Open Access

Multicentric solid pseudopapillary neoplasms of the pancreas diagnosed by endoscopic ultrasound-guided fine needle aspiration: a case report

  • Megumi Yamaguchi1Email authorView ORCID ID profile,
  • Toshikatsu Fukuda1,
  • Masahiro Nakahara1,
  • Mio Amano2,
  • Daisuke Takei1,
  • Masumi Kawashima1,
  • Yusuke Sumi1,
  • Hironobu Amano1,
  • Shuji Yonehara3,
  • Keiji Hanada2 and
  • Toshio Noriyuki4
Surgical Case Reports20151:110

https://doi.org/10.1186/s40792-015-0111-8

Received: 21 April 2015

Accepted: 16 October 2015

Published: 27 October 2015

Abstract

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare tumor. This neoplasm usually arises as a single mass; multicentricity is exceptionally rare. We report the preoperative diagnosis of multicentric SPNs by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA). A 32-year-old woman presented to the hospital with a pancreatic tumor that was detected on abdominal echography. Contrast-enhanced computed tomography (CT) scans revealed a 5-mm low-density mass in the body of the pancreas and a 10-mm mass in the tail of the pancreas. Magnetic resonance imaging (MRI) also revealed two tumors in the body and tail of the pancreas. On endoscopic ultrasonography (EUS), two indistinct and heterogeneous echogenic masses were found, and EUS-FNA was performed for each of these tumors. Cytological analysis revealed that the two masses were highly cellular with papillary groups of small, uniform, oval cells surrounding a fibrovascular core. Immunohistochemistry was positive for α-1 antitrypsin, vimentin, neuron-specific enolase (NSE), CD10, and progesterone receptor. These features confirmed the preoperative diagnosis of multicentric SPNs. The patient underwent laparoscopic distal pancreatectomy with splenectomy. The final pathologic diagnosis was multicentric SPNs. During 2 years of follow-up, she has not developed any recurrence.

Keywords

MulticentricSolid pseudopapillary neoplasm (SPN)Endoscopic ultrasound-guided fine needle aspiration (EUS-FNA)

Background

Solid pseudopapillary neoplasm (SPN) of the pancreas is rare, accounting for approximately 0.13–2.7 % of all pancreatic tumors and 1–2 % of all exocrine pancreatic tumors [1]. SPN usually occurs in young women and has low potential for malignancy. In rare cases, it may spread to the lymph nodes, resulting in distant metastasis. Therefore, complete surgical resection is the main treatment for SPN.

SPN is usually a solitary mass, and multicentricity is exceptionally rare. This case report describes the preoperative diagnosis of multicentric SPNs by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA).

Case presentation

A 32-year-old woman presented to our hospital with a pancreatic body tumor, which was identified by abdominal echography during health screening. No symptoms were evident, and her medical history was unremarkable.

A contrast-enhanced computed tomography (CT) scan revealed a 5-mm low-density mass in the pancreatic body, which was gradually enhanced in the portal vein phase, and a 10-mm mass in the pancreatic tail, which had the same density and enhancement as the other mass (Fig. 1).
Fig. 1

Contrast-enhanced computed tomography scan. (a-1) There was a 5-mm low-density mass in the pancreatic body. (a-2, 3) This mass was gradually enhanced in the portal vein phase and the late phase. (b-1) There was also a 10-mm low-density mass in the pancreatic tail. (b-2, 3) This mass had the same density and enhancement as the other mass

On magnetic resonance imaging (MRI), the two tumors in the body and tail of the pancreas had lower signal intensity on T1-weighted images and higher signal intensity on T2-weighted and diffusion-weighted images. There was no stenosis of the main pancreatic duct (MPD). We observed neither dilatation of the distal side of the pancreatic duct nor communication between the tumors and the MPD.

