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Learning Modules ❯ Gastrointestinal

Calcified Pancreatic and Peripancreatic Masses: Common to Uncommon Pathologies

Calcified Pancreatic and Peripancreatic Masses: Common to Uncommon Pathologies

Franco Verde, MD; Elliot K Fishman, MD

 

Disclosures

  • FV: None
  • EKF: Research support, Siemens AG Advisory Board, Siemens AG Research support, GE Company Advisory Board, HipGraphics, Inc co-founder

 

Introduction

  • Pancreatic calcifications are frequently related to chronic pancreatitis and is usually not a diagnostic dilemma
  • Beyond chronic pancreatitis, calcifications can be present in range of pancreatic lesions
  • We will review the appearances of common to uncommon masses that could contain calcifications

 

Outline

  • Pancreatic masses
    • Common
      • Ductal adenocarcinoma
    • Uncommon
      • Neuroendocrine tumor
      • Serous cystadenoma
      • Solid pseudopapillary epithelial neoplasm (SPEN)
      • Intraductal papillary mucinous tumor (IPMN)
      • Colloid carcinoma arising from IPMN
      • Mucinous cystic neoplasm
      • Lymphoepithelial cyst
  • Peripancreatic masses
    • Duodenal adenocarcinoma
    • Pseudocyst
    • Desmoplastic small cell
  • Pitfall
    • Chronic pancreatitis

 

Protocol Optimization

Recommended 64 MDCT protocol
  • Arterial (bolus triggered) and venous phases (60 second delay) at 4-5 mL/s
  • 120 kVp
  • 250 mAs
  • 0.8 pitch
  • 64 x 0.6 mm collimation
  • 0.75 mm axial and 3 mm axial, sagittal, and coronal reconstructions
  • 1000 mL water PO

 

Common pancreatic masses that may calcify: Ductal adenocarcinoma

  • Twelfth most commonest cancer, 4th leading cancer killer:
    • 48,960 new US cases (12.4:100k, 3% of all cancer cases) with 40,560 deaths (6.9%) in 2015 (SEER database)
  • 2005-2011 SEER: Mean 7.2% 5 year survival for all stages, 27% for localized disease, 2.4% for metastatic disease
  • 1.3:1::M:F, almost twice as common in blacks than whites, rare before 45 years. Numerous syndromes associated with increased cancer risk, usually tumor is sporadic
  • 85% of all pancreatic neoplasms
  • Imaging:
    • Up to 70% localized to pancreatic head, 25% body/tail
    • Solid, hypovascular, locally invasive mass with duct obstruction
    • 100% sensitive for tumor >2cm
    • Best seen on arterial phase, can be isodense on venous when small
    • Carcinomatosis can be subtle and difficult with CT having low sensitivity
    • CT must be performed within 25 days of planned surgery to maintain accuracy
    • Calcification is not a common feature

 

49 year old woman with pancreatic mass.
IV contrast enhanced CT with axial arterial phase (A), axial venous (B), coronal arterial (C) and coronal thick-slab MIP venous (D) images.
Obstructing pancreatic mass seen with ill-defined hypoattenuation and coarse calcification (white arrows). Peripancreatic necrotic adenopathy is present (gold arrow). A common bile duct stent is in place (arrow head).
ERCP brushing revealed adenocarcinoma of pancreatic primary.

49 year old woman with pancreatic mass

 

Uncommon masses that may calcify: Neuroendocrine tumors

  • Rare: <1:100,000 per year (US); 1-2% of all pancreatic tumors, 5-7th decade
  • Mostly sporadic or associated with MEN1 (80-100% lifetime risk) , VHL (20%), NF1 (10%) or TS (1%)
  • 50-75% “non-functional” (although still secrete substances but no hormonal syndrome). Insulinoma and gastrinoma are most common NETs to cause hormonal syndromes.
  • Imaging:
    • Dual phase is critical as smaller lesions may be isodense on venous phase CT >80% sensitive.
    • Appear as round, hypervascular masses
    • Smaller tumors are usually homogeneous whereas larger tumors can be heterogeneous with central necrosis
    • Duct obstruction is not typical unless large. Small serotonin secreting tumors may cause focal fibrosis causing duct obstruction
    • Larger tumors can have vascular invasion (up to a third in some series).
    • Calcifications are uncommon, suggesting malignant pNET, can be peripheral or central
    • Extra-pancreatic findings include vascular liver metastases, peri-portal adenopathy, and vascular invasion

 

58 year old man with pancreatic mass.
IV contrast enhanced CT in axial arterial (A) and portal venous phases (B and C) demonstrate a cluster of coarse calcifications in the pancreatic tail (white arrows) with adenopathy (short white arrows) and hepatic metastatic disease (black arrows).
Coronal thick slab MIP (D) well depict the calcifications throughout the mass and punctate calcifications in the metastatic gastrohepatic and peripancreatic adenopathy (arrow heads).
Liver biopsy revealed a well-differentiated neuroendocrine tumor. Patient was not a surgical candidate and received chemotherapy.

