visible on arterial phase CT and
angiography.
• Rare cystic lesions have been reported.
45. Glucagonoma
• Most lesions are malignant and present with equal incidence in middle-aged
men and women.
• Most patients present with a necrolytic migratory rash and various other
elements of the “glucagonoma syndrome” including diabetes mellitus,
stomatitis, diarrhea, anemia, and weight loss.
• The characteristic rash involves the lower extremities and the perineum, begins
with erythema and progresses to coalescent blisters and pustules, and is pruritic.
• An elevated plasma glucagon level establishes the diagnosis, but there are often
increased blood levels of other hormones, including insulin, serotonin, and
gastrin.
• Approximately 50% of patients survive at least 5 years after diagnosis.
• Tumor size is variable, but most are large and have metastasized at the time of
diagnosis.
• Most are located in the distal pancreas and are vascular.
• Tumors may be solid or contain central low-attenuation areas on CT. Cystic
lesions are rare.
46. Cystic glucagonoma in 34-year-old woman. CT scan shows large cystic mass in tail of
pancreas. Patient had diabetes and presented with characteristic eczematous dermatitis
termed “necrolytic migratory erythema.”
47. Nonepithelial Neoplasms of the Pancreas
1-Sarcoma
• Most sarcomas originate in the parapancreatic tissues and secondarily invade the
pancreas. Primary origin in the pancreas is rare.
• Leiomyosarcoma is the most common cell type, but only 21 cases have been
reported.
• These neoplasms are most often poorly differentiated and are diagnosed when
they are at an advanced stage.
• The prognosis is similar to that of adenocarcinoma of the pancreas.
• The gross morphology ranges from solid to cystic when central necrosis is
present.
• The solid lesions have an appearance similar to that of adenocarcinoma,
whereas necrotic lesions can stimulate mucinous cystic tumors.
48. Leiomyosarcoma in 44-year-old woman. CT scan shows cystic mass with well-defined wall in
body of pancreas. Note dilatation of pancreatic duct (arrow).
49. 2-Metastases to the Pancreas
• When lymphoma was excluded, breast, lung, melanoma, and
gastrointestinal tract are the most common primary sites.
• Several series of metastases identified by imaging studies have reported
renal cell carcinoma to be the most common primary neoplasm. Some of
these metastases developed many years after the initial diagnosis and
treatment.
• Cystic metastases can be the result of central necrosis or cystic
degeneration.
• The imaging characteristics of pancreatic metastases tend to mirror the
characteristics of the primary neoplasm.
50. Metastatic melanoma in 61-year-old man. CT scan
shows lobulated cystic mass in body of pancreas.
51. Metastatic renal cell carcinoma in 70-year-old woman. CT scan shows large cystic mass with
irregularly thickened wall in pancreatic body and tail. Note similarity to renal cell carcinoma
of right kidney. Pancreatic mass proved to be metastatic lesion.
52. Parapancreatic Neoplasms
• Lymphomas are the most frequent parapancreatic neoplasm and, in some cases
it may not be possible to differentiate parapancreatic lymphadenopathy from a
primary lesion in the pancreas.
• Lymphangiomas are most often homogeneous, thin-walled, fluid-filled cysts,
but they may have septa, thick walls, calcification, and internal debris. These
lesions have an epithelial lining but do not contain keratin, which differentiates
them from lymphoepithelial cysts.
• Paragangliomas are 10% extraadrenal. Most often located near the origin of
the inferior mesenteric artery in Zuckerkandl's organs or near the kidney.
Many produce hormones, and approximately 10% are malignant.
• Retroperitoneal cystic teratomas have been reported in children and young
adults. Reflects variable cystic, solid, fat, and calcific content.
53. Parapancreatic paraganglioma in
64-year-old man. CT scan shows
parapancreatic mass with cystic
component that deforms
pancreatic head and simulates
intrinsic pancreatic lesion.
Parapancreatic paraganglioma in
64-year-old man. CT image 2 cm
inferior to A shows same
findings.
54. Parapancreatic metastasis from
testicular carcinoma in 21-year-old
man. CT scan shows large low-
attentuation lymph node metastasis
displacing pancreatic head
anteriorly and inferior vena cava
laterally.
