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- •Disclaimer
- •Contents
- •Contributors
- •Embryology
- •Lymphatics
- •Nerves
- •Clinically Relevant Anatomic Variations
- •Duodenum Inversum
- •Pancreas Divisum
- •Annular Pancreas
- •Ectopic Pancreas
- •Ansa Pancreatica
- •Pancreaticobiliary Maljunction
- •Duplication Anomalies
- •Physiology
- •Duodenal Physiology
- •Mechanical Function
- •Endocrine Function
- •Pancreatic Physiology
- •Exocrine Physiology
- •Normal Anatomy
- •Duodenal Anatomy
- •Pancreatic Anatomy
- •Ductal Anatomy
- •Vasculature
- •Endocrine Physiology
- •References
- •Etiology
- •Pathophysiology
- •Diagnosis
- •Clinical Presentation
- •Laboratory Tests
- •Imaging
- •Medical Management
- •Fluid Resuscitation
- •Analgesics
- •Prophylactic Antibiotics
- •Nutrition
- •Complications
- •Long-Term Sequelae of Acute Pancreatitis
- •References
- •Introduction
- •Initial Treatment
- •Reducing Severity of Acute Pancreatitis
- •Fluid Resuscitation
- •Pain Management
- •Nutrition
- •Preventing Infectious Complications
- •References
- •Introduction
- •Sterile Pancreatic Necrosis
- •Antibiotic Therapy
- •Catheter Drainage
- •Video-Assisted Retroperitoneal Drainage (VARD) Procedure
- •Sinus Tract Necrosectomy
- •Open Necrosectomy
- •Open Trans-Gastric Cystogastrostomy
- •Disconnected Distal Pancreatic Duct Syndrome
- •Introduction
- •References
- •Introduction
- •Venous Thrombosis
- •Intra-Abdominal Hypertension
- •Thoracic Complications
- •Gastrointestinal Complications
- •References
- •Pain
- •Endocrine Dysfunction
- •Exocrine Dysfunction
- •Conclusion
- •References
- •Background
- •Postoperative Care
- •References
- •Background
- •Head-Dominant Disease
- •Tail-Dominant Disease
- •Perioperative Management
- •Procedure Steps
- •Open Whipple
- •MIS Whipple
- •Open Distal Pancreatectomy
- •MIS Distal Pancreatectomy
- •Pearls
- •References
- •Introduction
- •Procedures
- •Indications
- •Contraindications
- •Preoperative Workup
- •Pediatrics
- •Patient Selection
- •Contraindications
- •Key Steps
- •Common Steps
- •Pitfalls/Tricks
- •Local Complications
- •Systemic Complications
- •References
- •History/Introduction
- •Indications
- •Adults
- •Procedural Aspects
- •Preoperative Care
- •Total Pancreatectomy
- •Islet Infusion
- •Minimally Invasive Surgery (MIS)
- •Postoperative Care
- •Outcomes
- •Perioperative Data
- •Perioperative Complications
- •Endocrine Function
- •References
- •Introduction
- •Duodenal Adenomas
- •Duodenal Adenocarcinomas
- •Duodenal Neuroendocrine Tumors (D-NETs)
- •Other Non-neoplastic Epithelial Lesions
- •Duodenal Gastrointestinal Stromal Tumors (DGISTs)
- •Leiomyoma
- •Lipoma
- •Choledochal Cysts
- •Duodenal Lymphoma
- •Conclusion
- •References
- •Introduction
- •Pre-procedural Considerations
- •Indications
- •Resection Techniques
- •Sporadic Non-ampullary Adenomas: Cold Snare Polypectomy
- •Sporadic Non-ampullary Adenomas: EMR
- •Sporadic Non-ampullary Adenomas: ESD
- •Sporadic Non-ampullary Adenomas: Full-Thickness Resection Device
- •Ampullary Adenomas: Endoscopic Papillectomy
- •Sporadic Non-ampullary Adenomas: Cold Snare Polypectomy
- •Sporadic Non-ampullary Adenomas: EMR
- •Endoscopic Papillectomy
- •Surveillance
- •References
- •Introduction
- •Benign Tumors
- •Genetic Syndromes
- •Pre-Malignant Tumors
- •Low-Grade Malignancies
- •Alternatives
- •Inclusion Criteria
- •Preoperative Planning
- •Open Transduodenal Ampullectomy
- •Minimally Invasive (Robotic-Assisted) Transduodenal Ampullectomy
- •Outcomes
- •Conclusions
- •References
- •Introduction
- •Anatomy
- •Laparoscopic Segmental Duodenectomy
- •Robotic Segmental Duodenectomy
- •Technique
- •Open Segmental Duodenectomy
- •Patient Positioning
