Clinical Case Report Medicine ®

OPEN

Spontaneous rupture of solid pseudopapillary

tumor of pancreas
A case report and review of literature
Xiaofeng Xu, MDa, Diyu Chen, MDa,b, Linping Cao, MDa, Xiaode Feng, MDa,b, Rongliang Tong, MDa,b,
∗
Shusen Zheng, MDa,b,c, , Jian Wu, MDa,b,c

Abstract
Introduction: Solid pseudopapillary tumors (SPT) account for 1% to 3% of all pancreatic tumors. They have low malignant
potential with a favorable prognosis, and predominantly occur in young women. The pathogenesis and clinical behavior of SPT are
still uncertain. In addition, most ruptures of SPT were associated with blunt abdominal trauma, while spontaneous ruptures seemed
to be quite rare. Up to now, there have been only 3 spontaneous ruptured SPT cases reported worldwide.
Patient concerns: Here, we reported a 22-year-old female patient with left lower abdominal pain. Computed tomography (CT)
showed that a hemorrhagic complex solid cystic mass located in the lesser omentum sac.
Diagnosis: According to pathological findings of tumor specimen, the diagnosis of solid pseudopapillary tumor (SPT) of the
pancreas was made.
Interventions: Distal pancreatectomy and splenectomy was carried out.
Outcomes: The patient recovered to normal status within 10 days after surgery.
Conclusion: Besides, we reviewed about 50 cases in literatures to find out the clinical characteristics and differential diagnostic
strategies of SPT.
Abbreviations: CT = computed tomography, EUS = endoscopic ultrasound, MRI = magnatic resonance imaging, SPT = solid
pseudopapillary tumors.
Keywords: cyst, neoplasm, pancreas, rupture, solid pseudopapillary tumors

1. Introduction
Editor: N/A.
Solid pseudopapillary tumor (SPT) of the pancreas is a kind
XX, DC, and LC are joint first authors.
of rare neoplasm, which represents less than 3% of all
Patient has provided informed consent for publication of the case. exocrine pancreatic tumors. SPT is prevalent among young
This study was sponsored by grants from the National Natural Science females, with a median age of 20 to 30 years old.[1,2] When SPT
Foundation of China (No. 81874228), Zhejiang Provincial Program for Cultivation
represents in male, it has greater malignant potential with a
of High-Level Innovative Health Talents, the Science and Technology Department
of Zhejiang Province (grant no. 2015C03034). worse prognosis. Besides, most of the ruptures of SPT were
Informed written consent was obtained from the patient for publication of this
associated with blunt abdominal trauma, while the spontane-
case report and accompanying images. ous ruptures seemed to be quite rare. Furthermore, the
The authors have no conflicts of interests to disclose. symptoms of SPT are not typical in general. Symptoms can
a
Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First
occasionally occur due to the size and location of the tumor but
Affiliated Hospital, School of Medicine, Zhejiang University,, b Key Laboratory of usually are nonspecific.[3] Because of its unusual behavior, SPT
Combined Multi-organ Transplantation, Ministry of Public Health,, c Collaborative is often associated with diagnostic and therapeutic chal-
Innovation Center for Diagnosis Treatment of Infectious Diseases, Hangzhou, lenges.[4] Computed tomography (CT) and magnetic resonance
Zhejiang, China.
∗
imaging (MRI) is beneficial for the diagnosis of this tumor.[5]
Correspondence: Shusen Zheng, Department of Hepatobiliary Surgery, the First Surgical resection is now considered as the most efficient
Affiliated Hospital, Zhejiang University School of Medicine, No. 79 Qingchun
Road, Hangzhou 310003, Zhejiang Province, China (e-mail:
treatment option for patients with SPT, because it offers a good
shusenzheng@zju.edu.cn). chance of long-term survival.
Copyright © 2019 the Author(s). Published by Wolters Kluwer Health, Inc.
This is an open access article distributed under the Creative Commons 2. Case report
Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and
reproduction in any medium, provided the original work is properly cited.
The patient was a 22-year-old female, who presented with 2 days
history of left lower abdominal pain. She was taken to the local
How to cite this article: Xu X, Chen D, Cao L, Feng X, Tong R, Zheng S, Wu J.
Spontaneous rupture of solid pseudopapillary tumor of pancreas. Medicine hospital, and the computed tomography scan revealed a large
2019;98:44(e17554). occupying lesion in the peripancreatic clearance. She was treated
Received: 6 January 2019 / Received in final form: 20 August 2019 / Accepted: with antibiotics and intravenous fluid therapy, but the symptom
19 September 2019 still advanced. Then she was admitted to the emergency
http://dx.doi.org/10.1097/MD.0000000000017554 department of our hospital. Physical examination revealed the

