Vol.2, No.7, 415-421 (2013) Case Reports in Clinical Medicine
Copyright © 2013 SciRes. OPEN ACCESS
Solid-Pseudopapillary tumor of the p a ncreas: Clinical
and radiological features. Case report and review of
the literature
Slim Jarboui1*, Alifa Daghfous2, Karima Kacem3, Lamia Rezgui Marhoul2
1Department of General Surgery, Sidi Bouzid Hospital, Sidi Bouzid, Tunisia; *Corresponding Author: drslimjarboui@yahoo.fr
2Department of Radiology, Trauma Center of Ben Arous, Ben Arous, Tunisia
3Department of Hematology, Aziza Othmana Hospital,Tunis, Tunisia
Received 23 July 2013; revised 26 August 2013; accepted 10 September 2013
Copyright © 2013 Slim Jarboui et al. This is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Solid-Pseudopapillary Tumor (SPT) of the pan-
creas is considered to be a rare pancreatic tu-
mor that occurs in young females. Most SPTs
are considered to be benign. However, the na-
tural course of history has not y et bee n clarified.
We reported a case of 25-year-old women with a
p aten t histo r y of splenectomy fo r hemolytic ane-
mia, who presented intermittent abdominal pain
and vomiting of 6 months ago. Physical exami-
nation revealed a hard mass of 8 × 5 cm in the left
upper quadrant of the abdomen. Routine labo-
ratory tests were within the normal range. CT-
Scan showed an 8 × 6 cm lesion with irregular
low density in the body and the tail of the pan-
creas. MRI showed a mass of 8 cm, surrounded
by a capsule, with the irregular intensity both in
T1 and T2 enhanced-images. Distal pancreatic-
tomy was done as a definitive treatment via a Bi
sub costal incision. Histopathological examina-
tion confirmed the diagnosis of SPT. There was
no vascular invasion or other malignant features.
The resection margins are free of lesions. The
post operative course was uneventful. During
post operative follow-up of 24 months, there is no
sign of recurrence on CT-scan. SPT is a rare
condition with a low potential for malignancy
and favorable prognosis. Surgical resection is
generally curative. Characteristic CT and MRI
scans combined with age and sex profile should
be sufficient for the diagnosis and the decision
to operate.
Keywords: Soli-Pseudopapillary Tumor; Distal
Pancreatectomy; Pancreas
Solid-Pseudopapillary Tumor (SPT) of the pancreas or
Frantz tumor is a rare exocrine neoplastic tumor of the
pancreas with a high prevalence in young women. It ac-
counts for 0.13% - 2.7% of all pancreatic tumors and it’s
generally considered as a tumor with a low malignant po-
tential [1-4]. Although resection of the tumor provides a
5-year survival rate of 97%, local recurrence or distant
metastases can occur in a significant number of patients
For delineating the clinicopathological and radiologi-
cal features, and the surgical therapy strategy of this tu-
mor, we hereby report a clinical case with a review of the
A 25-year-old woman with a past medical history of
laparotomic splenectomy for hematologic anemia (mixed
α and β thalassemia) was transferred to our department
for abdominal mass. She was complaining of intermittent
epigastric pain with nausea and vomiting. An 8 × 6 cm
immobilized mass with distinct margin, and slight ten-
derness was palpable on the left upper quadrant. Neither
jaundice nor enlarg ed superficial lymph nodes were seen.
Laboratory findings were normal except anemia with
Hemoglobin at 8.5 g/dl and increased level of ASAT (85
UI/l, normal < 45UI/L) and ASAT (103 UI/l, normal <
35 UI/l). Abdominal ultrasonography demonstrated a
heterogeneous semi solid mass with cystic components
adjacent to the left lobe of the liver. CT-scan delineated a
well-demarcated solid-cystic lesion at the pancreatic tail
surrounded by an integrated cap sule with a diameter of 8 ×
6 cm. The solid areas were in the central and the cystic
areas were peripheral (Figure 1(a)). Intravenous contrast
injection revealed slight enhancement of the cystic wall
S. Jarboui et al. / Case Reports in Clinical Medicine 2 (2013) 415-421
Copyright © 2013 SciRes. OPEN ACCESS
and solid part in arterial phase (Figure 1(b)), and mod-
erate enhancement in portal phase (Figure 1(c)). No cal-
cification was seen. MRI showed an 8 cm avoid
solid-cystic mass of the pancreatic tail with distinct bor-
der. On T1 weight, heterogeneous iso intense lesion was
Figure 1. (a) Axial non enhanced CT-scan
demonstrating a well-demarcated solid-cystic
at the pancreatic tail surrounded by an inte-
grated capsule with a diameter of 8 × 6 cm; (b)
Arterial phase of contrast-enhanced CT dem-
onstrating slight enhancement of the cen-
trally solid portion of the lesion (Arrow); (c)
Portal phase of contrast-enhanced CT de-
monstrating papillae-like enhancement of
the solid portion and the peripheral cystic
wall (Arrow).
