Duodenal Carcinoma

Published on 19/07/2015 by admin

Filed under Radiology

Last modified 22/04/2025

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 With periampullary tumors

• Liver ± peritoneal metastases

TOP DIFFERENTIAL DIAGNOSES

• Pancreatic ductal carcinoma
• Ampullary carcinoma
• Intestinal metastases and lymphoma
• GI stromal tumor (GIST)
• Duodenal ulcer
• Crohn disease
• Tuberculosis
• Annular pancreas

PATHOLOGY

• Risk factors

image Familial polyposis syndromes (especially Gardner)
image Crohn disease
image Cigarette smoking and alcohol abuse

CLINICAL ISSUES

• Other signs/symptoms

image Nausea and vomiting, weight loss, anemia, upper GI bleed
image Periampullary tumors may present with jaundice
• Rare: Represents < 1% of all gastrointestinal neoplasms

DIAGNOSTIC CHECKLIST

• Most duodenal carcinomas cause focal stenoses or obstruction
• A large mass with cavitation is more likely to be lymphoma or GIST
image
(Left) Axial CECT in a 60-year-old man with weight loss and early satiety shows obvious liver metastases image.

image
(Right) Axial CECT in the same patient also shows paraduodenal lymph node metastases image.
image
(Left) Axial CECT in the same patient shows the relatively subtle mass that narrows the 3rd portion of the duodenum image. There is also a subtle extension of tumor along the superior mesenteric vessels image.

image
(Right) Film from an upper GI series in the same patient shows the duodenal carcinoma image more clearly. Note the “shoulder” or abrupt transition to tumor at its proximal extent. The lumen of the more proximal duodenum is dilated.

TERMINOLOGY

Abbreviations

• Duodenal carcinoma (CA)

Synonyms

• Duodenal adenocarcinoma

Definitions

• Primary malignant neoplasm arising in duodenal mucosa

IMAGING

General Features

• Best diagnostic clue

image Irregular intraluminal mass or “apple core” lesion at or distal to ampulla of Vater
• Location

image 15% in 1st portion of duodenum
image 40% in 2nd portion of duodenum
image 45% in distal duodenum
• Size

image Usually < 8 cm
• Morphology

image Polypoid, ulcerated, or annular constricting mass
image Intraluminal mass with numerous frond-like projections for carcinomas arising in villous tumors

Radiographic Findings

• Radiography

image Proximal obstruction pattern if lumen severely narrowed

Fluoroscopic Findings

• May have various appearances

image Ulcerated mass
image Polypoid mass
image Annular constricting “apple core” lesion
image “Soap bubble” reticulated pattern for villous tumors

CT Findings

• CECT

image Discrete mass or irregular thickening of duodenal wall
image Concentric narrowing of duodenal lumen
image Polypoid intraluminal mass
image Local lymphadenopathy
image Infiltration of adjacent fat
image Biliary ± pancreatic duct dilatation

– With periampullary tumors
image Liver ± peritoneal metastases

MR Findings

• MRCP

image May see pancreatic or biliary ductal dilatation with periampullary duodenal carcinomas

Ultrasonographic Findings

• Grayscale ultrasound

image Hypoechoic mass in duodenum with echogenic center: Pseudokidney sign
• Color Doppler

image May see invasion of adjacent vascular structures

Imaging Recommendations

• Best imaging tool

image Thin-section CECT with water for luminal distention and dual-phase arterial and venous imaging
• Protocol advice

image Multiplanar MIP and volume-rendered CT images

DIFFERENTIAL DIAGNOSIS

Neoplasms

• Ampullary and periampullary adenocarcinomas

image Pancreatic ductal carcinoma

– Hypodense mass centered in pancreas with ductal obstruction
image Ampullary carcinoma
image Cholangiocarcinoma

– Biliary obstruction with small mass
• Intestinal metastases and lymphoma

image Contiguous spread from pancreatic, colon, kidney, or gallbladder carcinoma
image Hematogenous metastases from melanoma, Kaposi sarcoma, lung or breast cancer
image Periduodenal lymph node metastases from other malignancies
• Other duodenal primary neoplasms

image Duodenal lymphoma

– Bulky, usually does not obstruct
image Malignant GI stromal tumor

– Hypervascular mass, mostly exophytic
image Duodenal carcinoid

Inflammatory

• Duodenal ulcer

image Spasm may narrow lumen
• Zollinger-Ellison syndrome

image Multiple post-bulbar ulcers, thickened folds, hypersecretion
• Crohn disease

image Usually with other sites of involvement in distal bowel

Infectious

• Tuberculosis

image May be indistinguishable from cancer on imaging

Congenital

• Annular pancreas

image CT findings are diagnostic (pancreatic tissue encircling 2nd duodenum)
• Enteric duplication cyst

image Encapsulated fluid collection; not soft tissue mass

Trauma

• Gastroduodenal trauma

image Duodenal hematoma is more common in children
image Spiked folds and luminal narrowing

PATHOLOGY

General Features

• Etiology

image Adenoma-carcinoma sequence

– Adenomatous polyps are most important risk factor
image Risk factors

– Familial polyposis syndromes (especially Gardner)
– Crohn disease
– Cigarette smoking
– Alcoholism
• Genetics

image Alterations in oncogenes ERB B2, KRAS, CCND1, and p53
• Adenocarcinomas represent 73-90% of malignant duodenal tumors
• Small bowel adenocarcinomas are rare, especially in relation to length of small bowel

image 45% of small bowel adenocarcinomas arise in duodenum
image 25% of all malignant small bowel tumors occur in duodenum

Staging, Grading, & Classification

• American Joint Committee on Cancer (AJCC) TNM staging system

image Primary tumor (T)

