A brief presentation on cystic neoplasms of pancreas. SOLID PSEUDOPAPILLARY TUMOR NEOPLASM: Relatively rare entity initially described by Frantz in 1959. Represent up to 3% of all pancreatic tumors and 6% to 12% of pancreatic cystic neoplasms. Designated as SPT by the World Health Organization in 1996, several other names, including Frantz tumors, Hamoudi tumors, and papillary cystic neoplasm.

1. CYSTIC NEOPLASM OF
PANCREAS
DR MAHESH MANI ADHIKARI
GENERAL SURGERY RESIDENT
DHULIKHEL HOSPITAL KUSMS

2. CASE DETAILS
• 32Y Female
• C/C
• Pain abdomen since 5 months
• Dull aching, gradual on onset with no radiation,
diffuse but more on epigastric region.
• CECT Abd and Pelvis
• Enhancing soft tissue attenuating isodense lesion in
pancreatic body possibility of endocrine tumor can be
considered.
• EUS
• Pancreatic body mass; D/D NET, Adeno Ca.
• FNA: SOLID PSEUDOPAPILLARY TUMOR OF PANCREAS

3. • Patient underwent Enucleation Of The Tumor with intraoperative
frozen section
• INTRAOP FINDINGS:
1. Solid well circumscribed mass 3 x 3 cm in the body of pancreas
2. Tumor was not involving main pancreatic duct and pancreatic tissue were normal
3. Liver, omentum, spleen and peritoneum were normal
4. Intraoperative frozen section was negative for malignancy

4. • Postoperative period uneventful.
• Drain Amylase: 600000
• Discharged on 8th POD.

5. • HPE:
• GROSS: Single piece of grey white nodular tissue 4
x 3x 2 cm, cut section- solid grey-white to
yellowish area
• MICRO:
• Partially capsulated tissue
• Tumor cells arranged in sheets, chords and
pseudopapillary pattern with central
fibrovascular core.
• Tumor of composed of uniform cells with
eosinophillic vacuolated cytoplasm and round
to oval nuclei with granular chromatin and
inconspicious nucleoli. Occasional nuclear
grooves are seen. Intervening stroma shows
hyalinization and myxoid changed along with
hemorrhage and collection of foamy cells.
Increased mitotic figures, necrosis and marked
pleomorphism not seen.
• CONSISTENT WITH SOLID-PSEUDOPAPILLARY
NEOPLASM OF PANCREAS.

6. SOLID PSEUDOPAPILLARY TUMOR NEOPLASM
• Relatively rare entity initially described by Frantz in 1959,
• Represent up to 3% of all pancreatic tumors and 6% to 12% of pancreatic cystic
neoplasms
• Designated as SPT by the World Health Organization in 1996
• Several other names, including Frantz tumors, Hamoudi tumors, and papillary
cystic neoplasm

7. CLINICAL FEATURES
• Young women
• average age at presentation in the early to mid-20s
• Pain or abdominal mass is the most common presenting clinical symptom or sign
• Other presentations include nausea, vomiting, fever, weight loss, and jaundice
• ~15% - may be asymptomatic
• May arise anywhere within the pancreas but are reported most commonly in the
tail or head.

8. CT IMAGING
• Large - mean diameter 5.4 cm,
• Heterogeneously enhancing lesions
with solid and cystic components, and
they frequently demonstrate peripheral
enhancement and central calcification
MRI
• Low signal intensity on T1-weighted
images
• High signal intensity on T2-weighted
images
Solid pseudopapillary tumor of the pancreas with solid and
cystic characteristics (long arrow) and central calcification (short
arrow and arrowheads)

9. ULTRASOUND IMAGING
• Hypoechoic or isoechoic lesion.
• Range from being completely cystic to completely
solid
• Cystic portion - not a true cyst, secondary to
necrotic degeneration
D/D:
• Other cystic neoplasms including
• Mucinous neoplasms or serous cystadenomas,
• Intraductal papillary mucinous neoplasms
• Cystic degeneration of a typically solid neoplasm,
such as a pancreatic neuroendocrine tumor or
ACC

10. • FNA biopsy may be useful when routine imaging is inconclusive and diagnostic
uncertainty exists
A. CT image of solid pseudopapillary tumor (SPT) of pancreatic
head (white arrow). B Gross tumor appearance after
pancreatoduodenectomy

11. HISTOLOGIC FEATURES
• Presence of solid cellular hypervascular regions
without gland formation, and
• Presence of branching papillary fronds with sheets
and degenerative pseudopapillae
• Cells have eosinophilic granules within the nuclei,
which are typically grooved
Solid pseudopapillary neoplasm. The
pseudopapillae are formed by a cuff of cells
adherent to a central small vessel. Many of the
cells contain large eosinophilic globules, and the
nuclei have longitudinal grooves.

