Abstract
Pancreatic tumors can be classified by their morphologic features on CT. The subtypes include solid tumors, mixed cystic and solid lesions, unilocular cysts, multilocular cystic lesions, and microcystic lesions. Endoscopic US and MRI can provide detailed information for classifying pancreatic lesions. Each subtype has different kinds of tumors and malignant potential, thus the classification can be useful for a better differential diagnosis and treatment planning. For this purpose, we suggest an appropriate modified classification system by using the imaging features of pancreatic tumors with an emphasis on CT findings and illustrate various findings of typical and atypical manifestations.
Keywords: Pancreatic tumors, Computed tomography, Magnetic resonance imaging (MRI), Endoscopic ultrasound (EUS)
INTRODUCTION
While clinical features such as age and sex are important in differentiating pancreatic tumors, imaging features can be very informative for both the detection and characterization of lesions. For example, most solid pancreatic tumors are malignant or potentially malignant, so surgical resection should be initially considered as a curative treatment. On the other hand, cystic pancreatic lesions may be either benign or have malignant potential. Therefore, accurate diagnosis of pancreatic lesions is critical for management planning. However, accurate characterization and differentiation are still challenging because of their morphologic overlap (1, 2).
Multi-detector computed tomography (MDCT) is usually used as an initial imaging tool for evaluating pancreatic lesions and is an excellent modality for both detection and characterization. Once pancreatic lesions are found on MDCT, magnetic resonance imaging (MRI) and endoscopic ultrasound (EUS) can be used for further characterization (3, 4). MRI has been accepted as a useful imaging modality for evaluating cystic lesions due to its excellent soft tissue contrast. EUS is valuable for precisely demonstrating internal structures such as septa and mural nodules, thanks to its high spatial resolution (5). Classification schemes for pancreatic cystic tumors have been previously proposed based on the morphologic features of the lesions (1, 5). However, we suggest another modified classification system based on the imaging features of pancreatic tumors, with an emphasis on CT findings for better differential diagnosis and treatment planning. The correlation between the pathologic specimens to the imaging features is briefly described along the figures.
Classification of the Pancreatic Tumors: Categorization Based on Gross Morphology and Malignant Potential
In this article, we classified pancreatic tumors into solid tumors, mixed cystic and solid lesions, unilocular cysts, multilocular cystic lesions, and microcystic lesions. This classification is based on the previously suggested classifications for pancreatic cystic tumors (1, 5). However, under the influence of the ovarian tumor classification of the International Ovarian Tumor Analysis (IOTA) group, we thought "solid tumors" should be included as a category in pancreatic tumor classification according to its morphologic features. Therefore we created a category for solid and cystic tumors. Multilocular cystic tumors were divided into either a lobulated, pleomorphic, or smooth shape with septation for better differentiation (Table 1). Because each pancreatic neoplasm has characteristic morphologic features and different malignant potential, this classification system of pancreatic tumors can be helpful in characterizing and managing the lesions (Fig. 1). Quite a number of potentially malignant cystic pancreatic tumors (e.g., mucinous cystic neoplasms [MCNs] or intraductal papillary mucinous neoplasms [IPMNs]), show typical morphologic features, allowing therapeutic planning to be determined based on imaging findings.
Table 1.
Solid Tumors
Although there are a wide variety of pancreatic neoplasms, the majority of them are epithelial tumors and 90% of these are ductal adenocarcinomas and their variants (e.g., adenosquamous carcinomas and undifferentiated carcinomas). Ductal adenocarcinoma is highly invasive and often elicits a desmoplastic reaction. Typically, ductal adenocarcinoma is a hypoattenuating solid mass relative to the normally enhancing pancreatic parenchyma in the pancreatic and portal venous phases because of fibroblastic proliferation and decreased vascularity. Tumors in the head and body of the pancreas frequently cause pancreatic or biliary duct obstruction with upstream ductal dilatation and pancreatic parenchymal atrophy. Neoplasms originating outside the exocrine ductal epithelium are rare. They include pancreatic endocrine tumors and metastasis. Pancreatic endocrine tumors are typically hyperattenuating in the arterial and venous phases on contrast-enhanced CT because of their rich capillary network, while most pancreatic metastases are hypodense except for those from renal cell carcinoma and melanoma (Fig. 2). Unlike ductal adenocarcinomas, pancreatic endocrine tumors are well demarcated and rarely result in pancreatic ductal dilatation. In addition, owing to their hypervascularity, the tumors intensely enhance on either contrast enhanced CT or MRI (6).
Mixed Cystic and Solid Lesions
This category includes true cystic tumors as well as solid neoplasm associated with a cystic component or cystic degeneration. Solid tumors associated with a cystic component include cystic pancreatic endocrine tumor (Fig. 3), solid pseudopapillary tumor (SPT), cystic change of ductal carcinoma, and metastasis (5). Usually a cystic pancreatic endocrine tumor is a single unilocular cyst that occupies the majority of the tumor. In rare cases, ductal adenocarcinoma with cystic change could also manifest as a unilocular cystic lesion (7). Cystic change of ductal carcinoma commonly has low-attenuating regions consistent with necrosis, dilated duct or mucin pools. In comparison with cystic pancreatic endocrine tumor, cystic change of ductal carcinoma appears as relatively poor enhancement of solid portion and has irregular outer margin due to a desmoplastic reaction. On the other hand, when central necrosis is present in pancreatic endocrine tumor, enhancement of the non-necrotic portions is typical on contrast enhanced CT or MRI (6). SPT appears as a well-demarcated mass with a fibrous capsule and varying amount of hemorrhage, necrosis, or calcification (8). Because all of these tumors are either malignant or have a high potential for being malignant, surgical resection is the treatment of choice.
