Figure 18
Solid-pseudopapillary neoplasm. Prominent pseudopapillary growth pattern is present (hematoxylin-eosin, original magnification 10).
Source publication
Cystic lesions of the pancreas are being recognized with increasing frequency and have become a more common finding in clinical practice because of the widespread use of advanced imaging modalities and the sharp drop in the mortality rate of pancreatic surgery. Consequently, in the past 2 decades, the nature of many cystic tumors in this organ has...
Similar publications
Pancreatic cysts, especially incidental asymptomatic ones seen on noninvasive imaging such as CT or MR imaging, remain a clinical challenge. The etiology of such cysts may range from benign cysts without any malignant potential such as pancreatic pseudocysts and serous cystadenomas to premalignant or frankly malignant cysts such as mucinous cystic...
The rate of incidentally detected pancreatic cystic lesions (PCLs) has increased over the past decade and was recently reported at 8%. These lesions pose a unique challenge, as each subtype of PCL carries a different risk of malignant transformation, ranging from 0% (pancreatic pseudocyst) to 34–68% (main duct intraductal papillary mucinous neoplas...
Citations
... MCN are unusual primary tumors that usually affect middleaged women in their fifth decade of life. Although these neoplasms are less common than SCNs, they have significant malignant potential [3]. Therefore, it is very important to identify and remove them. ...
The aim of this study was to introduce a patient with mucinous nonneoplastic cyst (MNNC) at an unusual age. MNNCs of the pancreas are uncommon primary tumors, which affect middle-aged women in their fifth decade of life and have significant malignant potential. Therefore, it is important to accurately diagnose and remove them. This case is a 28-year-old woman patient who presented with a pain in the right and upper abdomen from 3 months ago and worsened at night. A cystic lesion was observed near the upper bridge of the left kidney in abdominal ultrasonography. Contrast-enhanced abdominal computed tomography (CT) scan showed a cystic lesion in the trunk and umbilical cord of the pancreas. The patient underwent surgery and the mass was removed and the MNNC was diagnosed.
... We initially tested EV from a broad spectrum of patients with "cystic lesions of the pancreas" as the unifying theme because that is how patients present clinically. To represent the most common clinical spectrum, we included SCA (15), low-grade (benign) and high-grade (pre-malignant) IPMN (23), HC (8), MCNs (4), other cysts (8), and cystic pancreatic adenocarcinoma (10) (Fig. S3). Subsequently, a subset analysis was done on the 38 patients with (a)typical SCA and/or IPMN. ...
Increased use of cross-sectional imaging has resulted in frequent detection of incidental cystic pancreatic lesions. Serous cystadenomas (SCAs) are benign cysts that do not require surgical intervention unless symptomatic. Unfortunately, up to half of SCAs do not have typical imaging findings (“atypical SCAs”), overlap with potentially malignant precursor lesions, and thus pose a diagnostic challenge. We tested whether the analysis of circulating extracellular vesicle (EV) biomarkers using a digital EV screening technology (DEST) could enhance the discrimination of cystic pancreatic lesions and avoid unnecessary surgical intervention in these atypical SCAs. Analysis of 25 different protein biomarkers in plasma EV from 68 patients identified a putative biomarker signature of Das-1, Vimentin, Chromogranin A, and CAIX with high discriminatory power (AUC of 0.99). Analysis of plasma EV for multiplexed markers may thus be helpful in clinical decision-making.
... The exact etiology of the PLECs is unknown. But various theories such as epithelial remnants in lymph nodes, a cystic transformation of pancreatic ducts with squamous metaplasia, displaced branchial cleft cysts that were fused with the pancreas during embryogenesis, or a form of teratoma have been proposed [2,10]. PLECs do not appear to be associated with autoimmune disorders, HIV infection, or lymphoma [1,5]. ...
Pancreatic Lymphoepithelial Cysts (PLECs) are very rare and benign lesions of the pancreas. They are commonly detected incidentally during radiological examinations. Proper diagnosis of Pancreatic Cystic Lesions (PCLs) is very important, and physicians should be aware of the different types of cystic lesions and their characteristics before the treatment choice. However, preoperative diagnosis of PLECs, which have no malignant potential and rarely symptomatic or cause complication, are difficult due to nonspecific imaging and the presence of cytological features that overlap with other PCLs. Although many methods are used to avoid unnecessary surgery, most cases are diagnosed after surgery. We report a very rare case of PLEC with coexisting uroepithelial carcinoma.