- •Technique
- •Conclusion
- •References
- •Overview
- •Intraductal Papillary Mucinous Neoplasm (IPMN)
- •General Concepts
- •Novel Biomarkers
- •DNA-Based Biomarkers
- •MiRNA
- •Protein-Based Biomarkers
- •IPMNs
- •MCNs
- •SCNs
- •SPTs
- •Guidelines
- •Surveillance Discontinuation
- •Follow-Up Strategy
- •The Verona Policy
- •Conclusions
- •References
- •Introduction
- •Pathophysiology
- •Work-Up
- •Tissue Diagnosis
- •Serum Tumor Markers
- •Multidisciplinary Decision-Making
- •Adjuvant Trials
- •Systemic Chemotherapy
- •Chemoradiation
- •Neoadjuvant Trials
- •Chemotherapy
- •Chemoradiation
- •Pancreatectomy
- •Summary
- •References
- •Introduction
- •Diagnosis
- •Imaging
- •Functionality
- •Insulinoma
- •Gastrinoma
- •VIPoma
- •Glucagonoma
- •Staging/Surgical Decision-Making
- •Nonmetastatic Disease
- •Metastatic Disease
- •Multidisciplinary Decision-Making
- •Surgical Resection
- •Systemic Treatments
- •Open Trials
- •Surveillance
- •References
- •Renal Cell Carcinoma
- •Introduction/Epidemiology
- •Diagnosis/Radiology/Pathology
- •Treatment/Outcome
- •Colorectal Carcinoma
- •Introduction/Epidemiology
- •Diagnosis/Radiology/Pathology
- •Treatment/Prognosis
- •Melanoma
- •Introduction/Epidemiology
- •Diagnosis/Radiology/Pathology
- •Treatment/Prognosis
- •Sarcoma
- •Introduction/Epidemiology
- •Diagnosis/Radiology/Pathology
- •Treatment/Prognosis
- •Conclusion
- •References
- •Preoperative Considerations
- •Key Steps
- •Staging Laparoscopy
- •Specimen Removal
- •Vascular Resection
- •Reconstruction
- •Pancreaticojejunostomy
- •Hepaticojejunostomy
- •Gastro- or Duodeno-Jejunostomy
- •Final Steps
- •References
- •Randomized Controlled Trials
- •Surgical Technique
- •Resection Phase
- •Reconstruction Phase
- •Postoperative Course
- •Conclusions
- •References
- •Introduction
- •Preoperative Workup
- •Preoperative Planning
- •Surgical Management
- •Patient Preparation
- •Surgical Steps
- •Step 1: Kocher Maneuver
- •Step 4: Pancreatic Transection
- •Reconstruction
- •Hepaticojejunostomy
- •Pancreaticojejunostomy
- •Duodenojejunostomy
- •References
- •Introduction
- •Preoperative Planning
- •Diagnostic Laparoscopy
- •Radical Antegrade Modular Pancreatosplenectomy (RAMPS)
- •Splenic Vein Stump Length
- •Ligamentum Teres/Falciform Pedicle Flap
- •References
- •History
- •Early Exploration
- •Trends Over Time
- •Morbidity
- •Safety
- •Oncologic Safety
- •Preoperative Planning
- •Clinical Considerations
- •Anatomical Considerations
- •Surgical Technique
- •Conclusion
- •References
- •Introduction
- •Indications
- •Preoperative Testing
- •Operative Approach
- •Peritoneal Access
- •Specimen Extraction
- •Closure
- •Clinical Outcomes
- •Conclusions
- •References
- •Introduction
- •Preoperative Preparation
- •Key Shared Operative Steps
- •Trocar Placement
- •Splenic Flexure Mobilization
- •Pancreas Mobilization
- •Identify Pancreatic Pathology
- •Pancreatic Transection
- •Splenic Vein Dissection
- •Splenic Artery Dissection
- •Conclusion
- •References
- •Introduction
- •Historical Evolution
- •Perioperative Outcomes
- •Oncologic Outcomes
- •Neoadjuvant Therapy
- •Preoperative Adjuncts
- •Preoperative Coiling
- •Aortic Stenting
- •Robotic DP-CAR Surgical Technique
- •Positioning
- •Port Placement
- •Surgical Steps
- •Perioperative Care
- •Conclusion
- •References
- •Introduction
- •Preoperative Considerations
- •Laparoscopic Enucleation
- •Patient Positioning
- •Procedure
- •Robotic Enucleation
- •Patient Positioning
- •Procedure
- •Open Enucleation
- •Postoperative Management
- •Postoperative Outcomes
- •References
- •Introduction
- •Indications
- •Preoperative Assessment
- •Serologic Testing
- •Surgical Management
- •Patient Preparation
