www.elsevier.com/locate/jpedsurg

Management of children with pancreatic head mass

Jinyoung Parka, James C.Y. Dunnb, James B. Atkinsonb,*

aDepartment of Pediatric Surgery, School of Medicine, Kyungpook National University, Taegu, KoreabDivision of Pediatric Surgery, David Geffen School of Medicine, University of California, Los Angeles, USA

0022-3468/$ – see front matter D 2006

doi:10.1016/j.jpedsurg.2006.02.029

Presented at the 16th Annual Conf

Chapter of the American College of

January, 2005.

* Corresponding author. Tel.: +1 31

E-mail address: jatkinson@mednet.u

Index words:Obstructive jaundice;

Children;

Pancreatic head mass;

Pancreatitis

Abstract The management of children with a mass in the head of the pancreas is not well defined. The

medical records of 3 children with obstructive jaundice because of a mass in the head of the pancreas

over a 4-year period were reviewed retrospectively. Abdominal ultrasonography and computed

tomography showed intrahepatic and extrahepatic ductal dilatation and a pancreatic mass. Intraoperative

frozen section revealed no evidence of malignancy. These patients were separately managed by pylorus-

preserving pancreaticoduodenectomy, cholecystectomy and Roux-en-Y choledochojejunostomy, com-

mon duct exploration, and T-tube drainage. The final pathology of the pancreatic head mass in all cases

demonstrated chronic pancreatitis. Follow-up at an average of 7 months postoperatively showed no

recurrence of obstructive jaundice. Unlike adults with a mass in the head of the pancreas, it is

recommended that children with similar presentation should undergo biopsy and biliary diversion rather

than resection as the primary therapy. Adults presenting with similar radiologic and clinical features

would be treated by a pancreaticoduodenectomy in the absence of histologic evidence of malignancy.

This series would suggest histologic conformation should be obtained before radical surgery in children.

D 2006 Elsevier Inc. All rights reserved.

Obstructive jaundice in childhood encompasses a differ-

ential diagnosis that includes choledocholithiasis, pancrea-

titis, pancreatic pseudocyst, choledochal cyst, intrahepatic

neoplasm, and pancreatic tumors [1]. Unlike adults, children

rarely develop malignancy of the pancreas. Despite currently

available diagnostic imaging modalities, it is very difficult to

distinguish a benign pancreatic mass from pancreatic cancer

preoperatively. The optimal management of children with a

mass in the head of the pancreas is unknown.

We reviewed the medical records of 3 children with

biliary stricture because of a mass in the pancreatic head. A

review of the literature is included for comparison.

Elsevier Inc. All rights reserved.

erence of the Southern California

Surgeons, Santa Barbara, Calif,

0 206 2429; fax: +1 310 296 1120.

cla.edu (J.B. Atkinson).

1. Case reports

1.1. Case 1

A previously healthy boy presented at 5 1/2 years of age

with a 2-week history of painless jaundice. He complained

of abdominal pain and had clay-colored stool, dark urine,

easy bruising, and itching. On physical examination, he was

icteric, and his abdomen was soft, without a palpable mass.

Initial laboratory studies were notable for a total bilirubin of

7.2 mg/dL, conjugated bilirubin of 4.1 mg/dL, alkaline

phosphatase of 1298 U/L, serum glutamic-oxaloacetic

transaminase (SGOT) of 281 U/L, serum glutamic-pyruvic

transaminase (SGPT) of 332 U/L, serum amylase of

109 U/L, serum lipase of 34 U/L, and a negative hepatitis

panel. Abdominal ultrasonography (US) and computed

tomography (CT) scan showed intrahepatic and extrahepatic

ductal dilatation and mild pancreatic ductal dilatation.

Endoscopic retrograde cholangiopancreatography (ERCP)

Journal of Pediatric Surgery (2006) 41, E1–E4

Fig. 1 Endoscopic retrograde cholangiopancreatography

showed the stricture of common bile duct distal to takeoff of the

cystic duct.

Fig. 2 Abdominal CT scan showed the pancreatic head mass

with enhancement.

J. Park et al.E2

showed a distal stricture of the common bile duct (Fig. 1).

The biliary stricture was fibrotic and did not respond

to sphincterotomy. A biliary stent was placed to relieve

the obstruction. At operative exploration, he was found

to have a firm inflammatory mass in the head of the

pancreas, which was not seen on the preoperative radiologic

studies. A transduodenal biopsy of the head of the pancreas

was performed. Intraoperative frozen section showed

pancreatic tissue with benign fibrosis. A cholecystectomy

and Roux-en-Y choledochojejunostomy was performed.

