title: Institutional experience with solid pseudopapillary neoplasms: focus on computed tomography, magnetic resonance imaging, conventional ultrasound, endoscopic ultrasound, and predictors of aggressive histology.
abstract: OBJECTIVE
  Solid pseudopapillary neoplasms (SPNs) are low-grade malignancies with an excellent prognosis, albeit with the potential for metastatic disease. This study details our institution's experience with the diagnosis and treatment of SPN, including clinical presentation, multimodality imaging findings, and potential predictors of aggressive tumor behavior.
  
  
  METHODS
  The institutional pathology database was searched through for all cases of SPN since 1988, yielding 51 patients. The electronic medical record was searched for clinical and demographic information regarding these patients, including age, sex, presenting symptoms, type of surgery, postoperative length of stay, tumor markers, and postsurgical follow-up. All available imaging data were reviewed, including those of 30 patients who underwent multidetector computed tomography, those of 9 patients who underwent magnetic resonance imaging (MRI), those of 3 patients who underwent conventional ultrasound, and those of 11 patients who underwent endoscopic ultrasound.
  
  
  RESULTS
  A total of 84% of patients were females, with a mean age of only 33 years. Prognosis was excellent, with a mean follow-up of 3 years without recurrence. Only 1 of the 51 patients developed metastatic disease to the liver 8 years after the surgery. On computed tomography, lesions tended to be large (5.3 cm), well circumscribed (29/30), round/oval (20/30), and encapsulated (23/30). The lesions often demonstrated calcification (14/30) and typically resulted in no biliary or pancreatic ductal dilatation. The lesions ranged from completely cystic to completely solid. On MRI, the lesions often demonstrated a T2 hypointense or enhancing capsule (6/9) and demonstrated internal blood products (5/9). The lesions tended to be devoid of vascularity on conventional ultrasound. Ten patients were found to have "aggressive" histology at presentation (T3 tumor, nodal involvement, perineural invasion, or vascular invasion). No demographic, clinical, or multidetector computed tomographic imaging features were found to correlate with aggressive histology.
  
  
  CONCLUSIONS
  Certain imaging features (eg, well-circumscribed mass with calcification, peripheral capsule, internal blood products, and lack of biliary/pancreatic ductal obstruction) on computed tomography and MRI are highly suggestive of the diagnosis of SPN, particularly when visualized in young female patients. However, it is not possible to predict aggressive histology on the basis of imaging findings, clinical presentation, or patient demographic features.
authors.affiliation: From the *Department of Radiology, Johns Hopkins University; †Division of Gastroenterology, Johns Hopkins Hospital; ‡Division of Oncology Biostatistics, Johns Hopkins University School of Medicine; §Department of Surgery, Johns Hopkins University School of Medicine; and ∥The Sol Goldman Pancreatic Cancer Research Center, Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD.
authors.forename: Siva P
authors.initials: SP
authors.lastname: Raman
authors.forename: Satomi
authors.initials: S
authors.lastname: Kawamoto
authors.forename: Joanna K
authors.initials: JK
authors.lastname: Law
authors.forename: Amanda
authors.initials: A
authors.lastname: Blackford
authors.forename: Anne Marie
authors.initials: AM
authors.lastname: Lennon
authors.forename: Christopher L
authors.initials: CL
authors.lastname: Wolfgang
authors.forename: Ralph H
authors.initials: RH
authors.lastname: Hruban
authors.forename: John L
authors.initials: JL
authors.lastname: Cameron
authors.forename: Elliot K
authors.initials: EK
authors.lastname: Fishman
mesh_terms: D000328:Adult; D017677:Age Distribution; D000368:Aged; D002291:Carcinoma, Papillary; D003952:Diagnostic Imaging / Q000706:statistics & numerical data*; D005260:Female; D006801:Humans; D008297:Male; D008396:Maryland / Q000453:epidemiology; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery*; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D017678:Sex Distribution; D016896:Treatment Outcome
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