British Journal of Radiology (2005) 78, 441-443
© 2005 British Institute of Radiology
doi: 10.1259/bjr/91312352
Solid-pseudopapillary tumour of the pancreas associated with dorsal agenesis
S Ulusan, MD1,
N Bal, MD2,
O Kizilkilic, MD1,
F Bolat, MD2,
S Yildirim, MD3,
T Yildirim, MD1 and
E A Niron, MD1
Departments of 1 Radiology, 2 Pathology and 3 Surgery Baskent University Adana Teaching and Medical Research Centre, 01250 Yuregir, Adana, Turkey
 |
Abstract
|
|---|
Solid-pseudopapillary tumour of the pancreas is a rare benign or low-grade malignant epithelial tumour; its association with pancreatic dorsal agenesis has been reported only once before. We present the radiological and histological findings of a case of pancreatic solid-pseudopapillary tumour associated with total pancreatic dorsal agenesis. A 49-year-old woman presented with abdominal pain radiating to the back for several months. Radiological findings showed absence of the dorsal pancreas and an 8 cm x 6 cm diameter tumour arising from the head of the pancreas. She underwent successful complete resection of the tumour. Histopathology revealed a diagnosis of solid-pseudopapillary tumour.
|
Introduction
|
|---|
Solid-pseudopapillary tumour (SPT) of the pancreas is a rare benign or low-grade malignant epithelial tumour occurring predominantly in women, accounting for 1–2% of exocrine pancreatic tumours at most institutions. It was first described by Frantz in 1959, and is also known as a solid and cystic tumour, solid and papillary epithelial neoplasm, papillary-cystic neoplasm, papillary cystic tumour and Frantz tumour. In 1996, The World Health Organization (WHO) gave a new name to this tumour as solid-pseudopapillary tumour of the pancreas [1–3]. Pancreatic anomalies are occasionally reported and association of partial agenesis of the dorsal pancreas has been reported once [4–8].
|
Case report
|
|---|
A 49-year-old woman presented with abdominal pain that radiated to the back for several months. Physical examination was unremarkable. She had non-insulin dependent diabetes mellitus diagnosed 12 months earlier. Laboratory investigations including bilirubin, aspartate transaminase, carcinoembryogenic antigen (CEA), alpha-fetoprotein (AFP), and serum glucose level were all normal. Carbohydrate antigen (CA) 19-9 was elevated at 70.42 U ml–1 (normal range 0–37 U ml–1).
Abdominal ultrasound (US) revealed an 8 cm x 6 cm mass containing peripheral calcification in the pancreatic head. The biliary tree was not dilated. Non contrast abdominal and pelvic CT confirmed a well defined 8 cm x 6 cm mass lesion in the pancreatic head, with linear peripheral calcification, containing central cystic-necrosis. The mass displaced the adjacent superior mesenteric vein minimally and infiltrated into the second part of duodenum (Figure 1a,b
). There was no normal cephalic pancreatic tissue separate from the mass and complete absence of dorsal pancreatic parenchyma anterior to the splenic vessels artery and vein was also noted (Figure 1c).
View larger version (101K):
[in this window]
[in a new window]
|
Figure 1. (a) Non-contrast enhanced CT scan shows a large mass (thin black arrow) in the head of the pancreas with characteristic solid and cystic components and peripherally calcification. (b) Contrast-enhanced CT scan shows contrast enhancement of solid component (open arrow) and peripheral calcification (thin white arrow). (c) Axial curved reformatted CT image shows the mass (open arrow) and splenic vein (arrowhead).
|
|
The patient was referred for surgical resection. At laparotomy, there was no body or tail of the pancreas present, and the 8 cm x 6 cm mass in the head of the pancreas appeared to have grown exophytically. Invasion into the second part of the duodenum, with adherence to the superior mesenteric and portal veins was observed. The patient underwent a pancreatoduodenectomy (Whipple's procedure). In the post-operative period she developed insulin-dependent diabetes. At post-operative follow-up her of CA 19-9 levels returned to normal.
On pathological examination; the tumour mass was greyish brown in colour and measured 6 cm x 6 cm x 8 cm in size macroscopically with a lobulated surface. The cut surface of the mass had solid, cystic, haemorrhagic and calcified areas. Microscopic examination revealed a solid, cystic and pseudopapillary growth pattern. Haemorrhage, calcification and hyalinization were seen. Tumour cells had small round nuclei with fine chromatin, inconspicuous nucleoli and moderate eosinophilic cytoplasm. The cystic areas were filled with erythrocytes and foamy histiocytes. Immunohistochemical examination revealed diffuse positivity for vimentin, and focal positivity for keratin (Figure 2), consistent with a SPT of the pancreas.
|
Discussion
|
|---|
SPT of the pancreas is a rare primary pancreatic neoplasm of unknown aetiology, occurring most commonly in young women. Its malignancy rate is low and has a good prognosis after surgical removal [1–3, 9, 10]. Grossly, these large tumours have solid, haemorrhagic, calcified and cystic areas. Histologically, they have, cystic and pseudopapillary components. Kosmahl et al [11] reported that the immunocytochemical phenotype of SPT of the pancreas were characterized by vimentin, neuron-specific enolase (NSE) and alpha-1 antitrypsine stain positivity in over 90% of cases, whereas cytokeratinstains, positive in most epithelial tumours are usually negative. Our patient had all the macroscopic features and microscopic components described previously.
