Imaging Pearls ❯ Pancreas ❯ SPEN
|
-- OR -- |
|
- “While 10 %–20 % of SPTs demonstrate malignant behavior, the vast majority are indolent neoplasms that display excellent prognosis after surgical resection. Metastases, though rare, have also been reported in up to 2 % of cases, with the liver being the most common site of metastatic spread. Given their low malignant potential and excellent outcomes if appropriately treated, correct and timely radiological diagnosis is crucial in optimizing patient management and prognosis. Due to the rarity of SPTs however, in conjunction with the heterogeneity of their imaging features, establishing correct diagnosis has previously proven to be challenging. Prior studies have demonstrated that among cases of pathologically proven SPTs, preoperative suspicion of SPT based on imaging features was correctly raised in only 24 % of cases.”
Cinematic rendering of solid pseudopapillary tumors: Augmenting diagnostics of an increasingly encountered tumor
Taha M. Ahmed, MD, Elliot K. Fishman, MD, Linda C. Chu, MD*
Current Problems in Diagnostic Radiology (in press) - “Cinematic rendering is completed on an independent workstation equipped with Siemens Syngo Via (VB40) running cinematic rendering software. We have developed optimized rendering parameters for the pancreas which we have used for a range of pancreatic pathology. These pre-defined parameters are adjusted in real time to optimize output on a case-by-case basis. The final images are subsequently exported to PACS where they can be reviewed by the referring clinician.”
Cinematic rendering of solid pseudopapillary tumors: Augmenting diagnostics of an increasingly encountered tumor
Taha M. Ahmed, MD, Elliot K. Fishman, MD, Linda C. Chu, MD*
Current Problems in Diagnostic Radiology (in press) - “CT attenuation in cystic regions ranges from that of fluid attenuation equal to that of water to soft tissue attenuation of areas rich in blood. Internal hemorrhage, in the appropriate context of other imaging findings, is highly characteristic of these tumors and is present in between 29 and 88.9 % of cases. Calcifications are present in roughly one third of tumors and are more frequently encountered in larger tumors.19,20 Although peripheral curvilinear calcifications are most classic, calcification patterns may vary. The majority of SPTs (59.3 %) are located within the body and tail of the pancreas with a mean tumor size of 6.1 cm at the time of presentation.”
Cinematic rendering of solid pseudopapillary tumors: Augmenting diagnostics of an increasingly encountered tumor
Taha M. Ahmed, MD, Elliot K. Fishman, MD, Linda C. Chu, MD*
Current Problems in Diagnostic Radiology (in press) - “Differential diagnoses for predominantly solid SPTs consists of well differentiated pancreatic neuroendocrine tumor (PNET), pancreatic ductal adenocarcinoma (PDAC), serous adenoma (SCA) and metastases. For larger mixed solid and cystic SPTs, differential diagnoses consists of such as mucinous cystic neoplasm (MCN), SCA, intraductal papillary mucinous neoplasm (IPMN), cystic PNETs, calcified hemorrhagic pseudocyst, pancreatoblastoma in pediatric patients, and exophytic gastrointestinal stromal tumor (GIST) when arising in close relation to the pancreas.”
Cinematic rendering of solid pseudopapillary tumors: Augmenting diagnostics of an increasingly encountered tumor
Taha M. Ahmed, MD, Elliot K. Fishman, MD, Linda C. Chu, MD*
Current Problems in Diagnostic Radiology (in press) - “Computed tomography (CT) is the primary imaging modality used to assess SPT. At imaging, SPTs typically appear as well encapsulated mixed-density lesions composed of varying proportions of solid components and cystic components. Infrequently, they may be mostly solid or mostly cystic. SPTs are hypothesized to grow as solid tumors that outgrow their vascular supply and subsequently undergo cystic degeneration. As a result, cystic components tend to be centrally located while enhancing solid components are present peripherally. CT attenuation in cystic regions ranges from that of fluid attenuation equal to that of water to soft tissue attenuation of areas rich in blood. Internal hemorrhage, in the appropriate context of other imaging findings, is highly characteristic of these tumors and is present in between 29 and 88.9 % of cases.”
Cinematic rendering of solid pseudopapillary tumors: Augmenting diagnostics of an increasingly encountered tumor
Taha M. Ahmed, MD, Elliot K. Fishman, MD, Linda C. Chu, MD*
Current Problems in Diagnostic Radiology (in press) - “CR in these cases can better visualize internal septations, increase depth perception of cystic components and characterize the true cystic nature of the mass. Through this, the internal architecture of cystic components can be better appreciated which can help in differentiating between cystic neoplasms. In rare cases, SPT may also present as a purely cystic mass, making differentiation between SPT and MCNs challenging. In these equivocal cases, patient demographics, particularly age, may be used to help differentiate between the two entities.”