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Xu et al. Medicine (2019) 98:44 Medicine

Figure 1. The CT scan demonstrated a cystic lesion (about 95  75 mm) located in the pancreas.

tenderness and rebound-tenderness of the whole abdomen and In order to stop bleeding in time, the patient underwent
abdominal muscular tension was obvious. Laboratory findings emergency excision laparotomy of the cyst based on clinical and
revealed elevated leukocytosis (12.3  109/L), neutrophile gran- radiological findings. We located the tumor in the body of the
ulocytes (86.3% of the leukocytes) and decreased hemoglobin pancreas (about 8  7 cm), and it had invaded to the spleen.
(Hb) (105 g/L). Then a CT scan was performed again to assess the Therefore, distal pancreatectomy (including the cyst, the body
properties of the abdominal lesion. The review result of the CT and tail of pancreas) and splenectomy were performed. The
scan showed a hemorrhagic complex solid cystic mass located in pathology results reported a solid-cystic mass (8  6.5  5 cm)
the lesser omentum sac, which was considered to be originated with heterogenesis. Histological examination indicated that a
from pancreas (Fig. 1). solid and vascular pattern with pseudo-papillary cores (Fig. 2).

Figure 2. A. H&E staining shows that gland-like structure was lined by round tumor cells in solid pseudopapillary tumors specimen (original magnification  100).
B–H: The immunohistochemical results of solid pseudopapillary tumors specimen.

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Figure 3. Trend of platelet change after surgery of this patient.

Immunohistochemical (IHC) stains for CK (pan), CD-10, PR, 3. Discussion

CyclinD1, CD-56 and b-Catenin showed positivity, but CgA and
3.1. Description of solid pseudopapillary tumors of
E-cadherin staining were negative. On the whole, final
pathological diagnosis was solid pseudopapillary tumor of the pancreas
pancreas. In the postoperative period, the patient had high level SPT is an uncommon and enigmatic pancreatic neoplasm firstly
of platelet (PLT) (12.3  109/L, Fig. 3), and it was thought to be described by Frantz in 1959,[3] which is considered to be low
induced by the resection of spleen. After treated by acute malignant potential.[6] It represents 0.2% to 2.7% of tumors in
preoperative plateletpheresis, PLT level came back to normal. A the pancreas.[7] Frequently, it is identified as solid and cystic
follow-up 10 months later showed neither signs of tumor tumor, solid and papillary epithelial neoplasm, papillary-cystic
recurrence nor endocrine and exocrine insufficiency of the neoplasm, papillary-cystic epithelial neoplasm, papillary-cystic
pancreas (Fig. 4). tumor or Franz tumor. The concept of SPT for the international

Figure 4. Abdominal ultrasound scan at 10 months after operation.

