Surg Today DOI 10.1007/s00595-014-0860-1

CASE REPORT

Primary adenocarcinoma of the minor duodenal papilla with mass-forming chronic pancreatitis: report of a case Kazuhiro Suzumura • Seikan Hai • Nobukazu Kuroda • Tadamichi Hirano • Yasukane Asano • Toshihiro Okada • Yuji Iimuro • Shogo Tanaka • Keiji Nakasho • Jiro Fujimoto

Received: 5 April 2013 / Accepted: 16 December 2013 Ó Springer Japan 2014

Abstract A 70-year-old male was treated for gastric ulcers. Follow-up upper gastrointestinal endoscopy revealed an irregular, elevated tumor in the second portion of the duodenum. Upon pathological inspection of a biopsy specimen, a diagnosis of adenocarcinoma was made, and the patient was admitted to our hospital. Computed tomography showed an irregular mass in the pancreatic head and dilatation of the main pancreatic duct and bile duct. Pancreatic head carcinoma with infiltration of the duodenum was diagnosed, and pylorus-preserving pancreaticoduodenectomy was performed. A histopathological examination of the resected specimen showed moderately differentiated adenocarcinoma in the minor duodenal papilla and chronic pancreatitis in the pancreatic head. Therefore, primary adenocarcinoma of the minor duodenal papilla with mass-forming chronic pancreatitis was diagnosed. Currently, the patient is alive without recurrence 17 months after the surgery. Primary adenocarcinoma of the minor duodenal papilla is extremely rare. We herein report this case, and also provide a review of the literature. Keywords Adenocarcinoma  Minor duodenal papilla  Mass-forming  Pancreatitis  Pancreaticoduodenectomy

K. Suzumura (&)  S. Hai  N. Kuroda  T. Hirano  Y. Asano  T. Okada  Y. Iimuro  S. Tanaka  J. Fujimoto Department of Surgery, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya 663-8501, Japan e-mail: [email protected] K. Nakasho Department of Pathology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya 663-8501, Japan

Introduction The minor duodenal papilla is an opening for the accessory pancreatic duct, and is located in the anterior wall of the second portion of the duodenum approximately 2 cm proximal to the major duodenal papilla [1]. Tumors of the minor duodenal papilla are rare. Some cases of tumors occurring in the minor duodenal papilla have been reported, including cases of adenoma [2], adenomyoma [3], carcinoid tumors [4], somatostatinoma [5] and gangliocytic paraganglioma [6]. Adenocarcinoma of the minor duodenal papilla is extremely rare. The presence of mass-forming chronic pancreatitis may lead to difficulties in differentiating such cancers from other pancreatic tumors [7]. We herein report a case of adenocarcinoma of the minor duodenal papilla with mass-forming chronic pancreatitis.

Case report A 70-year-old male was treated for gastric ulcers at another hospital. There was no history suggestive of alcohol abuse. Follow-up upper gastrointestinal endoscopy revealed an irregular, elevated tumor in the second portion of the duodenum. Upon the pathological inspection of a biopsy specimen, a diagnosis of adenocarcinoma was made, and the patient was admitted to our hospital. We performed upper gastrointestinal endoscopy and found a tumor located 2 cm proximal to the duodenal papilla (Fig. 1). The serum levels of alkaline phosphatase (ALP) and c-glutamyl transpeptidase (c-GTP) were elevated to 1,309 U/l (normal range 115–359 U/l) and 632 U/l (normal range 11–58 U/l), respectively. The level of carbohydrate antigen 19-9 (CA19-9) was elevated to 109.8 U/ml (normal range \37 U/ml), while all other laboratory findings,

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Fig. 1 Upper gastrointestinal endoscopy revealed an irregular, elevated lesion in the second portion of the duodenum (arrows)

Fig. 3 Magnetic resonance cholangiopancreatography (MRCP) showed obstruction of the main pancreatic duct in the pancreatic head and dilatation of the distal main pancreatic duct and bile duct