Unilocular Cysts
Unilocular cysts are probably the most difficult to manage because they are frequently small and consist of a broad-spectrum of benign to potentially malignant pathologies, including MCN (Fig. 4), IPMN, and serous cystadenoma. A unilocular cyst in a patient with a clinical history of pancreatitis is almost always a pseudocyst. Other less common unilocular cysts include simple cyst or lymphoepithelial cyst (Fig. 5). Although accurate characterization of unilocular cysts is challenging, CT findings, including the location in the pancreatic head, lobulated contour, absence of wall enhancement, and lack of mural nodule, are specific for the service of serous cystadenoma (9). The presence of peripheral tumoral calcification has a significant association with mucinous cystic neoplasms (10). Moreover, when differentiation is not possible at imaging, symptomatic patients can be further treated with EUS-guided cyst aspiration or surgical resection (5).
Multilocular Cystic Lesions
Multilocular cystic lesions can be divided into three categories: (a) lobulated; (b) pleomorphic, (c) smooth shape with septation (1, 5). A "lobulated" shape is defined as the shape of a simple closed curve that could not be described as the borders of the same circle, with or without internal septation and corresponding with oligocystic serous cystadenomas. A "pleomorphic" shape is defined as one containing three or more cysts, including more than one oval or tubular cyst, and is typical of branch duct type IPMNs. A "smooth shape with septation" is defined as a simple closed curve with the borders of the same circle, and this is a typical finding of MCNs (Fig. 6).
The cystic tumors in this category are also difficult to be characterized because of their overlapping morphology. The differentiation of oligocystic serous cystadenoma from MCN is important because of the malignancy potential of the mucinous tumors. Oligocystic serous cystadenoma appears as a multicystic or lobulated cystic lesion with septation (Fig. 7), while MCN shows a smooth shape, with or without septations. Calcification in serous cystadenomas is typically central within the fibrous stroma, whereas MCN may have a peripheral eggshell calcification (1, 9).
A branch duct type or combined type IPMN can have the morphologic features of pleomorphic cysts. They arise from the epithelial lining of the pancreatic duct and typically produce a copious amount of mucin, which may cause ductal dilatation and obstruction resulting from mucus plugs. Histologically, IPMN represents a spectrum ranging from hyperplasia to carcinoma. Identification of a pleomorphic cyst that communicates with the main pancreatic duct is highly suggestive of a branch duct type or combined type IPMN (Fig. 8). Usually solid tumors, serous cystadenomas, and most mucinous cystic neoplasms do not show ductal communication. For predicting ductal communication and differentiating IPMNs from other lesions, MRI is generally known to show a better diagnostic performance than MDCT (4). However, the lack of communication with the main pancreatic duct does not exclude the possibility of an IPMN. Features predictive of invasive carcinoma in IPMN on CT and other imaging studies include involvement of the main pancreatic duct, marked dilatation of the main pancreatic duct, diffuse or multifocal involvement, the presence of a large mural nodule or solid mass, large size of the mass, and obstruction of the common bile duct (Fig. 9) (11).
Endoscopic US is helpful in excluding a pseudocyst to determine the specific type of the cystic pancreatic tumors, and identifying a malignant transformation by evaluation of the cyst wall (thickness, focal irregularity, mass, or papillary projections) and intracystic structures (septations, mucous, or debris). EUS features that correlate with malignancy include the presence of focal cyst wall thickening or irregularity, septal thickening, an adjacent solid mass, and the presence of collateral vessels (12).
Microcystic Lesions
Serous microcystic cystadenoma is the only cystic lesion included in this category. The typical CT appearance of this tumor is similar to that of a sponge or honeycomb with innumerable tiny cystic spaces septated by thin septa. The septa may coalesce into a characteristic central stellate scar that may calcify itself, which is considered to be pathognomonic for serous cystadenoma and found in about 20% of tumors. The small size of the cysts and the innumerable enhancing septa may cause the mass to appear solid on CT. Microcysts may be seen as numerous discrete foci with bright signal intensity on T2-weighted MR images (Fig. 10), and have little free fluid in the locules on EUS (5, 10, 12, 13).
Limitations and Pitfalls in the Differentiation of the Cystic Pancreatic Tumors
Although MCN usually manifests itself as a multilocular cyst with a smooth contour, with or without septation, there is definite overlap between MCN and serous cystadenoma. In our case (Fig. 11), the cystic tumor in the tail of the pancreas has a lobulated contour with internal septation and calcification in the central area, but it was confirmed as MCN (Fig. 11).
Serous cystadenomas typically have a polycystic, honeycomb, or oligocystic appearance. However, they can also atypically show giant tumors with ductal dilatation, intratumoral hemorrhages, solid variants, unilocular cystic tumors, interval growth, and disseminated forms, so that imaging findings may be unlikely to be diagnostic (14). Even though almost all serous cystic pancreatic neoplasms are benign, serous cystadenocarcinomas rarely are benign, but also have been reported (15).
Identification of a pleomorphic cyst that communicates with the main pancreatic duct is highly suggestive of a branch duct type or combined type IPMN. However, the lack of communication with the main pancreatic duct does not exclude an IPMN. EUS and EUS-guided fine needle aspiration (FNA) may provide additional information about the malignant transformation. A three-dimensional MR pancreatography can be used for differentiation of IPMN from other pancreatic cystic lesions by showing ductal communication (16) (Fig. 12).
CONCLUSION
Pancreatic tumors can be classified by morphologic features on CT and their pathologic features are correlated with CT. Each category has a different malignant potential. Therefore, knowledge of the spectrum of morphologic features observed in pancreatic tumors is useful for differential diagnosis and treatment planning. EUS and MRI can provide detailed information for classifying the pancreatic lesions.
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