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Table 1. 
Comparison of Clinical Characteristics and Surgical Outcome Between Adults and Children
Comparison of Clinical Characteristics and Surgical Outcome Between Adults and Children
Table 2. 
Predictive Factors of Malignant Potential
Predictive Factors of Malignant Potential
1.
Martin  RCKlimstra  DSBrennan  MFConlon  KC Solid-pseudopapillary tumor of the pancreas: a surgical enigma? Ann Surg Oncol 2002;9 (1) 35- 40
PubMedArticle
2.
Pettinato  GManivel  JCRavetto  C  et al.  Papillary cystic tumor of the pancreas: a clinicopathologic study of 20 cases with cytologic, immunohistochemical, ultrastructural, and flow cytometric observations, and a review of the literature. Am J Clin Pathol 1992;98 (5) 478- 488
PubMed
3.
Petrakis  IVrachassotakis  NKogerakis  NHatzidakis  AZoras  OChalkiadakis  G Solid pseudopapillary neoplasm of the pancreas: report of a case after a 10-year follow-up and review of the literature. Pancreatology 2001;1 (2) 123- 128
PubMedArticle
4.
Ward  HCLeake  JSpitz  L Papillary cystic cancer of the pancreas: diagnostic difficulties. J Pediatr Surg 1993;28 (1) 89- 91
PubMedArticle
5.
Poustchi-Amin  MLeonidas  JCValderrama  E  et al.  Papillary-cystic neoplasm of the pancreas. Pediatr Radiol 1995;25 (7) 509- 511
PubMedArticle
6.
Wang  KSAlbanese  CDada  FSkarsgard  ED Papillary cystic neoplasm of the pancreas: a report of three pediatric cases and literature review. J Pediatr Surg 1998;33 (6) 842- 845
PubMedArticle
7.
Jung  SEKim  DYPark  KWLee  SCJang  JJKim  WK Solid and papillary epithelial neoplasm of the pancreas in children. World J Surg 1999;23 (3) 233- 236
PubMedArticle
8.
Peng  CHChen  DFZhou  GW  et al.  The solid-pseudopapillary tumor of pancreas: the clinical characteristics and surgical treatment. J Surg Res 2006;131 (2) 276- 282
PubMedArticle
9.
Sun  CDLee  WJChoi  JSOh  JTChoi  SH Solid-pseudopapillary tumours of the pancreas: 14 years experience. ANZ J Surg 2005;75 (8) 684- 689
PubMedArticle
10.
Salvia  RBassi  CFesta  L  et al.  Clinical and biological behavior of pancreatic solid pseudopapillary tumors: report on 31 consecutive patients. J Surg Oncol 2007;95 (4) 304- 310
PubMedArticle
11.
Papavramidis  TPapavramidis  S Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature. J Am Coll Surg 2005;200 (6) 965- 972
PubMedArticle
12.
Choi  SHKim  SMOh  JTPark  JYSeo  JMLee  SK Solid pseudopapillary tumor of the pancreas: a multicenter study of 23 pediatric cases. J Pediatr Surg 2006;41 (12) 1992- 1995
PubMedArticle
13.
Rebhandl  WFelberbauer  FXPuig  S  et al.  Solid-pseudopapillary tumor of the pancreas (Frantz tumor) in children: report of four cases and review of the literature. J Surg Oncol 2001;76 (4) 289- 296
PubMedArticle
14.
Goh  BKTan  YMCheow  PC  et al.  Solid pseudopapillary neoplasms of the pancreas: an updated experience. J Surg Oncol 2007;95 (8) 640- 644
PubMedArticle
15.
de Castro  SMSinghal  DAronson  DC  et al.  Management of solid-pseudopapillary neoplasms of the pancreas: a comparison with standard pancreatic neoplasms. World J Surg 2007;31 (5) 1130- 1135
PubMedArticle
16.
Ng  KHTan  PHThng  CHOoi  LL Solid pseudopapillary tumor of the pancreas. ANZ J Surg 2003;73 (6) 410- 415
PubMedArticle
17.
Nishihara  KNagoshi  MTsuneyoshi  MYamaguchi  KHayashi  I Papillary cystic tumors of the pancreas: assessment of their malignant potential. Cancer 1993;71 (1) 82- 92
PubMedArticle
18.
Matsunou  HKonishi  F Papillary-cystic neoplasm of the pancreas: a clinicopathologic study concerning the tumor aging and malignancy of nine cases. Cancer 1990;65 (2) 283- 291
PubMedArticle
19.
Hibi  TOjima  HSakamoto  Y  et al.  A solid pseudopapillary tumor arising from the greater omentum followed by multiple metastases with increasing malignant potential. J Gastroenterol 2006;41 (3) 276- 281
PubMedArticle
20.
Tipton  SGSmyrk  TCSarr  MG  et al.  Malignant potential of solid pseudopapillary neoplasm of the pancreas. Br J Surg 2006;93 (6) 733- 737
PubMedArticle
21.
Fried  PCooper  JBalthazar  E  et al.  A role for radiotherapy in the treatment of solid and papillary neoplasms of the pancreas. Cancer 1985;56 (12) 2783- 2785
PubMedArticle
22.
Maffuz  ABustamante Fde  TSilva  JA  et al.  Preoperative gemcitabine for unresectable, solid pseudopapillary tumour of the pancreas. Lancet Oncol 2005;6 (3) 185- 186
PubMedArticle
Original Article
December 15, 2008