- •Diagnostic Laparoscopy
- •Surgical Steps
- •Step 1: Gastric Mobilization
- •Step 2: Pancreatic Resection
- •Step 3: Reconstruction
- •Jejunojejunostomy
- •Pancreaticojejunostomy
- •Discussion
- •References
- •Introduction
- •Biliary Obstruction
- •Endoscopic Interventions
- •Plastic Versus Metal Stents
- •Covered Versus Uncovered Metal Stents
- •Stent Obstruction
- •Surgical Options
- •Endoscopic Versus Surgical Intervention
- •Duodenal Obstruction
- •Duodenal Stents
- •Venting Percutaneous Gastrostomy Tubes (PEG)
- •Surgical Gastrojejunostomy (Duodenal Bypass)
- •Endoscopic Versus Surgical Intervention
- •Abdominal Pain
- •Celiac Plexus Neurolysis
- •Surgical Celiac Plexus Block
- •Summary
- •References

242
Fig. 14.12 View of
pancreatoduodenal groove
during dissection of a
distal duodenal lesion. D
duodenum, P pancreas,
Red arrow
pancreatoduodenal groove.
With permission from D
Asbun
Fig. 14.13 Intraoperative
cholangiogram with a
laparoscopic bowel clamp
across planned duodenal
transection line during a
distal segmental
duodenectomy. Contrast
ows freely through the
patent ampulla into the
duodenum. With
permission from HJ Asbun
D. Asbun et al.
transected. Meticulous dissection ensures the surgeon remains in the pancreatoduodenal groove without deviating into the duodenum or pancreas.
A bowel clamp is placed across the planned transection line and—after identication, clipping, and cannulation of the cystic duct—an IOC is obtained (Fig.14.13).
Free ow of contrast into the duodenum assures the ampulla is not near the bowel
clamp and will not be included in the staple line. Less frequently, an EGD can be
performed and the ampulla visualized directly. The authors recommend this only for
patients in whom IOC cannot be performed, as endoscopic visualization of the
ampulla can be challenging, and endoscopy may potentially apply undue traction on

14 Segmental Duodenectomy
the dissected duodenum. After conrming the ampulla is patent, the clamp is
replaced with a stapler and the proximal duodenum transected as described for
PSD.Specimen extraction likewise is as described above.
Reconstruction is usually performed as a stapled side-to-side duodenojejunostomy. The jejunum is brought up behind the mesenteric vessels to lay adjacent to the
remaining duodenum. The antipancreatic side of the duodenum is anastomosed to
the antimesenteric side of the jejunum in a side-to-side stapled duodenojejunostomy. Care is taken to avoid leaving a blind loop of stapled duodenum distal to the
anastomosis. The common enterotomy is closed in two layers of absorbable suture,
or with a transverse ring of a surgical stapler.
A cholecystectomy is completed following dissection in the hepatocystic triangle
started with the IOC.The duodenojejunal anastomosis is inspected for proper perfusion and closure. Fluorescent angiography can aid in assuring proper perfusion to
the anastomosis, and an air leak test can also be performed to further assure anastomotic integrity.
243
Robotic Segmental Duodenectomy
Patient Positioning andPort Placement
The patient is positioned supine with legs split and arms tucked. Robotic trocars and
assistant ports are placed as shown in Fig.14.14. Two robotic instrument trocars are
in the right hemiabdomen, the camera periumbilical, and one robotic instrument
trocar in the left. A 12mm assistant port is placed roughly between the camera and
the left-sided robotic trocar, to aid with passing of sutures, surgical sponges, and
surgical staplers (if robotic surgical staplers are not used). Another 5mm port is
placed in the right infracostal region for a self-retaining liver retractor. This setup
can be used for both PSD and DSD.