Cinematic rendering of solid pseudopapillary tumors: Augmenting diagnostics of an increasingly encountered tumor
Taha M. Ahmed, MD, Elliot K. Fishman, MD, Linda C. Chu, MD*
Current Problems in Diagnostic Radiology (in press) - “Vascular encasement, narrowing, occlusion and invasion in SPTs is rare and up to 95 % of SPT do not demonstrate any vascular involvement. CR in these cases can better characterize any vascular stretching, abutment, and compression from a large SPT and differentiate it from true vessel involvement from a PDAC or PNET. This can help rule in the correct diagnosis with more certainty. While most SPTs do not demonstrate vascular invasion, a small aggressive subset (4.6 %) of tumors can present with vascular involvement. Vascular invasion does not necessarily preclude surgical resection of SPT however, and previous reports have demonstrated long term improved outcomes when radical resection with vascular reconstruction is pursued. CR in such cases can better define the extent of vascular involvement through characterizing the length of involved vessel and degree of luminal narrowing."
Cinematic rendering of solid pseudopapillary tumors: Augmenting diagnostics of an increasingly encountered tumor
Taha M. Ahmed, MD, Elliot K. Fishman, MD, Linda C. Chu, MD*
Current Problems in Diagnostic Radiology (in press) - “Second, the display of CR images needs to be optimized to accurately depict the anatomy and pathology of interest. This requires the specialized expertise of radiologists who must tailor the display settings for each unique pathology. Utilization of incorrectly optimized parameters can hinder visualization and potentially lead to incorrect diagnoses. While this aspect of CR implementation may become more standardized and user-friendly in the future, and potentially automated with the help of artificial intelligence algorithms, currently radiologists must dedicate time to manually render each case. It takes an experienced radiologist approximately 5–7 min to render each case.”
Cinematic rendering of solid pseudopapillary tumors: Augmenting diagnostics of an increasingly encountered tumor
Taha M. Ahmed, MD, Elliot K. Fishman, MD, Linda C. Chu, MD*
Current Problems in Diagnostic Radiology (in press)
- “Pancreatic solid pseudopapillary tumors (SPTs) are a rare subset of pancreatic neoplasms, accounting for under 2% of exocrine pancreatic tumors. The incidence of SPTs has shown a significant increase in the past two decades, attributed to heightened cross-sectional imaging utilization. These tumors often present with nonspecific clinical symptoms, making imaging a crucial tool in their detection and diagnosis. Cinematic rendering (CR) is an advanced 3D post-processing technique that generates highly photorealistic realistic images by accurately modeling the interaction of light within the imaged volume. This allows improved visualization of anatomic structures which holds potential to improve diagnostics.”
Cinematic Rendering of Solid Pseudopapillary Tumors: Augmenting Diagnostics of an Increasingly Encountered Tumor
Taha M. Ahmed, Elliot K. Fishman, Linda C. Chu
Current Problems in Diagnostic Radiology, 2023 (in press) - “Up to 40% of cases may be detected incidentally with most patients being asymptomatic at time of detection given the slow growing nature of these tumors. This renders the role of imaging central to the detection and diagnosis of these tumors. While 10%-20% of SPTs demonstrate malignant behavior, the vast majority are indolent neoplasms that display excellent prognosis after surgical resection. Metastases, though rare, have also been reported in up to 2% of cases, with the liver being the most common site of metastatic spread. Given their low malignant potential and excellent outcomes if appropriately treated, correct and timely radiological diagnosis is crucial in optimizing patient management and prognosis.”
Cinematic Rendering of Solid Pseudopapillary Tumors: Augmenting Diagnostics of an Increasingly Encountered Tumor
Taha M. Ahmed, Elliot K. Fishman, Linda C. Chu
Current Problems in Diagnostic Radiology, 2023 (in press) - “Calcifications are present in roughly one third of tumors and are more frequently encountered in larger tumors. Although peripheral curvilinear calcifications are most classic, calcification patterns may vary. The majority of SPTs (59.3%) are located within the body and tail of the pancreas with a mean tumor size of 6.1cm at the time of presentation. SPTs generally do not obstruct the pancreatic duct or lead to pancreatic atrophy, even in cases where the tumor is large and bulky. While most SPTs present with these imaging findings, atypical presentations that deviate from the outlined characteristics have also been reported.”
