CASE 13671 Published on 22.06.2016

Partial agenesis of the pancreas with superimposed chronic pancreatitis

Section

Abdominal imaging

Case Type

Clinical Cases

Authors

Vella Adriana, MD; Minorati Davide, MD; Tonolini Massimo, MD.

"Luigi Sacco" University Hospital,
Radiology Department;
Via G.B. Grassi 74
20157 Milan, Italy;
Email:mtonolini@sirm.org

Patient

58 years, female

Categories

Area of Interest Pancreas ; Imaging Technique CT, MR, MR-Functional imaging

Procedure Diagnostic procedure ; Special Focus Congenital ;

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Clinical History

Woman with unremarkable past medical history and recent diagnosis of T2N+ breast carcinoma one year earlier, which was treated with neoadjuvant chemotherapy followed by quadrantectomy, radiotherapy and further chemotherapy.
Currently undergoing oncologic reassessment after treatment completion. Laboratory assays revealed mildly increased serum glucose, lipase (68 U/l) and gamma-glutamyl transpeptidase.

Imaging Findings

The patient underwent total-body CT (Fig. 1) which excluded distant metastases from her primary tumour. Incidentally, rounded truncation of the pancreas at the neck was noted, with absent pancreatic body and tail, dilated splenic vein. The pancreatic head contained several tiny calcifications suggesting chronic pancreatitis. The patient denied history of alcoholism, gallstones and previous episodes of acute abdominal pain. Serum bilirubin, amylase and tumour markers were within normal limits.
The presumptive diagnosis of congenital-developmental abnormality was further investigated with MRI (Fig. 2) two months later, which confirmed truncated pancreas with absent body and tail, inhomogeneous pancreatic head with two cystic lesions, the largest measuring 2 cm located in the uncinate process. MR-cholangiopancreatography visualized normal biliary tract and short Wirsung duct draining the pancreatic remnant into the Vaterian ampulla.
Imaging findings were consistent with dorsal pancreatic agenesis with superimposed chronic pancreatitis and impaired glucose tolerance. Imaging and laboratory follow-up was planned.

Discussion

The pancreatic gland develops from dorsal and ventral buds arising from the caudal region of the embryonic foregut. Alterations of the normal embryogenesis result in congenital anomalies or anatomic variants of the pancreatic gland and ductal system, which encompass altered course or configuration of the pancreatic duct, duplication anomalies, pancreas divisum (PD), annular pancreas, ectopic pancreas, accessory pancreatic lobe, pancreatic agenesis and hypoplasia. Most of these forms are more or less uncommon, except for PD which is encountered in up to 10% of the general population [1-4].
Most congenital pancreatic anomalies (CPAs) do not cause symptoms and biochemical changes, and therefore are generally undetected until adulthood and usually diagnosed as incidental findings during cross-sectional imaging studies performed for unrelated reasons. Alternatively, unspecific complaints such as recurrent abdominal pain, nausea and vomiting may be present. Radiologists need familiarity with CPAs to correctly classify them and avoid misinterpretation as other disorders. Furthermore, awareness of the presence of a CPA is warranted when planning endoscopic or surgical therapies, particularly to prevent inadvertent ductal injury [1-4].
While complete pancreatic agenesis is incompatible with life, partial agenesis (hypoplasia) results from absence of either ventral or dorsal pancreatic bud. Albeit very rare, dorsal pancreatic agenesis (DPA, “short pancreas”) is relatively more common than agenesis of the ventral pancreas, and may be isolated or part of heterotaxia syndromes. Generally asymptomatic, DPA may sometimes cause maldigestion, abdominal pain, acute or chronic pancreatitis. Serum amylase and lipase levels are usually within normal limits. Furthermore, patients with DPA have an increased risk of diabetes mellitus, since most islet cells are located in the distal pancreas [5-10].
Nowadays the diagnosis of DPA is generally made at multidetector CT or MRI. The typical appearance includes a short pancreas “truncated” at the level of the neck, with absent body and tail. MR- (MRCP) and endoscopic cholangiopancreatography (ERCP) allow depicting the ductal anatomy, and generally show absence of the Santorini and main pancreatic ducts, with a short ventral (Wirsung) duct draining the pancreatic head and uncinate process [1-4, 11-13].
The differential diagnosis of DPA mostly includes two entities: pancreatic head carcinoma with upstream ductal dilatation and atrophied gland; and fatty replacement of body and tail following chronic pancreatitis with spared pancreatic head: the latter is associated with a relevant clinical history and with abnormal enzymes increase [1-4, 11-13].