Fig. 2 Computed tomography (CT) showed an irregular mass measuring 4 cm in diameter in the pancreatic head (arrows)

including the levels of total bilirubin and the carcinoembryonic antigen (CEA) level, were within the normal ranges. The level of the IgG4 was not elevated, at 60.4 mg/dl (normal range 4.8–106 mg/dl). Computed tomography (CT) showed an irregular mass measuring 4 cm in diameter in the pancreatic head and dilatation of the main pancreatic duct and bile duct without any apparent distant metastasis (Fig. 2). Magnetic resonance cholangiopancreatography (MRCP) showed obstruction of the main pancreatic duct in the pancreatic head and dilatation of distal main pancreatic duct and bile duct (Fig. 3). Based on these findings, the most probable preoperative diagnosis was considered to be pancreatic

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Fig. 4 The resected specimen showed an irregular, elevated tumor measuring 12 9 11 mm in the minor duodenal papilla (white arrow minor duodenal papilla, black arrow major duodenal papilla)

head carcinoma with infiltration of the duodenum, and pylorus-preserving pancreaticoduodenectomy with regional lymph node (12 lymph nodes) dissection was performed. After the pylorus-preserving pancreaticoduodenectomy, reconstruction by the modified Child method was done. We were able to perform a curative resection. The resected specimen showed an irregular, elevated tumor measuring 12 9 11 mm in the minor duodenal papilla (Fig. 4). The specimen exhibited diffuse pancreatic sclerosis with an ill-defined tumor in the pancreatic head. A

Surg Today Fig. 5 The histopathological examination of the resected specimen. a There was moderately differentiated adenocarcinoma in the minor duodenal papilla (H&E staining 9 100) (arrow orifice) and b chronic pancreatitis in the pancreatic head (H&E staining 9 100). c) The tumor had not invaded the main pancreatic duct (H&E staining 9 40)

histopathological examination of the resected specimen revealed moderately differentiated adenocarcinoma in the minor duodenal papilla (Fig. 5a) and chronic pancreatitis in the pancreatic head (Fig. 5b). The carcinoma cells were primarily located in the minor duodenal papilla with invasion into the muscularis propria of the duodenal wall and the pancreatic parenchyma, but the tumor had not reached the main pancreatic duct (Fig. 5c). There were no findings of lymph node metastasis. According to the TNM classification system, the final pathological stages were pT3, pN0, M0, pStage IIA for adenocarcinoma of the minor duodenal papilla. Based on these pathological findings, we diagnosed the patient with primary

adenocarcinoma of the minor duodenal papilla and massforming chronic pancreatitis of the pancreatic head. The patient’s postoperative course was uneventful, and he was discharged from the hospital on the 30th day after surgery. He is currently alive without recurrence 17 months after undergoing surgery.

Discussion The minor duodenal papilla, which is occasionally difficult to distinguish macroscopically, can be identified in virtually all male cases. The minor duodenal papilla is located

123

123

Died from other disease a

Our case 10

Adenoca. Adenocarcinoma, Mod. diff. adenoca. Moderately differentiated adenocarcinoma, ND not described, Pap. adenoca. Papillary adenocarcinoma, PD pancreaticoduodenectomy, PPPD pylorus-preserving pancreaticoduodenectomy, SSPPD subtotal stomach-preserving pancreaticoduodenectomy, Well diff. adenoca. Well differentiated adenocarcinoma

3 m, alive (no recurrence)

17 m, alive (no recurrence) (-) (-) Normal Mod. diff. adenoca. 12 9 11 PPPD None M 70

16 m, alive (no recurrence)

Fujimoto [18] 9

2013

(-) (-) Normal Well diff. adenoca. 896 PPPD None M 74

Alive

Okuma [17] 8

2012

(-) (-) Normal Well diff. adenoca. 12 9 10 PPPD None M 76

24 m, alive (no recurrence)

Takami [16] 7

2011

(-) (?) Normal Pap. ? well ? mod. diff. adenoca. 20 9 15 SSPPD None M 81

8 m, alive (no recurrence)

Matheus [15] 6

2011

(-) ND Adenoca. Mod. diff. adenoca. 10 PPPD Abdominal pain, jaundice F 50

32 m, alive (no recurrence)

Parthasarathy [14]

2008

(-) (?) Adenoca. Mod. diff. adenoca. 15 9 12 PD Fever, jaundice F 60

Alive

5

2008

(-)

(-) (?)

