Solid pseudo papillary-cystic tumour

Clinical features

  • Rare tumour accounting for 0.17% to 2% of all nonendocrine tumours of the pancreas
  • Adolescents girls and young women
  • Benign tumour

 

Fig 83- Solid –pseudopapillary neoplasm -– Delicate papillary groups (Giemsa)
Fig 84 - Solid –pseudopapillary neoplasm – Delicate papillary groups covered by monomorphic tumor cells and with visible vascular stalks. Individual scattered cells (H&E)
Fig 85 - Solid –pseudopapillary neoplasm – cells have nuclei with fine chromatin, small nucleoli and in some nuclear grooves (H&E)
Fig 86  - Solid –pseudopapillary neoplasm – aspiration smear of a papillary-cystic tumor showing a branched vascular stalk. Nuclear grooves can also be noted. (Giemsa).
Fig 87 - Solid –pseudopapillary neoplasm – aspiration smear of a papillary-cystic tumor showing a branched vascular stalk. Nuclear grooves can also be noted. (Giemsa).

 

 

  • Branching delicate papillary structures covered by monomorphic tumour cells
  • Individual scattered cells
  • Nuclei with fine chromatin and small nucleoli
  • Nuclear grooves
  • Foamy histiocytes
  • Multinucleated  giant cells
  • Diastase-resistant PAS-positive hyaline globules

 

Immunocytochemistry

  • Vimentin: Positive
  • NSE: Positive
  • Synaptophysin: Positive
  • Alpha1-antitrypsin :Positive intracytoplasmic globules
  • Alpha1-antichymotrypsin:Positive intracytoplasmic globules
  • CD56- Positive-Intense and diffuse
  • CD10: Positive
  • Chromogranin: negative
  • CEA: Negative

 

Genetic Studies

  • Most tumours are diploid

 

Differential Diagnosis

  • Mucinous cystic neoplasms /microcystic adenomas
    • Presence of mucoid material in the background
  • Acinar cell carcinoma
    • Older patients
    • No female predominance
    • Cytological atypia

 

Main points

  • 95% cure after resection
  • All the attempts to predict an aggressive behaviour have been unsuccessful
  •  In 10% of the cases it can metastasize to the liver and peritoneum, generally associated to venous invasion and high grade
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