Introduction
Duodenal tumors occur as sporadic lesions and are a common finding in patients with
familial adenomatous polyposis (FAP) [1]. Up to 80 % of adult patients with FAP may develop duodenal tumors [2]. However, these tumors are becoming increasingly diagnosed even in patients without
FAP due to the increasing number of endoscopies being performed [3]. The majority of these tumors arise in the major duodenal papilla and account for
approximately 5 % of gastrointestinal\ neoplasms [3]. In contrast, tumors arising in the minor papilla are uncommon and only described
in case reports [4]
[5]
[6]
[7]
[8]
[9]
[10]. Tumors of the minor papilla are rare and are almost always reported as adenoma,
or neuroendocrine tumors such as carcinoid, or somatostatinoma [4]
[6]. Adenocarcinoma of the minor papilla is quite rare. A surgical resection is the
primary treatment for both minor and major papillary adenocarcinomas [4].
Benign lesions of the papilla have the potential to undergo malignant transformation
to papillary carcinomas [11]. The incidence of malignant transformation to carcinoma in situ or invasive carcinoma
has ranged from 25 % to 85 % [11]. Papillary and duodenal carcinoma are aggressive cancers with poor 5-year survival
rates. Like colorectal cancer, papillary carcinomas are also thought to follow the
adenoma-carcinoma sequence. To prevent malignant transformation of minor papillary
tumors, complete resection or surveillance of these lesions is advisable.
There have been some case reports on endoscopic papillectomy and endoscopic mucosal
resection of minor papillary tumors, however all of these studies include no more
than one patient [10]
[12]. This lack of data compels further evaluation of managing minor papillary lesions
by endoscopy. We report a multicenter case series of endoscopic papillectomy in the
management of minor papillary tumors.
Patients and methods
Consecutive patients undergoing papillectomy for minor papillary tumors at four hospitals
were included in this study over a period of 5 years. Inclusion and exclusion criteria
are detailed below:
Inclusion criteria:
-
Any patient found to have minor papillary tumor with history of abdominal pain or
pancreatitis, deemed to be secondary to the tumor, or tumor felt to be at high risk
of progression to carcinoma were included in the study with intention of endoscopic
resection.
Exclusion criteria:
-
Inability or refusal to provide informed consent
-
Contraindication to MAC sedation or general anesthesia
-
Contraindication to endoscopic resection including severe coagulopathy, immunosuppression.
A total of six patients were included in the study and all six patients underwent
endoscopic retrograde cholangiopancreatography (ERCP) for the purpose of minor papillectomy
([Table 1]). Magnetic resonance cholangiopancreatography and endoscopic ultrasound was performed
on all patients prior to ERCP to rule out invasion ([Fig. 1]). All patients underwent ERCP, and resection of the minor papilla was performed
using snare polypectomy technique. An ERBE generator was used for all cases with the
following Endocut Q settings: Effect 2, cut duration 1, cut Interval three. The specimens
that were retrieved were sent for histopathologic analysis. Pancreatic stents were
placed in the duct of Santorini (minor duct) after papillectomy in five patients,
3 F × 8-cm single pigtail stents in four patients, and one 5 F × 5-cm straight stent
in one patient. All stents were removed after approximately 2 weeks. Stent placement
was not successful in one patient.
Table 1
Patient demographics and minor papillary tumor characteristics.
|
Patient 1
|
Patient 2
|
Patient 3
|
Patient 4
|
Patient 5
|
Patient 6
|
|
Gender
|
M
|
M
|
F
|
M
|
F
|
M
|
|
Age
|
45
|
56
|
61
|
67
|
52
|
41
|
|
Tumor type
|
Carcinoma
|
Adenoma
|
Adenoma
|
Adenoma
|
Carcinoid
|
Adenoma with High-grade dysplasia
|
|
Tumor Size
|
1 cm
|
2 cm
|
1.5 cm
|
1.7 cm
|
2.5 cm
|
3 cm
|
|
FAP
|
Yes
|
No
|
No
|
No
|
No
|
Yes
|
|
Pancreatic divisum
|
Type 1
|
None
|
Type 3
|
None
|
None
|
None
|
FAP, familial adenomatous polyposis.
Fig. 1 Endoscopic ultrasound demonstrating no deep invasion of tumor.
Complications were assessed postoperatively and by close outpatient follow-up. At
10 to 12 weeks, all six patients underwent repeat endoscopy with a standard duodenoscope
for evaluation of residual neoplastic tissue. Results were reported as success, residual
lesion, recurrence, and complications. Success was defined as a complete resection
of the tumor regardless of the number of required procedures with no recurrences on
follow-up endoscopies. A residual lesion was one in which gross or microscopic adenomatous
tissue was present on follow up endoscopies. Complications included pancreatitis,
bleeding, perforation, and delayed papillary stenosis [13]. Pancreatitis was defined by a three-fold increase in serum amylase or lipase in
presence of abdominal pain. Bleeding was defined as a drop in hemoglobin of at least
2 grams or if clinical suspicion led to performing endoscopy to evaluate for possible
bleeding. Recurrence was defined as the presence of a new tumor on repeat endoscopy.