Cinematic Rendering of Solid Pseudopapillary Tumors: Augmenting Diagnostics of an Increasingly Encountered Tumor
Taha M. Ahmed, Elliot K. Fishman, Linda C. Chu
Current Problems in Diagnostic Radiology, 2023 (in press) - “Differential diagnoses for predominantly solid SPTs consists of well differentiated pancreatic neuroendocrine tumor (PNET), pancreatic ductal adenocarcinoma (PDAC), serous adenoma (SCA) and metastases.14 For larger mixed solid and cystic SPTs, differential diagnoses consists of such as mucinous cystic neoplasm (MCN), SCA, intraductal papillary mucinous neoplasm (IPMN), cystic PNETs, calcified hemorrhagic pseudocyst, pancreatoblastoma in pediatric patients, and exophytic gastrointestinal stromal tumor (GIST) when arising in close relation to the pancreas.”
Cinematic Rendering of Solid Pseudopapillary Tumors: Augmenting Diagnostics of an Increasingly Encountered Tumor
Taha M. Ahmed, Elliot K. Fishman, Linda C. Chu
Current Problems in Diagnostic Radiology, 2023 (in press) - While most SPTs are large and well encapsulated, and may therefore be easily visualized, smaller SPTs are increasingly being detected.3 These smaller SPTs often present with atypical imaging features. Cystic change may be less prevalent amongst smaller SPTs and they may instead appear as solid soft tumors with varying degrees of fibrosis.21 These tumors are also often not as sharply circumscribed, may appear unencapsulated and are therefore not as well demarcated from adjacent pancreatic parenchyma. CR imaging allows for photorealistic rendering quality that enhances the visibility of subtle textural compared to the surrounding pancreatic parenchyma. This improved visualization can be beneficial in the identification and evaluation of these more subtle cases, particularly when they arise among an older age group not typically associated with SPTs
Cinematic Rendering of Solid Pseudopapillary Tumors: Augmenting Diagnostics of an Increasingly Encountered Tumor
Taha M. Ahmed, Elliot K. Fishman, Linda C. Chu
Current Problems in Diagnostic Radiology, 2023 (in press) - “Although smaller SPTs are associated with atypical imaging findings, on the other end of the spectrum, larger SPTs pose their own set of diagnostic challenges. Though these tumors may be easily visualized, it can become difficult to determine the true pancreatic origin of these tumors, particularly if they are large. While the average size at detection ranges from 5-10cm, given their indolent behavior, these tumors can often grow much larger with prior studies reporting cases ranging up to 22cm at time of detection. At these sizes, differentiating between a potential adrenal, retroperitoneal, or gastric origin of these masses can become challenging. CR can potentially improve appreciation of the spatial relationship between the mass and adjacent structures. This can subsequently improve the diagnostic confidence of the pancreas as the organ of origin in ambiguous cases.”
Cinematic Rendering of Solid Pseudopapillary Tumors: Augmenting Diagnostics of an Increasingly Encountered Tumor
Taha M. Ahmed, Elliot K. Fishman, Linda C. Chu
Current Problems in Diagnostic Radiology, 2023 (in press) - “Vascular encasement, narrowing, occlusion and invasion in SPTs is rare and up to 95% of SPT do not demonstrate any vascular involvement.26 CR in these cases can better characterize any vascular stretching, abutment, and compression from a large SPT and differentiate it from true vessel involvement from a PDAC or PNET. This can help rule in the correct diagnosis with more certainty. While most SPTs do not demonstrate vascular invasion, a small aggressive subset (4.6%) of tumors can present with vascular involvement.2 Vascular invasion does not necessarily preclude surgical resection of SPT however, and previous reports have demonstrated long term improved outcomes when radical resection with vascular reconstruction is pursued.2CR in such cases can better define the extent of vascular involvement through characterizing the length of involved vessel and degree of luminal narrowing.”
Cinematic Rendering of Solid Pseudopapillary Tumors: Augmenting Diagnostics of an Increasingly Encountered Tumor
Taha M. Ahmed, Elliot K. Fishman, Linda C. Chu
Current Problems in Diagnostic Radiology, 2023 (in press) - “Second, the display of CR images needs to be optimized to accurately depict the anatomy and pathology of interest. This requires the specialized expertise of radiologists who must tailor the display settings for each unique pathology. Utilization of incorrectly optimized parameters can hinder visualization and potentially lead to incorrect diagnoses. While this aspect of CR implementation may become more standardized and user-friendly in the future, and potentially automated with the help of artificial intelligence algorithms, currently radiologists must dedicate time to manually render each case. It takes an experienced radiologist approximately 5-7 minutes to render each case.”
