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Everything you need to know about Computed Tomography (CT) & CT Scanning

Pancreas: Spen Imaging Pearls - Educational Tools | CT Scanning | CT Imaging | CT Scan Protocols - CTisus
Imaging Pearls ❯ Pancreas ❯ SPEN

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  • 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 Neoplasms
    SPEN



    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
© 1999-2020 Elliot K. Fishman, MD, FACR. All rights reserved.