Uncommon masses that may calcify: Neuroendocrine tumors

 

67 year old woman with pancreatic mass.
IV contrast enhanced CT in axial and coronal arterial (A &C) and portal venous phases (B) demonstrate a 6 cm heterogenous peripherally enhancing mass with a punctate calcification (arrow).
Coronal thick slab MIP (D) demonstrates the vascularity of the mass with punctate calcification (arrow).
FNA demonstrated a well-differentiated neuroendocrine tumor. Patient was not a surgical candidate and received chemoradiation.

Uncommon masses that may calcify: Neuroendocrine tumors

 

Uncommon masses that may calcify: Serous cystadenoma

  • Frequently incidental cystic tumors found in woman in the 5th to 7th decades.
  • Tumors >4 cm are more likely to be symptomatic
  • Three morphologic types: polycystic (70%), honeycomb (20%) or oligocystic (10%)
  • Imaging
    • Polycystic: cysts are <2cm; oligocystic cysts are > 2cm
    • Usually in head, 40% in tail
    • Fibrous enhancing septations
    • Central scar with coarse calcification (30%)
    • Does not communicate with duct. Can obstruct duct when large
    • Atypical findings: giant >10 cm, intratumoral hemorrhage, solid appearance (pNET mimic), unilocular with calcification (pseudocyst or mucinous mimic)

 

71 year old woman with symptomatic pancreatic mass.
IV contrast enhanced CT in axial arterial (A) and portal venous phases (B) demonstrate a 8.6 x 6 cm hypovascular mass with microcystic appearance and prominent rim vascularity. Central cluster of coarse calcifications are present (arrows).
Patient was a surgical candidate and underwent Whipple procedure demonstrating a serous cystadenoma without infiltrating carcinoma.

Uncommon masses that may calcify: Serous cystadenoma

 

Uncommon masses that may calcify: Solid pseudopapillary epithelial neoplasm (SPEN)

  • 1-2% of exocrine pancreatic tumors
  • Much more common in woman (9.5:1), range 15-35 (mean 24) years
  • 95-100% 5 year survival after resection
  • Low percentage are malignant
  • Imaging
    • Encapsulated solid and cystic mass
    • Usually in tail
    • Peripheral enhancing solid components
    • 30% contain calcification, usually peripheral
    • Can spontaneously hemorrhage
    • Duct obstruction is rare
    • Can have hepatic metastases, >5 cm associated with increased risk
    • Capsule discontinuity has been described in malignant SPENs

 

30 year old woman with pancreatic mass.
IV contrast enhanced CT in axial arterial (A) and venous (B) phases with coronal thick slab MIP (C) and 3D rendering (D) demonstrate a large pancreatic mass with rim interrupted calcifications (arrows). Mass demonstrates soft tissue and cystic components.
MIP imaging fully depicts all the calcification in one image (black arrows).
Mass was resected and revealed a solid pseudopapillary epithelial neoplasm.

Uncommon masses that may calcify: Solid pseudopapillary epithelial neoplasm (SPEN)

 

Uncommon masses that may calcify: Intraductal papillary mucinous tumor

  • 1-3% of exocrine pancreatic neoplasms, 20-50% of pancreatic cystic neoplasms
  • Older patients, 0.7 to 1.8 M:F, 5th to 7th decade
  • Histological spectrum from low grade, moderate, high grade dysplasia and invasive carcinoma
    • Branch duct (BD), main duct or mixed
    • 5 different subtypes; gastric type more common for branch duct type
    • Branch duct lower risk of malignant transformation than main duct type
  • Imaging (branch-duct type):
    • Usually incidentally seen
    • Usually well demarcated small simple cystic mass
    • Can be multiple
    • 60% occur in head/uncinate process
    • Up to 20% can contain calcification: punctate, coarse, or eggshell, more frequently seen in larger lesions
    • Presence of calcification is not associated with malignancy
    • > 3cm, solid component, MPD/CBD dilation, and adenopathy suggest malignancy

 

68 year old man with pancreatic mass.
IV contrast enhanced CT in axial arterial phase (A) and axial, coronal, and coronal 3D portal venous phase (B, C, and D) demonstrates 1.5 x 2 cm pancreatic head cystic mass with eccentric cluster of coarse calcifications (arrow). Additional punctate calcification seen cranial to larger cluster (figure A arrow).
EUS/FNA was performed demonstrating mucinous features and communication with pancreatic duct, consistent with an intraductal papillary mucinous tumor.

