Solid Pseudopapillary Tumor of the Pancreas

Daisuke Watanabe
1
, Kouichi Miura
2
, Takashi Goto
2
,
Hiroshi Nanjo
3
, Yuzo Yamamoto
4
, Hirohide Ohnishi
2
1
Department of Gastroenterology, Noshiroyamamoto Medical Association Hospital.
Noshiro, Japan. Departments of
2
Gastroenterology,
3
Pathology, and
4
Gastroenterological Surgery,
Akita University Graduated School of Medicine. Akita, Japan
ABSTRACT
Context Solid pseudopapillary tumor of the pancreas is a rare neoplasm which affects young women. On the other hand, pancreas
divisum is an anomaly which develops at 7 weeks of gestation. Here, we report a case of a solid pseudopapillary tumor of the
pancreas with concomitant pancreas divisum. Case report A 26-year-old woman was diagnosed as having a pancreatic tumor with
solid and cystic components in the pancreatic head. Pancreatograms obtained by ERCP and MRCP showed no communication
between the ventral and dorsal pancreatic ducts, indicating that pancreas divisum was present. Microscopically, the resected tumor
had solid and cystic components. Immunohistochemical study demonstrated that the tumor cells were positive for alpha-1-
antitrypsine, vimentin and progesterone receptors but negative for estrogen receptors, NSE, insulin or glucagon. The tumor was
diagnosed as a solid pseudopapillary tumor of the pancreas. Although more than 700 cases of solid pseudopapillary tumors of the
pancreas have been reported in the English literature, a search of PubMed turned up no reports of concomitant solid pseudopapillary
tumor and pancreas divisum. Conclusion Solid pseudopapillary tumors of the pancreas with concomitant pancreas divisum are
extremely rare.
INTRODUCTION
Solid pseudopapillary tumors of the pancreas are rare
pancreatic neoplasms accounting for 0.13% to 2.7% of
all pancreatic tumors [1]. Frantz first described the
unique characteristics of this tumor in 1959 [2], and
then Klöppel et al. clearly documented this distinct
clinical entity in 1981 [3]. Due to its variety of gross
appearances and histological features, this tumor has
been referred to with multiple names such as papillary
cystic neoplasm, solid and papillary epithelial
neoplasm, and papillary and solid neoplasm. This
tumor is currently designated as a solid
pseudopapillary tumor of the pancreas by WHO [4].
In contrast to pancreatic cancer, a solid pseudopapillary
tumor of the pancreas primarily affects young females,
suggesting that this tumor is associated with some
female hormones and their receptors. In addition, solid
pseudopapillary tumors are occasionally found as
extra- pancreatic tumors, indicating that the origin of
the tumor cells is distinct from other pancreatic tumors.
Although many investigators have proposed the origin
of solid pseudopapillary tumors of the pancreas,
including pluripotent stem cells, ductal cells, acinar
cells, neuroendocrine cells, neurocrest and ovaries [1,
5], the origin of the tumor cells and promoting factors
have not yet been determined.
Pancreas divisum is an anomaly caused by
inappropriate fusion of the dorsal and the ventral
pancreatic ducts during embryogenesis. Recent data
have shown that pancreas divisum is highly associated
with pancreatic tumors [6]. One of the proposed
mechanisms by which pancreatic tumors occur in
patients with divisum is recurring pancreatitis.
Chronically damaged pancreatic cells may transform
into cells having a malignant potential. However, the
association between solid pseudopapillary tumor and
pancreas divisum is still unknown. We herein report
the case of a solid pseudopapillary tumor of the
pancreas with concomitant divisum in the same
pancreas.
CASE REPORT
A 26-year-old woman, who had complained of
intermittent abdominal pain for 8 years, was referred to
our hospital for further evaluation of a pancreatic
tumor. She was 159 cm tall and weighed 48 kg. Her
Accepted October 3rd, 2009 - Received November 2nd, 2009
Key words Neoplasms /etiology; Progesterone
Correspondence Kouichi Miura
Akita University Graduated School of Medicine, Department of
Gastroenterology, 1-1-1 Hondo Akita-shi, Akita 010-8543, Japan
 

