Solid and Papillary Epithelial Neoplasm of the Pancreas: Case Report
Chen-Hsien Su , Tsann-Long Hwang , Chuen Hsueh* , Yi-Yin Jan , Miin-Fu ChenSolid and papillary epithelial neoplasm of the pancreas is a rare pancreatic tumor, and its
histogenesis is still controversial. It behaves either as a benign or low grade maligant neoplasm that
occurs predominantly in young women and adolescents. (1,3) Generally the biologic characteristics of
the tumor indicate a favorable prognosis. There have been relatively few reports of metastatizing
solid and papillary epithelial neoplasms of the pancreas. We found less than thirty cases of high grade
maligant tumors during the course of this disease reported in the English literature. The majority
presented with local invasion, liver metastasis or postoperative recurrence. We present a rare advanced
case with presentation of obstructive jaundice.
Case report
A 20-year-old woman experienced yellowish discoloration of the skin, tea-colored urine, anorexia,
poor appetite and post-prandial vomiting two weeks prior to admission
.
Physical examination revealedpale conjunctiva, icteric sclera and yellowish skin. The abdomen was soft and mildly distended on
examination, with an 8×6 cm ill-defined hard mass over the epigastric and left hypochondrial area and
another 5 cm ill-defined mass over the right iliac fossa. Complete blood count revealed Hemoglobin
8.8 mg/dL, Hemotocrit 25.4 %, white blood count 6,400/mL and platelets 220,000/mL. Initial
biochemical data were as follows: bilirubin 7.6 / 15.4 mg/dl (direct / total), glutamic-oxaloactic
transaminase (GOT) 156 U/L, glutamic-pyruvic transaminase (GPT)182 U/L, and alkaline phosphatase
574 U/L. Stool occult blood analysis was positive (3+). The tumor marker levels of carcinoembryonic
antigen (CEA) and beta-chorionic gonadotropins (beta-HCG) were within the normal range.
Abdominal ultrasonography showed an 8.6 × 6.6 cm heterogenous tumor with necrosis at segments 1
and 2 of the liver with bulging. The common bile duct (CBD), common hepatic duct (CHD) and
bilateral intra-hepatic duct ( IHD) were dilated.
The chest X-ray was normal. Abdominal CT scan demonstrated multiple masses at the Douglas
pouch, supra-urinary bladder, mesentery, omentum, lesser sac and liver. (Fig.1-2) The pancreas was
hard to identify because of the huge mass at the lesser sac. Calcification was noted in parts of the
masses. Endoscopic retrograde cholangio-pancreatogram (ERCP) was attempted. The papilla was
normal and external compression of the duodenal bulb was seen. The pancreatic duct abruptly
terminated at pancreatic head (Fig. 3) but cannulation of the CBD was difficult due to poor axis.
Percutaneous transhepatic cholangic drainage (PTCD) was performed via the left IHD. Tapering at the
CHD with mild proximal biliary tree dilation was noted. (Fig.4) There was no passage of contrast
medium beyond the CHD.A gynecologist was consulted and ultrasonography showed bilateral ovary
tumors. Metastatic rather than primary ovary tumor was favored. An echo-guided biopsy was
performed and a papillary tumor was found on histopathologic examination. Inguinal lymph node open
biopsy revealed lymphoid hyperplasia. Laparoscopy revealed an 8 cm mass, which was white and
irregular in appearance and hard in consistency, in the left upper abdomen near the round ligament.
Several masses (most within 2 cm) were found over the omentum, mesentery and especially in the
pelvis. Two well-defined masses of about 1 cm at omentum and in the pelvis were taken for
pathological diagnosis. Histopathologic examination showed proliferation of solid sheets of
monotonous cells arranged in a pseudopapillary pattern. The tumor cells had round to oval nuclei and
eosinophilic granular cytoplasm. The tumor masses were considered to originate from the pancreas on
the basis of the pathological findings. (Fig. 5) After a serial workup, solid and papillary epithelial
neoplasm of the pancreas with multiple distal metastases and carcinomatosis was diagnosed. The
patient was transferrd to the oncology department for systemic chemotherapy but the tumor mass
enlarged progressively , which was identified in the follow-up abdominal computed tomography . She
started to take chinese herb for treatment and was regularly followed up at our clinics for near one year.