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histological classification of tumors of the exocrine head-body junction (4/50) or, in the body-tail junction of
pancreas was firstly put forward by the World Health pancreas (10/50).
Organization (WHO) in 1996.[8] Then we collected 50 SPT
cases from pubmed database to ensure the characters of SPT.
3.2. Spontaneous rupture of solid pseudopapillary tumors
Until now, the cases reported showed that SPT commonly occurs
of pancreas
in the head or tail region of pancreas. As shown in Table 1, 17 of
the cases had the SPT in the head of pancreas. While, 5 The symptoms of SPT are usually nonspecific, with abdominal
reported cases had SPT in the body, and 8 cases occurred in pain being the most common. SPT which was discovered after
the tail. The remaining cases reported the tumor located in the rupture and hemoperitoneum was rare.[9] According to the cases

Table 1
Summary information of the 50 cases reviewed in literatures.
Year Authors Nation Sex Age Size Clinical Treatment
2018 Hooper et al [19]
Spain Female 31 1.7  1.4 cm pancreatoduodenectomy
2018 Liang B et al[20] Spain Female 35 2  2 cm distal pancreatectomy with splenectomy
2018 Estifan et al[21] USA Female 15 10  12 cm distal pancreatectomy
2018 Burk et al[22] India Female 12 NM distal pancreatectomy with splenectomy
2018 Filatov et al[23] USA Female 27 2.1 x 1.8 cm distal pancreatectomy with splenectomy
2018 Bender et al[24] India Female 49 8.2  14  11 cm tumor resection
2018 Lanke et al[25] Brazil Female 19 3.0  2.2 cm pancreatoduodenectomy
2017 Kim SS et al[26] Brazil Female 18 4.1  3.3 cm pancreatoduodenectomy
2017 Lawlor et al[27] Sri Lanka Female 19 15  18cm distal pancreatectomy with splenectomy
2017 Kriger et al[28] Poland Female 15 4.8  4.2  5 cm pancreatoduodenectomy
2017 Gozdowska et al[29] Poland Female 12 5.3  3.2  5 cm distal pancreatectomy with splenectomy
2017 Azagoh-Kouadio et al[30] France Female 69 6  4 cm pancreatoduodenectomy
2017 Ruzzenente et al[31] Russia Female 31 5  4.5 cm tumor resection
2017 Aikot S et al[32] Qatar Female 32 5.1  4.6 cm distal pancreatectomy with splenectomy
2016 Yang et al[33] Turkey Female 13 9  7.2 cm distal pancreatectomy
2016 Chinnusamy et al[34] USA Female 27 5.6  4.5 cm pancreatoduodenectomy
2016 Michalova et al[35] India Female 18 10  8  6 cm distal pancreatectomy with splenectomy
2015 Coronel et al[36] Brazil Female 47 11  6  9 cm distal pancreatectomy
2015 Snajdauf et al[37] Argentina Female 28 2.8 cm in diameter distal pancreatectomy with splenectomy
2015 Xiang et al[38] China Female 25 4.5 cm in diameter distal pancreatectomy
2015 Sharma et al[39] India Male 10 11  10  9.2 cm tumor resection
2015 Wu et al[40] China Female 19 14.8  8.9 cm distal pancreatectomy with splenectomy
2012 Cho et al[41] Korea Male 10 11  10 cm NM
2012 Manfredi et al[42] Porland Female 39 15 cm in diameter distal pancreatectomy
2012 Luchini et al[43] Porland Female 36 17  13  8 cm distal pancreatectomy
2012 Jung et al[44] China Female 24 4.0  5.0 cm distal pancreatectomy
2012 Park et al[45] Portugal Female 35 4 cm tumor resection
2010 Sandlas et al[46] Greece Female 55 5 cm distal pancreatectomy with splenectomy
2010 Lee et al[47] Italy Female 15 12  13  10 cm distal pancreatectomy with splenectomy
2010 Nishida et al[48] Japan Female 32 1.5 cm pancreatoduodenectomy
2009 Dumitru et al[49] Iran Female 17 NM pancreatoduodenectomy
2009 Jiang et al[50] Mexico Female 37 NM distal pancreatectomy with splenectomy
2006 Branco et al[51] Japan Female 26 5  4.5  5.2 cm pancreatoduodenectomy
2006 Murayama et al[52] USA Female 20 6.1  6.6 cm distal pancreatectomy with splenectomy
2006 Fukuda et al[53] Turkey Female 27 14  16 cm distal pancreatectomy with splenectomy
2006 Dalla Bona et al[54] Tunisia Female 14 6.8  6.3 cm distal pancreatectomy
2006 Okamoto et al[55] Turkey Female 29 11 cm in diameter distal pancreatectomy with splenectomy
2004 Lakhtakia et al[56] India Female 22 5  4  4 cm EUS-FNA
2004 Hanada et al[57] Japan Female 33 2.5  2.5  2cm NM
2004 Kanngurn et al[58] Italy Female 49 10  10  9.5 cm distal pancreatectomy
2004 Karamarković et al[59] Greece Female 17 6.5  5.4 mm EUS-FNA
2004 Aydiner et al[60] Turkey Female 29 8.5 cm in diameter pancreatoduodenectomy
2004 Ulusan et al[61] Turkey Female 17 NM pancreatoduodenectomy
2004 Levy et al[62] USA Male 31 9 cm pancreatoduodenectomy
2002 Mancini et al[63] USA Male 43 11 cm distal pancreatectomy with splenectomy
2002 Mancini et al[63] USA Female 47 3.5  2.3 cm distal pancreatectomy
2002 Coleman et al[64] USA Female 28 4.5  3.0 cm distal pancreatectomy
2001 Potrc et al[2] Slovenia Female 14 10  10cm pancreatoduodenectomy
2001 Molino et al[65] Italy Female 67 2 cm pancreatoduodenectomy
2001 Casanova et al[66] Italy Female 44 6 cm pancreatoduodenectomy
EUS-FNA = endoscopic ultrasonography guided fine needle aspiration, NM = not mentioned.

