Ann Hepatobiliary Pancreat Surg.  2024 Feb;28(1):92-98. 10.14701/ahbps.23-083.

Solid pseudopapillary epithelial neoplasm of pancreas in pregnancy: A case report and review of literature

• Naik RKH
• Amudhan A
• Ashokkumar A
• Inbasekaran A
• Thangasamy S
• Sathyanesan J

• KMID: 2553370
• DOI: http://doi.org/10.14701/ahbps.23-083

Abstract

The solid pseudopapillary epithelial neoplasm (SPEN) of the pancreas is an uncommon tumor that accounts for approximately 1%–2% of exocrine pancreatic neoplasms. It predominantly affects female in their second and third decades of life. In this case report, we present a clinical scenario of a 21-year-old pregnant woman who incidentally discovered a solid cystic lesion in her pancreas, exhibiting features suggestive of SPEN. The patient underwent surgery during the second trimester. Management of pregnant females with SPEN poses challenges due to the absence of definitive treatment guidelines, particularly in determining the ideal timing for surgical intervention. Notably, during pregnancy, the presence of a small SPEN does not necessarily require immediate resection. However, if the tumor is of significant size, it can give rise to complications such as tumor rupture, multivisceral resection, recurrence, spontaneous abortion, intrauterine growth restriction, or premature delivery if not addressed. In the existing literature, a common finding is that approximately two-thirds of pregnant females with SPEN underwent surgery in the second trimester, often without complications for the mother or fetus. All these tumors were larger than 8 cm. The decision to operate before or after birth can be individualized based on team discussion. However, delay in surgery may lead to larger tumors and higher risks like bleeding, rupture, multivisceral resection, and recurrence. Therefore, second-trimester surgery seems safer, and lessens dangers, emergency surgery, and tumor recurrence.

Keyword

Pancreatic neoplasms; Pregnancy; Distal pancreatectomy; Splenectomy

Figure

• 

  Fig. 1 Ultrasound and MRI of the abdomen and pelvis. (A) Ultrasound showing solid cystic lesion in the body of the pancreas. (B) Gestational sac with the 10-week fetus. (C) MRI of the abdomen showing T1-heterogeneous lesion in the body of the pancreas. (D) Solid & cystic lesion with T2 heterogeneity.

• 

  Fig. 2 Intraoperative picture distal pancreatectomy and splenectomy with histopathological pictures. (A) Intraoperative finding – 13 × 12 × 10 cm in the body of the pancreas. T- tumor in the body of pancreas. (B) Distal pancreatectomy- splenectomy specimen. (C) Cut-open specimen showing solid and cystic area with intra tumoral hemorrhage. (D, E) Cells arranged in sheets, nests, and pseudopapillary pattern, eosinophilic cytoplasm, oval nuclei with fine chromatin and nucleoli grooving (H&E, ×10). SV, splenic vein.

• 

  Fig. 3 Pictorial depiction of SPEN in pregnancy reported in the literature. (A) Showing yearly reported cases, a total of 22 cases have been reported (including the current case). (B) Timing of surgery performed during pregnancy, 15 cases underwent surgery during the prenatal period, 6 cases during the postpartum period, and one case was managed conservatively with MTP due to DCLD. (C) Complication occurred in pregnancy, 5 cases of MTP/SA, 3 cases of multivisceral resection, and 3 cases of emergency surgery for tumor rupture (1) and intractable pain (1). (D) A total of 21 patients underwent surgeries, various surgeries were performed including 8 Whipple’s, 5 distal pancreatectomy, 3 distal pancreato-splenectomy, 3 multivisceral resections, and 2 enucleations. SPEN, solid pseudopapillary epithelial neoplasm; MTP, medical termination of pregnancy; SA, spontaneous abortion; DCLD, decompensated chronic liver disease.

Reference

1. Humphreys GH 2nd. 1968; In memoriam. Virginia Kneeland Frantz, M.D. 1896-1967. Am J Clin Pathol. 49:429–430. DOI: 10.1093/ajcp/49.3.429. PMID: 4868790.

2. Yu Y, Teng L, Liu J, Liu X, Peng P, Zhou Q, et al. Pregnancy complicated with a giant pancreatic tumor and decompensation of liver cirrhosis: a case report and literature review. Matern-Fetal Med. 2022; 10.1097/FM9.0000000000000168. DOI: 10.1097/FM9.0000000000000168.

3. Rafay Khan Niazi M, Dhruv S, Polavarapu A, Toprak M, Mukherjee I. 2021; Solid pseudopapillary neoplasm of the uncinate process of the pancreas: a case report and review of the literature. Cureus. 13:e15125. DOI: 10.7759/cureus.15125.

4. Huang TT, Zhu J, Zhou H, Zhao AM. 2018; Solid pseudopapillary neoplasm of pancreas in pregnancy treated with tumor enucleation: case report and review of the literature. Niger J Clin Pract. 21:1234–1237. DOI: 10.4103/njcp.njcp_39_18. PMID: 30156213.