Cinematic Rendering of Solid Pseudopapillary Tumors: Augmenting Diagnostics of an Increasingly Encountered Tumor
Taha M. Ahmed, Elliot K. Fishman, Linda C. Chu
Current Problems in Diagnostic Radiology, 2023 (in press)
- Objective: To review all pancreatic tumors seen in children over a decade at a large tertiary pediatric institution and to describe multimodality imaging findings. Materials and methods: We conducted a retrospective review of imaging studies performed in children with pancreatic tumors between January 2000 and December 2012, analyzing imaging features on available US, CT and MR examinations. We reviewed patient charts for clinical features, management and final diagnosis.
Results: We included 23 children in this study. Of these, 12 had solid and papillary epithelial neoplasms (SPEN), 3 had neuroendocrine tumors, 3 had lymphoma, and 1 each had hemangioendothelioma, benign congenital cysts with adipose tissue, dendritic cell sarcoma, metastatic rhabdomyosarcoma, and lipoma. All children with SPEN were teenage girls. SPEN showed characteristic imaging features including well-defined margins with surrounding capsule, solid-cystic components and hemorrhage.
Conclusion: Pancreatic tumors are uncommon in children. SPEN is the most common tumor and is seen predominantly in teenage girls and shows characteristic imaging features.
Imaging features of pancreatic tumors in children: 13-year experience at a pediatric tertiary hospital.
Ahmed TS et al.
Pediatr Radiol. 2013 Nov;43(11):1435-43. - Pancreatic tumors are rare in children. Malignant pancreatic tumors diagnosed in children and adults younger than 20 years account for only 0.1% of all malignant pancreatic tumors. Even though pancreatoblastoma is considered the most common malignant tumor in children, recent studies have indicated that solid pseudopapillary epithelial neoplasm (SPEN) is the most common pancreatic tumor in children. Other pancreatic tumors seen in children include neuroendocrine tumors, lymphoma and acinar cell carcinoma, among others. The 5-year overall survival is approximately 88% in SPEN, 66% in pancreatoblastoma, 58% in neuroendocrine tumors and 33% in carcinomas, with overall survival worse in boys, probably reflecting the higher incidence of SPEN in girls. SPENs are seen predominantly in teenagers, whereas the median age for pancreatoblastoma is 4 years.
Imaging features of pancreatic tumors in children: 13-year experience at a pediatric tertiary hospital.
Ahmed TS et al.
Pediatr Radiol. 2013 Nov;43(11):1435-43. - “Large series of SPEN have been reported recently in adults and children. Interestingly, these large series are from Asia. In our study, out of 23 cases SPEN was the most common tumor, accounting for 12 cases. SPEN is a slow-growing tumor of the pancreas that is seen predominantly in teenage girls and young women. In a large series involving 390 cases, the female-to-male ratio was 7.3:1 and the average age of the patients was 25.3 years, with more than 50% age 10–24 years.”
Imaging features of pancreatic tumors in children: 13-year experience at a pediatric tertiary hospital.
Ahmed TS et al.
Pediatr Radiol. 2013 Nov;43(11):1435-43. - “SPEN tends to be moderately large, with an average size (mean of maximum dimensions) reported in the literature ranging between 7.9 cm and 9.5 cm. In our study, the average size of the SPEN was 6.9 cm. A small number of SPENs can be malignant, presenting with recurrence and even metastases. On imaging these tumors can show irregular borders and signs of invasion of adjacent structures. In our series one tumor showed irregular borders but no SPEN has shown evidence of recurrence or metastases. In general, SPEN has good prognosis, with >95% of patients with local disease cured by complete resection.”
Imaging features of pancreatic tumors in children: 13-year experience at a pediatric tertiary hospital.
Ahmed TS et al.
Pediatr Radiol. 2013 Nov;43(11):1435-43. - “Solid pseudopapillary pancreatic neoplasms are rare pancreatic tumors accounting for 1% to 2% of exocrine pancreatic lesions. Recent surveys report a 7-fold increase in the incidence of SPEN cases since the past two decades.This increase is likely representative of improved quality and techniques of radiological imaging and increased usage of screening tests.The etiology of this tumor remains unclear. Suggestions are that these neoplasms develop from either pluripotent pancreatic stem cells or cells of female genital epithelial origin due to increased prevalence in younger females.”
Solid and Papillary Epithelial Neoplasm.