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reported, most ruptured SPTs are induced by the blunt abdominal Clinical presentation of solid pseudopapillary tumor in pancreas
trauma, and spontaneous ruptures seem to be quite uncommon. is various. Abdominal discomfort or vague pain can be the most
Since the cystic part of SPT consisted of the degeneration after the common symptom, but the minority of patients can be also
intramural hemorrhage, SPT had a natural tendency to asymptomatic and the tumors are detected incidentally. However,
hemorrhage inside the tumor.[10] Abrupt massive hemorrhage some symptoms including vomiting, discomfort in the epigastrium
and increased pressure of the tumor are considered to be the main or jaundice occur more rarely.[11] We collected about 50 cases of
reasons for the spontaneous rupture of SPT. When we face with SPT between 2018 to 2001 (Table 1). As the reported cases showed
such patient, the enhanced CT and emergency laparotomy would (Table 2), most patients presented with abdominal pain (24/50),
be helpful to make a correct diagnosis. and others complained with the symptoms of nausea, vomiting (7/
50), abdominal distension (3/50), or jaundice (1/50). However,
when the tumors invade to neighboring organs, such as the adrenal
3.3. Clinical findings of solid pseudopapillary tumors of
glands, patients present with specific clinical manifestations, such
pancreas
as acute kidney injury (AKI) with rhabdomyolysis. Therefore, the
We reviewed 50 case reports of SPT in literatures. The patients clinical presentations may be the necessary clues to find the origin
included 46 females and 4 males, the ages ranged from 10 to 69, of the tumor and make the differential diagnosis.
and the mean age was 31.5 years old (Table 1). Totally, SPT, as
an uncommon, typically benign tumor, is found mainly in young
3.4. Diagnosis of solid pseudopapillary tumors of
non-Caucasian women between the 2nd and 3rd decades of life.
pancreas
In addition, we found that most of the patients were Asians (20/
50) and Americans (18/50), and Europeans comprised 11/50, Image examination is significant in diagnosis of SPT. On X-ray, solid
respectively (Table 2). This observation suggests that it seems to pseudopapillary tumors appear as large masses, which sometimes
have a predilection for Asian and American women, although could displace adjacent structures like stomach or bowels. The
rare cases have been reported in children and men. Some experts majority of tumors are diagnosed through ultrasound or CT scan of
considered that female predominance may attribute to the the abdomen. Ultrasound shows a well-defined mass with solid and
proximity of primordial pancreatic cells to the ovarian ridge cystic components and increased vascularity.[12,13] Besides, CT
during the development of SPT. imaging is superior for the diagnosis of SPT. Through contrast
enhanced CT, it shows an encapsulated lesion with enhancing solid
and non-enhancing cystic areas with some showing calcific foci. If
Table 2 spontaneous bleeding was occurred in the tumor, the hemorrhagic
Epidemiological and pathological results of the 50 cases in the density can be found within the lesion. Solid pseudopapillary tumors
literature review. may grow to large sizes with a mean diameter ranging from 6cm to 10
cm.[14] Through magnetic resonance image (MRI), it reveals the
Item Statistical result
hyper-vascular, well-encapsulated, round tumors with mixed cystic
Age range (years) 10–69 (31.5) and solid components. Furthermore, echo-endosonography may
Sex (F:M) 46:4 provide FNA biopsy with the possibility of pre-operative pathologic
Location
diagnosis. So SPT requires differential diagnosis to be made with other
Head 17
Body 5
pancreatic tumors, such as mucinous neoplasm, serous cystadenoma,
Tail 8 pseudocyst, nonhyperfunctioning islet cell tumor and pancreatic
Body-tail junction 10 adenocarcinoma (Table 3). Thus, to ensure a better surgical approach,
Head-body junction 4 a clear preoperative diagnosis of SPT is preferable.
All of pancreas 0 As for the confusion between pancreatic SPTs and cystic
Not described 6 neoplasms, histological differential diagnosis is crucial. SPT is
Race composed of poorly cohesive, monomorphic cells forming solid,
Asian 20 and pseudopapillary structures are lined by neoplastic cells.
American 18 Moreover, cystic spaces containing blood and necrotic debris.[15]
European 11
Histologically, the neoplastic cells are characteristically strongly
Other Races 1
Symptoms
positive for vimentin, a1-antitrypsin, a1-antichymotrypsin, CD-
Abdominal pain 24 10, progesteron receptor (PR), neuronspecific enolase, CD-56
Abdominal pain/nause/vomitting 7 and cyclin D1.[6,16] In our review, we found that neoplastic cells
Painless mass 8 were strongly and diffusely positive for vimentin in 12 cases,
Abdominal distension 3 positive for CD-10 in 10 cases. Besides, a total of 9 patients in our
Jaundice 1 review showed positive results for PR expression and 8 patients
Not described 7 showed positive results for a1-antitrypsin expression (Table 4).
Treatment Thus, the combination of multiple IHC markers including
EUS-FNA 2 vimentin, CD-10, PR anda1-antitrypsin, may contribute to
Distal pancreatectomy 11
improving the diagnosis rate of SPT.
Distal pancreatectomy with splenectomy 16
Pancreaticoduodenectomy 15
Tumor resection 2 3.5. Treatments for solid pseudopapillary tumors of
Others 3 pancreas
Not described 1
Despite the large tumor size at the time of diagnosis, surgery is the
EUS-FNA = endoscopic ultrasonography guided fine needle aspiration.
preferred treatment option for solid pseudopapillary tumors of