Embryologically the pancreas grows dorsal and ventral buds originating from the endodermal lining of the duodenum. During the seventh gestational week, the ventral buds turn posteriorly and to the left, connecting with the dorsal bud. Each of the pancreatic buds grow into a pair of branching, arborized ductal systems. The neck, body, tail and the cephalic aspect of the head of the pancreas originate from the dorsal bud. The ventral bud becomes the inferior portion of the head and the uncinate process [5–7]. The pancreas divisum is the most common anomaly of the pancreas in autopsy series with prevalence as high as 5–10% [5–7]. Other common pancreatic anomalies include an annular pancreas and agenesis of either the ventral or the dorsal bud. Complete agenesis of the pancreas is extremely rare and not compatible with life [5–7]. The exact frequency of dorsal agenesis of the pancreas is not known, and has been reported only rarely.
Since most islets cells are located in the tail and body of the pancreas, agenesis of the dorsal pancreas results in significant loss of beta-cells which impaired insulin secreting capacity [8] and as in our patient may result in the development of diabetes.
A CT diagnosis of dorsal pancreatic agenesis can be made when the presence of normal pancreatic head and absence of pancreatic tissue (body and tail) ventral to the splenic vein are demonstrated [4, 5, 7]. The CT findings of SPT of the pancreas are: non-contrast images show a well encapsulated mass with varying solid and cystic components owing to haemorrhagic degeneration, and contrast enhanced images show enhancement of solid areas peripherally, with cystic areas more centrally located [1–3, 11]. The presence of calcification in this tumour is not uncommon [1–3], as in our case.
The distribution of SPT of the pancreas are within the head in 32%, and body and/or tail of the pancreas in 68% of cases [8].
The tumour markers (CEA, CA19-9) are almost always negative in SPT of the pancreas [1–3, 8, 9], although our patient had an elevated serum levels of CA19-9. The elevation of tumour markers may be helpful diagnostically and on follow-up.
The only case of previously reported SPT of pancreas associated with pancreatic dorsal agenesis was by Nakamura et al, and as they demonstrated a pancreatic accessory duct with endoscopic retrograde cholangiopancreatography, they suggested there was partial pancreatic dorsal agenesis [8]. In contrast, our CT findings were of a calcified part solid and cystic mass with complete pancreatic dorsal agenesis.
Received for publication August 10, 2004.
Revision received November 18, 2004.
Accepted for publication January 5, 2005.
|
References
|
|---|
- Coleman KM, Doherty MC, Bigler SA. Solid-pseudopapillary tumour of the pancreas. Radiographics 2003;23:1644–8.[Free Full Text]
- Klimstra DS, Wenig BM, Heffess CS. Solid-pseudopapillary tumour of the pancreas: a typically cystic carcinoma of low malignant potential. Semin Diagn Pathol 2000;17:66–80.[Medline]
- Pettinato G, Di Vizio D, Manivel C, Pambuccian SE, Somma P, Insabato L. Solid-pseudopapillary tumour of the pancreas: a neoplasm with distinct and highly characteristic cytological features. Diagn Cytopathol 2002;27:325–34.[CrossRef][Medline]
- Nonent M, Linard J, Leveque E, Larroche P, Bobeuf J, Senecail B. Dorsal pancreas agenesis: computed tomography appearance with three-dimensional volume rendering reconstruction. Surg Radiol Anat 2003;25:161–3.[CrossRef][Medline]
- Shah KK, DeRidder PH, Schwab RE, Alexander TJ. CT diagnosis of dorsal pancreas agenesis. J Comput Assist Tomogr 1987;11:170–1.[Medline]
- Macari M, Giovanniello G, Blair L, Krinsky G. Diagnosis of agenesis of the dorsal pancreas with MR pancreatography. AJR Am J Roentgenol 1998;170:144–6.[Free Full Text]
- Soler R, Rodríguez E, Comesana ML, Pombo F, Marini M. Agenesis of the dorsal pancreas with polysplenia syndrome: CT features. J Comput Assist Tomogr 1992;16:921–3.[Medline]
- Nakamura Y, Egami K, Maeda S, Hosone M, Onda M. Solid and papillary tumour of the pancreas complicating agenesis of the dorsal pancreas. J Hepatobiliary Pancreat Surg 2001;8:485–9.[CrossRef][Medline]
- Potrc S, Kavalar R, Horvat M, Gadzijev EM. Urgent Whipple resection for solid-pseudopapillary tumour of the pancreas. J Hepatobiliary Pancreat Surg 2003;10:386–9.[CrossRef][Medline]
- Mancini GJ, Dudrick PS, Grindstaff AD, Bell JL. Solid-pseudopapillary tumour of the pancreas. Two cases in male patients. Am Surg 2004;70:29–31.[Medline]
- Kosmahl M, Seada LS, Janig U, Harms D, Kloppel G. Solid-pseudopapillary tumour of the pancreas: its origin revisited. Virchows Arch 2000;436:473–80.[CrossRef][Medline]
Top
Abstract
Introduction