Endoscopic ultrasonography (EUS) showed two indistinct and heterogeneous echo-poor masses (Fig. 2). The patient subsequently underwent EUS-FNA for each mass. On cytological analyses, the tumors were found to be highly cellular masses with papillary groups of small and uniform cells with oval nuclei surrounding a fibrovascular core. Immunohistochemically, the two masses were positive for α-1 antitrypsin, vimentin, neuron-specific enolase (NSE), CD10, and progesterone receptor. There was no histologic difference between the tumors. These features confirmed the preoperative diagnosis of multicentric SPNs (Fig. 3).
Fig. 2

Endoscopic ultrasonography revealed indistinct and heterogeneous echogenic masses in the pancreatic body and tail. a body, b tail. SV splenic vein

Fig. 3

Endoscopic ultrasound-guided fine needle aspiration. a Cytological analyses revealed that the two masses were highly cellular and had papillary groups of small and uniform cells with oval nuclei surrounding a fibrovascular core. Immunohistochemistry was positive for b CD10, c α-1 antitrypsin, d vimentin, e neuron-specific enolase, and f progesterone receptor

The patient underwent laparoscopic distal pancreatectomy (LDP). In order to achieve bleeding control, it became necessary to perform splenectomy because bleeding from the splenic vein developed while separating this vein from the pancreas. The final pathologic diagnosis was multicentric SPNs (Fig. 4). She recovered well during the immediate postoperative period and was discharged from the hospital 15 days later. During 2 years of follow-up, she has not developed any recurrence.
Fig. 4

Histopathological findings. On macroscopic examination, both tumors were uncoated and solid lesions. (a-1) The tumor in the pancreatic body and (a-2) the tumor in the pancreatic tail. b Cytologic analysis revealed characteristic branching papillae with myxoid stroma in the tumor of pancreatic tail (hematoxylin and eosin [HE], ×100). c The tumor of the pancreatic body had the same characteristic features as the other tumor in HE staining (HE, ×100). d The tumor composed of small and uniform cells with oval nuclei surrounding a fibrovascular core (HE, ×400)

SPN is classified as an epithelial neoplasm of uncertain differentiation in the General Rules for the Study of Pancreatic Cancer, 6th Edition. Historically, SPN accounts for only 0.13–2.7 % of all pancreatic tumors and 1–2 % of exocrine pancreatic tumors [1]. SPNs usually occur in young women and have low malignant potential. In rare cases, SPNs may spread to the lymph nodes, resulting in distant metastasis. Therefore, complete surgical resection is necessary in patients with SPNs.

In this report, we have described a case of multicentric pancreatic tumors. The patient received a confirmed preoperative diagnosis of SPNs based on EUS-FNA findings, and she was therefore able to undergo laparoscopic surgery. SPN is usually a solitary mass, and multicentricity is exceptionally rare. To examine the rarity of multicentric SPNs, we performed searches of the PubMed database using the following keywords: (multicentric OR multi-centric OR two synchronous) AND (SPN). These searches revealed only 10 cases of multicentric SPNs that were reported recently (Table 1) [29]. Nine of these 10 cases involved two pancreatic tumors, as assessed based on abdominal CT scans; however, these patients did not receive a preoperative SPN diagnosis. Only one case [5] included EUS-FNA and a special stain of beta-catenin that suggested multicentric SPN. The present case is the first reported instance in which the patient received laparoscopic surgery.
Table 1

Recently reported cases of multicentric SPNs

 

Case 1 [2]

Case 2 [3]

Case 3 [4]

Case 4 [5]

Case 5 [6]

Case 6 [7]

Case 7 [7]

Case 8 [7]

Case 9 [8]

Case 10 [9]

This case

Sex

Female

Female

Female

Female

Female

Female

Male

Female

Female

Female

Female

Age

26

17

31

34

24

57

56

44

24

16

32

Site

Head

Body

Head

Body

Head

Head

Body

Head

Head

Head

Body

Tail

Tail

Tail

Tail

Tail

Body

Tail

Body

Tail

Tail

Tail

Size (cm)