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Table 3
Immunohistochemical markers of the SPT specimens in the literature review.
Author CK Vimentin CHROMO NSE CD10 CD56 SYNAPTO Ki67 PR b-catenin
[24]
Bender Am et al NM NM + NM NM + + NM + +
Lanke G et al[25] + + NM NM + + NM NM NM +
Kim SS et al[26] NM NM - NM NM NM NM NM NM +
Lawlor RT et al[27] NM NM NM NM + NM NM NM NM NM
Kriger AG et al NM NM NM NM + + NM NM NM NM
Gozdowska J et al[28] NM NM NM NM + + NM NM + +
Azagoh-Kouadio R et al[30] NM + NM NM NM NM NM NM NM NM
Ruzzenente A et al[31] NM NM NM NM NM NM NM + NM NM
Aikot S et al[32] NM + NM NM + + NM NM NM +
Yang JR et al[33] NM NM + NM NM NM NM NM + NM
Chinnusamy P et al[34] NM + NM + + NM + + NM NM
Michalova K et al[35] NM NM + NM + NM + + + NM
Coronel M et al[36] - + - + NM NM - NM + NM
Snajdauf J et al[37] NM + - NM + NM NM NM NM NM
Xiang D et al[38] NM + + NM + + NM NM + +
Sharma M et al[39] NM + NM - NM NM NM NM + NM
Wu H et al[40] + NM NM NM NM NM + NM NM NM
Cho YJ et al[41] NM + NM NM NM NM NM NM NM NM
Manfredi R et al[31] + NM NM + NM NM NM NM NM NM
Luchini C et al[43] NM + NM + + NM NM NM NM NM
Jung MJ et al[44] NM + + + NM NM + NM NM NM
Park HJ et al[45] - + - NM NM NM + NM + NM
Coleman KM et al[31] NM NM - NM NM NM + - + NM
NM = Not Mentioned, SPT = solid pseudopapillary tumors.