Technique
Abdominal entry/insufation, inspection, and lysis of any adhesions is started laparoscopically. Once the robotic platform is docked, the technical steps for the roboticassisted segmental duodenectomy mirror the steps in the laparoscopic PSD and
DSD described above.
The primary robotic instruments are generally the robotic monopolar scissors or
hook for the right hand, and fenestrated bipolar graspers for the left. Alternatively,
robotic harmonic shears can be used in the right hand if available. The secondary
left-sided instrument is an atraumatic grasper. During transection of the mesentery,
a bipolar vessel-sealing instrument should be used in the right hand.

244
Fig. 14.14 Trocar
placement for robotic
segmental duodenectomy.
A assistant’s 12mm trocar,
C camera robotic trocar, L
liver retractor 5mm trocar,
R1–R3 robotic instrument
trocars. With permission
from D Asbun
D. Asbun et al.
For transection of the duodenum and jejunum, a robotic stapler can be used in
either the right or left hand of the surgeon although the left may provide a better
angle for transection. Alternatively, the assistant can pass a laparoscopic stapler
through the 12mm assistant port.
Open Segmental Duodenectomy
Patient Positioning
The patient is positioned supine with arms out.
Technique
A midline laparotomy incision is made, with adequate length usually extending
from just under the xiphoid to just above the umbilicus. A wound protector is placed
around the wound. A self-retaining retractor, such as a Bookwalter retractor, is
important for adequate exposure.

14 Segmental Duodenectomy
245
The subsequent technical steps follow those described in the laparoscopic PSD
and DSD.Bimanual palpation and manipulation of the pancreatoduodenal complex
and the bowel facilitates certain aspects of the operation, such as bowel mobilization and exposure. However, the open approach lacks the magnied view from various angles that is possible during a laparoscopic or robotic approach, and meticulous
dissection along the correct planes is paramount.
Postoperative Management andOutcomes
Nasoenteric tubes and abdominal drains are not routinely used postoperatively
although this is left at the discretion of the surgeon. In cases with difcult dissection
in the pancreatoduodenal groove and concerns for pancreatic injury, a closedsuction drain can be left in place.
Usually sips of clear liquids are allowed after surgery, with advancement to liquid diet on postoperative day 1. The patient is not advanced to a soft diet until
approximately 5days after tolerating a liquid diet. Patients are placed on enhanced
recovery pathways, which include measures such as minimizing opioid use and
early ambulation. If there are concerns over the integrity of the anastomosis (due to
difcult dissection, friable tissue, etc.), an upper gastrointestinal X-ray series with
water-soluble contrast can be obtained in the early postoperative period before
allowing unrestricted access to a clear liquid diet.
Early postoperative complications specic to PSD and DSD include delayed gastric emptying, pancreatitis, anastomotic bleeding, and anastomotic leak. Pancreatic
stulas have been reported but are rare, as dissection should not involve pancreatic
parenchymal disruption. Longer term complications include anastomotic stricture,
marginal ulceration, and cancer recurrence. Surveillance is dependent on the primary pathology.
Several studies have shown favorable outcomes after segmental duodenectomy
for duodenal neoplasms, with PD providing no clear benet over segmental duodenectomy [20–22]. Although most series are single-center, retrospective reviews, a
population-level analysis of the Surveillance, Epidemiology, and End Results
(SEER) database supported these ndings [7]. It evaluated patients with duodenal
adenocarcinoma who underwent resection between 1988 and 2010, comparing
those with simple resection (duodenal only) with those who had a radical resection
(duodenum and surrounding organs, meant to represent PD). A radical resection
was not associated with an improved overall or disease specic survival, even after
adjusting for confounding factors. These ndings are supported by a more recent
systematic review and meta-analysis [9].

246
D. Asbun et al.
Pearls andPitfalls
– Alternative methods of identifying the duodenal lesion include intraoperative
EGD or intraoperative ultrasound. In the author’s experience, these may be more
prone to error than preoperative endoscopy with tattooing.