Clinical Features and Outcome of Solid Pseudopapillary NeoplasmDifferences Between Adults and Children

Author Affiliations

Author Affiliations: Department of Surgery, Seoul National University College of Medicine, Seoul, Korea.

Arch Surg. 2008;143(12):1218-1221. doi:10.1001/archsurg.143.12.1218
Abstract

Objectives  To delineate the clinical and pathological characteristics of solid pseudopapillary neoplasm (SPN), compare them between adults and children, and determine the predictive features suggesting malignant potential.

Design  Retrospective analysis of patients who underwent surgery for a pathologically confirmed SPN.

Setting  Tertiary care referral center.

Patients  Sixty-two consecutive patients who underwent surgery for a pathologically confirmed SPN between 1985 and 2006.

Main Outcome Measures  Demographic information and clinical presentation, radiological details, surgical data, pathological characteristics, postoperative course, and long-term survival.

Results  Among 62 patients, 47 patients were adults (mean age, 36 years; range, 18-63 years) and 15 patients were children (mean age, 12 years; range, 8-13 years). A palpable mass was the most common presenting symptom in children (9 of 15; 60%) and an incidentally detected pancreatic mass, in adults (18 of 47; 38.3%) (P = .001). The mean tumor size in children was significantly larger than in adults (8.0 vs 6.0 cm; P < .03). In children, the tumor was located in the head of the pancreas (10 of 15; 66.7%) and in adults, in the body or tail (38 of 47; 80.9%) (P = .001). Nine patients (14.5%) had malignant SPN. There was no significant clinical factor suggesting malignant potential. Two patients had a tumor recurrence. They were still alive after debulking surgery. There were no tumor-related deaths.

Conclusion  Solid pseudopapillary neoplasm had different clinical features in adults and children. Because long-term survival can be achieved, even with the synchronous or metachronous metastatic lesions, SPN should be treated aggressively, with complete resection, even if this requires metastatectomy.

A solid pseudopapillary neoplasm (SPN) is a rare primary neoplasm of the pancreas with low-grade malignant potential. Despite a recent increase in recognition, the pathogenesis and guidelines for SPN treatment remain unclear. Even in the event of metastases, extension into adjacent structures, or nodal involvement, radical surgical resection provides long-term survival.13 Solid pseudopapillary neoplasm in children is rare.47 Because of the rarity of this neoplasm, the reported information available is limited. To our knowledge, there is no prior report of a comparison of the clinical and pathological features in adults and children. In this study, we evaluated the clinical and pathological characteristics of SPN, the features suggesting malignant potential, and the long-term outcome, comparing them between adults and children, by examining a single institution's experience.

METHODS

Between January 1985 and December 2006, 62 consecutive patients who underwent surgery for a pathologically confirmed SPN at Seoul National University Hospital and Seoul National University Children's Hospital were retrospectively reviewed. Of the 62 patients, 47 patients were adults (mean age, 36 years; range, 18-63 years) and 15 were children (mean age, 12 years; range, 8-13 years). Demographic information and clinical presentation, radiological details, surgical data, pathological characteristics, postoperative course, and long-term survival were evaluated. We compared the outcomes of the clinical and pathological characteristics between females and males and between benign and malignant SPN in the adults and children. Pathologically, SPN was defined as malignant if it demonstrated deep pancreatic invasion, perineural invasion, vascular invasion, or lymph node metastasis.