Table 4
Differential diagnosis of SPT.
Local Morphology US CT MRI
Solid pseudopapillary Head and tail Wellcircumscribed, heterogeneous Well-circumscribed, encapsulated, Degradation blood products: high SI
tumor (hypoechoic solid and anechoic round or lobulated lesion. onT1-wi,low or inhomogeneou s SI
cystic components) and Variable internal architecture on T2-wi Solid component without
hypovascular (solid, mixed solid and cystic, hemorrhagic foci: low SI on T1-wi,
thick-walled cyst) depending on high SI on T2-wi Fibrous capsule:
the degree of hemorrhagic low SI on T1 and T2-wi
necrosis.
Mucinous neoplasm Tail (Uni/ multilocular cyst) Large unilocular or multilocular Water HU of the cyst Distant High SI on T1 and T2-wi (but
cysts metastasis (mucinous variable with concentration)
adenocarcinoma)
Serous cystadenoma Body and tail (small cysts) Multiple milimetric hypoechoic or Honeycomb pattern of multiple Hypointense on T1 and hyperintense
anechoic cysts milimetric cysts on T2-wi clustered cysts
Pseudocyst Head 50% (Large or small Uni/Multiple cysts of of the upper Round or lobulated like low- High SI on T1wi and T2wi
Uni/ multilocular cysts) abdomen density area
Lymphangioma Head and body (cystic/ Multilocular lesions with internal Homogeneous cystic mass with Cystic spaces appear hypointense on
cavernous mass) septa thin walls and multiple fine T1-weighted images and
intervals,and the wall of the hyperintense on T2-weighted
tumour may be enhanced after images.
intravenous contrast-
administration
Nonhyperfunctioning islet Small or large in size Homogeneously hypoechoic lesion Isoattenuating to the parenchyma Low SI or isointensity on T1-wi High
cell tumors Distant metastasis to isointense on T2-wi
Pancreatic Head (Lesion with contour Hypoechoic lesion Dilated Isodense to the parenchyma Low SI on T1wi Variable SI on T2-wi
adenocarcinoma deformity of the gland) pancreatic duct Atrophic gland Dilated pancreatic duct and Contiguous organ invasion and
atrophic gland Obliteration of distant metastases
peripancreatic fat Contiguous
organ invasion, vascular
invasion and distant metastases

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