Paluri R, Babiker HM
[Updated 2020 Nov 19]. In: StatPearls [Internet]. - "Solid pseudopapillary epithelial neoplasms (SPEN), also known Frantz tumors, are rare pancreatic exocrine neoplasms with age and gender predisposition. This pathology was first described by Dr. Virginia Kneeland Frantz in 1959 and hence the name. Other names include solid pseudopapillary tumor (SPT) of the pancreas, solid pseudopapillary neoplasm (SPN), papillary cystic neoplasm of the pancreas, Hamoudi tumor, or Gruber-Frantz tumor. These are low grade with low malignant potential neoplasms commonly seen in young and adolescent females and rarely affects men. Accurate diagnosis of SPEN is of utmost importance since it has a low malignant potential, and with prompt diagnosis and surgery, the patients could have a long life expectancy including possible cure.”
Solid and Papillary Epithelial Neoplasm.
Paluri R, Babiker HM
[Updated 2020 Nov 19]. In: StatPearls [Internet]. - Pancreas Tumors in the Pediatric Population
- SPEN
- Pancreaticoblastoma
- Islet Cell Adenomatosis and Congenital Hyperinsulinemia
- Islet cell tumors
- Pseudocysts and True Cysts
- Cystic Neoplasms
- Metastases to the Pancreas - “Pediatric pancreatic masses are rarely encountered at imaging, but a variety of tumors should be considered. The most likely diagnostic considerations include solid pseudopapillary tumor and pancreatoblastoma, which have differing peak ages of incidence. Endocrine lesions, including islet cell adenomatosis and islet cell tumors, are another possible group of epithelial neoplasms. Among cystic lesions, pseudocyst is the most common lesion in the pediatric pancreas. True cysts should be considered in the context of a genetic syndrome affecting multiple abdominal organs. Among nonepithelial tumors, primary or secondary pancreatic lymphoma is the most common consideration, though neurogenic tumors such as neuroblastoma may be considered.”
Imaging of Pediatric Pancreatic Neoplasms With Radiologic Histopathologic Correlation
Narendra S. Shet et al.
AJR 2014; 202:1337–1348
- “SPNs are usually large, well-demarcated lesions of the pancreas with both cystic and solid components. A review of more than 2700 patients suggested a mean tumour diameter of 8.6 cm, while smaller reviews point at a tumour size of 6 cm. Similarly, in our cohort, we found lesions of variable size ranging from 1.5 to 15.3 cm with a median size of 5 cm. The sheer size of these lesions makes non-specific abdominal pain the most common presenting symptom at the time of diagnosis [8], which was also reiterated by our cohort. Other presenting symptoms may include non-specific abdominal symptoms and jaundice. As many as 30% may be asymptomatic and are diagnosed incidentally, such as following screening for unrelated reasons or deranged blood tests like in our experience.”
Solid Pseudopapillary Neoplasms of the Pancreas: a Single‐Center Experience
Moustafa Allam et al.
Journal of Gastrointestinal Cancer 2021 (in press) - "As many as 30% may be asymptomatic and are diagnosed incidentally, such as following screening for unrelated reason or deranged blood tests like in our experience. Moreover, SPNs in our cohort, in concordance with what is reported in the literature, appear to be most commonly localized to either the tail or the head of the pancreas, with only a small minority found in the pancreatic body and tail- or head-body junction.”
Solid Pseudopapillary Neoplasms of the Pancreas: a Single‐Center Experience
Moustafa Allam et al.
Journal of Gastrointestinal Cancer 2021 (in press) - "Cross-sectional imaging is a widely accepted pre- operative diagnostic modality. On CT, SPNs usually appear as a large mass of mixed solid and cystic appearance originating in the pancreas. According to the extent of degenerative changes, their appearance may range from almost completely solid to almost completely cystic. Cystic areas are most commonly found in the centre, while solid components tend to predominate in the periphery. The attenuation of the cystic components can vary from density similar to water to dense haemorrhagic debris . Areas of calcification are also frequent, and these calcifications may be most prominent around the rim of the lesion but may also be found centrally.”
Solid Pseudopapillary Neoplasms of the Pancreas: a Single‐Center Experience
Moustafa Allam et al.
Journal of Gastrointestinal Cancer 2021 (in press) - "SPNs have an uncertain malignant potential, and as such, in 1996, the WHO classified them as borderline tumours. Although mostly benign, some SPNs have been shown to metastasize preferentially to the liver, but also to the lungs and peritoneum in most aggressive cases. As the vast majority of SPNs resected are benign, the overall survival is good; nevertheless, there is an increasing body of evidence recognizing the malignant potential of these lesions. Studies have identified several predictors of malignancy including large tumour size, focal discontinuity of SPN capsule, Ki-67 proliferative index, and exophytic growth pattern. Current literature has shown a recurrence rate after resection of around 10–15%.”
Solid Pseudopapillary Neoplasms of the Pancreas: a Single‐Center Experience
Moustafa Allam et al.