Discussion
Solid and papillary epithelial neoplasms of the pancreas are uncommon benign or low-grade tumors
that typically occur in adolescents and young women(1). They were first described as “ Papillary tumor,
benign or maligant ? “ in 1959 by Frantz (2). Different names had been coined since then, such as
Frantz’s tumor, papillary epithelial neoplasm, papillary cystic neoplasm, solid and cystic acinar cell
tumors, papillary and solid neoplasm, papillary cystic epithelial neoplasm, low-grade papillary
neoplasm, solid and papillary tumor, papillary cystic carcinoma, papillary cystic tumor, solid and cystic
tumor , and solid-pseudopapillary tumor (3 ,31). The estimated frequency is from 0.17% to 2.1% of all
nonendocrine tumors of the pancreas (4). The mean age of most young women at onset is between 22
and 27 years, with the ages ranging from 10 to 73 years (5,6,7,30)
.
There is a black racial predilectionin some reports (8). Clinically, the tumors are almost always diagnosed because of their large size and
the presentation of an asymptomatic abdominal mass. Abdominal pain, dyspepsia, nausea, jaundice
(7), body weight loss and rupture with hemoperitoneum(9) are sometimes seen. Many cases are
asymptomatic until they are found incidentally at laparotomy or on radiographic studies.
Ultrasonography and CT scan are the most useful tools for diagnosis but they are not specific. The
tumor is sharply defined, nonhomogenous and lacks central enhancement on ultrasonography (7).
Abdominal CT scan is the most useful diagnostic method for the evaluation of not only the primary
mass but also the status of the liver as well. The tumors are demonstrated as well-encapsulated
nonhomogenous, large pancreatic masses, and internal architecture of the mass varies from solid
muscle density through mixed solid and cystic to thick-wall cyst , depending on the degree of
hemorrhagic necrosis (5,10). A rim of calcification in the tumor wall is also noted on radiographic
examination, suggesting that this tumor has a slow-growing and long-standing process. The
calcification is not specific for these tumors and can be present in other neoplastic cysts and pseudocyst
of the pancreas. Angiography is also helpful preoperatively, especially in cases involving hepatic
metastasis, where the possibility of hepatic resection is to be considered. Fine-needle aspiration of the
tumor may play an important role in pre-operative planning by distinguishing it from other pancreatic
lesions with significantly different prognoses and treatments(11)
.
The tumors may occur anywhere in the pancreas but are most frequently found in the tail. On gross
examination, the tumors are usually large, averaging about 9-10 cm in diameter, and
well-circumscribed with a compressed pseudocapsule at the periphery. Their cut surface shows a solid
area mostly located at the periphery with cystic degeneration in the center (3, 30, 31)
.
In fact, theinternal structure may be either mostly homogenous and solid, mixed cystic and solid, or mostly cystic.
(12)
The histologic hallmark of this tumor is a mixture of solid and pseudopapillary patterns with cystic
degeneration. The tumor cells are monomorphic with uniform small nuclei and a rim of pale,
eosinophilic cytoplasm. Mitosis is rare and nuclear pleomorphism is minimal. However, hemorrhage
and necrosis are common (13,31). In some tumors with long-standing necrosis, various degenerated
changes such as foamy cell aggregation and formation of cholesterol granulomas can be observed in
the transition to the necrotic portion (13,31).
Surgical excision is the primary form of treatment if the tumor is resectable, i.e.,
pancreatoduodenectomy for head lesions and distal or subtotal pancreatectomy for body and tail lesions
(30)
.
Long-time survival is the rule even though they are locally invasive. The treatment ofunresectable primary tumor or metastatic lesions is controversial. A partial response has been reported
after radiotherapy and chemoembolism, but the unpredictable nature of this tumor makes it
questionable(6,17,18)
.
Tumor aggression is usually limited to capsule or local invasion. Rarely, metastasis has been
reported. (1,3-6,13-29) In a review of 56 cases by Sclafani at 1991, 16% of patients had major organ or
blood vessel invasion, and 7% had liver metastasis at some time during the course of their disease
illustrating the maligant nature of the tumor (16). A review by Gonzalez-Campora reported on 131
reports representing a total of 351 cases. Of these cases , 88% presented with disease limited to the
pancreas, 6.2% had local peri-pancreatic infiltration or recurrence, and 5.6 % developed distal
metastasis. (4)
In conclusion, maligant solid and papillary epithelial neoplasm of the pancreas with liver metastasis
and severe carcinomatosis is rare. Obstructive jaundice was found in the initial presentation in our
patient. In the English literature we reviewed, less than thirty cases of high grade maligant tumors
during the course of the disease were reported. The majority presented with local invasion or liver
metastasis. Rare cases presented with advanced disease initially as in our patient. It is important to
recognize that this unusual presentation may occur in patients with solid and papillary epithelial
neoplasms of the pancreas.