observed (Figure 2(a)), on T2 weight, papillae-like hy-
pertensed and irregular hypointense area in the mass was
seen (Figure 2(b)). The mass was irregularly enhanced
with the significantly enhanced papillary solid centrally
portion and the cystic wall after gadolinium injection
(Figure 2(c)). There was no evidence of locoregional or
distant spread on CT-scan or MRI.
Figure 2. (a) T1 weigh showing iso-
intense lesion in the pancreatic tail
with hyper-intense hemorrhage in the
centre (Arrow); (b) T2 Weigh MRI
showing papillae-like hypo-intense and
peripheral cystic hyper-intense areas
in the mass; (c) Enhanced-MRI show-
ing heterogeneous enhancement of the
mass with obvious enhancement of the
solid centrally portion and the wall of
the cystic lesion.
S. Jarboui et al. / Case Reports in Clinical Medicine 2 (2013) 415-421
Copyright © 2013 SciRes. OPEN ACCESS
The patient was qualified to the scheduled surgical
procedure. The Bi sub costal incision was performed.
The tumor was resected completely via a distal pancre-
atectomy (Figure 3). The region of pancreatic tail was
managed using the manual suture and the omental pack.
The post operative period was uneventful. She was dis-
charged 6 days after the surgical procedure.
On gross examination of the specimen, an 8 × 6 cm,
oval, well defined and mixed solid-cystic mass was seen.
Cut surface was grayish white, with areas of necrosis and
hemorrhage. Surrounding pancreas at resection margins
appeared normal. On histological examination, the tumor
was very cellular and well-circumscribed without in-
volvement of adjacent pancreas. The tumor cells were
arranged in solid sheets and pseudopapillae. Mitotic fig-
ures were few. No vascular invasion was seen. Immuno-
histochemical staining of viementin, neuron-specific
enolase (NSE) were positive, but negative reactio ns were
found for chromogranine A, cytokeratine and CEA. With
these histological features, final diagnosis of SPT of the
pancreas was made.
24 months after surgery, the patient is asymptomatic
and CT-scan shows no signs of recurrences or metas-
SPTs of the pancreas are unusual neoplasms of the
pancreas of uncertain histogenesis that occur mostly, but
not exclusively in young women in the second or third
decade. It is a rare neoplasm that has shown a progres-
sive increase of incidence, passing from 0.17% - 2.7% of
711 exocrine tumors of the pancreas in the 1980’s, to 6%
in recent report in 2003 [1-10]. Until 2006, there have
been 718 cases in the English literature: female domi-
nance has been found with a ratio of 10:1 and there was a
mean age of 22 years [11]. In the review of 553 cases in
Chinese literature, Peng-Fei Yu et al. had reported a
mean age of patients of 27.2 years, and the male to fe-
male ratio was 1:8.37 [12].
Figure 3. Operative specimen showing a well-
circumscribed mass occupying the tail of the
The histogenesis of these ep ithelial neoplasms remains
unclear. They have been suggested to have a ductal epi-
thelial, neuroendocrine multipotent primordial cell, or
even an extra pancreatic genital related cell origin [2,3,
7,12-17]. Deshpande et al. have described three cases of
SPT of the ovary that resembling those of the pancreas
[15]. The predominantly young female distribution of
this tumor led to suggestions that estrogen and proges-
terone hormones may have a role in the pathogenesis
The y are slo w grow ing tumo rs w ith indo lent c ourse as
was seen in our case. The diagnosis in most cases is
based on compressive symptoms, pain or finding of a
palpable mass, while about 20% to 30% of the patients,
the diagnosis is made during abdominal imaging per-
formed for others pathologies [1-3,5,7,12,13,18-22]. Even
more unusual is, its presentation as an acute abdomen
symptom with hemoperitoneum from spontaneous rup-
ture or intratumoral bleeding [23-29]. They commonly
located in the body and tail of pancreas also like our pa-
tient [1-7,11,12,18]. In the review of Peng-Fei Yu et al.