... 13,14 A recent study showed that EUS-TTNB has a higher diagnostic yield in the preoperative assessment of PCL than the combination of EUS-FNA cytology and fluid CEA. 15 Pancreatic neoplastic cysts can be subclassified into two categories: mucinous (intraductal papillary mucinous neoplasms, mucinous cystic neoplasms, and intraductal oncocytic papillary neoplasms) and non-mucinous (serous cystadenomas). 6,16 Non-neoplastic pancreatic cysts are usually injury-related and/or inflammation-related, and these are seen in approximately 30%-40% of patients with chronic pancreatitis. Other non-neoplastic cysts include congenital cysts, LEC, and squamoid cysts of the pancreatic duct (SCOPD), among others. ...
... Other non-neoplastic cysts include congenital cysts, LEC, and squamoid cysts of the pancreatic duct (SCOPD), among others. In general, mucinous pancreatic neoplasms have serious clinical implications, as they possess malignant potential representing the adenoma-adenocarcinoma sequence, 16 whereas non-mucinous neoplastic cysts do not appear to harbor malignant potential. Also, in assessing PCL, the rare occurrence of degenerative or necrotic changes in non-mucinous solid malignant tumors such as cystic degeneration in ductal adenocarcinoma and neuroendocrine tumors must be considered, since these are malignant, with variable degrees of aggressiveness. ...
... Also, in assessing PCL, the rare occurrence of degenerative or necrotic changes in non-mucinous solid malignant tumors such as cystic degeneration in ductal adenocarcinoma and neuroendocrine tumors must be considered, since these are malignant, with variable degrees of aggressiveness. 16 In addition, the presence of secondary changes in non-neoplastic cysts such as goblet cell metaplasia that was present in our case is an important feature to be included in the differential diagnosis and not to be interpreted as a mucinous neoplasm, particularly on fine-needle aspiration specimens that would impact further patient management. To our knowledge, this is the first case of pancreatic LEC that demonstrated such metaplastic features. ...
Pancreatic lymphoepithelial cysts are rare, benign, non-neoplastic unilocular or multilocular cystic lesions. These circumscribed pancreatic lesions are filled with keratinous material grossly and exhibit distinct microscopic features. Pancreatic lymphoepithelial cysts are like the more common lymphoepithelial cysts of the parotid glands, which have been associated with the diffuse infiltrative lymphocytosis syndrome often seen in patients with HIV infection. However, pancreatic lymphoepithelial cysts are rare and their association with HIV infection has not been established. The presence of secondary changes in non-neoplastic cysts such as goblet cell metaplasia that was present in our case is an important feature to be included in the differential diagnosis and not to be interpreted as a mucinous neoplasm, particularly on fine-needle aspiration specimen microscopic evaluation that would impact further management. Here we describe the diagnosis and treatment of lymphoepithelial cysts in a patient who was on highly active antiretroviral therapy for HIV infection and we provide a brief literature review. Defining the clinical characteristics of lymphoepithelial cysts in patients with HIV and determining accurate preoperative diagnostic procedures will be critical for establishing effective surgical and medical approaches to treating these cysts, which differ substantially from other more serious pancreatic cystic lesions.
... Although many studies have been reported, it remains extremely difficult to diagnose LEC correctly and to rule out malignancies based on the preoperative imaging examinations. It is important to differentiate LEC from malignant pancreatic tumors to avoid unnecessary surgery [14]. In general, measurement of tumor markers may be useful in differentiating malignancies originating from benign tumors. ...
Background
Pancreatic lymphoepithelial cyst (LEC) is a rare nonmalignant cyst consisting of a benign collection of keratinizing squamous epithelial cells with lymphoid tissue. Diagnosing LEC preoperatively is considered difficult because of its non-specific clinical features; therefore, LEC is generally treated the same as a malignant tumor.
Case presentation
Our case was a 65-year-old man who underwent pancreatoduodenectomy 3 years previously for carcinoma arising from the ampulla of Vater. A pancreatic mass in the remnant pancreatic tail was detected through follow-up abdominal contrast-enhanced computed tomography (CT). This revealed two adjacent ring-enhanced masses that had been in tight contact with the left diaphragm and were enlarged. The tumors had high signal intensity in diffusion-weighted images of magnetic resonance imaging, and fluorodeoxyglucose-positron emission tomography (FDG-PET) showed abnormal uptake (standardized uptake value maximum: 17.4). Therefore, we conducted a partial resection of the remnant pancreas with concomitant resection of the left diaphragm. Microscopically, one of the tumors revealed rare fragments of apparently benign squamous epithelium on a background of keratinous debris, cyst contents, and scattered lymphocytes, and the adjacent mass revealed infiltrated neutrophils. The histopathological diagnosis was an LEC with chronic abscess. The patient recovered uneventfully and was discharged on postoperative day 10.
