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01.10.2009 | Case Report | Ausgabe 10/2009 Open Access

Journal of Gastrointestinal Surgery 10/2009

Pancreatic Serous Cystadenocarcinoma: A Case Report and Review of the Literature

Zeitschrift:
Journal of Gastrointestinal Surgery > Ausgabe 10/2009
Autoren:
Jonathan C. King, Tina T. Ng, Stephen C. White, Galen Cortina, Howard A. Reber, O. Joe Hines

Introduction

Malignant cystic neoplasms are rare entities that account for only 1% of all pancreatic tumors. 1 Serous and mucinous cystic neoplasms are tumors of the exocrine pancreas with different biological behaviors. Mucinous cystic tumors are typically slow-growing but carry a significant potential for malignancy, and thus, resection is often indicated. 2, 3 In contrast, serous cystadenomas are considered benign tumors with almost no malignant potential. They are often observed with serial imaging or managed expectantly. 4 In the absence of symptoms, surgery is not usually recommended.
The first case of a pancreatic serous cystadenocarcinoma was reported by George et al. in 1989. The authors described the malignant characteristics of a serous cystic tumor of the pancreas with invasion into the spleen, stomach, and liver. The patient expired intra-operatively due to hemorrhage. 5 Subsequently, additional reports have documented similar findings of serous cystic neoplasms with malignant behavior. The histological characteristics of serous cystadenocarcinoma are indistinguishable from its benign counterpart, making the presence of invasion the sole distinguishing characteristic between the two. 5 In this report, we present a case of serous cystadenocarcinoma with duodenal, vascular, and neural invasion. We also review the literature and discuss the current diagnostic techniques and principles of management.

Materials and Methods

A systematic review of the literature was performed utilizing PubMed and MEDLINE searches. Articles were identified using the search terms: pancreas and serous cystadenocarcinoma. Nineteen articles were included in the analysis. Data are presented as mean ± standard error of the mean.

Results

A 70-year-old man presented with upper gastrointestinal (GI) bleeding and abdominal pain. There was a duodenal ulcer with no evidence of malignancy on esophagogastroduodenoscopy (EGD), and an abdominal computed tomography (CT) scan revealed a 5.7-cm cystic mass in the head of the pancreas which was diagnosed by core needle biopsy as a serous cystadenoma. The patient was treated for presumed duodenal ulcer disease leading to resolution of symptoms and scheduled for observation of his pancreatic mass.
Three months later, the patient returned with recurrent coffee-ground emesis and abdominal pain. CT scan showed enlargement of the pancreatic mass to 6.5 × 8 cm and central dystrophic calcifications with new pancreatic and biliary ductal dilatation (Fig.  1). Repeat EGD identified a bleeding duodenal ulceration, and biopsies were consistent with a “benign” serous cystadenoma.
Upon surgical consultation, an elective pancreaticoduodenectomy was planned due to the increased size of the tumor, recurrent bleeding, and erosion into the duodenum. Laparotomy revealed a large mass in the head of the pancreas with no evidence of gross metastatic disease or invasion of the mesenteric vessels. The patient’s post-operative course was complicated by delayed gastric emptying requiring temporary gastrostomy and feeding jejunostomy tubes.
On gross examination, the mass measured 9 × 8 × 6 cm, and there was marked, aggressive invasion of the duodenum beyond the level of the muscularis propria (Fig.  2). Histology demonstrated microcysts lined by clear cells without mucinous cytoplasm (Fig.  3). Microscopic vascular and perineural invasion were also seen, further distinguishing this lesion from a benign serous cystadenoma (Figs.  4 and 5). All resection margins and 17 lymph nodes were uninvolved.
Immunochemical stains for keratin AE1/3, 7, and 19 and CAM 5.2 were positive. Stained samples of the mass also showed weak immunoreactivity for carcinoembryonic antigen (CEA). Keratin 20 staining was negative. DNA content analysis by flow cytometry demonstrated no evidence of aneuploidy.
The final diagnosis was serous cystadenocarcinoma with duodenal, vascular, and neural invasion. At last follow-up 7 years post-operatively, the patient is doing well without clinical or radiographic evidence of recurrent disease.
Literature review yielded 25 reports of serous cystadenocarcinoma (Table  1). 523 The average age at presentation is 68 ± 2 years, and 60% of patients affected are female (28% male; in 12% of cases, sex was not reported). Presenting complaints included abdominal pain (24%), upper GI bleeding (12%), weight loss (8%), palpable mass (8%), jaundice or abnormal serum liver enzymes (8%), and nonspecific abdominal complaints (8%).
Table 1
Characteristics of Pancreatic Serous Cystadenocarcinoma Reported in the Literature
Author
Publication year
Patient age
Patient gender
Signs/symptoms
Tumor size (cm)
Metastases
Procedure
Outcome
Note
George et al. 5
1989
70
M
Hemorrhage from gastric varices
11
Synchronous in stomach and liver
DP
Operative death due to hemorrhage
 
Friedman 6
1990
74
F
NA
19 × 16 × 10
Synchronous in liver, lungs, bone marrow, adrenal glands, LN
NA
NA
 
Kamei et al. 7
1991
72
F
Jaundice
10
No
Total pancreatectomy
NA
 
Okada et al. 8
1991
63
F
Abdominal pain
12
Metachronous in liver
DP
Alive 1 year later
 