– Port positioning, especially in laparoscopic procedures, may need to be adjusted
based on body habitus. For example, patients with an obese abdomen may
require the ports to be slightly more cephalad. Patients with a smaller abdomen
or narrow costal margin may require some ports to be placed more laterally/
inferiorly to allow for adequate space between ports.
– Repositioning the bed as necessary is important to allow gravity to aid in retrac-
tion, especially in minimally invasive approaches. The bed is positioned in
reverse Trendelenburg position with left side down during early dissection and
medialization of the duodenum. The bed is more level during separation of the
duodenum from the pancreas, duodenal transection, and reconstruction.
– If the transverse colon protrudes into the surgical eld despite adequate mobili-
zation, it can be gently retracted downward for better exposure. This is done with
a blunt grasper or a snake-type liver retractor from the epigastrium/left upper
quadrant, held by the assistant or by a static retractor secured to the bed.
– Division of the gastroepiploic pedicle in PSD can be challenging in patients with
excessive intra-abdominal fat, peri-pyloric adhesions, or omental adhesions to
the duodenum/pylorus. Careful takedown of these adhesions and exploiting
known anatomic planes is helpful. A nger-type esophageal retractor can be
helpful to gently encircle the pedicle from behind and create a window through
which a Penrose drain can be placed. This window can be enlarged prior to pass-
ing a stapler.
– For transection of the duodenum and jejunum, a stapler with approximately
1.5mm closed staple height is usually adequate.
– The proximal duodenum in PSD is usually adequately perfused through collat-
eral blood ow coming from the stomach. If there are concerns for ischemia,
indocyanine green or other methods of intraoperative assessment of perfusion
can be employed.
– Difculty in taking down the ligament of Treitz from right to left during DSD
can be made easier by identifying and taking down the ligament of Treitz from
inferior to the transverse mesocolon. Dissection will meet with the prior dissec-
tion performed superior to the mesentery.
– In patients who have had a prior cholecystectomy, it is necessary to identify the
cystic duct stump to perform the IOC.
– If the duodenal lesion is in the more distal aspect of D4, it may be amenable to
an inframesocolic approach. The colon is lifted cephalad, the ligament of Treitz
identied and taken down, and the distal duodenum mobilized from caudal to
cephalad. Care must be taken to avoid inadvertent injury to the mesenteric ves-
sels, either by traction or dissection. Attempts are made to preserve the inferior
mesenteric vein although it may be sacriced if necessary.

14 Segmental Duodenectomy
247
Conclusion
A partial duodenectomy is a feasible procedure for a variety of duodenal lesions. It
has good outcomes and spares patients the morbidity associated with more extensive resections. There are inherent technical challenges associated with the procedure. However, with a thorough understanding of anatomy, technical expertise, and
good preoperative planning, surgeons can offer their patients the benets of this
operation.
References
1. Bilimoria KY, Bentrem DJ, Wayne JD, Ko CY, Bennett CL, Talamonti MS.Small bowel cancer in the United States: changes in epidemiology, treatment, and survival over the last 20
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2. Hatzaras I, Palesty JA, Abir F, etal. Small-bowel tumors: epidemiologic and clinical characteristics of 1260 cases from the Connecticut tumor registry. Arch Surg. 2007;142(3):229–35.
3. Ochiai Y, Kato M, Kiguchi Y, etal. Current status and challenges of endoscopic treatments for
duodenal tumors. Digestion. 2019;99(1):21–6.
4. Onkendi EO, Boostrom SY, Sarr MG, etal. 15-year experience with surgical treatment of duodenal carcinoma: a comparison of periampullary and extra-ampullary duodenal carcinomas. J
Gastrointest Surg. 2012;16(4):682–91.
5. Schneider L, Contin P, Fritz S, Strobel O, Büchler MW, Hackert T.Surgical ampullectomy: an
underestimated operation in the era of endoscopy. HPB (Oxford). 2016;18(1):65–71.
6. Vega EA, Salehi O, Nicolaescu DC, etal. Laparoscopic pancreatic head preserving total duodenectomy: the parenchymal sparing alternative to a whipple. Ann Surg Oncol. 2021;28(1):131–2.
7. Cloyd JM, Norton JA, Visser BC, Poultsides GA.Does the extent of resection impact survival
for duodenal adenocarcinoma? Analysis of 1,611 cases. Ann Surg Oncol. 2015;22(2):573–80.