– T1: Tumor invades lamina propria or submucosa
– T2: Tumor invades muscularis propria
– T3: Tumor invades through muscularis propria and ≤ 2 cm into adjacent tissues
– T4: Tumor perforates visceral peritoneum, directly invades other organs, or extends > 2 cm into adjacent tissues
image Regional lymph nodes (N)

– N0: No regional nodes involved
– N1: Regional lymph node metastasis
image Distant metastasis (M)

– M0: No distant metastasis
– M1: Distant metastasis
image Staging

– Stage I: T1 or T2, N0, M0
– Stage II: T3 or T4, N0, M0
– Stage III: Any T, N1, M0
– Stage IV: Any T, any N, M1

Gross Pathologic & Surgical Features

• Duodenal mass may be flat, stenosing, ulcerative, infiltrating, or polypoid in growth pattern
• Secondary cancers far more common than primary cancers in proximal small bowel

image Often difficult to distinguish primary duodenal CA from secondary GI adenocarcinoma even with special stains
• Proximal small bowel adenocarcinoma may be marker for familial or multicentric cancer syndrome

Microscopic Features

• Similar histology to other GI adenocarcinomas

image Cellular and nuclear pleomorphism
image Dysplasia
image Gland-in-gland appearance
image Invasion into adjacent normal tissues
• Most duodenal carcinomas are moderately differentiated with variable mucin production
• 20% of duodenal carcinomas are poorly differentiated

CLINICAL ISSUES

Presentation

• Most common signs/symptoms

image Upper abdominal pain secondary to obstruction
image Other signs/symptoms

– Nausea and vomiting, weight loss, anemia, upper GI bleed
– Periampullary tumors may present with jaundice
• Clinical profile

image Increased incidence of duodenal CA in familial polyposis syndromes

– Peutz-Jeghers syndrome, Gardner syndrome

Demographics

• Age

image 7th decade

– Median age: 60 years
image Low incidence in patients under 30
• Gender

image Slight male predominance
• Epidemiology

image Rare: Represents < 1% of all gastrointestinal neoplasms
image Incidence rises with age

Natural History & Prognosis

• Spreads by direct extension to adjacent organs and through serosa to peritoneal cavity
• Metastasizes hematogenously to liver, lungs, and bone
• Metastasizes via lymphatics to regional nodes
• 22-71% of patients have positive nodes at presentation
• Prognosis depends on resectability, lymph node involvement, and somewhat on histologic grade
• Vascular invasion makes lesion unresectable

Treatment

• Options, risks, complications

image Surgery for resectable lesions

– Pancreaticoduodenectomy for 1st and 2nd portion of duodenum lesions
– Segmental duodenectomy and primary reanastomosis for 3rd and 4th portions of duodenum lesions
image Unresectable tumors: Palliation with radiation, chemotherapy, stenting

DIAGNOSTIC CHECKLIST

Consider

• Check for vascular invasion, especially for lesions of 2nd and 3rd duodenum
• Look for regional lymph nodes and liver metastases

Image Interpretation Pearls

• Most duodenal carcinomas cause focal stenoses or obstruction; large mass with cavitation is more likely lymphoma or GIST
• Scrutinize duodenum when periduodenal lymphadenopathy is present on CT without obvious source

image
(Left) Axial CECT in a 49-year-old man with abdominal pain and abnormal liver function shows dilation of the intrahepatic bile ducts image.
image
(Right) Axial CECT in the same patient shows that the pancreatic duct image is also dilated. The findings, to this point, suggest a malignant obstructing neoplasm at or near the ampulla of Vater.
image
(Left) Axial CECT in the same patient shows that the pancreatic head image is normal, while the distal common bile duct image remains dilated.

image
(Right) Axial CECT in the same patient shows a large mass image present within the lumen of the 2nd and 3rd portions of the duodenum that obstructed the ducts at the site of a relatively low-lying ampulla. Endoscopy confirmed a duodenal carcinoma arising in a villous adenoma.
image
(Left) Axial NECT in an 88-year-old man with early satiety and weight loss showed gastric distention (not seen on this section). The lumen of the 2nd portion of duodenum is abruptly and eccentrically narrowed image. There is extensive tumor infiltration of the adjacent fat planes image and regional lymphadenopathy image.

image
(Right) Film from an upper GI series in the same patient confirms high-grade obstruction of the 2nd duodenum by a scirrhous mass image, the primary duodenal carcinoma.
image
Axial CECT shows an irregular mass distorting the duodenal lumen and extending into adjacent fat medially image.

image
Axial CECT shows an irregular low-attenuation mass in the 2nd portion of the duodenum. Note the central low-density lumen image, which shows the mass to be arising within the duodenum rather than the pancreas.
image
Double-contrast upper GI series shows an “apple core” lesion of the 2nd portion of the duodenum image, representing duodenal carcinoma. (Courtesy H. Harvin, MD.)
image
Coronal CECT thin-slab-average image shows a low-attenuation annular constricting mass in the transverse duodenum image.
image
Axial CECT shows bulky duodenal carcinoma in the 2nd portion of duodenum. This patient has a gastrojejunostomy image and biliary stent image for palliation of obstruction.
image
Axial CECT shows duodenal wall thickening image, a low-density lymph node image, and liver metastasis image in a patient with duodenal carcinoma.
image
Single-contrast upper GI series shows an ulcerated annular constricting mass in the descending duodenum image, which proved to be duodenal carcinoma. (Courtesy M. Nino-Murcia, MD.)
image
Axial CECT shows a soft tissue mass image along the medial border of the 2nd portion of the duodenum, which proved to be duodenal carcinoma. (Courtesy M. Nino-Murcia, MD.)

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