Parapancreatic retroperitoneal cystic
teratoma in 8-year-old girl. CT scan
shows heterogeneous lesion
displacing pancreas and kidney and
containing large central calcification,
peripheral water-attenuation cystic
areas, and small focus of fat (arrow).
57. D.D. of microcystic lesions
“Grandmother Lesion”
• Serous cystadenoma
(Only lesion included in this category)
• May grow up to approx 4 mm/year
• 70% cases demonstrate:
– Polycystic/microcystic pattern
– Collection of cysts (>6)
– Range: few mm – 2 cm
– External lobulations
– Enhancing septa, walls
• 30% demonstrate fibrous central scar +/- stellate calcifcation
• Other variants (macrocystic + oligocystic)
59. D.D. of cystic lesions with solid component
“Daughter cyst”
• Mucinous cystic neoplasms
• IPMNs
• Islet cell tumor
• Solid pseudopapillary tumor (SPEN)
• Adenocarcinoma
• Metastasis
N.B. All are malignant or have a high malignant potential
Surgical management
60.
61. • Age & Gender
– “Daughter Lesion”: SPEN
– “Mother Lesion”: Mucinous cystic
– “Grandmother Lesion”: Serous cystadenoma
• Location
– Head/neck for serous & side branch IMPN
– Body/tail for mucinous cystic neoplasm
• Calcification
– Peripheral in mucinous cystic
– Central in serous cystadenoma
• Mural Nodularity (enhancement = neoplasm)
• Duct communication (narrow neck) favors IPMN
62.
63. Fine-Needle Aspiration Biopsy of Cystic Pancreatic Lesions
• A simple, cost-effective procedure with relatively low risk.
• Analysis of the aspirate includes 1-cytology; 2-viscosity; and 3-the presence of
mucin and glycogen, enzymes (amylase and lipase), and antigenic tumor
markers.
• The fluid in most pseudocysts contains high levels of amylase, microcystic
adenomas contain intracellular glycogen, and mucinous cystic tumors stain
positively for mucin.
• Fewer data are available for the accuracy of tumor markers, but some reports are
promising.
• Several studies report high sensitivity in differentiating pseudocysts from cystic
neoplasms and microcystic adenomas from mucinous cystic tumors on the
basis of the results of fine-needle aspiration.
69. Putting in mind “ American Society of GI endoscopy”
ASGE Guidelines 2005:
• Cystic lesion may represent malignancy.
• EUS findings are not accurate enough.
• Cytology – low sensitivity, high specificity.
• Markers – may provide useful information but cannot
provide a definitive diagnosis or determine malignancy.
• FNA – 2-3% risk of pancreatitis.
• Proph Abx for EUS-FNA or ERCP.
• ERCP : -Bx should be performed whenever possible
-A pancreatic orifice exuding mucous is specific
but not sensitive for IPMN
• “There are currently no established endoscopic therapies
for cystic neoplasms of the pancreas”.
70. Incidence of cystic neoplasm - 0.7%
19% increase in size over 16 months
Gender and size did not predict malignancy
Symptoms predicted malignancy (60%)
Age >70 predicted malignancy (60%)
—Autosomal dominant polycystic kidney disease in 41-year-old man. CT scan shows small cyst (arrow) in body of pancreas. Note enlarged polycystic kidneys and small hepatic cysts.
—Von Hippel-Lindau disease in 37-year-old man. CT scan shows small pancreatic cysts (arrows), multiple renal cysts, and renal cell carcinoma.
—Lymphoepithelial cyst in asymptomatic 54-year-old woman. CT scan shows nonspecific 2-cm cyst (arrow) in tail of pancreas. Diagnosis was made after tail was resected.
—Pseudocyst in 10-year-old girl. CT scan shows posttraumatic thin-walled pseudocyst of pancreas.
—Calcified pseudocyst in 44-year-old man with chronic pancreatitis. CT scan shows pseudocyst with calcified wall in head of pancreas.
—Pancreatic abscess containing gas in 54-year-old man. CT scan shows large fluid collection containing gas bubbles in pancreatic bed due to abscess complicating acute pancreatitis. Note infiltration of peripancreatic fat and calcified gallstones.