2.3

3

8

3.7

5

1.5

3

4

Unclear

10

0.5

9

6

19

2

8

3

12

11

7

1

Chief complaint

Abdominal pain

Abdominal pain

Abdominal pain

No

No

No

Abdominal mass

Abdominal mass

No

Abdominal pain

No

Preoperative diagnosis

Single mass

Not definite

Endocrine neoplasms

SPN

SPN considered

Not definite

Not definite

Endocrine carcinoma

SPN considered

Not definite

SPN

Treatment

TP

Unclear

DP

DP

DP

Unclear

DP

PD

LP

Tumor enucleation

LDP

PD

PPPD

PD

TP total pancreatectomy, DP distal pancreatectomy, PD pancreaticoduodenectomy, PPPD pulorus-preserving pancreaticoduodenectomy, LP left pancreatectomy, LDP laparoscopic distal pancreatectomy, SPN solid pseudopapillary neoplasm

In this case, it was difficult to diagnose SPN before surgery because the tumors had atypical features that did not resemble SPN: they were uncoated, solid, and multicentric lesions. CT scans are useful for diagnosing SPN, which is usually described as an encapsulated lesion with cystic degeneration on CT. However, small SPNs without cysts and capsules have been reported [1]. The present case involved two uncircumscribed tumors that were 5 and 10 mm in diameter, which may have been a reason for the atypical tumor features on CT. The differential diagnosis of multicentric pancreatic tumors can include endocrine neoplasms, intraductal papillary mucinous tumors, serous cystic neoplasms, mucinous cystic neoplasms, tumor-forming pancreatitis, pancreatic carcinoma, other complicated tumors, and metastatic pancreatic tumors. SPNs were considered in the differential diagnosis in this case because the patient was a young woman. However, a definitive diagnosis was not possible because the tumors were uncoated, solid, and multicentric lesions.

EUS-FNA is a useful diagnostic tool for pancreatic tumors—it has been reported to have a 91 % sensitivity and a 94 % specificity for diagnosing these tumors [10]. Song et al. found that the cytological features of SPN, as assessed by EUS-FNA, are highly characteristic and distinct from those of other cystic or solid pancreatic tumors [11]. Additionally, Law et al. reported that the addition of EUS-FNA increased the diagnostic yield to 82.4 %, as compared with CT (23.5 %), EUS (41.2 %), or CT and EUS (52.9 %) [12]. Furthermore, the overall complication rate of EUS-FNA was reported to be <1 % in large centers [13]. In one study, the complications of EUS-FNA included hemorrhage (0.96 %), acute pancreatitis (0.19 %), and duodenal perforation (0.09 %) [14]. It has generally been reported that EUS-FNA is a useful and safe method.

In recent years, the number of laparoscopic pancreatic procedures has increased because of the growing experience with laparoscopic surgery and availability of the relevant technology. LDP has gained worldwide acceptance because it does not require anastomosis or other reconstruction. In a recent comparison with conventional open surgery, LDP decreased blood loss and morbidity and promoted early recovery and shorter hospital stays [15]. It is important to confirm the LDP diagnosis before the operation; LDP should not be carried out in patients with invasive pancreatic carcinoma because the oncological consequences of laparoscopic pancreatic surgery remain quite controversial.

Conclusions

We have reported a case of multicentric pancreatic SPN that was diagnosed preoperatively by EUS-FNA. No consensus exists on the prognosis and biological features of SPN. Thus, for tumors with atypical features that do not suggest SPN, EUS-FNA is a useful and safe method of definitively diagnosing SPN preoperatively.

Consent

Witten informed consent was obtained from the patient for publication of this case report and any accompanying images.

Abbreviations

CT: 

computed tomography

EUS: 

endoscopic ultrasonography

EUS-FNA: 

endoscopic ultrasound-guided fine needle aspiration

LDP: 

laparoscopic distal pancreatectomy

MRI: 

magnetic resonance imaging

NSE: 

neuron-specific enolase

SPN: 

solid pseudopapillary neoplasm

Declarations

Acknowledgements

None

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

Authors’ Affiliations

(1)
Department of Surgery, Onomichi General Hospital
(2)
Department of Gastroenterology, Onomichi General Hospital
(3)
Department of Diagnostic Pathology, Onomichi General Hospital
(4)
Faculty of Medicine, Hiroshima University