Statistical comparisons between the 2 groups were made using the χ2 test or the Fisher exact test for categorical parameters and the t test for continuous variables. P values <.05 were considered statistically significant.

RESULTS
COMPARISON OF CLINICAL AND PATHOLGICAL FEATURES BETWEEN ADULTS AND CHILDREN

The clinical and pathological features of the 62 patients are summarized in Table 1. In the adult group, the diagnosis was usually made incidentally during screening by detection of a mass. By contrast, all of the children were symptomatic. This difference in presentation was significant (P = .001). Only 38 patients' (61.3%) SPN was diagnosed before surgery. The mean diameter of the tumors based on pathological examination was 6.0 cm (range, 1.5-14 cm) in adults and 8.0 cm (range, 3.5-14 cm) in children. The tumor size was significantly larger in children than in adults (P = .03). The most common location of the tumor in all patients was the pancreatic body or tail (43 of 62; 69.4%). In adults, the pancreatic body or tail was the most common location of the tumor. However, in children, the pancreatic head was the most common site (P = .001).

SURGICAL OUTCOMES

All patients underwent surgical resection for the tumor. Pancreaticoduodenectomy was performed in 18 patients; distal pancreatectomy, in 38 patients; central pancreatectomy, in 2 patients; and duodenum-preserving pancreatic head resection, in 1 patient (Table 1). Concurrent resection of other organs was performed in 4 patients (6.5%). Two patients underwent a transverse colon resection and anastomosis, and 1 patient underwent portal vein resection and anastomosis because tumor infiltration was suspected; pathology reports showed no tumor involvement. One child underwent a left lateral sectionectomy of the liver because of a synchronous liver metastasis. There was no surgery-related mortality. Postoperative complications were noted in 19 patients (30.6%), including pancreatic fistula in 6 patients, intra-abdominal fluid collection in 6 patients, and delayed gastric emptying in 3 patients. None of the complications required surgical intervention.

CHARACTERISTICS OF MALIGNANT SPN

The pathological findings suggesting malignancy are listed in Table 1. Nine patients (14.5%) had malignant SPN. Perineural invasion was the most common pathological finding. A 13-year-old girl had a synchronous liver metastasis; she underwent near total pancreatectomy with left lateral sectionectomy and was treated with chemotherapy with the OCCG321P2 (combination of cyclophosphamide, cisplatin, doxorubicin hydrochloride, and etoposide phosphate) regimen for a total of 13 cycles. At 3 years' follow-up, this patient was living, with no evidence of recurrence. During the follow-up period that ranged from 5 months to 20 years (mean, 47.5 months), 2 patients had tumor recurrence. A 25-year-old patient underwent debulking surgery because of SPN in the tail of the pancreas and peritoneal seeding. This patient rejected adjuvant chemotherapy, and 13 years after the initial operation, the size of seeding nodules increased and debulking surgery was performed again. Sixteen years after the initial operation, liver metastasis was detected and a left lateral sectionectomy was performed. Twenty years after the initial operation, the patient was still living. The other patient was 8 years of age and she underwent spleen-preserving distal pancreatectomy because of a ruptured SPN. There were no pathological features suggesting a malignancy. Seven years after the initial operation, peritoneal seeding was detected. Debulking surgery was performed. There were no tumor-related deaths and all of the patients are still living.

PREDICTIVE FEATURES OF MALIGNANT SPN

We compared the clinical features of malignant and benign SPN. On univariate analyses, none of the preoperative features, including age, sex, tumor size, tumor location, elevated carcinoembryonic antigen levels, and elevated carbohydrate antigen 19-9 levels, were predictive of a malignant SPN (Table 2).

COMMENT

Recently, the number of cases of SPNs reported in the literature has been steadily increasing. However, there have been only a few large series reporting a single institution's experience.1,810 Furthermore, to our knowledge, there is no prior report comparing the clinical and pathological features in adults and children.