12. • Tumor typically expresses
• Vimentin, progesterone receptors, CD10, CD117, and some of the
neuroendocrine markers, CD56
• Chromogranin, the most specific neuroendocrine marker, is negative, which is
important for the differential diagnosis with PanNETs,
• Pancreatic enzymes are not expressed
• Often stain positive for progesterone receptors, whereas estrogen receptor
positivity is more variable.

13. TREATMENT
• Unpredictable but real metastatic potential
• Surgical resection is recommended for all patients with localized SPT.
• Regardless of size, most lesions are usually amenable to complete resection.
• Pancreaticoduodenectomy or distal pancreatectomy can be performed with en
bloc resection of involved adjacent organs when indicated
• Other available surgical approaches
• Distal pancreatectomy spleen-preserving
• Pylorus-preserving pancreaticoduodenectomy (PD/PPPD)
• Duodenum preserving pancreatic head resection (DPPHR)
• Middle pancreatectomy (MP) or
• Enucleation

14. CYSTIC NEOPLASMS OF PANCREAS
• Cystic tumors are the second most common exocrine pancreatic neoplasm,
following only adenocarcinomas of the pancreas in incidence.
• Becoming more common with improved imagings.
• Cystic neoplasms include
• Benign entities such as serous cystadenomas (SCAs),
• Premalignant cysts such as intraductal papillary mucinous neoplasms (IPMNs),
and
• Cystic lesions with invasive carcinoma

15. Klöppel’s Classification of Cystic Neoplasms of the Pancreas
From Kosmahl M, et al: Cystic neoplasms of the
pancreas and tumor-like lesions with cystic
features: a review of 418 cases and a
classification proposal. Virchows Arch 445:168-
178, 2004.

16. SEROUS CYSTIC NEOPLASMS
• First characterized by Compagno and Oertel in 1978 as microcystic and glycogen
rich.
• Clearly distinguished SCAs from mucinous cysts (MCNs and IPMNs)
• Variable size, and when large (>10 cm) and may cause symptoms from local
compression
• More in women (3 : 1 F:M ratio), Mean age : 62 years
• Four subtypes :
• Serous microcytic adenoma,
• Serous macrocytic (oligocystic) adenoma,
• Von Hippel-Lindau (VHL)-associated pancreatic cysts, and
• Serous cystadenocarcinoma

17. GROSSLY
• Cysts are characterized by septations and
thick fibrous walls
• Appear to be innumerable small cysts
containing clear thin fluid.
• There is often a classic honeycomb
appearance
• And a calcified central scar with or without
hemorrhage
Microcystic serous cystadenoma, gross appearance. The tumor
is well circumscribed with a central stellate fibrous scar. The
cystic spaces are small, ranging from 1 mm to several
millimeters.

18. DEFINING MICROSCOPIC FEATURE
• A simple, nonmucinous, cuboidal
epithelium that contains
intracytoplasmic glycogen,
resulting in characteristic clear
cytoplasm,
• Along with a distinctive, rich
capillary network of the
epithelium
Microcystic serous cystadenoma. The individual cysts are
lined by a flattened-to-cuboidal layer of uniform epithelial
cells with clear cytoplasm and round hyperchromatic
nuclei

19. TREATMENT
• Nearly all SCNs are benign.
• The general principle has been to observe SCNs.
• In older or frail patients, conservative approaches continue to be the best
ideology
• Main indication for operative management
• Presence of symptoms.
• Other indications - include cyst size >4 cm and uncertainty of diagnosis
despite appropriate radiologic assessment.

20. • Type of surgical resection
• Based on the position of the cyst within the pancreas.
• These include
• Anatomic pancreatectomy (pancreaticoduodenectomy, distal
pancreatectomy) or
• Tissue-preserving procedures (segmental central pancreatectomy).
• Enucleation of the cyst – limited literature
• Associated with high morbidity (approximately 40%) due to the
development of a pancreatic fistula.
• No role for lymphadenectomy or extended resections due to the inherent benign
nature of SCNs

21. MUCINOUS CYSTIC NEOPLASMS
• Lower incidence than serous cysts or IPMNs
• Perimenopausal women (>95% female; mean age, 48
years)
• Body and tail of the pancreas
• Approximately 6 to 10 cm at the time of diagnosis but
range from 1.5 to 35 cm in diameter
• ~50% present with vague abdominal pain
• ~20% with history of pancreatitis – misdiagnosed as
Pseudocyst.

22. CT- IMAGING:
• MCNs are macrocystic and
unilocular (80%) but can also be
multilocular (20%).
• Presence of
• Eggshell calcification,
• Larger tumor size, or
• A mural nodule
Is suggestive of malignancy.
Macrocystic form: note septum
and lack of surrounding
inflammatory reaction
CT scan of the tail of the pancreas MCN showing a large multiloculated
cyst in the absence of pancreatic ductal communication.