References

  1. Nakagawa N, Murakami Y, Uemura K, Hayashidani Y, Sudo T, et al. Eight cases of solid―pseudopapillary tumors (SPT) of the pancreas. Suizo. 2008;23:132–9.View ArticleGoogle Scholar
  2. Orlando CA, Bowman RL, Loose JH. Multicentric papillarycystic neoplasm of the pancreas. Arch Pathol Lab Med. 1991;115:958–60.PubMedGoogle Scholar
  3. Kobayashi T, Kimura T, Takabayashi N, Sugimura H. Two synchronous solid and cystic tumors of the pancreas. J Gastroenterol. 1998;33:439–42.View ArticlePubMedGoogle Scholar
  4. Lloyd Jr FP, Kang J. Multifocal papillary-cystic neoplasm of the pancreas. J Natl Med Assoc. 2003;95:1204–7.PubMed CentralPubMedGoogle Scholar
  5. Matsumoto K, Hara K, Sawaki A, Mizuno N, Hijioka S, et al. Pre-operative diagnosis of synchronous solid-pseudopapillary neoplasms of the pancreas. Clin J Gastroenterol. 2010;3(6):301–6.View ArticlePubMedGoogle Scholar
  6. Hu S, Lin X, Song Q, Chen K. Multidetector CT of multicentric solid pseudopapillary tumor of the pancreas: a case report and review of the literature. Cancer Imaging. 2011;11:175–8.PubMed CentralView ArticlePubMedGoogle Scholar
  7. Li HX, Zhang Y, Du ZG, Tang F, Qi XQ, et al. Multi-centric solid-pseudopapillary neoplasm of the pancreas. Med Oncol. 2013;30:330.View ArticlePubMedGoogle Scholar
  8. Cheng K, Shen BY, Peng CH, Na LM, Cheng DF, et al. Middle-preserving pancreatectomy: report of two cases and review of the literature. World J Surg Oncol. 2013;11:106.PubMed CentralView ArticlePubMedGoogle Scholar
  9. Jurić I, Pogorelić Z, Stepan JG, Kuzmić IP. Extremely rare presentation of Frantz's tumour: synchronous localisation in the pancreatic head and tail. Scott Med J. 2014;59(3):e8–e12.View ArticlePubMedGoogle Scholar
  10. Hewitt MJ, McPhail MJ, Possamai L, Dhar A, Viavianos P, et al. EUS-guided FNA for diagnosis of solid pancreatic neoplasms: a meta-analysis. Gastrointest Endosc. 2012;75:319–31.View ArticlePubMedGoogle Scholar
  11. Song JS, Yoo CW, Kwon Y, Hong EK. Endoscopic ultrasound-guided fine needle aspiration cytology diagnosis of solid pseudopapillary neoplasm: three case reports with review of literature. Korean J Pathol. 2012;46:399–406.PubMed CentralView ArticlePubMedGoogle Scholar
  12. Law JK, Stoita A, Wever W, Gleeson FC, Dries AM, et al. Endoscopic ultrasound-guided fine needle aspiration improves the pre-operative diagnostic yield of solid-pseudopapillary neoplasm of the pancreas: an international multicenter case series (with video). Surg Endosc. 2014;28:2592–8.View ArticlePubMedGoogle Scholar
  13. Fujii M, Saito H, Kato H, Kojima T, Ito M, et al. Diagnosis of a solid pseudopapillary neoplasm using EUS-FNA. Intern Med. 2013;52:1703–8.View ArticlePubMedGoogle Scholar
  14. Carrara S1, Arcidiacono PG, Mezzi G, Petrone MC, Boemo C, et al. Pancreatic endoscopic ultrasound-guided fine needle aspiration: complication rate and clinical course in a single centre. Dig Liver Dis. 2010;42(7):520–3.View ArticlePubMedGoogle Scholar
  15. Iacobone M, Citton M, Mitti D. Laparoscopic distal pancreatectomy: up-to-date and literature review. World J Gastroenterol. 2012;18(38):5329–37.PubMed CentralView ArticlePubMedGoogle Scholar

Copyright

© Yamaguchi et al. 2015