2014, Volume 30, Number 3, Page(s) 228-232
Solid Pseudopapillary Neoplasm of the Pancreas: Report of a Rare Case and Review of the Literature
Arzu Neşe YENER1, Manuk MANUKYAN2, Ahmet MİDİ1, Rahmi ÇUBUK3
1Department of Pathology, Maltepe University, Faculty of Medicine, İSTANBUL, TURKEY
2Department of General Surgery, Maltepe University, Faculty of Medicine, İSTANBUL, TURKEY
3Department of Radiology, Maltepe University, Faculty of Medicine, İSTANBUL, TURKEY
Keywords: Pancreatic neoplasm, Cystic, Papillary
Solid pseudopapillary neoplasm, a rare primary neoplasm of the
pancreas that typically affects young women, is a relatively indolent
entity with favorable prognosis. We here report a 20-year-old young
girl with solid pseudopapillary neoplasm who presented with mild
dull abdominal discomfort without any significant laboratory findings.
On MRI, a heterogenous mass was found at the distal pancreas. The
patient underwent en-block distal pancreatectomy with splenectomy
with the presumptive diagnosis of cystic neoplasm of the pancreas.
The tumor was well-circumscribed, encapsulated, 5.5 cm in the
greatest dimension and showed typical papillary and pseudopapillary
structures. Capsular invasion was seen on focal areas. The patient was
not given any adjuvant therapy and shows no sign of disease after six
months follow-up. It is important to differentiate this tumor from other
pancreatic neoplasms because this neoplasm is amenable to cure after
complete surgical resection even in cases with capsular invasion, unlike
malignant tumors of the pancreas.
Solid pseudopapillary neoplasm (SPN) of the pancreas
almost exclusively affects young women and is a rare
. Although symptoms of SPNs
are usually nonspecific and the preoperative diagnosis
is often inaccurate, it has distinct pathologic features. Its
importance comes from the fact that it may be misdiagnosed
as a malignant tumor of the pancreas1
. In this study, we
aimed to present the case of a 20-year-old young girl with
an SPN located in the distal part of the pancreas and briefly
review the literature on this rare entity.
A 20-year-old young girl presented with history of
mild, dull left sided-abdominal pain for the last two
months. Laboratory examinations were unremarkable. Computerized tomography and magnetic resonance
imaging revealed a well-circumscribed, partly cystic and
partly solid mass measuring 57x48 mm in the tail of the
pancreas (Figure 1a,b
). The patient was operated on
with the presumptive diagnosis of SPN or cystadenoma/
cystadenocarcinoma of the pancreas. Distal pancreatectomy
with splenectomy was performed. There was no evidence of
ascites or intraabdominal metastasis during surgery. Frozen
section of a regional lymph node revealed no metastasis
but reactive changes. The patient had an unremarkable
postoperative course and was discharged 5 days after the
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|Figure 1: CT (a) & MRI (b) revealed a well circumscribed,
mainly cystic and partly solid mass (arrows) located in the tail of
On gross examination, there was a well-circumscribed
and totally encapsulated mass measuring 55x50x45 mm
located in the tail of the pancreas. On the cut surface, it was heterogenous and predominantly a cystic tumor. Focal solid
areas were also present (Figure 2). The cystic parts were
mainly filled with dark brown hemorrhagic debris. Normal
pancreatic tissue was identified at the periphery. The spleen
weighed 150 gm and was in normal limits.
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|Figure 2: Grossly, the tumor was separated from the adjacent
pancreatic tissue with a thin fibrous capsule.
On microscopic examination, we observed a tumor
mainly composed of cystic cavities lined by friable tissue
and cholesterol clefts (Figure 3a). The solid parts grossly
described were showing diffuse growth pattern with
minimal supporting fibrovascular stroma. They were
composed of papillary, pseudopapillary and microcystic
structures with dyscohesive neoplastic cells (Figure 3b).
The neoplastic cells in these areas had polygonal, uniform,
centrally located grooved nuclei and vacuolated or
eosinophilic large cytoplasm (Figure 3c). There were rare
mitotic figures in solid areas. No atypical cells were found.
The tumor was separated from the normal pancreatic tissue
with a thick fibrous capsule in which there was infiltration
of the tumor cells in some areas (Figure 3d). Surgical
margins were free of tumor. Immunohistochemistry
evaluation was performed. The tumor cells showed strong
cytoplasmic positivity for vimentin (Figure 4a) and both
nuclear and cytoplasmic positivity for β catenin (Figure
4b). Weak or moderate staining for p53, pancytokeratin
(PanCK), cyclinD1, CD10, synaptophysin, estrogen
(ER) and progesterone receptors (PR) were observed. No
immunostaining was seen for chromogranine A, CD34,
CD117, cytokeratin 7 (CK7) and cytokeratin 20 (CK20).
