**12. Clinical cases**

This chapter can be completed with the presentation of some cases of cystic pancreatic neoplasms treated in our Service. These detailed examinations can contribute to clarify several clinical pathological features.

**•** *First case study*: female, 35 years old. Anamnestic data: non‐specific vague upper abdominal pain and postprandial fullness since 4 months. The diagnosis is incidental by US and CT. The multislice CT shows cystic mass located in the tail of the pancreas, size 8.5 cm, uniloc‐ ular, fluid content, and wall well defined, with contact but not infiltration of posterior gastric wall and splenic vessels. The cystic pancreatic lesion, with this radiologic features, may be also a postnecrotic pseudocyst.

Differential diagnosis, for a cystic pancreatic lesion with these imaging features, may be discussed between MCNs and postnecrotic pseudocysts (**Figure 1**).

**Figure 1.** MCN of the tail of the pancreas (arrow).

This knowledge crucial for the diagnosis and management should be integrated by the classification that separates pancreatic cystic lesions in two categories. There are pancreatic cysts benign, not premalignant, such as SCNs, pseudocysts, lymphoepithelial cysts, and lymphangioma, and pancreatic cysts premalignant and malignant such as MCNs, IPMNs, SPPNs, and CPENs [70]. Roughly, the first conclusion can be the indication of surgical resection for premalignant lesions and observation for benign or indolent lesions. The indeterminate cystic lesions can be located between the cysts frankly benign such as pseudocysts or serous cystadenoma or lymphangioma and, on the other hand, the cystic lesions frankly malignant or with clear findings of malignant evolution such as MD‐IPMNs, IPMNs associated with invasive carcinoma, MCNs with increased size, cyst‐wall irregularity, and intracystic solid regions. In the indeterminate cystic lesions, the management choices can be debatable and uncertain. In this group, small cysts with not certain diagnosis, small BD‐IPMNs, or MCNs can be considered. Characteristic in this setting is the asymptomatic pancreatic cyst inciden‐ tally detected on abdominal CT. The improvement of an unclear diagnosis can be achieved with MRI and MRCP. If the data obtained with these examinations are not conclusive (e.g., main duct <1 cm; thick cyst wall size >2 cm), the diagnostic process can continue with invasive procedure such as EUS‐FNA. The detection of nodule or solid mass or main duct >1 cm and cytology positive for malignancy is crucial for the surgical resection. In the patients without these diagnostic data, the conservative option marked by periodic controls with CT or MRI or EUS (repeat the control test in 6 months) can be evaluated [70]. In the patients with clear diagnosis (CT, MRI, EUS, and clinical data), serous cystadenoma asymptomatic can be followed with periodic imaging control with MRI or CT (repeat the control test in 1 year); if symptomatic, overall in young patient (<65 years), surgery should be considered. Patients with MD‐IPMNs, mixed‐type IPMNs, SPPNs, and MCNs should be proposed for surgical resection. BD‐IPMNs characterized by main pancreatic duct >1 cm, cystic lesion in the head of pancreas, jaundice, solid component, main duct with thickened wall, and mural nodule, which are features concerning malignancy, can undergo surgical resection, if, without these findings, CT,

MRI, and EUS (repeat the control test in 6 months) may be followed conservatively.

is more than 3 cm unless there are features concerning for potential malignancy.

**12. Clinical cases**

108 Challenges in Pancreatic Pathology

clinical pathological features.

There is almost unanimously consensus [32, 71] for surgical indications in patients with MCNs, SPPNs, MD‐IPMNs, and mixed‐type IPMNs. Patients with serous cystadenoma should be directed to conservative management. Surgery can be proposed only in symptomatic patients or if the diagnosis is uncertain. Patients with BD‐IPMN can be observed also if the size lesion

This chapter can be completed with the presentation of some cases of cystic pancreatic neoplasms treated in our Service. These detailed examinations can contribute to clarify several

**•** *First case study*: female, 35 years old. Anamnestic data: non‐specific vague upper abdominal pain and postprandial fullness since 4 months. The diagnosis is incidental by US and CT. The first question is whether other examinations for preoperative diagnosis can be useful. In these cases, the anamnestic data are most important: this patient had not in the past acute pancreatitis that can explain pseudocyst. Consequently in our opinion, other abdominal imaging cannot add other information. The preoperative diagnosis is MCN with the surgical indication: distal pancreatectomy and splenectomy.

The second question regards the method of treatment of proximal pancreatic stump. The transection (pancreatic body and splenic vein) with linear stapler and tubular drainage can be suggested. The splenic artery is treated separately.

The third question regards the incidence of pancreatic fistula in distal pancreatectomy. The most important and frequent complication of distal pancreatectomy is the pancreatic fistula. The incidence of pancreatic fistula ranges from 5 to 30% [72–75]. This variability is explained because there are no the standard definition of the fistula: there are a little gatherings or a few drainage in the postoperative period that are not diagnosed as fistula. The criteria for grading pancreatic fistula have been proposed by ISGPF [76, 77] based on drain and amylase level, persistent drainage (>3 weeks), signs of infections, sepsis, clinical conditions, and need for reoperation. The fistula can be classified, with increase of severity, as grades A, B, and C. The grade A and B usually can be treated with non‐invasive approach: parenteral nutrition, somatostatine, etc. CT control can be useful. Pathological feature shows cystic lesion, size 8.5 cm, mucoid content with smooth surfaces, and thickened, glistening wall. Histological diagnosis was mucinous cystadenoma. Lymph node is negative.