Journal of Gastrointestinal Cancer 2021 (in press)
- Background: Solid-pseudopapillary neoplasms (SPNs) are rare pancreatic tumors with malignant potential. Clinicopathologic characteristics and outcomes of patients with SPN were reviewed.
Study Design: Long-term outcomes were evaluated in patients with an SPN who were followed from 1970 to 2008.
Conclusions: SPNs are rare neoplasms with malignant potential found primarily in young women. Formal surgical resection may be performed safely and is associated with long-term survival.
Reddy, S., Cameron, J. L., Scudiere, J., Hruban, R. H., Fishman, E. K., Ahuja, N., Pawlik, T. M., Edil, B. H., Schulick, R. D., & Wolfgang, C. L. (2009).
Surgical management of solid-pseudopapillary neoplasms of the pancreas (Franz or Hamoudi tumors): a large single-institutional series.
Journal of the American College of Surgeons, 208(5), 950–959. - Thirty-seven patients were identified with an SPN. Thirty-three (89%) were women, and median age at diagnosis was 32 years. Most patients were symptomatic; the most common symptom was abdominal pain (81%). Thirty-six patients underwent resection; one patient with distant metastases was not operated on. There were no 30-day mortalities. Median tumor size was 4.5 cm. Thirty-four patients underwent an R0 resection, 1 had an R1 resection, and 1 had an R2 resection. Two patients had lymph node metastases, and one patient had perineural invasion. After resection, 34 (94%) patients remain alive. One patient died of unknown causes 9.4 years after resection, and another died of unrelated causes 25.6 years after operation. The patient with widespread disease who didn’t have resection died 11 months after diagnosis. Thirty-five of the 36 patients having resection remained disease free, including those who died of unrelated causes (median followup, 4.8 years). One patient developed a recurrence 7.7 years after complete resection. She was treated with gemcitabine and remains alive 13.6 months after recurrence.
Reddy, S., Cameron, J. L., Scudiere, J., Hruban, R. H., Fishman, E. K., Ahuja, N., Pawlik, T. M., Edil, B. H., Schulick, R. D., & Wolfgang, C. L. (2009).
Surgical management of solid-pseudopapillary neoplasms of the pancreas (Franz or Hamoudi tumors): a large single-institutional series.
Journal of the American College of Surgeons, 208(5), 950–959. - “Solid-pseudopapillary neoplasms (SPNs) of the pancreas are rare neoplasms, comprising only 1% to 2% of all pancreatic tumors. There is a strong female preponderance, and most SPNs present in the third and fourth decade of life. The first published description of an SPN was by Frantz in 1959. This report consisted of a pathologic description of three patients with SPN. Hamoudi and colleagues added an additional patient to the literature in 1970 and detailed the electron microscopic appearance of the tumor. The first report in the surgical literature of an SPN was by Sanfey and associates in 1983.SPNs are also called solid and papillary tumors, papillary cystic tumors, solid cystic tumors, Frantz tumors, and Hamoudi tumors. SPN is synonymous with the preceding names and is the preferred terminology.”
Reddy, S., Cameron, J. L., Scudiere, J., Hruban, R. H., Fishman, E. K., Ahuja, N., Pawlik, T. M., Edil, B. H., Schulick, R. D., & Wolfgang, C. L. (2009).
Surgical management of solid-pseudopapillary neoplasms of the pancreas (Franz or Hamoudi tumors): a large single-institutional series.
Journal of the American College of Surgeons, 208(5), 950–959.
- “Solid pseudopapillary neoplasms (SPEN), also known Hamoudi tumors or Franz tumors, are rare pancreatic neoplasms which are almost always seen in young women (usually under 35 years of age), with only a small minority of cases diagnosed in men (~12 %). As a result of this age and gender predisposition, these tumors are frequently referred to as “daughter” tumors. Although less certain, some studies have suggested a slightly higher incidence in patients of African or Asian ethnicities.”
Multidetector Computed Tomography in the Evaluation of Cystic Tumors of the Pancreas Siva P. Raman, Elliot K. Fishman (in) Cystic Tumors of the Pancreas: Diagnosis and Treatment Marco Del Chiaro, StephanL.Haas, RichardD.Schulick Editors © Springer International Publishing Switzerland 2016 - “While these lesions are classified as low-grade malignancies, they have an excellent prognosis after surgical resection, with fewer than 10% of patients demonstrating distant metastases (usually to the liver) or locoregional lymphadenopathy and reported 5-year survival rates as high as 94–97 %. In fact, given the relative indolence of these lesions, it is not surprising that these tumors are increasingly being identified incidentally on imaging, with over 40% of all cases found in patients undergoing imaging for unrelated reasons.”