The postoperative course was uneventful. Follow-up T-tube

cholangiogram at 2 months was unremarkable. Five months

after operation, a follow-up US showed air in the biliary

tract without biliary dilatation.

1.2. Case 2

A previously healthy adolescent boy presented at 15 years

of age with a 4-week history of jaundice. He complained of

abdominal pain and had clay-colored stool. On physical

examination, he was icteric but was otherwise normal-

appearing. His abdomen was soft, without palpable mass.

Initial laboratory studies included a total bilirubin of

5.9 mg/dL, conjugated bilirubin of 3.4 mg/dL, alkaline

phosphatase of 400 U/L, SGOT of 105 U/L, SGPT of

380 U/L, amylase of 100 U/L, lipase of 109 U/L, and a

negative hepatitis panel. Abdominal US and CT scan

showed intrahepatic and extrahepatic ductal dilatation and

a pancreatic head mass measuring 3 � 4 cm. Endoscopic

retrograde cholangiopancreatography with brushing cytolo-

gy was negative for malignant cells. A preoperative fine-

needle aspiration biopsy showed normal pancreas with no

evidence of malignancy. At operative exploration, the boy

was found to have a firm mass in the head of the pancreas

measuring 4 � 5 cm, and the tail and body of the pancreas

were inflamed and thickened. The pancreatic mass was

obstructing both the bile duct and the pancreatic duct,

causing secondary pancreatitis. A pancreaticoduodenectomy

was performed. The pathologic diagnosis was chronic

pancreatitis. The postoperative course was notable for an

episode of intestinal obstruction that required adhesiolysis at

1 year. The decision to proceed with radical resection in this

case was based upon the need to relieve obstruction of both

the pancreatic and bile ducts, as well as a remote possibility

of malignancy.

1.3. Case 3

A previously healthy male infant presented at with a

2-week history of jaundice. He was transferred to our

hospital after an exploratory laparotomy with pancreatic

biopsy performed at another institution. This biopsy was

reviewed and showed benign inflammatory pancreatic

tissue. On physical examination, he was icteric but was

otherwise healthy-appearing. Initial laboratory studies in-

cluded a total bilirubin of 13.6 mg/dL, conjugated bilirubin

of 8.4 mg/dL, alkaline phosphatase of 534 U/L, SGOT of

177 U/L, SGPT of 82 U/L, amylase of 50 U/L, lipase of

19 U/L, and a negative hepatitis panel. Abdominal US and

CT scan showed a dilated common hepatic duct and a

dilated common bile duct, as well as a pancreatic head mass

with enhancement (Fig. 2). Although ERCP showed a

normal appearing pancreatic duct, a stent could not be

passed through the common bile duct. A percutaneous

transhepatic biliary stent was performed to relieve the

biliary obstruction. At operative exploration 3 months later,

he was found to have a firm inflammatory mass in the head

of the pancreas. A transduodenal biopsy of the head of

the pancreas was performed. Intraoperative frozen section

showed chronic pancreatitis. Intraoperative cholangiogram

showed an irregular pattern at the distal bile duct with-

out filling defects. Common duct exploration revealed

inflamed common duct mucosa without stones. The stric-

ture was assumed to be inflammatory in nature, possibly

Management of children with pancreatic head mass E3

related to a history of gallstone pancreatitis. Therefore,

no resection was performed. The need for an internal

biliary-enteric drainage procedure was considered; however,

an external drainage was performed. A T tube was inserted

into the choledochotomy site. The postoperative course was

uneventful. Follow-up T-tube cholangiogram 2 months

after the operation was unremarkable, and the T tube

was removed.

2. Discussion

Obstructive jaundice in childhood encompasses a differ-

ential diagnosis that includes choledocholithiasis, pancrea-

titis, pancreatic pseudocyst, choledochal cyst, intrahepatic

neoplasm, and pancreatic tumors. Pancreatic tumors are

uncommon in childhood. They account for 0.2% of all

childhood malignancies as compared with 3% in adults [2].

The differential diagnosis of a pancreatic mass in a child is

different than that in an adult. Although adenocarcinoma of

the pancreas is the most clinically important pancreatic mass

in adults, it is rare in children [3]. Pancreatic masses in

children may represent an epithelial neoplasm such as

adenoma, pancreatoblastoma, solid and papillary neoplasm,

tumors of endocrine origin, or nonepithelial tumor, such as

lymphoma, primitive neuroectodermal tumor, neuroblasto-

ma, and rhabdomyosarcoma.