The fourth question is whether surgical treatment with laparoscopic approach can be pro‐ posed. A laparoscopic approach is possible for small or medium size mucinous cystic tumors located in the body or tail of the pancreas. The laparoscopic duodenopancreatectomy is a very complex procedure not yet worldwide performed. But there are two important considerations: not to break the cyst during the intervention because the spillage of mucoid material could lead to tumor spread; moreover, the cyst should be removed intact because the pathologist can do an appropriate examination of the complete wall of the cyst.

**•** *Second case study*: male, 80 years old. Anamnestic data: recurrent episodes of pancreatitis with upper abdominal pain, hyperamilasemia, diabetes, mild alteration of cholestasis tests, and no alcohol consumption since 10 months. The imaging examinations (US and CT) show cystic lesions of the head of the pancreas, its size is 7 cm, mild dilation of main pancreatic duct, and choledocal duct. The MRCP confirms the same lesion and no stones or sludge in the bile duct (**Figure 2**).

**Figure 2.** MD‐IPMN of the head of pancreas (arrow).

Preoperative diagnosis: Because of previous episodes of acute pancreatitis and no biliary stones and alcohol consumption, the proposed diagnosis may be cystic neoplasm. In addition, in this case, we have had the pathognomonic sign: mucus extrusion through a bulging papilla at endoscopy. The diagnosis was intraductal papillary mucinous neoplasm. There are clear surgical indications: duodenpancreatectomy has been proposed.

Pathological description: head of the pancreas, increased in size (7.5 × 5.3 × 4.5 cm), and dystrophic with cystic lesions with mucus. Histology: IPMN not invasive in the pancreatic ductal ectasia with squamous metaplasia of epithelium. There is no neoplastic invasion in the lymph nodes.

In the surgical management, how to regulate the extension of pancreatic resection in IPMN is very important. First consideration: IPMNs encompass a spectrum of epithelial changes from adenoma to invasive adenocarcinoma; in addition, there is the propensity of the tumor to spread microscopically along the pancreatic ducts. Because of these histopathological features, the most simple therapeutic choice is the intraoperative control (by frozen section) to rule out the presence of the tumor in the transection margin (over the all on main duct). In this perspective, the extension of pancreatic resection is possible once or twice, but is corrected to make a total pancreatectomy? The standard choices are difficult. In the experience of Massa‐ chusetts, General Hospital has performed 63% duodenopancreatectomy, 17% distal pancrea‐ tectomy, and 19% total pancreatectomy [28]. The positive frozen‐section intraoperative examination ranges from 23 to 52%. If recurrence occurs in the pancreas after first intervention, a second resection may be possible.

**•** *Third case study*: male, 78 years old. Anamnestic data: the patient has been operated for lung cancer 3 years ago.

In the follow‐up, US of abdomen shows cystic lesion of the pancreatic head. As the most patients with serous cystadenoma, our patient was asymptomatic and the diagnosis incidental.

CT and MRI confirm cystic tumor (size 1.5 cm) in the head of pancreas, well circumscribed and multinodular. There are also mild dilation of main bile duct and Wirsung. Our conclusion was for SCN (**Figure 3**).

**Figure 3.** SCN of the head of the pancreas (arrow).

The fourth question is whether surgical treatment with laparoscopic approach can be pro‐ posed. A laparoscopic approach is possible for small or medium size mucinous cystic tumors located in the body or tail of the pancreas. The laparoscopic duodenopancreatectomy is a very complex procedure not yet worldwide performed. But there are two important considerations: not to break the cyst during the intervention because the spillage of mucoid material could lead to tumor spread; moreover, the cyst should be removed intact because the pathologist can

**•** *Second case study*: male, 80 years old. Anamnestic data: recurrent episodes of pancreatitis with upper abdominal pain, hyperamilasemia, diabetes, mild alteration of cholestasis tests, and no alcohol consumption since 10 months. The imaging examinations (US and CT) show cystic lesions of the head of the pancreas, its size is 7 cm, mild dilation of main pancreatic duct, and choledocal duct. The MRCP confirms the same lesion and no stones or sludge in

Preoperative diagnosis: Because of previous episodes of acute pancreatitis and no biliary stones and alcohol consumption, the proposed diagnosis may be cystic neoplasm. In addition, in this case, we have had the pathognomonic sign: mucus extrusion through a bulging papilla at endoscopy. The diagnosis was intraductal papillary mucinous neoplasm. There are clear

Pathological description: head of the pancreas, increased in size (7.5 × 5.3 × 4.5 cm), and dystrophic with cystic lesions with mucus. Histology: IPMN not invasive in the pancreatic ductal ectasia with squamous metaplasia of epithelium. There is no neoplastic invasion in the

do an appropriate examination of the complete wall of the cyst.

the bile duct (**Figure 2**).

110 Challenges in Pancreatic Pathology

**Figure 2.** MD‐IPMN of the head of pancreas (arrow).

lymph nodes.

surgical indications: duodenpancreatectomy has been proposed.

In this patient, the diagnosis may be serous cystadenoma and the therapeutic choice is the organized controls. At present, we have made three controls (by imaging) every 6 months: There is no clinical or morphological modification of the lesion.

The first question is in which patients, the prolonged observation of the cystic tumor of the pancreas may be reasonable? Serous cystadenomas are indolent, slow‐growing tumors, with a very low incidence of malignancy (3%) [17]. These lesions become symptomatic with the increase in size. The reasonable therapeutic organization for serous tumors may be the following: first to take the certainty of the clinical diagnosis (serous cystic neoplasm).

Patient with little lesion (<4 cm) asymptomatic (in addition take in mind the great incidence of this tumors in sixth–seventh decade) with certain diagnosis can be undergone to non‐operative treatment and followed up. Patient with the lesion bigger in size (>4 cm) symptomatic, in particular if younger, may be undergone to surgical treatment with pancreatic resection.