Multidetector Computed Tomography in the Evaluation of Cystic Tumors of the Pancreas Siva P. Raman, Elliot K. Fishman (in) Cystic Tumors of the Pancreas: Diagnosis and Treatment Marco Del Chiaro, StephanL.Haas, RichardD.Schulick Editors © Springer International Publishing Switzerland 2016 - “In some cases, the presence of gross intratumoral or peritumoral hemorrhage may be present and is very strongly suggestive of SPEN, although blood within the lesion is often easier to perceive on MRI compared to CT . In rare instances, SPEN can be associated with large retroperitoneal hemorrhage that may result in the patient’s presentation. Calcification is very frequent and may be seen either within the center of the lesion or peripherally.”
Multidetector Computed Tomography in the Evaluation of Cystic Tumors of the Pancreas Siva P. Raman, Elliot K. Fishman (in) Cystic Tumors of the Pancreas: Diagnosis and Treatment Marco Del Chiaro, StephanL.Haas, RichardD.Schulick Editors © Springer International Publishing Switzerland 2016
- Solid and Papillary Epithelial Neoplasm (SPEN): Demographics
• Age range usually teens to late twenties
• Females in the 2nd and 3rd decade with a mean age of 27 but a range of 8-72 years old
• Female to male ration nearly 10-1
• Clinical presentation commonly vague abdominal pain
• It is most often a benign neoplasm, but 10–15% of the cases are malignant.
• Long term outcome good with surgery and 5 year survival 95-100% - Solid and Papillary Epithelial Neoplasm (SPEN): CT Findings
• Average size is 5 cm but range is 1-10 cm
• Cystic and Solid Mass with capsule common
• Calcifications may be extensive especially around the border of the lesion
• Calcifications present in up to one third of cases
• Average size is 5 cm but range is 1-10 cm
• Lesion location more common in the tail of the pancreas - Solid and Papillary Epithelial Neoplasm (SPEN): Differential Diagnosis
• Cystic Pancreatic Masses
• Serous Cystadenoma (SC)
• Mucinous Cystic Neoplasm (MCN)
• Intraductal Papillary Mucinous Neoplasm (IPMN)
• Cystic Neuroendocrine NeoplasmsSPEN 
SC
MCN
- “Solid pseudopapillary neoplasms of the pancreas are rare tumors accounting for 1–2% of pancreatic exocrine neoplasms. This entity was first described by Dr. Frantz in 1959 and was defined by the World Health Organization in 1996 as “solid pseudopapillary tumor.” It is most often a benign neoplasm, but 10–15% of the cases are malignant. Over the past decades, the incidence of this tumor is increasing. However, many surgeons are still unfamiliar with this neoplasm and its unique characteristics, which can lead to pitfalls in the diagnosis and treatment. The correct diagnosis of SPNP is of utmost importance since it has a low malignant potential and with the appropriate treatment, patients have a long life expectancy.”
Solid Pseudopapillary Neoplasms of the Pancreas: A Surgical and Genetic Enigma Leon Naar et al. World J Surg (2017). doi:10.1007/s00268-017-3921-y
- “The presence of hemorrhage within a mixed solid and cystic tumor suggests solid and pseudopapillary tumor.”
Imaging features of rare pancreatic tumors M. Barrala, S.A. Faraound, E.K. Fishman, A. Dohan, C. Pozzesserea, M.-A. Berthelina,P. Bazeries, M. Barat, C. Hoeffel, P. Soyer Diagnostic and Interventional Imaging (2016) (in press)
- “ SPT, or Frantz tumor, accounts for 3% of all pancreatic tumors and 6% of all exocrine pancreatic tumors . SPT is mostly found in women in the 2nd and 3rd decade because of its progesterone dependency. The mean size of SPT is 50 mm at the time of diagnosis and the pancreatic head is the most common location. SPT is considered as a benign condition even if distant metastases or recurrence after resection have been reported. Histopathologically SPT consists of an encapsulated mixed tumor with cystic and pseudopapillary component.”
Imaging features of rare pancreatic tumors M. Barrala, S.A. Faraound, E.K. Fishman, A. Dohan, C. Pozzesserea, M.-A. Berthelina,P. Bazeries, M. Barat, C. Hoeffel, P. Soyer Diagnostic and Interventional Imaging (2016) (in press) - “Unenhanced CT shows calcifications in one third of the tumors. After intravenous administration of iodinated contrast material, SPT shows vivid enhancement during the arterial phase that persists during the portal and late phases.”