The proliferative index assessed by Ki-67 immunoreactivity
was 1-2% in solid areas of the tumor (Figure 4c). With the
histopathological and immunohistochemical findings, we
signed out the case as “solid pseudopapillary neoplasm of
the pancreas”. The patient did not undergo any adjuvant
therapy and has been doing well in the year after her
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|Figure 3: A) Cystic cavities within the tumor contained hemorrhagic material, cholesterol clefts and aggregated histiocytes (H&E).
B) The characteristic papillary and pseudopapillary structures lined by dyscohesive neoplastic cells (H&E). C) The neoplastic cells with
polygonal, uniform, centrally grooved nuclei and vacuolated and with eosinophilic large cytoplasm (H&E). D) The tumor cells infiltrated
the fibrous capsule in some areas (H&E).
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|Figure 4: The tumor cells strongly stained with vimentin (a), β
catenin (b). Both nuclear and cytoplasmic staining for β catenin
was seen. Ki-67 labeling index was 1-2% (c).
SPN was first described by Gruber Frantz in 1959 and
many cases or case series have been reported so far2-7
. It is usually seen in young women who present with
abdominal pain, palpable abdominal mass or occasionally
with mild or no clinical signs and symptoms2,5,6
Imaging studies consistently demonstrate a well-defined
solid-cystic mass with variable degrees of hemorrhagic
degeneration. Calcification is common. Characteristic
fluid-debris levels and signal intensities seen with MRI
indicate blood products7
. Our case presented with mild
dull abdominal discomfort without any other clinical or
laboratory abnormalities. MRI revealed a well-defined mass
that was mainly cystic and only focally solid. The cystic parts corresponding to the areas of hemorrhage showed
hyperintensity on T1-weighted MRI sequences.
Grossly, the tumor is well circumscribed and may reach a
huge size in some instances2,8. Invasion into the capsule,
peritumoral tissues or adjacent pancreatic parenchyme3
as well as distant metastasis to adjacent organs such as liver2,5, spleen8 and regional lymph nodes2 are reported
in some series.
Immunohistochemically, SPN cells strongly and diffusely
express vimentin, α-1 antitrypsin, α-1 antichymotrypsin
(AACT), neuron specific enolase, PR and β form of ER1,7.
CD10, CD56, CD 117, FLI-1, and also epithelial markers
such as CK, AE1/AE3, CAM 5.2 can be focally positive1.
Chromogranine A, a specific endocrine marker, is typically
negative or only very focally positive1.
Abnormal nuclear localization of β catenin gene results in
nuclear staining1,3,6 and also this genetic abberation activates
the Wnt-signaling pathway resulting in overexpression
of cyclinD1, but not in overt malignancy of this tumor9. In our case, we observed strong immunoreactivity for
vimentin, β catenin and weak staining for synaptophysin,
PR and ER. Moderate expression of cyclinD1 was observed.
There was no immunoreactivity for CD117, CD34, Chromogranine
A, CK20 and CK7. Our immunohistochemistry
results were consistent with the literature findings and supported
our diagnosis of SPN.
The pathogenesis and the cell of origin of SPN remain
unknown, since it lacks evidence of ductal, acinar or frank
endocrine differentiation1-5. The strong preponderance
in young women and the common expression of PR suggests that during early embryogenesis, especially the left
genital ridge cells come into contact with the pancreas and
follow a different line of differentiation10.
On the other hand, genetic changes involved in SPNs are
different from the genetic changes involved in conventional
ductal adenocarcinomas. Mutation in exon 3 of the
β-catenin gene is a well-known genetic abberation in SPN1.
Clinical behavior of SPN is still unclear. The long-term
follow up of the patients showed that distant metastasis or
invasion of the peritumoral tissues itself does not indicate
aggressive clinical behavior of this tumor2,8. High-grade
malignant transformation into undifferentiated carcinoma
has been reported to be the only reliable predictor of clinical
aggressiveness of this tumor till now2. In our case, we
noticed capsular invasion in focal areas and the tumor
cells were very close to the adjacent pancreatic tissue. Our
patient is well and free of any sign of disease on her second
follow up one year after her operation.
In conclusion, SPT is a rare tumor of low malignant potential
with uncertain origin. Capsular invasion or invasion of the
tumor to the adjacent pancreas does not correlate with
aggressive behavior. Radical resection of the lesion, where
technically feasible, should be considered as the treatment
of choice since it is safe and effective in controlling the
We would like to thank to Prof Dr Volkan Adsay of Emory
University, Department of Pathology, Atlanta, USA for his
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