This study evaluated 47 adults and 15 children with pathologically proven SPN. The adults and children had different clinical features. A palpable mass was the most common symptom in children with SPN (9 of 15; 60%) and no child's diagnosis was made incidentally. By contrast, an incidentally detected pancreatic mass was the most common presentation in adults (18 of 47; 38.3%) (P = .001). These results are different from the results of other reported studies. Abdominal pain was reported as the most common symptom.11 The incidental diagnosis in adults may be because of the improved generalized screening programs in Korea. The mean tumor size in children was significantly larger than in adults (6.0 vs 8.0 cm; P < .03). Because only a few cases of children have been reported and prior reports did not divide adult and childhood cases for comparison, we cannot confirm our findings by comparing the results with previous studies. In children, the tumor was more commonly located in the head of the pancreas (10 of 15; 66.7%), and in adults, location in the body or tail of the pancreas was more common (38 of 47; 80.9%) (P = .001). The tail of the pancreas has been the most commonly reported location of the tumor,12,13 except for 1 report from our institution.7 However, all prior reports analyzed the adults and children together, and therefore, potential differences could not be identified.

Although our study is one of the largest clinical and pathological studies to date, consistent with prior reports, there was no factor identified that predicted the malignant potential of an SPN.1,14 Malignant behavior (recurrence or metastasis) could not be completely excluded even in the absence of pathological features suggesting a malignant potential; therefore, aggressive tumor behavior was not predictable. In fact, 2 patients with recurrence in our study had no pathological features suggesting malignant potential. Therefore, regardless of the malignant potential, all patients with SPN must be observed closely. A few cases of SPN with peritoneal seeding have been reported who lived for several years after surgery.1519 Solid pseudopapillary neoplasm is considered to be a tumor that grows slowly and can have stable metastatic disease. For these reasons, despite the large size of these tumors and their ability to extend locally, a complete excision can provide benefits for most patients.

The role of adjuvant or neoadjuvant chemotherapy and radiotherapy for SPN is currently poorly defined because of the difficulty in obtaining data on adjuvant or neoadjuvant therapy.2022 In our study, 2 patients were given adjuvant chemotherapy. To our knowledge, there are only 2 published case reports showing successful use of radiotherapy21 and neoadjuvant chemotherapy22 for locally advanced unresectable SPN. Further studies are needed to determine the radiosensitivity and chemosensitivity of SPN.

In summary, SPN demonstrated different clinical features in adults and children. Further study is required to elucidate the pathophysiology of these differences. Because long-term survival can be achieved even with the synchronous or metachronous metastatic lesions, SPN of the pancreas should be treated aggressively, with surgical resection, even if this requires metastasectomy.

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Article Information

Correspondence: Sun-Whe Kim, MD, PhD, Department of Surgery, Seoul National University College of Medicine, 28 Yeongeon-dong, Jongno-gu, Seoul 110-744, Korea (sunkim@plaza.snu.ac.kr).

Accepted for Publication: December 20, 2007.

Author Contributions:Study concept and design: Lee, Jang, Hwang, Park, and Kim. Acquisition of data: Lee. Analysis and interpretation of data: Lee, Jang, Hwang, Park, and Kim. Drafting of the manuscript: Lee, Jang, Hwang, Park, and Kim. Critical revision of the manuscript for important intellectual content: Lee, Jang, Park, and Kim. Statistical analysis: Lee, Jang, Hwang, and Kim. Obtained funding: Lee and Jang. Administrative, technical, and material support: Lee, Jang, Hwang, and Kim. Study supervision: Jang, Park, and Kim.

Financial Disclosure: None reported.