Chronic pancreatitis with a mass effect shares similar

clinical presentations with cancer of the head of the

pancreas, such as abdominal and back pain, weight loss,

steatorrhea, and obstructive jaundice [4]. Pancreatic cancer

can also cause chronic pancreatitis by obstructing the

pancreatic duct. It is well known that chronic pancreatitis

and pancreatic cancer might coexist.

In adults, acute and chronic pancreatitis is usually caused

by alcohol abuse or biliary tract disease. In children, the

most common etiologies are trauma [5], heredity [6],

systemic diseases [7], choledocholithiasis, drug-induced

such as didanosine, azathioprine, and mercaptopurine,

malformations of the pancreaticobiliary duct system, such

as pancreaticobiliary malunion, pancreas divisum, hyper-

tensive sphincter of Oddi and annular pancreas, and

idiopathic [8,9]. In our cases, there were no specific

causative factors identified.

Despite currently available diagnostic imaging modali-

ties, it is very difficult to distinguish preoperatively a

benign pancreatic head mass from pancreatic cancer.

Computed tomography is the most important imaging

modality of the pancreas. Endoscopic retrograde cholan-

giopancreatography or magnetic resonance cholangiopan-

creatography may provide ductal anatomy that is useful for

operative planning. The contours of the pancreatic duct are

more irregular in chronic pancreatitis than those in tumor-

related obstructions [10-12].

Ragozzino and Scaglione [13] reported that magnetic

resonance imaging is much more sensitive and specific

than CT in pancreatic lesion detection. Although not

pathognomonic, the typical cholangiographic pattern on

magnetic resonance cholangiopancreatography sequences

may be helpful in the differential diagnosis between

pancreatitis and pancreatic head tumor. Typical cholangio-

graphic patterns secondary to pancreatic cancer are the

double duct sign (dilatation of both the biliary and

pancreatic ducts) and the mouse tail sign (reduction of

the bile duct caliber because of sudden obstruction at the

pancreatic head). In chronic pancreatitis, the biliary tract

may have a tapered or an irregular appearance on magnetic

resonance imaging with alterations of dilated and stenotic

biliary segments.

Although the pancreatic head masses can be documented

by imaging, the correct diagnosis remains difficult. Because

the management of patients with stricture of the extrahepatic

bile duct depends on the underlying cause, it is useful to

obtain tissue for a pathologic diagnosis. Generally, the

amount of tissue obtained by ERCP is limited, and the use

of brush cytology as an adjunct has proved to be

increasingly useful. Cohen et al [14] reported that endo-

scopic retrograde wire-guided brushing of strictures using

3 cytologic features (nuclear molding, chromatin clumping,

and increased unclear-cytoplasmic ratio) to be useful in

discriminating malignant from benign stricture of the

extrahepatic biliary tract.

Even intraoperatively, the differentiation between chronic

pancreatitis and pancreatic cancer is difficult. Olah et al [15]

reported that intraoperative biopsy had a false-negative rate

of 12.5%, and the diagnosis was uncertain in 35%. These

data showed that a benign biopsy result does not exclude the

presence of malignancy. In cases of mass lesions with

obstructive symptoms, resection is a reasonable option in

adults, even in the case of chronic pancreatitis. These

recommendations in adults are based upon a much higher

probability of malignant tumors.

The best surgical procedure for children with a

pancreatic head mass is much less clear. In adults, chronic

pancreatitis with a mass in the pancreatic head is best

managed by a pylorus-preserving pancreaticoduodenec-

tomy. Diversion of the biliary tract can also be safely

performed using Roux-en-Y hepaticojejunostomy or chol-

edochojejunostomy over a transanastomotic tube. Smith et

al [1] reported a child with pancreatic head adenocarcino-

ma, which was treated with pancreaticoduodenectomy, and

suggested that aggressive surgical therapy is not only

possible but also essential. In contrast, the present series of

cases revealed the benign nature of the masses in the head

of the pancreas in all 3 children. Unlike adults with a mass

in the head of the pancreas, children with similar

presentation should undergo biopsy and biliary diversion

rather than resection as the primary therapy, unless

histologic evidence of malignancy can be demonstrated.

In rare cases, where both the pancreatic and biliary ducts

are obstructed, pancreaticoduodenectomy may be indicated

even for benign disease.

J. Park et al.E4

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