Uncommon masses that may calcify: Intraductal papillary mucinous tumor

 

Uncommon masses that may calcify: Colloid carcinoma arising from IPMN

  • IPMNs that progress to invasive carcinoma can be either tubular (similar to ductal adenocarcinoma) or colloid type (resembles breast and skin cancer)
    • Typically main duct type more than branch duct type
  • Risk of developing carcinoma varies with the histologic subtype of IPMN
  • Colloid carcinoma develops in 30 to 50 percent of patients with intestinal-type IPMN
  • Colloid have better prognosis than tubular
  • Imaging:
    • Solid enhancing components arising in a dilated main duct (main duct type IPMN) or large peripheral cystic mass (branch duct type)
    • Coarse or fine calcifications possible

 

69 year old man with pancreatic mass.
IV contrast enhanced CT in axial arterial phase (A) and in axial, coronal, and MIP portal venous phase (B, C, and D) demonstrates massive dilation and sacculation of the main duct with extensive mural nodularity (arrows heads) and areas of coarse calcification (arrows).
Partially imaged pancreatic head is enlarged with acute pancreatitis (not labelled).
Patient underwent distal pancreatectomy revealing colloid carcinoma arising from IPMN. Nodal and omental metastases were present (not shown).

Uncommon masses that may calcify: Colloid carcinoma arising from IPMN

 

Uncommon masses that may calcify: Mucinous cystic neoplasm (MCN)

  • Uncommon tumor. Predominately woman (>80%) with mean age 54
  • Can be found incidentally on imaging (20% of cases) or present with abdominal pain, recurrent pancreatitis, gastric outlet obstruction, and/or a palpable mass.
    • Jaundice and/or weight loss are more common with malignant lesions.
  • EUS-FNA can obtain fluid for cyst analysis
    • Cytology: Mucin-containing cells
    • CEA: High concentrations of CEA (no correlation with malignancy)
  • MCNs have malignant potential. Curative if resected.
  • Imaging
    • Round or ovoid
    • Homogenous to heterogeneous cyst contents
    • Usually septated but can be unilocular
    • Eccentric calcifications in 15% of patients
    • Malignant features: >5cm, thickened cyst wall, internal solid components
    • DDx: unilocular serous tumor, cystic neuroendocrine tumor, pseudocyst

 

48 year old woman with pancreatic mass.
IV contrast enhanced CT in axial arterial phase (A) axial venous (B), coronal arterial (C), and coronal venous (D) phases.
Well demarcated, 3 cm, partially exophytic, cystic mass, arising from the pancreatic body. No internal septation or debris present. Two punctate calcifications are present (arrows). No communication with duct was noted.
Patient underwent EUS-FNA revealing very high CEA levels, consistent with a mucinous cystic neoplasm.

Uncommon masses that may calcify: Mucinous cystic neoplasm (MCN)

 

Uncommon masses that may calcify: Lymphoepithelial Cyst

  • Part of 3 types of morphologically similar “squamous” cysts
    • lymphoepithelial cysts
    • dermoid cysts (monodermal teratomas)
    • epidermoid cysts in intrapancreatic accessory spleen. 
  • Predominantly older men, 4:1::M:F, mean age 56 (35-74 years). 
  • Characterized microscopically by stratified squamous epithelium surrounded by a band of mature lymphoid tissue with intervening well-formed germinal centers
  • Imaging
    • Any part of the pancreas (head, body, or tail).
    • Well-delineated cysts that may be multilocular (60%) or unilocular (40%)
    • Variable size: 1 to 17 cm
    • Peripheral calcifications may be present

 

66 year old man with peripancreatic mass.
IV contrast enhanced CT with selected axial arterial (A), axial venous (B), coronal arterial (C) and coronal thick-slab MIP arterial (D) images.
Large round cystic mass arising near the tail of the pancreas, measuring 14 x 12 x 15 with mass displacement of the stomach. Note punctate and coarse calcifications along the wall (arrows).
Resection demonstrated lymphoepithelial cyst without evidence of carcinoma.

Uncommon masses that may calcify: Lymphoepithelial Cyst

 

Peripancreatic masses that can calcify: Duodenal adenocarcinoma

  • Overall rare tumor depending on series 1-3 to 14.8 cases per 100,000
  • Adenocarcinoma most common subtype affecting the duodenum
  • Associated with HNPCC, Peutz-Jeghers and Familial Polyposis syndromes. Crohn’s disease increases risk of small bowel adenocarcinoma.
  • Patients commonly present with abdominal pain then weight loss, nausea, vomiting, GI bleeding, obstruction, and rarely perforation
  • Imaging
    • Usually near the ampulla
    • Annular narrowing with abrupt concentric or irregular edges
    • Ulceration
    • Obstruction
    • Mucinous subtypes can calcify

 

65 year old man with peripancreatic mass.
IV contrast enhanced CT with selected axial arterial (A) and portal venous phases axial (B), coronal (C) and coronal 3D (D) images.
Cluster of calcifications noted near the uncinate process abutting third portion duodenum (white arrows). Subtle thickening of the 2nd portion duodenum near the ampulla also seen, best depicted on 3D image (gold arrow).
Histology revealed a 4.1 cm mucinous adenocarcinoma with pancreatic invasion and regional metastatic adenopathy (not shown).