Differential Diagnosis List

Dorsal pancreatic agenesis with superimposed calcifications from chronic pancreatitis

Segmental pancreatic atrophy

Chronic pancreatitis

Pancreatic head carcinoma with upstream atrophic gland

Heterotaxy syndrome

Final Diagnosis

Dorsal pancreatic agenesis with superimposed calcifications from chronic pancreatitis

References

[1] Borghei P, Sokhandon F, Shirkhoda A, et al. (2013) Anomalies, anatomic variants, and sources of diagnostic pitfalls in pancreatic imaging. Radiology 266:28-36. (PMID: 23264525)

[2] Mortele KJ, Rocha TC, Streeter JL, et al. (2006) Multimodality imaging of pancreatic and biliary congenital anomalies. Radiographics 26:715-731 (PMID: 16702450)

[3] Turkvatan A, Erden A, Turkoglu MA, et al. (2013) Congenital variants and anomalies of the pancreas and pancreatic duct: imaging by magnetic resonance cholangiopancreaticography and multidetector computed tomography. Korean J Radiol 14:905-913 (PMID: 24265565)

[4] Yu J, Turner MA, Fulcher AS, et al. (2006) Congenital anomalies and normal variants of the pancreaticobiliary tract and the pancreas in adults: part 2, Pancreatic duct and pancreas. AJR Am J Roentgenol 187:1544-1553 (PMID: 17114549)

[5] Lang K, Lasson A, Muller MF, et al. (2012) Dorsal agenesis of the pancreas - a rare cause of abdominal pain and insulin-dependent diabetes. Acta Radiol 53:2-4. (PMID: 22139719)

[6] Oldenburg B, van Leeuwen MS, van Berge Henegouwen GP, et al. (1998) Pancreatitis and agenesis of the dorsal pancreas. Eur J Gastroenterol Hepatol 10:887-889 (PMID: 9831413)

[7] Pasaoglu L, Vural M, Hatipoglu HG, et al. (2008) Agenesis of the dorsal pancreas. World J Gastroenterol 14:2915-2916 (PMID: 18473421)

[8] Rakesh K, Choung OW, Reddy DN (2006) Agenesis of the dorsal pancreas (ADP) and pancreatitis - is there an association?. Indian J Gastroenterol 25:35-36 (PMID: 16567893)

[9] Schnedl WJ, Piswanger-Soelkner C, Wallner SJ, et al. (2009) Agenesis of the dorsal pancreas and associated diseases. Dig Dis Sci 54:481-487 (PMID: 18618254)

[10] Shimodaira M, Kumagai N, Sorimachi E, et al. (2012) Agenesis of the dorsal pancreas: a rare cause of diabetes. Intern Emerg Med 7:83-84 (PMID: 21573988)

[11] Macari M, Giovanniello G, Blair L, et al. (1998) Diagnosis of agenesis of the dorsal pancreas with MR pancreatography. AJR Am J Roentgenol 170:144-146.

[12] Shah KK, DeRidder PH, Schwab RE, et al. (1987) CT diagnosis of dorsal pancreas agenesis. J Comput Assist Tomogr 11:170-171 (PMID: 3805410)

[13] Zhou Y, Chen M, Liu Y (2014) Agenesis of dorsal pancreas confirmed by three-dimensional reconstruction CT. Int J Clin Exp Med 7:3110-3112 (PMID: 25356189)

Case information

URL:	https://www.eurorad.org/case/13671
DOI:	10.1594/EURORAD/CASE.13671
ISSN:	1563-4086

License

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Figure 1

Unenhanced and portal venous phase post-contrast multidetector CT

Unenhanced images (a, detail b) showed truncated pancreas (arrowheads) at transition between neck and body; pancreatic head containing tiny calcifications, a demarcated 2-cm hypodense ovoid lesion (arrow) at the uncinate process.

Unenhanced images (a, detail b) showed truncated pancreas (arrowheads) at transition between neck and body; pancreatic head containing tiny calcifications.

Corresponding portal venous phase enhanced images (c, detail d) confirmed rounded, truncated pancreas (arrowhead) at transition between neck and body; tiny pancreatic calcifications; a non-enhancing demarcated 2-cm ovoid lesion (arrow) at the uncinate process.

Coronal contrast-enhanced image confirmed pancreatic head with tiny pancreatic calcifications; a ductal structure (thin arrow), a non-enhancing demarcated 2-cm ovoid lesion (arrow) at the uncinate process. Note absent pancreatic body and tail, dilated splenic vein (*).

Cranial axial image confirmed absent pancreatic body and tail; patent dilated splenic vein (*).

Figure 2

Unenhanced MRI and MR-cholangiopancreatography (MRCP)

Axial T2-weighted image confirmed absent pancreatic body and tail in their normal anatomic location adjacent to the splenic vein (*).

Axial (b), coronal (c) T2- and axial T1-weighted (d) images confirmed inhomogeneous pancreatic head with two cystic lesions (arrows), the largest measuring 2 cm in the uncinate process.

Coronal (c) T2- and axial T1-weighted (d) images confirmed inhomogeneous pancreatic head with two cystic lesions (arrows), the largest measuring 2 cm in the uncinate process. Note absent pancreatic body and tail, splenic vein (*).

Axial T1-weighted image confirmed truncated pancreas (arrowhead) at transition between neck and body, absent pancreatic body and tail.

MRCP (e, f) confirmed two cystic pancreatic lesions (arrows), the largest in the uncinate process, and visualized a normal-calibre pancreatic duct (thin arrows) draining into the Vaterian ampulla. Normal biliary tract.