Complete excision was confirmed by reviewing the pathology of the prior tissue biopsy.
Results
Papillectomy was technically successful in all six patients. One patient required
two ERCPs for complete papillectomy. One patient developed post ERCP pancreatitis
and was kept in the hospital for 2 days. No other major complications were noted.
Two patients had abdominal pain for one day post ERCP which was considered a minor
complication.
The tumors varied in size from 1 cm to 3 cm ([Fig. 2]). Pathology revealed adenoma in three patients, adenoma with high-grade dysplasia
in one patient, carcinoma in one patient, and carcinoid tumor in one patient.
Fig. 2 Endoscopy of minor papillary tumor, carcinoid.
Follow-up for these patients ranged from 2 to 5 years with esophagogastroduodenoscopy
using duodenoscope at 3 months, at 1 year and yearly thereafter. One patient had an
additional tumor identified at 2 years, which was found to be a recurrence of the
original adenoma (4 mm). This patient was treated with repeat papillectomy. The patient
with carcinoma had endoscopies every 3 months for a year followed by yearly endoscopy;
no recurrence was noted during the 3 years of follow-up. Confocal laser endomicroscopy
was performed at the time of each endoscopy to look for tumor recurrence, showing
no further evidence of tumor at the cellular level. Two of six patients (33 %) had
FAP. Two patients had pancreas divisum, one with Type 1, and other with Type 3 Divisum.
Discussion
Tumors of the minor papilla are rare [6]. In contrast to tumors of the major papilla, those of the minor papilla are less
symptomatic unless they grow large or cause pancreatic duct anomalies [5]
[6]. Historically, tumors of major papilla are removed surgically but given the increased
risk of post-surgical complications, endoscopic resection is now becoming a safe and
effective treatment option in these patients [3]. Surgical or endoscopic resection is indicated for all tumors to prevent progression
to carcinomas [11]. There are several existing case reports on endoscopic resection of minor papilla
tumors [4]
[5]
[6]
[7]
[8]
[9]
[10]. However, very little data are available regarding the long-term outcome for patients
after endoscopic treatment of tumors of minor papilla in order to ascertain the long-term
safety and efficacy of such treatment. One study identified three cases of minor papillary
tumors that were managed by endoscopic resection, but the follow-up duration was only
12 months [9]. Our case series is unique as not only was the follow-up period longer between 2
to 5 years, consistent with follow-up period suggested by recent guidelines on papillary
tumor management [14], but it also highlighted that endoscopic resection of benign tumors and early malignant
tumors of the minor duodenal papilla is a relatively safe procedure associated with
favorable long-term outcomes.
Pancreatitis is a well-recognized complication after endoscopic resection of tumor
of major papilla. Recent evidence has suggested prophylactic pancreatic duct stenting
to reduce the risk of pancreatitis [14]. With our case series, stents were placed after minor papillary resection into the
duct of Santorini, or minor duct. Our case series showed a comparable rate of procedure-related
complication to reported findings in the literature, as one out of six patients was
found to have pancreatitis [3]. The other five patients had no major complications and were discharged within 24
hours. In addition, none of our patients were noted to have delayed stenosis. Among
the patients who followed up, there were no recurrences of tumor and no patient was
found to have cancer develop over a mean follow-up period of 2 to 5 years. This demonstrates
that complete removal of these lesions via endoscopic resection is safe and has favorable
outcomes.
Confocal laser endomicroscopy (CLE) is an advanced endoscopic imaging technology that
facilitates the observation of gastrointestinal epithelia at a magnified, cellular
level [15]. CLE is utilized in the detection of dysplasia, adenoma, and carcinoma. CLE has
a high diagnostic accuracy for conditions affecting the gastrointestinal tract, such
as esophageal, gastric, and colonic neoplasia, pancreatic cysts and solid lesions,
and malignant pancreatobiliary strictures [15]. One single-center study comparing dual-focus narrow band imaging and CLE for real-time
diagnosis of adenomatous polyps in patients with FAP, showed that CLE had a similar,
high degree of diagnostic value as compared with narrow band imaging [16]. With respect to duodenal papilla, there has been evidence to suggest that CLE provides
adequate diagnostic accuracy similar to histopathologic specimens [17]. In our study, we were able to effectively demonstrate CLE as a reliable tool for
detection of recurrence of minor papilla tumors.
Conclusions
In our pilot study, endoscopic papillectomy appears safe and effective in the management
of minor papillary tumors. Larger studies with long-term follow-up are needed to further
demonstrate the safety and efficacy of endoscopic resection for minor papillary tumors.