23. GROSS EXAMINATION
• Spherical and infrequently
encapsulated by a calcified fibrous wall
(approximately 15%)
• The cyst can be filled with mucin,
blood, or a watery fluid.
• more viscous than in SCN, due to the
presence of mucus.
• MCNs do not communicate with the
pancreatic ductal system except when
there is erosion into the duct or the
formation of a fistula.
Gross appearance of macrocysts with thin walls and mucinous
cystic fluid

24. HISTOLOGICALLY
• Lined by tall mucin-producing
columnar cells
• Epithelial cells - papillary or flat ;
tendency toward gastric or
intestinal differentiation
• Stroma resembles an ovarian
corpora albicantia due to
luteinized cells and foci of
hyalinization.
• The ovarian-like stroma is
pathognomonic of MCN.
• Varying degrees of cytologic and
architectural atypia
• Invasive carcinoma is seen in
about 15% of the cases
Mucinous cystic
neoplasm. A layer of
hypercellular ovarian-like
stroma underlies
columnar mucinous
epithelium that lines
the cystic spaces
Mucinous ovarian tumor

25. TREATMENT
• Potential malignant degeneration shown by varying degrees of
metaplasia, dysplasia, carcinoma in situ, and tissue invasion
• General principle - is surgical resection irrespective of location in the
pancreas or size
• MCN in head of the pancreas – pancreaticoduodenectomy
• Body and tail of the pancreas- distal pancreatectomy
• Controversial data supporting or against a concurrent splenectomy
• If there is invasion on frozen section, a completion splenectomy with
splenic vessels and regional lymph node dissection should be
performed

26. IPMN (INTRADUCTAL PAPILLARY MUCINOUS
NEOPLASM)
• First recognized in 1982 by Ohashi
• 3 types based on duct involvement
1. Main-duct IPMN (~25% of IPMNs):
• Segmental or diffuse dilation - MPD (>5 mm) in the absence of other causes
of ductal obstruction.
2. Branch-duct IPMN (~57% of IPMNs):
• Pancreatic cysts (>5 mm) that communicate with the MPD (not dilated).
3. Mixed type IPMN (~18% of IPMNs):
• Meets criteria for both main and branch duct.

27. • ~3% to 5% of all pancreatic tumors; ~15% to 30% of all cystic lesions
• Prevalence in M > F
• Peak incidence 6th – 7th decade
• Majority of IPMNs are discovered incidentally, most are asymptomatic.
• Symptoms tend to be nonspecific
• Unexplained weight loss, anorexia, abdominal pain, and back pain
• Jaundice - mucin obstructing the ampulla or with an underlying invasive
carcinoma.
• The obstruction of the pancreatic duct can also lead to pancreatitis
• IPMNs may represent genomic instability of the entire pancreas. This concept,
known as a “field defect,” has been described as a theoretical risk of developing a
recurrent IPMN or pancreatic adenocarcinoma at a site remote from the original
IPMN

28. HISTOLOGICALLY,
• The mucosa of IPMN can express a
range of dysplasia.
• Categories of
• Low-grade dysplasia,
• Moderate dysplasia,
• High-grade dysplasia, and
• Carcinoma
Gross (A) and microscopic (B) features of
intraductal papillary mucinous neoplasm

29. BD-IPMNs –
• Slightly younger population
• Less associated with malignancy
• Most frequently - uncinate process
• Risk of Malignancy – 10- 15%
• Gross examination,
• Grapelike structures that are multicystic,
containing mucin-filled ducts
• The adjacent pancreas is usually normal
due to noninvolvement of the main
pancreatic duct

30. MD-IPMN
• Abnormal dilation of MPD >5mm
• The ampulla of Vater on endoscopy tends to
have a characteristic “fish-mouth”
appearance with thick mucinous secretions
oozing from a patulous papilla on
endoscopic evaluation.
• Mostly near the head of the pancreas and
grow along the main duct.
• EUS: aspirated fluid – viscous, clear and
mucin rich
• CEA increased (>192ng/ml)- not predictive
of invasiveness.
Cross-sectional imaging of MD-IPMN throughout the entire
pancreatic gland and a prominent ampulla of Vater

31. MIXED-IPMN
• BD-IPMN extending into MPD
• Upstream dilatation of MPD
• Carries ~30- 50% risk of invasive nature at presentation.
• Surgical resection is warranted.

32. TREATMENT
MD-PIMN
• ~60% of MD-IPMNs are malignant (carcinoma in situ and invasive cancer), 45%
have invasive adenocarcinoma,
• In general, patients with MD-IPMN should undergo resection.
• Features strongly associated with invasive IPMN:
• Jaundice
• Increased ALP
• Mural nodules
• Diabetes
• MPD >7mm
• Careful assessment of local resectability should be performed before operative
intervention.