43.6% of tumors were localized in the body and tail of
the pancreas [12].
SPT have not been associated with specific clinical
laboratory test findings including serum tumor markers.
The finding in the macroscopic pathological analysis
corresponds to images in the CT-scans and MRI. Ultra-
sosnography shows the tumor as a well-defined mass
consisting of solid as well cystic components [1,8,14,18,
25,28-31]. Typical SPT is characterized by a well-en-
capsulated mass with ranging amounts of intraluminal
hemorrhage [1,7,8,12,25]. However, the tumor can have
atypical appearance, such as metastasis, ductal obstruc-
tion, parenchymal and extra capsular invasion, simula-
tion of islet cell tumor, intratumoral calcification, and
occurrence in male patient. Knowledge of the spectrum
of variable features observed in SPT is useful for differ-
entiating this lesion from other pancreatic neoplasm and
formulating the correct diagnosis. The classic CT fea-
tures of SPT are a large well-encapsulated mass with
varying solid and cystic components caused by hemor-
rhagic degeneration. Their mutual proportions can be
various and the tumor can be differentiated from entirely
solid to completely cystic [1,6,8 ,11,18,25-27,31,32]. Cal-
cifications and enhancing solid areas may be present at
the periphery of the mass. MRI typically shows a well-
defined lesion with a mix of high and low signal inten-
sity on T1 and T2 weighted images. T2-weighted images
show a thick fibrous capsule, which is seen as a discon-
tinuous rim of low signal inten sity. Gado linium enhan ced
dynamic MRI shows early peripheral heterogeneous en-
hancement of the solid portion with progressive fill-in
[7,12,18,22,26,30-35]. Endoscopic ultrasound (EUS) may
provide fine needle puncture biopsy with a supplemen-
S. Jarboui et al. / Case Reports in Clinical Medicine 2 (2013) 415-421
Copyright © 2013 SciRes. OPEN ACCESS
tary option of preoperative diagnosis. Fine-needle aspira-
tion (FNA) can be effective in obtaining a preoperative
diagnosis of SPT since the tumor has characteristic cy-
tologic features [6,12,26,30,36-42]. On aspirated materi-
als, the most frequent features are the presence of marked
cellularity with pseudopapillary fragments composed of
fibrovascular stalks lined with one to several layers of
tumor cells [30,35,36,43-45]. In our case, clinical and
radiological patterns are so specific to make preopera-
tively the diagnosis of SPT. Grossly, the tumors present
as large, round, solitary masses that are usually well de-
marcated from the remaining pancreas. The cut surface
of the tumors reveals lobulated, light brown solid areas
admixed with zones of hemorrhage and necrosis as well
as cystic spaces files with necrotic debris [1,3,5,8,12,
26,28,31,38]. However, cystic changes can be less pro-
minent in smaller tumors. Both a capsule and intratu-
moral hemorrhage are important clues to the diagnosis
because these features are rarely found in other pancre-
atic neoplasms. Histologically, the tumors exhibit a solid
monotonous pattern with sclerosis and a pseudopapillary
pattern. Immunohistochemically, most tumor cells are
positive for vimentin, alpfa-1-antitrypsin, alpha-1-antichy-
motrypsine, occasionally positive for neuron-specific
enolase (NSE) and like in our patient, negative for cy-
tokeratin, chromgranin, synaptophysin, and S-100 pro-
tein [7,11,12,30,32]. Recently, CD10 was demonstrated
in SPT in a diffuse pattern, and therefore it can be helpful
in differential diagnosis [11,12,20,30,45]. Many studies
have used a large number of stains to document an im-
munohistochemical profile associated with SPT. They
conclude that nuclear/cytoplasmic expression of Beta
Catenin and loss of E-Cadherin can be also used in the
diagnosis of SPT even in small biopsy specimens [11,
13,46-48]. The differential diagnosis includes pancreatic
neuroendocrine tumor (PNET), acinar-cell carcinoma,
papillary mucinous carcinomas, intraductal papillary mu-
cinous tumor, pancreaticoblastoma and adenocarcinoma
of the pancreas. PNET predominantly occur in older pa-
tients and can be associated with a variety of clinical
syndromes. Additionally, these tumors express neuroen-
docrine markers such as chromgranin, NSE, and synap-
tophysin [6,7,18,20,21,30,32,35]. Acinar cell carcinoma
occurring in a wide range of age is more commonly ob-
served in males, and no predilection in location on the
pancreas. Aspirates are cellular and composed only of
acinar neoplastic cells containing enlarged nuclei with
irregular membrane [7,21,30,32,35]. Papillary mucinous
carcinoma is often presented as a single, large, unilocular
cystic mass and the neoplastic cells are columnar with
cytoplasmic vacuoles, prominent nucleoli and mucinous
background. Also, the thick and viscid mucus almost
always present in intraductal papillary mucinou s tumor is
an important feature that distinguishes this neoplasm
from SPT [7,20,21,30]. Pancreaticoblastoma is a disease
of childhood. Pancreatic adenocarcinoma is also seen in
older patients. It does not grow as large as SPT. Calcifi-
cation is quite unusual and cystic or hemorrhagic degen-
eration is uncommon [6,7,32].