8. Bakaeen FG, Murr MM, Sarr MG, etal. What prognostic factors are important in duodenal
adenocarcinoma? Arch Surg. 2000;135(6):635–41; discussion 641–2.
9. Meijer LL, Alberga AJ, de Bakker JK, etal. Outcomes and treatment options for duodenal adenocarcinoma: a systematic review and meta-analysis. Ann Surg Oncol. 2018;25(9):2681–92.
10. Stauffer JA, Adkisson CD, Riegert-Johnson DL, Goldberg RF, Bowers SP, Asbun
HJ.Pancreas-sparing total duodenectomy for ampullary duodenal neoplasms. World J Surg.
2012;36(10):2461–72.
11. Skandalakis JE.Congenital anomalies and variations of the pancreas and pancreatic and extraphepatic bile ducts. The Pancreas. 1998;1:27–59.
12. Kimura W.Surgical anatomy of the pancreas for limited resection. J Hepato-Biliary-Pancreat
Surg. 2000;7(5):473–9.
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14. Mittal PK, Harri P, Nandwana S, et al. Paraduodenal pancreatitis: benign and malignant
mimics at MRI. Abdom Radiol (NY). 2017;42(11):2652–74. https://doi.org/10.1007/
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15. Stolte M, Weiss W, Volkholz H, Rösch W.A special form of segmental pancreatitis: “groove
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D. Asbun et al.

Chapter 15
Cystic Neoplasms
G.Corvino, G.Perri, R.Salvia, andG.Marchegiani
Abbreviations
EUS Endoscopic ultrasound
IPMN Intraductal papillary mucinous neoplasm
MCN Mucinous cystic neoplasm
SCN Serous cystic neoplasm
SPT Solid pseudopapillary tumor
Overview
Despite being dened as rare entities in the past, pancreatic cystic neoplasms
(PCNs) are common, with a prevalence of up to 50% of the population and an incidence estimated to be 12%. The risk of developing a PCN increases with age [1].
Most patients are asymptomatic and diagnosed incidentally [2]. Due to the extensive use of cross-sectional imaging, while the early diagnosis is growing, the size of
newly diagnosed PCNs is reducing. Therefore, the clinical approach toward PCNs
is changing over time, with a relative decrease in surgical resections in favor of the
G. Corvino · R. Salvia
Department of General and Pancreatic Surgery, The Pancreas Institute Verona, University of
Verona Hospital Trust, Verona, Italy
G. Marchegiani (
Hepatopancreatobiliary and Liver Transplant Surgery, Department of Surgery, Oncology and
Gastroenterology (DiSCOG), University of Padua, Padua, Italy
e-mail: giovanni.marchegiani@unipd.it
Switzerland AG 2025
E. P. Ceppa et al. (eds.), The SAGES Manual of Evolving Techniques in
Pancreatic Surgery, https://doi.org/10.1007/978-3-031-78409-5_15
*) · G. Perri
249© The Author(s), under exclusive license to Springer Nature

250
G. Corvino et al.
enrollment of patients in surveillance protocols, with some critical implications for
sustainability and cost to healthcare systems.
According to World Health Organization (WHO) classication [3], PCNs can be
sub-classied into benign, precursors, and malignant entities (Table15.1).
Intraductal Papillary Mucinous Neoplasm (IPMN)
IPMNs are dened as preinvasive, intraepithelial, mucin-producing neoplasms that
grow within the ducts of the pancreas, representing around 80% of PCNs [2, 4, 5].