References
1.
Martin  RCKlimstra  DSBrennan  MFConlon  KC Solid-pseudopapillary tumor of the pancreas: a surgical enigma? Ann Surg Oncol 2002;9 (1) 35- 40
PubMedArticle
2.
Pettinato  GManivel  JCRavetto  C  et al.  Papillary cystic tumor of the pancreas: a clinicopathologic study of 20 cases with cytologic, immunohistochemical, ultrastructural, and flow cytometric observations, and a review of the literature. Am J Clin Pathol 1992;98 (5) 478- 488
PubMed
3.
Petrakis  IVrachassotakis  NKogerakis  NHatzidakis  AZoras  OChalkiadakis  G Solid pseudopapillary neoplasm of the pancreas: report of a case after a 10-year follow-up and review of the literature. Pancreatology 2001;1 (2) 123- 128
PubMedArticle
4.
Ward  HCLeake  JSpitz  L Papillary cystic cancer of the pancreas: diagnostic difficulties. J Pediatr Surg 1993;28 (1) 89- 91
PubMedArticle
5.
Poustchi-Amin  MLeonidas  JCValderrama  E  et al.  Papillary-cystic neoplasm of the pancreas. Pediatr Radiol 1995;25 (7) 509- 511
PubMedArticle
6.
Wang  KSAlbanese  CDada  FSkarsgard  ED Papillary cystic neoplasm of the pancreas: a report of three pediatric cases and literature review. J Pediatr Surg 1998;33 (6) 842- 845
PubMedArticle
7.
Jung  SEKim  DYPark  KWLee  SCJang  JJKim  WK Solid and papillary epithelial neoplasm of the pancreas in children. World J Surg 1999;23 (3) 233- 236
PubMedArticle
8.
Peng  CHChen  DFZhou  GW  et al.  The solid-pseudopapillary tumor of pancreas: the clinical characteristics and surgical treatment. J Surg Res 2006;131 (2) 276- 282
PubMedArticle
9.
Sun  CDLee  WJChoi  JSOh  JTChoi  SH Solid-pseudopapillary tumours of the pancreas: 14 years experience. ANZ J Surg 2005;75 (8) 684- 689
PubMedArticle
10.
Salvia  RBassi  CFesta  L  et al.  Clinical and biological behavior of pancreatic solid pseudopapillary tumors: report on 31 consecutive patients. J Surg Oncol 2007;95 (4) 304- 310
PubMedArticle
11.
Papavramidis  TPapavramidis  S Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature. J Am Coll Surg 2005;200 (6) 965- 972
PubMedArticle
12.
Choi  SHKim  SMOh  JTPark  JYSeo  JMLee  SK Solid pseudopapillary tumor of the pancreas: a multicenter study of 23 pediatric cases. J Pediatr Surg 2006;41 (12) 1992- 1995
PubMedArticle
13.
Rebhandl  WFelberbauer  FXPuig  S  et al.  Solid-pseudopapillary tumor of the pancreas (Frantz tumor) in children: report of four cases and review of the literature. J Surg Oncol 2001;76 (4) 289- 296
PubMedArticle
14.
Goh  BKTan  YMCheow  PC  et al.  Solid pseudopapillary neoplasms of the pancreas: an updated experience. J Surg Oncol 2007;95 (8) 640- 644
PubMedArticle
15.
de Castro  SMSinghal  DAronson  DC  et al.  Management of solid-pseudopapillary neoplasms of the pancreas: a comparison with standard pancreatic neoplasms. World J Surg 2007;31 (5) 1130- 1135
PubMedArticle
16.
Ng  KHTan  PHThng  CHOoi  LL Solid pseudopapillary tumor of the pancreas. ANZ J Surg 2003;73 (6) 410- 415
PubMedArticle
17.
Nishihara  KNagoshi  MTsuneyoshi  MYamaguchi  KHayashi  I Papillary cystic tumors of the pancreas: assessment of their malignant potential. Cancer 1993;71 (1) 82- 92
PubMedArticle
18.
Matsunou  HKonishi  F Papillary-cystic neoplasm of the pancreas: a clinicopathologic study concerning the tumor aging and malignancy of nine cases. Cancer 1990;65 (2) 283- 291
PubMedArticle
19.
Hibi  TOjima  HSakamoto  Y  et al.  A solid pseudopapillary tumor arising from the greater omentum followed by multiple metastases with increasing malignant potential. J Gastroenterol 2006;41 (3) 276- 281
PubMedArticle
20.
Tipton  SGSmyrk  TCSarr  MG  et al.  Malignant potential of solid pseudopapillary neoplasm of the pancreas. Br J Surg 2006;93 (6) 733- 737
PubMedArticle
21.
Fried  PCooper  JBalthazar  E  et al.  A role for radiotherapy in the treatment of solid and papillary neoplasms of the pancreas. Cancer 1985;56 (12) 2783- 2785
PubMedArticle
22.
Maffuz  ABustamante Fde  TSilva  JA  et al.  Preoperative gemcitabine for unresectable, solid pseudopapillary tumour of the pancreas. Lancet Oncol 2005;6 (3) 185- 186
PubMedArticle
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