5. Huang SC, Wu TH, Chen CC, Chen TC. 2013; Spontaneous rupture of solid pseudopapillary neoplasm of the pancreas during pregnancy. Obstet Gynecol. 121(2 Pt 2 Suppl 1):486–488. DOI: 10.1097/AOG.0b013e31826d292f. PMID: 23344418.

6. Al-Umairi RS, Kamona A, Al-Busaidi F. 2015; Solid pseudopapillary tumor in a pregnant woman: imaging findings and literature review. Oman Med J. 30:482–486. DOI: 10.5001/omj.2015.94. PMID: 26673875. PMCID: PMC4678441.

7. Ganepola GA, Gritsman AY, Asimakopulos N, Yiengpruksawan A. 1999; Are pancreatic tumors hormone dependent? A case report of unusual, rapidly growing pancreatic tumor during pregnancy, its possible relationship to female sex hormones, and review of the literature. Am Surg. 65:105–111. DOI: 10.1177/000313489906500202. PMID: 9926740.

8. Mahabane R, Khaba M. 2020; Solid pseudopapillary tumour of the pancreas in pregnancy - a case report and literature review. South Afr J Obstet Gynaecol. 26:35–37. DOI: 10.7196/sajog.1623.

9. Ahmad R, Baia M, Naumann DN, Mahmood F, Tirotta F, Ford S, et al. 2022; Emergency multivisceral resection for spontaneous haemorrhage rupture of huge solid pseudopapillary neoplasm of the pancreas during pregnancy. J Surg Case Rep. 2022:rjac331. DOI: 10.1093/jscr/rjac331. PMID: 35903665. PMCID: PMC9322990.

10. Santos D, Calhau A, Bacelar F, Vieira J. 2020; Solid pseudopapillary neoplasm of pancreas with distant metastasis during pregnancy: a diagnostic and treatment challenge. BMJ Case Rep. 13:e237309. DOI: 10.1136/bcr-2020-237309. PMID: 33298487. PMCID: PMC7733072.

11. Feng JF, Chen W, Guo Y, Liu J. 2011; Solid pseudopapillary tumor of the pancreas in a pregnant woman. Acta Gastro-Enterol Belg. 74:560–563.

12. MacDonald F, Keough V, Huang WY, Molinari M. 2014; Surgical therapy of a large pancreatic solid-pseudopapillary neoplasm during pregnancy. BMJ Case Rep. 2014:bcr2013202259. DOI: 10.1136/bcr-2013-202259. PMID: 24445849. PMCID: PMC3902659.

13. Tanacan A, Orgul G, Dogrul AB, Aktoz F, Abbasoglu O, Beksac MS. 2018; Management of a pregnancy with a solid pseudopapillary neoplasm of the pancreas. Case Rep Obstet Gynecol. 2018:5832341. DOI: 10.1155/2018/5832341. PMID: 29850316. PMCID: PMC5926515.

14. Chhabra M, Daver RG. 2017; Solid pseudopapillary epithelial neoplasm of pancreas in pregnancy: case report of a rare co-occurrence. J Med Sci Clin Res. 5:26978–26983. DOI: 10.18535/jmscr/v5i8.164.

15. Sharanappa V, Tambat RM, Nm S, Razack A. 2015; Solid pseudopapillary tumour of pancreas in pregnancy: case report. Sch J Med Case Rep. 3:40–46.

16. Tanaka K, Nagamine M, Kihara Y, Yokomizo H. 2020; A case of solid-pseudopapillary neoplasm diagnosed during pregnancy. Nihon Rinsho Geka Gakkai Zasshi J Jpn Surg Assoc. 81:570–575. DOI: 10.3919/jjsa.81.570.

17. Ganzoui I, Nouri D, Balti M, Ayed K. 2021; Pancreatitis during pregnancy revealing solid pseudopapillary tumor of the pancreas: case report. Acta Sci Womens Health. 3:38–41. DOI: 10.31080/ASWH.2021.03.0285.

18. Hajdú N, Pohárnok Z, Oláh A. 2009; Successfully resected pancreatic tumor in pregnancy. Eur Surg. 41:48–50. DOI: 10.1007/s10353-009-0443-3.

19. Yee AM, Kelly BG, Gonzalez-Velez JM, Nakakura EK. 2015; Solid pseudopapillary neoplasm of the pancreas head in a pregnant woman: safe pancreaticoduodenectomy postpartum. J Surg Case Rep. 2015:rjv108. DOI: 10.1093/jscr/rjv108. PMID: 26294703. PMCID: PMC4542138.

20. Rathi J, Anuragi G, J R LJ, R P, C S, O L NB. 2021; Prediction of recurrence risk in solid pseudopapillary neoplasm of the pancreas: single-institution experience. Cureus. 13:e17541. DOI: 10.7759/cureus.17541. PMID: 34646598. PMCID: PMC8478690.