Yoshimi et al. 9
1992
63
F
Abdominal pain
12
Metachronous in liver
DP
Alive 3 years later
 
Ohta et al. 10
1993
64
M
Urinary frequency
2.5 × 2.5 × 2
No
Enucleation
Alive 9 months later
 
Widmaier et al. 11
1996
71
M
Abnormal liver function
4
Synchronous in LN
Pylorus-preserving partial pancreatico-duodenectomy
Alive 1 year later
 
Ishikawa et al. 12
1998
63
F
Abdominal pain
12
Metachronous in liver
DP
NA
 
Siech et al. 13
1998
NA
NA
NA
NA
NA
NA
NA
2 cases reported
Eriguchi et al. 14
1998
65
F
Palpable abdominal mass
16
Synchronous and meta-chronous in liver
DP, Microwave coagulo-necrotic therapy
Alive 10 years later
 
Abe et al. 15
1998
71
F
Palpable abdominal mass
12 × 8.5 × 5
Synchronous in LN
DP, splenectomy
Alive 2 years later
 
Schmidt-Rohlfing et al. 16
1998
52–74
2 M, 2 F
NA
NA
NA
NA
NA
4 cases reported
Kimura and Makuuchi 17
1999
53, 66
F, M
NA
5, 3
No
NA
NA
2 cases reported
Horvath and Charbot 18
1999
81
F
NA
6
NA
NA
NA
 
Wu et al. 19
1999
57
F
Hematemesis
NA
Synchronous and meta-chronous in liver
NA
NA
 
Strobel et al. 20
2001
56
F
Abdominal pain, weight loss
14 × 7 × 4
Metachronous in liver
Pylorus-preserving total pancreatico-duodenectomy
Alive 3 years later
 
Shintaku et al. 22
2005
85
F
Fatigue, intermittent diarrhea
12 × 9 × 7
Direct extension to spleen
Distal gastrectomy, DP
Alive 10 months later
 
Friebe et al. 21
2005
80
F
Abdominal pain, anorexia, weight loss
8 × 7 × 7
Direct extension to spleen
DP, splenectomy
Alive 1 year later
 
Galanis et al. 23
2007
NA
NA
NA
NA
Synchronous and meta-chronous lesion in liver
NA
NA
2 cases reported
Current
70
M
Hematemesis, abdominal pain
9 × 8 × 6
Direct extension to duodenum
PPW
Alive 7 years later
 
DP distal pancreatectomy, NA not available, LN lymph node, PPW pylorus-preserving Whipple resection
The mean diameter of serous cystadenocarcinoma was 10 ± 1 cm (range, 2.5–19 cm). Lesions exhibit both of the hallmarks of malignancy: local invasiveness and distant metastasis with most tumors associated with local invasion of the spleen (8%), small intestine (4%), stomach (4%), adrenal gland (4%), or microscopic invasion of vascular and neural tissues. Synchronous or metachronous liver metastases were frequently noted (36%), along with metastasis to regional lymph nodes (12%), bone marrow (4%), and lung (4%). Mean survival was 36 ± 11 months (range, <30 days to 120 months) among cases with follow-up ( n = 11), and ten (91%) of these patients were still alive when reports were published including seven (64%) patients with metastatic disease.

Discussion

The preoperative differentiation between a benign serous cystadenoma and malignant serous cystadenocarcinoma remains difficult. Indeed, the correct diagnosis of serous cystadenocarcinoma was not made pre-operatively in any of the cases, including the current one. 523 The benign and malignant variants appear identical histologically, with the only distinguishing feature being gross or microscopic evidence of invasiveness. Thus, the utility of cytology or histology obtained from core needle biopsy is limited. 18
The current practice for management of serous cystadenomas of the pancreas is to observe asymptomatic lesions thereby avoiding the potential morbidity and mortality associated with a major operation. 13, 14, 18, 24, 25 We agree with this conservative approach. Nevertheless, clinicians should be aware of the possibility for malignant transformation in serous cystic neoplasms and should maintain an index of suspicion when certain clues appear. These include the onset of new symptoms, worsening of symptoms, or rapid enlargement of the mass. In these cases, resection may be indicated, despite the lack of objective evidence for malignancy obtained from preoperative imaging, endoscopy, and biopsies.
In the current case, a serous cystadenocarcinoma was diagnosed without evidence of distant metastasis but with extensive tumor invasion into surrounding structures, both grossly and microscopically. To our knowledge, this is the first example of serous cystadenocarcinoma with extensive duodenal, vascular, and neural invasion but no distant metastases.

Conclusion

Our case report is illustrative of the management strategy for serous cystic lesions of the pancreas despite the presence of an initially unrecognized malignancy: the progression of symptoms and increase in size of the mass triggered curative resection. The excellent prognosis associated with serous cystadenocarcinoma justifies an aggressive approach to surgical resection, even in older patients. This is especially so since major pancreatic resections are now done with very low mortality and morbidity rates in major centers around the world. 26

Open Access

This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited.
Open AccessThis is an open access article distributed under the terms of the Creative Commons Attribution Noncommercial License ( https://​creativecommons.​org/​licenses/​by-nc/​2.​0), which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited.

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