Peripancreatic masses that can calcify: Duodenal adenocarcinoma

 

Peripancreatic masses that can calcify: Pancreatic Pseudocyst

  • Develop in 10% of chronic pancreatitis, after 4 weeks and without necrotic material
  • Adjacent structures (stomach, omentum, transverse mesocolon) form the walls of the pseudocyst
  • Imaging
    • Small to large
    • Intra- or extra-pancreatic
    • Expansion can cause duodenal or biliary obstruction, vascular occlusion, fistula formation
    • Can get infected
    • Can cause vascular comprise by pressure erosion into adjacent vessels (splenic or gastroduodenal arteries common)
    • Uncommonly pseudocysts can calcify, punctate to eggshell

 

42 year old woman with peripancreatic mass.
IV contrast enhanced CT in axial arterial phase (A) demonstrates a 4cm finely rim calcified mass with central hypoattenuation (white arrow). Available prior from 7 years ago (B) demonstrates acute interstitial edematous pancreatitis with pancreatic head enlargement (black arrow) and extensive acute pancreatic fluid (yellow arrows). Current findings are consistent with a peripancreatic pseudocyst. No intervention was performed.

Peripancreatic masses that can calcify: Pancreatic Pseudocyst

 

Peripancreatic masses that can calcify: Desmoplastic Small Round Cell Tumor (DSRCT)

  • DSRCT is a soft tissue sarcoma that can occur throughout the body, primarily occurring in the abdomen or pelvis
  • DSRCT of the abdomen is a result of translocation of Ewing sarcoma gene (EWSR1) and Wilms tumor gene (WT1): t(11;22)(p13;q12) EWSR1-WT1
  • Usually occurs in young men (5:1::M:F), mean age 20 years, range 15-35
  • Patients usually present with mass effect: abdominal distension, palpable mass, pain, obstruction, ascites, weight loss
  • Prognosis is very poor, despite aggressive chemotherapy and debulking
  • Imaging
    • Large mass. Variable enhancement depending on necrosis
    • Small peritoneal implants usually present
    • Hypovascular hepatic metastases can be present
    • Tumors can surround or directly infiltrate organs
    • Calcifications frequently present

 

22 year old man with history of metastatic desmoplastic small cell tumor.
IV contrast enhanced CT with selected axial arterial (A) and portal venous phases axial (B), coronal (C) and coronal MIP (D) images.
Evidence of extensive metastatic disease is seen on all images as numerous cystic and hypodense masses throughout the liver (*). A large peripancreatic heterogeneous hypodense mass is seen with central scattered coarse calcifications (arrows). Note distal pancreatic parenchymal atrophy and duct dilation related to mass compression (gold arrow).

Peripancreatic masses that can calcify: Desmoplastic Small Round Cell Tumor (DSRCT)

 

Peripancreatic masses that can calcify: Pitfall - Chronic pancreatitis

  • Chronic pancreatitis is usually related to alcohol abuse, ductal obstruction (stones, pseudocysts, tumor), systemic disease (SLE, hypertriglyceridemia), autoimmune and idiopathic pancreatitis
    • Repetitive acute pancreatitis can lead to chronic pancreatitis
  • Distinguished from acute pancreatitis: not painful, progressive parenchymal fibrosis, pancreatic insufficiency, mononuclear infiltrate vs acute pancreatitis neutrophilic infiltrate
  • Imaging
    • Parenchymal atrophy
    • Mild main duct dilation. Prominence of side branches (“acinar filling”)
    • Parenchymal punctate calcification and intraductal stones
    • Focal inflammation can mimic ductal adenocarcinoma requiring biopsy for definitive management

 

64 year old man with abdominal pain
IV contrast enhanced CT with selected axial arterial (A,B,C) and venous oblique coronal (D) images.
Evidence of chronic pancreatitis seen with parenchymal atrophy, punctate parenchymal calcifications (short arrows) and 8 mm duct dilation. Intraductal stone present (gold arrow). Additional ill-defined low attenuation anterior to pancreatic neck and head (long arrows) concerning for adenocarcinoma. EUS-FNA performed demonstrating only inflammation.

Peripancreatic masses that can calcify: Pitfall - Chronic pancreatitis

 

Summary

  • Numerous etiologies exist for calcifications within pancreatic and peri-pancreatic lesion
  • Knowledge of lesions that can calcify can help tailor a well constructed differential diagnosis

 

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