Imaging features of rare pancreatic tumors M. Barrala, S.A. Faraound, E.K. Fishman, A. Dohan, C. Pozzesserea, M.-A. Berthelina,P. Bazeries, M. Barat, C. Hoeffel, P. Soyer Diagnostic and Interventional Imaging (2016) (in press)
- SPEN Tumors of the Pancreas
• Majority occur in the first three decades of life
• Age range is 8-72 years with a mean age of 27 years
• Female to male ratio is 10-1
- Solid-pseudopapillary tumor of the pancreas (SPEN)
Solid-pseudopapillary tumor of the pancreas is an uncommon low-grade malignant neoplasm that is usually found in young women. It was first described by Frantz in 1959 as a “papillary tumor of the pancreas, benign or malignant” . This tumor was formerly referred to as solid and papillary epithelial neoplasm, papillary cystic neoplasm, papillary cystic epithelial neoplasm, papillary cystic tumor, solid and cystic tumor, and Frantz’s tumor. In 1996, The World Health Organization renamed this tumor as solid-pseudopapillary tumor for the international histologic classification of tumor of the exocrine pancreas. - Solid-pseudopapillary tumor of the pancreas (SPEN)
A reported incidence of solid-pseudopapillary tumor of the pancreas is 0.13 to 2.7% of all pancreatic tumors . Most cases are found in young women and a majority of this tumor occurs in the first three decades of life. Earlier reports indicated that this neoplasm tends to occur in non-Caucasian women . The male:female ratio is 1:9.5 . It is associated with a favorable prognosis, and after complete removal, most patients are cured . - Solid-pseudopapillary tumor of the pancreas (SPEN)
Treatment is surgical, and the overall prognosis is excellent after surgical treatment with a reported 5-year survival of 97% . However, solid-pseudopapillary tumor of the pancreas uncommonly results in local recurrence and metastases, and distant metastasis predominantly occurs in the liver . Lam et al. reviewed 452 reported cases of solid-pseudopapillary tumor of the pancreas in the English literature, and reported that 66 of 452 neoplasms (15%) were found to be malignant defined as having evidence of metastases or invasion of adjacent structures . Metastasis reportedly occurred at a mean interval of 8.5 years , and long-term follow-up for metastasis is required . Early diagnosis of tumor is crucial to allow the curative resection . - Solid-pseudopapillary tumor of the pancreas (SPEN)
Calcifications are commonly seen in solid-pseudopapillary tumors. By a study by Buetow et al., 16 of 56 patients (29%) had calcifications. Calcifications may be seen predominantly peripherally but internal calcifications may also be seen . Occasionally, dense, extensive peripheral calcifications may be associated with solid-pseudopapillary tumor of the pancreas. - Solid-pseudopapillary tumor of the pancreas (SPEN)
Acute presentation of solid-pseudopapillary tumor of the pancreas with rupture of the capsule and resulted hemoperitoneum is rare, but 8 cases were found among 292 reported cases in the literature reviewed by Mao et al . Five of these cases were without any identifiable cause . Acute trauma may cause rupture of the capsule and hemoperitoneum, and result in acute presentation of solid-pseudopapillary tumor of the pancreas. These patients may require urgent surgery. CT may reveal disruption of capsule of tumor, and retroperitoneal or interaperitoneal hemorrhage. Tumor may simulates a peri- or intrapancreatic hematoma .
"A focal lobulated margin and a focal discontinuity of the capsule may suggest a malignant SPN, whereas a round or smoothly lobulated margin and a complete encapsulation were more commonly seen in benign SPN."
Differentiation of Benign and Malignant Solid Pseudopapillary Neoplasms of the Pancreas
Chung YE et al.
J Comput Assist Tomogr 2009; 33: 689-694- Solid Pseudopapillary Neoplasm of the Pancreas: Facts
- Up to 30% contain calcification
- Occassionally these lesions can spontaneously bleed
- Pancreatic duct obstruction or common bile duct obstruction is rare
- Solid Pseudopapillary Neoplasm of the Pancreas: Facts
- 1-2% of exocrine pancreatic tumors
- Woman common especially young women
- 5 year survival up to 95-100%
- 15-20% of cases develop metastases with liver most common site of metastases - Solid and Papillary Epithelial Neoplasm: CT Findings
- Mixed cystic and solid mass
- Lesion may have increased vascularity
- Calcifications do occur but are uncommon
- Solid and Papillary Epithelial Neoplasm: Facts
- 1% of pancreatic masses
- More common in woman
- Age range is 15-35 with a mean of 24 yrs of age
- Low grade malignancy
- More common in the tail of the pancreas
- Hamoudi tumor (solid papillary epithelial neoplasm): facts
- Most common in young woman with mean age of 24 yrs
- May contain calcification
- Cysts components may be thick walled on CT