Conclusions
We reported a rare case of LEC with chronic abscess that was positively visualized on FDG-PET. When a pancreatic malignancy cannot be excluded, surgical resection is considered inevitable.
... arising from mucinous cysts early and to improve the overall survival of patients with this dismal disease. However, pancreatic cysts encompass a wide variety of lesions to include congenital, inflammatory and neoplastic cysts 61 . Despite advances in CT and MRI, contemporary cross-sectional imaging is imperfect in differentiating mucinous cysts from non-mucinous cysts 62 . ...
Due to its poor prognosis and the late stage at which it is typically diagnosed, early detection of pancreatic cancer is a pressing clinical problem. Advances in genomic analysis of human pancreatic tissue and other biospecimens such as pancreatic cyst fluid, pancreatic juice and blood have opened the possibility of DNA-based molecular approaches for early detection of pancreatic cancer. In this Review, we discuss and focus on the pathological and molecular features of precancerous lesions of the pancreas, including pancreatic intraepithelial neoplasia, intraductal papillary mucinous neoplasm and mucinous cystic neoplasm, which are target lesions of early detection approaches. We also discuss the most prevalent genetic alterations in these precancerous lesions, including somatic mutations in the oncogenes KRAS and GNAS as well as tumour suppressor genes CDKN2A, TP53 and SMAD4. We highlight the latest discoveries related to genetic heterogeneity and multifocal neoplasia in precancerous lesions. In addition, we review specific approaches, challenges and clinically available assays for early detection of pancreatic cancer using DNA-based molecular techniques. Although detection and risk stratification of precancerous pancreatic neoplasms are difficult problems, progress in this field highlights the promise of molecular approaches for improving survival of patients with this disease.
... Pancreatic cysts may be nonneoplastic or neoplastic. 13,14 The most common nonneoplastic pancreatic cystic lesions represent the post inflammatory sequelae of acute and/or chronic pancreatitis and result in the formation of nonepithelial degenerative cysts (ie, pseudocysts), which account for 75% of all cystic pancreatic lesions. Other nonneoplastic cysts with true epithelial lining include squamous epithelium-lined lymphoepithelial cysts (LECs) and squamoid cysts of the pancreatic duct. ...
Context.—
Because of new and improved imaging techniques, cystic/intraductal pancreatobiliary tract lesions are increasingly being discovered, and brushings or endoscopic ultrasound/computed tomography/magnetic resonance imaging–guided fine-needle aspiration biopsies from these lesions have become an integral part of pathologists' daily practice. Because patient management has become increasingly conservative, accurate preoperative diagnosis is critical. Cytologic distinction of low-risk (pseudocysts, serous cystadenoma, lymphoepithelial cysts, and squamoid cysts of the pancreatic duct) from high-risk pancreatic cysts (intraductal papillary mucinous neoplasm and mucinous cystic neoplasm) requires incorporation of clinical, radiologic, and cytologic findings, in conjunction with chemical and molecular analysis of cyst fluid. Cytopathologists must ensure appropriate specimen triage, along with cytologic interpretation, cyst classification, and even grading of some (mucinous) cysts. Epithelial atypia in mucinous cysts (intraductal papillary mucinous neoplasm and mucinous cystic neoplasm) has transitioned from a 3-tiered to a 2-tiered classification system, and intraductal oncocytic papillary neoplasms and intraductal tubulopapillary neoplasms have been separately reclassified because of their distinctive clinicopathologic characteristics. Because these lesions may be sampled on brushing or fine-needle aspiration biopsy, knowledge of their cytomorphology is critical.
Objective.—
To use an integrated, multidisciplinary approach for the evaluation of cystic/intraductal pancreatobiliary tract lesions (incorporating clinical, radiologic, and cytologic findings with [chemical/molecular] cyst fluid analysis and ancillary stains) for definitive diagnosis and classification.
Data Sources.—
Review of current literature on the cytopathology of cystic/intraductal pancreatobiliary tract lesions.
Conclusions.—
Our knowledge/understanding of recent updates in cystic/intraductal pancreatobiliary lesions can ensure that cytopathologists appropriately triage specimens, judiciously use and interpret ancillary studies, and incorporate the studies into reporting.
... Another reason for lower ADC in SCAs might be due to internal cyst hemorrhage, which can change the ADC value of the cyst. Hypervascularization of septa in SCAs can result in spontaneous internal hemorrhage in these cysts resulting in high signal intensity on T1-WI [22,23]. ...
