Solid Pseudopapillary Tumor of the Pancreas – Rare Neoplastic Disease in 20-Year-Old Woman

Česká verzia

Authors: Fichtl Jakub ¹; Skalický Tomáš ¹; Vodička Josef ¹; Třeška Vladislav ¹; Tupý Radek ²; Hes Ondřej ³
Authors place of work: Chirurgická klinika LF UK a FN Plzeň ¹; Klinika zobrazovacích metod LF UK a FN Plzeň 3 Šiklův ústav patologie, LF UK a FN Plzeň ²
Published in the journal: Klin Onkol 2018; 31(5): 376-379
Category: Kazuistika
doi: https://doi.org/10.14735/amko2018376

Summary

Introduction:

Benign cystic tumors represent only 2% of all pancreatic tumors (pancreatic cancer – PC). In contrast to malignant cystic tumors, these tumors occur typically in young women. A solid pseudopapillary tumor is a relatively rare affliction representing less than 4% of cystic PC. Although the tumor is considered benign, metastasis, especially to the spleen, has been reported in approximately 0.5–4% patients. Despite R0 resection, vascular and perineural invasion is monitored in 20% of cases. Invasion is the cause of tumor relapse in up to one third of affected patients. Characteristic features of the disease are latent clinical indicators such as signs of pain and malfunction of intestinal passage. The diagnostics is based on MR, sometimes in combination with positron emission tomography. Medical treatment is specifically surgical.

Case history:

Authors present a case of a 20-year-old female patient who was examined due to pain in the epigastrium, further exasperated by a voluminous expansion of the abdominal cavity. An initial ultra-sonographic examination was conducted to examine for possible nodular focal nodular hyperplasia of the liver; however, an MRI scan revealed the likelihood of a malignant tumor in the subhepatic region. During laparotomy, a tumor protruding from the head of the pancreas was discovered and removed. Histological examination showed it was a solid pseudopapillary pancreatic tumor. After a month of good post-operative progress, the patient was re-operated because of the presence of pancreatic fistula. Complete healing of the fistula was achieved after total parenteral nutrition and administration of sandostatin. At her last examination, the patient was without any problems.

Conclusion:

Solid pseudopapillary pancreatic tumors are rare, mainly benign lesions. It is essential to consider them in the differential diagnostics of afflictions of the subhepatic region, especially in young women. The only generally accepted cure nowadays is surgical resection. It is necessary to monitor patients consistently considering the rather high frequency of relapse of disease despite R0 resections. In the case of surgical removal, the 5-year survival rate is near 97%.

Key words:

solid pseudopapillary tumor of pancreas – diagnostics – therapy

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers.

Submitted: 17. 4. 2018

Accepted: 13. 8. 2018

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