33. • Goal of surgery in MD-IPMN is removal of all high-risk disease and resection of
malignancy.
• If disease - limited to a part with no overt radiographic evidence of malignancy,
• Resection of the IPMN-containing pancreas with
• Pancreaticoduodenectomy for the head of the pancreas and
• Distal pancreatectomy for the body and tail of the pancreas
• Intraoperative frozen-section - high-grade dysplasia or an occult invasive cancer
at the margin
• Data suggest - high-risk disease at the margin - significant predictor of future
remnant recurrence
• Results of QoL assessment performed by European Organization for Research and
Treatment of Cancer (EORTC), suggest that Total Pancreatectomy is a viable
option for patients with main-duct IPMN with diffuse involvement of pancreas

34. BD-IPMN
• All patients with Worrisome features
should under go EUS evaluation.
• All with high risk features should
undergo Pancreatic resection.
• Differentiation between a small
oligocystic SCN and a BD-IPMN is
challenging and may require EUS-
FNA, where a very low CEA level and a
usually acellular cytology favor the
diagnosis of SCN

35. Tanaka M, Fernández-Del Castillo C,
Kamisawa T, et al. Revisions of
international consensus Fukuoka
guidelines for the management of IPMN of
the pancreas. Pancreatology 2017; 17:
738-753
Algorithm for the management of suspected
BD-IPMN. a. Pancreatitis may be an
indication for surgery for relief of symptoms.
b. Differential diagnosis includes mucin.
Mucin can move with change in patient
position, may be dislodged on cyst lavage
and does not have Doppler flow. Features of
true tumor nodule include lack of mobility,
presence of Doppler flow and FNA of nodule
showing tumor tissue. c. Presence of any one
of thickened walls, intraductal mucin or mural
nodules is suggestive of main duct
involvement. In their absence main duct
involvement is inconclusive

36. Tumors with a significant invasive component
• Pancreatoduodenectomy, Left Pancreatectomy, Or Total Pancreatectomy
according to the site and extent of the disease with lymph node dissection
remains the standard treatment
• Limited resections or even focal non-anatomic resections (excision,
enucleation, uncinatectomy) may be considered for BD-IPMN without
clinical, radiologic, cytopathologic, or serologic suspicion of invasive
carcinoma
• Non-anatomic resections - possible, leakage of mucin followed by
pseudomyxoma peritonei and also have a higher incidence of pancreatic
fistulae and risk of recurrence from potentially residual neoplasm

37. Multifocal disease
• Segmental anatomic pancreatectomy- lesion limited to a pancreatic
region.
• Threshold for total pancreatectomy - lowered in patients with a
strong family history of PDAC, because of increased prevalence of
higher-grade lesions.

39. Take home message
• Cystic neoplasms of the pancreas have become a well-defined radiographic entity
during the last decade.
• With increasing use of cross-sectional imaging, there has been increasing number
of individuals being encountered.
• Differential includes benign nonneoplastic pancreatic pseudocysts and cystic
neoplasms.
• With an increasing knowledge of the clinicopathologic variables predictive of
malignancy, management has changed to one of selective resection.
• Despite the improvement, significant number of lesions that are indeterminate
pose the challenge to clinicians to balance the risk of malignancy with the
morbidity and mortality associated with pancreatic resection.

40. References
• Yeo CJ. Shackelford's Surgery of the Alimentary Tract, 8th Edition. Elsevier Health Sciences; 2019.
• Jarnagin WR, Belghiti J, Blumgart LH. Blumgart's surgery of the liver, biliary tract, and pancreas. 6th Edition.
Elsevier Saunders; 2017.
• Beauchamp RD, Evers BM, Mattox KL. Sabiston textbook of surgery: the biological basis of modern surgical
practic. Elsevier/Saunders; 2017.
• Hasan A, Visrodia K, Farrell JJ, Gonda TA. Overview and comparison of guidelines for management of
pancreatic cystic neoplasms. World J Gastroenterol 2019; 25(31): 4405- 4413 URL:
https://www.wjgnet.com/1007-9327/full/v25/i31/4405.htm DOI:
https://dx.doi.org/10.3748/wjg.v25.i31.4405
• Gandhi D, Sharma P, Parashar K, Kochar PS, Ahuja K, Sawhney H, Sharma S. Solid pseudopapillary Tumor of
the Pancreas: Radiological and surgical review. Clinical imaging. 2020 Nov 1;67:101-7.
• Tanaka M, Fernández-del Castillo C, Kamisawa T, et al. Revisions of international consensus Fukuoka
guidelines for the management of IPMN of the pancreas. Pancreatology. 2017 Sep 1;17(5):738-53.

41. Thank you!!