Purpose
To evaluate the use of volumetric multiparametric MRI in differentiating pancreatic intraductal papillary mucinous neoplasms (IPMNs) from serous cystadenomas (SCAs)
Methods
Included patients (123 patients with pancreatic cystic neoplasms (PCNs) measuring ≥ 10 mm) were stratified into two groups based on cyst type. Axial cyst size, region of interest (ROI)-based apparent diffusion coefficient (ADC) and volumetric data, including cyst volume, volumetric apparent diffusion coefficient (vADC), and volumetric venous enhancement (vVE) were extracted and compared between the two groups. Univariate and multiple logistic regression was used to develop models for distinguishing between IPMNs and SCAs.
Results
Volume and size of the cysts, vVE and vADC and ROI-ADC were significantly different between the two groups. Cyst volume was significantly larger in SCAs (median = 14.1cm³, IQR 3.5–42.5) than in IPMNs (median = 2.5 cm³, IQR 1.1–6) (p < 0.001). IPMNs had a higher volumetric ADC value in comparison to SCAs (2925 ± 294 × 10–6 mm²/s vs 2521 ± 202 × 10–6 mm²/s, p < 0.001). However, IPMNs had lower vVE values compared to SCAs (37 signal intensity (SI) vs 86 SI, p < 0.001). Area under the ROC Curve (AUC) of the model that included vADC and cyst volume had 95% accuracy in distinguishing between the two groups. In comparison, the AUC of the model that included ROI-ADC and axial cyst size had 84% accuracy in distinguishing between the two groups.
A threshold of 2615 × 10⁻⁶ mm²/s for volumetric ADC resulted in the identification of IPMNs from SCAs with sensitivity and specificity of 90.8% and 73.5%, respectively.
Conclusion
IPMNs had smaller cyst volume, higher volumetric ADC and lower volumetric VE values compared to SCAs. Volumetric multiparametric MRI could be useful in differentiating between the IPMN and SCA groups.
... 4 Necrosis and cystic degeneration are common features in these tumors. 5,6 Clinically, SPTPs are usually nonsymptomatic and are discovered incidentally as abdominal masses during physical examinations. 3 Laboratory tests are normal most of time. ...
A solid pseudopapillary tumor should be included in the differential diagnosis of every pancreatic cystic lesion. A constellation of microscopic morphology and immunohistochemistry, in addition to the clinical history, aids in reaching the correct diagnosis. A solid pseudopapillary tumor should be included in the differential diagnosis of every pancreatic cystic lesion. A constellation of microscopic morphology and immunohistochemistry, in addition to the clinical history, aids in reaching the correct diagnosis.
... Organization as an SPT of the pancreas in 1996 (11). Since then, the tumour has been described just over 500 times in pediatric patients (12)(13)(14) The tumor is predominantly diagnosed in women in their third decade of life (6,7,(15)(16)(17), with pediatric patients making up approximately 20-25% of those diagnosed (2,5,7,18). The female to male ratio is between 8-20:1 (6,7,(15)(16)(17)19,20). ...
... Since then, the tumour has been described just over 500 times in pediatric patients (12)(13)(14) The tumor is predominantly diagnosed in women in their third decade of life (6,7,(15)(16)(17), with pediatric patients making up approximately 20-25% of those diagnosed (2,5,7,18). The female to male ratio is between 8-20:1 (6,7,(15)(16)(17)19,20). In both adults and children, SPT is usually diagnosed following presentation of abdominal pain or through incidental findings following imaging (21)(22)(23). ...
... SPT has low malignant potential, with 10-15% of cases following a malignant course (2,(24)(25)(26). Metastases have been observed in 5% to 20% of cases at diagnosis (17,24,27). Given this, radical resection offers an excellent prognosis (10), with a 1-year and 5year survival of 90% and 78% in adults, respectively (19,27,28). ...
Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm, comprising less than 3% of all pancreatic tumors. SPT tumors usually occur in women between 20 and 35 years, are extremely rare in children, and have a favorable prognosis. It is necessary to differentiate STP from other pancreatic neoplasms because, unlike malignant tumors of the pancreas, including those with capsular invasion, it is amenable to cure following complete surgical resection. Here, we report a case of a 7-year-old girl who presented with right upper quadrant pain and recent history of abdominal trauma, having fallen on her abdomen two days before admission. CT confirmed pancreatic mass. MRI findings revealed solid and cystic features with hemorrhage regions, including a mixture of high- and low-signal intensity on T1-and T2-weighted images. Distal pancreatectomy was performed, and the patient's postoperative course was uneventful. The patient was not given any adjuvant therapy. There was no evidence of tumor recurrence or metastases at 3-months follow-up. SPT diagnosis was confirmed following histology findings, including characteristic dot-like patterns. This case highlights incidental detection of SPT following recent abdominal trauma in a young female child. Typical radiological appearance of SPT is an indication for surgery.