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46
pulse rate was 70 min-1 and her blood pressure was
120/80 mmHg. There was no evidence of jaundice. The
abdomen was flat and soft but tenderness was present
at the upper abdomen. The tumor was not palpable.
Other physical examinations were unremarkable.
Laboratory data including hematology, blood
chemistry including amylase and lipase, and tumor
markers including CEA and CA 19-9 were within the
reference limits. US imaging showed a pancreatic
tumor with solid and cystic components (Figure 1a). A
CT scan demonstrated that the well-circumscribed
tumor was located in the pancreatic head (Figure 1b).
Pancreatograms obtained by MRI and ERCP showed
no communication between the tumor and the
pancreatic duct. In addition, the ventral pancreatic duct
did not communicate with the dorsal pancreatic duct
(Figure 2ab). Duodenum-preserving pancreatic head
resection was performed [7, 8]. Briefly, the subtotal
pancreatic head was resected preserving the bile duct.
Reconstitution with drainage of the pancreatic
secretion from the remnant pancreas took place with
Roux-en Y end-to-side pancreaticojejunostomy. The
resected tumor was 50x45x52 mm in size and 50 g in
weight, accompanied by hemorrhagic degeneration and
surrounded by a red fibrotic pseudocapsule (Figure 3a).
Microscopically, the tumor had a cystic component
(Figure 3b). The tumor cells were small to intermediate
in size with faintly eosinophilic cytoplasm. The nuclei
had slight indentations and no mitosis (Figure 3c).
Immunohistochemical examination demonstrated that
the tumor cells were positive for the progesterone
receptor (Figure 3d). In addition, the tumor cells were
also stained with alpha-1-antitrypsine and vimentin but
not with estrogen receptors, NSE, insulin or glucagon
(data not shown).
DISCUSSION
A solid pseudopapillary tumor of the pancreas
primarily affects young women, suggesting that
hormonal factors contribute to the tumor growth. In the
present case, abdominal symptoms were noted at 16
years of age, at which age female hormones and their
receptors function. Progesterone, estrogen, and their
receptors might play a role in the development of solid
pseudopapillary tumor of the pancreas. Our case
showed that the tumor cells were positive for the
progesterone receptor but negative for estrogen
receptors according to an immunohistochemical study.
Large clinical studies show that the progesterone
receptor is expressed by all cases of solid
Figure 2. a. MRCP. The ventral pancreatic duct does not
communicate with the dorsal pancreatic duct. b. ERCP. The ventral
duct attenuates and ends at the pancreatic body. In addition, the
ventral pancreatic duct does not communicate with the dorsal
pancreatic duct.
Figure 1. a. Abdominal US. The tumor has solid (arrow) and cystic
components (arrowhead). b. CT scan. The tumor is well encapsulated
with peripheral enhancement (arrow).

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pseudopapillary tumors [9, 10]. In addition, Yeh et al.
have reported that the progesterone receptor is
uniquely expressed in solid pseudopapillary tumors
while both estrogen and progesterone receptors are
expressed in mucinous cystic neoplasm [9]. These
clinical and histological findings imply that
progesterone and its receptor contribute to tumor
growth.
Why do pancreatic tumors express the progesterone
receptor? The hypothesis proposed by Zamboni et al. is
an excellent explanation for pancreatic tumors which
primarily affect women [11]. Pancreatic anlages are
very close to the genital ridge during embryogenesis.
Primitive ovarian tissue may be incorporated into
pancreatic tissue during the process of pancreatic
fusion, after which dislocated ovarian tissue may start
growing in response to female hormones during
adolescence. Indeed, immunoprofiles of pancreatic
tumors which affect women are similar to those in
certain ovarian tumors [10, 11]. Ten percent of solid
pseudopapillary tumors of the pancreas affect men, in
whom the solid pseudopapillary tumors also express
the progesterone receptor [10]. Even in men,
progesterone is produced by the adrenal glands and
testes. However, the origin of male solid
pseudopapillary tumors might be different from that of
females. Further studies are necessary to elucidate the
origin of solid pseudopapillary tumor.
Interestingly, the present case had pancreas divisum, an
anomaly which develops at 7 weeks of gestation.
Pancreas divisum is associated with pancreatic
carcinoma due to recurring pancreatitis [6]. However,
the present case had no histological evidence of
pancreatitis, indicating that chronic pancreatitis does
not account for the development of solid
pseudopapillary tumors. Although more than 700 cases
of solid pseudopapillary tumors of the pancreas have
been reported in the English literature [12], a search of
PubMed turned up no reports of concomitant solid
pseudopapillary tumors and pancreas divisum in the
same pancreas. Therefore, the association between
solid pseudopapillary tumors of the pancreas and
divisum is currently unknown.
In summary, we have reported a rare case of a solid
pseudopapillary tumor of the pancreas with
concomitant divisum. Little information is currently
available on the association between pancreatic solid
pseudopapillary tumor and divisum.
Figure 3. a. Gross appearance. The tumor is a well-circumscribed solid mass. Hemorrhagic degeneration is seen (arrow). b. A low power view shows
the tumor having cystic components (arrowheads). (H&E, magnification x100, Bar, 200µm). c. A high power view demonstrates that the tumor cells,
faintly eosinophilic cytoplasm, have slight indentations and no mitosis of nuclei. (H&E, magnification x400). d. Immunohistochemistry. The tumor
cells are positive for progesterone receptor (Magnification x400).

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48
Acknowledgement We thank Kazuo Yoneyama, Akita
Kumiai General Hospital and Shigetoshi Ohshima,
Wataru Sato,Takahiro Dohmen, Akita University
Graduated School of Medicine, for preparation of the
present manuscript
Conflict of interest The authors have no potential
conflicts of interest
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