Their typical appearance (at least in the early phases) is secondary to excessive
mucin secretion that produces cystic dilatation of the pancreatic ducts [4]. The prevalence and the risk of malignant progression increase with age, while there are no
differences by gender or location [6]. Most IPMNs are asymptomatic and in patients
dened as “symptomatic,” the symptoms are usually nonspecic (bloating, abdominal heaviness, postprandial fullness, and atulence) and difcult to correlate directly
with the disease, at the point of questioning the actual existence of such a correlation. A prospective study did not nd any differences in the prevalence of pain in
cases of presumed mucinous cystic neoplasm (MCN), serous cystic neoplasm
(SCN), or IPMN, compared to the general population [7]. In a minority of patients,
specic signs and symptoms directly related to the presence of IPMNs can be found
(especially in the presence of a solid component), namely recurrent acute pancreatitis, obstructive jaundice, new-onset or worsening diabetes mellitus, and steatorrhea
Table 15.1 WHO classication, 2019 [3]
Histological classication of pancreatic cystic tumors
Benign and precursors
• Serous cystadenoma
• Serous cystadenocarcinoma
• Glandular intraepithelial neoplasia, low grade
• Glandular intraepithelial neoplasia, high grade
• Intraductal papillary mucinous neoplasm with low-grade dysplasia
• Intraductal papillary mucinous neoplasm with high-grade dysplasia
• Intraductal papillary mucinous neoplasm with an associated invasive carcinoma
• Intraductal tubulopapillary neoplasm (ITPN)
• Intraductal tubulopapillary neoplasm (ITPN) with associated invasive carcinoma
• Intraductal oncocytic papillary neoplasm (IOPN)
• Intraductal oncocytic papillary neoplasm (IOPN) with associated invasive carcinoma
• Mucinous cystic neoplasms with low-grade dysplasia
• Mucinous cystic neoplasms with high-grade dysplasia
• Mucinous cystic neoplasms with associated invasive carcinoma
Malignant
• Acinar cell carcinoma
• Solid pseudopapillary neoplasm
• Pancreatoblastoma

15 Cystic Neoplasms
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due to endocrine or exocrine insufciency [7]. However, among these symptoms,
only jaundice appears to be an independent predictor of high-grade dysplasia (HGD)
or invasive cancer (IC) [8]. Acute pancreatitis symptoms are believed to be due to
temporary partial or complete occlusion of the main pancreatic duct with viscid
mucin. Persistent occlusion may result in pancreatic insufciency, presenting with
diabetes, steatorrhea, or both [4]. Obstructive jaundice could be present in case of
an invasive solid component in the pancreatic head [7, 9].
Two different classications of IPMNs are described: one is morphological,
depending on the relationship with the ductal system, and another one is
histological.
According to morphology, IPMNs can be classied as:
– Main duct IMPN (MD-IPMN): It is dened by the presence of segmented or dif-
fuse dilation of the main pancreatic duct (MPD) ≥5mm in diameter, without any
other evident cause of obstruction
– Branch duct IPMN (BD-IPMN): dilatation of a secondary duct ≥5mm without
the involvement of MPD
– Mixed type IPMN (MT-IPMN): both main and branch ducts are involved
Different subtypes bear different risks of malignancy, the lowest being in case of
BD-IPMN (6–46%) and the higher in case of MD- or MT-IPMN (60–92%) [10, 11].
However, it must be noticed that these data are extrapolated from surgical series and
risk is therefore overestimated due to selection bias. The malignancy risk development during follow-up of BD-IPMN, in a recent systematic review, was indeed as
low as 2.7%, with an overall malignancy rate of 3.5% [12].
According to histology [13–15], IPMNs can be further sub-classied
depending on:
– The degree of atypia of the epithelial cells: low-grade dysplasia (LGD),
HGD, and IC.
– The types of differentiation of epithelial cells are intestinal, gastric, pancreato-
biliary, and oncocytic. Furthermore, IC arising from IPMNs is subtyped accord-
ing to cytological characteristics: ductal/tubular (usually arising from gastric and
pancreato-biliary type), colloid/muco-nodular (usually arising from intestinal
type), and oncocytic (arising from oncocytic type) [16, 17].
The most frequent BD-IPMNs are of the gastric subtype with LGD and low risk
of progression to develop IC, but when this occurs, it is usually of the tubular type,
with a similar prognosis to that of pancreatic ductal adenocarcinoma (PDAC).
MD-IPMNs, on the other hand, are generally of the intestinal subtype which presents a higher risk of progression to IC but when it occurs, it is often of the colloid
type, with a relatively less dismal prognosis. Intraductal oncocytic papillary neoplasm (IOPN) has been recognized by the current WHO classication as rare
lesions, distinct from IPMNs [3]. They are more frequent in males and usually
involve the MPD.Although IOPNs are associated with invasive carcinoma in up
to 30% of cases and high risk for recurrence, even many years after surgical resection, survival outcome is extremely favorable [18, 19]. A positive resection margin
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