Once the diagnosis of SPT is made, surgery is the first
choice of treatment even in the case of distant hepatic
metastases or local recurrence which is not contraindica-
tions for surgical therapy. The surgical treatment de-
pended on the location of the neoplasm. Enucleation,
partial pancreatectomy, distal pancreatectomy with or
without splenectomy, Whipple procedure can be per-
formed [1,2,5 ,7 ,8 ,10, 12 ,23 ,25,2 7 ]. Th e surgical treatment
has to be radical since the malignancy can only be de-
fined by postoperative histological examination. Overall
5-year survival is high as 97% in patients undergoing
surgical resection [1,4-7,12,21,25-28]. Metastases de-
velop in less than 15% of cases and the liver is the most
common site of metastasis [8,10,20,22,23,27,29,34,35,
45,49-55]. Liver metastatic lesions can be multiple but
are generally solitary and may be amenable to resection.
Given the reports that even patients with SPT with local
recurrence as well as liver and peritoneal metastasis,
could still have long-term survival, the presence of me-
tastases is not a contraindication for surgery [1,19,23,
35,48,49]. Several features such as old age, male patient,
invasion into a capsule or adjacent normal pancreatic
parenchyma, and both vascular and perineural invasion,
are described as associated with an increase in potential
for malignancy [7,8,10,22,27,28,35,50-55]. Yang et al.
[54] in their study of 26 consecutive SPT concluded that
there were no significant association between clinicopa-
thological features and malignancy. High proliferative
index (KI67) may predict outcome of malignant SPT.
Tang et al. [8] in their review of 36 consecutive patients,
they described two cases of SPT exhibiting an aggressive
clinical course and both died of disease at 6 and 1
months after diagnosis. The pathologic features of these
patients included a diffuse growth pattern, extensive tu-
mor necrosis, significant nuclear atypia, high mitosis rate
and in one case a component of sarcomatoid carcinoma.
Nishihara et al. [9] have reported that venous invasion,
diffuse growth pattern, extensive tumor necrosis, signifi-
cant nuclear atypia and high mitotic rate are indicatio n of
aggressive behavior. Mackado et al. [36] in their series of
34 patients demonstrated that SPT in male patients have
distinct pattern of onset and aggressiveness when com-
pared with female patients. Geers et al. [16] suggest that
Galactin 3 is a useful marker to distinguish SPT from
PNET, and it is also indicator of behavior because of its
low expression is associates with metastatic spreading.
Our patient have nor of these predictive signs of malig-
nancy in histological examination. In fact, the follow up
of a large number of cases has shown that the majority of
S. Jarboui et al. / Case Reports in Clinical Medicine 2 (2013) 415-421
Copyright © 2013 SciRes. OPEN ACCESS
SPTs are benign. They have been differentiated by the
WHO classification into SP neoplasms with borderline
malignant potential and SP carcinomas [46]. Criteria for
this distinction are angioinvasion, perineural invasion
and deep invasion in the surrounding pancreatic paren-
chyma. There is limited experience regarding chemo-
therapy and radiotherapy with or without the presence of
metastatic disease [2,11]. Regular follow-up is manda-
tory for all patients having undergone potentially cura-
tive resection and particularly on patients with tumor
with histology suggesting a possible malignant behavior.
In conclusion, SPT is a low grade tumor with a good
prognosis. The presence of a huge mass in the pancreas
of young female should prompt suspicion for a SPT.
Given its low malignant potential, and the presence of
specific radiographic patterns, its diagnosis should be
accurate, as the radical surgical treatment is effective.
Surgical resection is the mainstay of treatment and is
possible in the majority of cases.
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