(-) Normal

Normal Well diff. adenoca.

Well diff. adenoca. 11 9 8

50 9 30 SSPPD

PPPD None

Transient abdominal pain M

M 70

60

Wakatsuki [1] 4

2008

Kajiwara [12] 3

2007

42 m, deada

ND ND

ND (?)

ND Normal

Normal Mod. diff. adenoca.

Adenoca. 10

25 9 20 PPPD

PD Gastric discomfort

Transient epigastric pain M

M 70

77 1998

2006

Yamao [10]

Ohya [11]

1

2

Operation Chief complaint(s) Sex Age Year Author [ref] Case

Table 1 Reported cases of adenocarcinoma of the minor duodenal papilla

Size (mm)

Histology

Major papilla

Invasion of the pancreas

Lymph node metastasis

Prognosis

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in the anterior wall of the second portion of the duodenum approximately 2 cm proximal to the major duodenal papilla, and its primary function is to drain pancreatic fluid from the dorsal pancreas to the duodenum in the embryo [8, 9]. Neoplasms of the minor duodenal papilla are rare, and most reported cases have been found to be benign tumors, such as carcinoid tumors and adenomas. Adenocarcinoma of the minor duodenal papilla is considered to be an extremely rare disease, with only 10 cases having been reported, including our case (Table 1) [10–18]. Among these cases, the tumors affected patients from 50 to 81 years (average 69 years) of age, consisting of eight males and two females. Two cases involved synchronous carcinomas of both the major and minor duodenal papilla. No lymph node metastasis was observed in any of these cases. The lack of specific symptoms makes diagnosing primary adenocarcinoma of the minor duodenal papilla difficult. Among the reports of this tumor, five patients, including our patient, had no symptoms, four patients experienced abdominal pain and one patient developed jaundice. No specific symptoms for tumors of the minor duodenal papilla have been identified, and all reported cases were discovered incidentally on upper gastrointestinal endoscopy. Therefore, careful observation of the minor duodenal papilla is necessary when routine upper gastrointestinal endoscopy is performed. The cause of the mass-forming chronic pancreatitis in the present case was unclear. There was no history suggestive of alcohol abuse, and the IgG4 level was within the normal range. In addition, the tumor had not invaded the main pancreatic duct, so the etiology of the patient’s massforming chronic pancreatitis of the pancreatic head is unknown. Regarding the treatment of primary adenocarcinoma of the minor duodenal papilla, pancreaticoduodenectomy has been performed in all reported cases. Recently, the use of endoscopic resection (papillectomy) was reported in patients with tumors of the major duodenal papilla, and a few malignant cases in the early stage were treated with papillectomy, although a few complications were observed [19–21]. For the evaluation of tumor staging, endoscopic ultrasonography (EUS) for tumors of the minor duodenal papilla is considered to be useful [10]. The diagnosis of whether or not the tumor has infiltrated into the pancreatic parenchyma is important. However, it is difficult to determine the depth of invasion of the tumor and the presence of lymph node metastasis using EUS and CT. Therefore, expanding the indications for papillectomy of malignant tumors of the duodenal papilla will require further accumulation of case information. In our case, EUS was not performed because we preoperatively diagnosed pancreatic head carcinoma with infiltration of the duodenum.

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With respect to the prognosis, all patients who underwent curative resection were still alive at the time of the publication of the report, with the exception of one patient who died from another disease. Therefore, curative resection is an effective treatment for primary adenocarcinoma of the minor duodenal papilla, and can provide long-term survival. Conflict of interest Kazuhiro Suzumura and the co-authors have no conflicts of interest to declare.

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Primary adenocarcinoma of the minor duodenal papilla with mass-forming chronic pancreatitis: report of a case.

A 70-year-old male was treated for gastric ulcers. Follow-up upper gastrointestinal endoscopy revealed an irregular, elevated tumor in the second port...
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