A rare cause of obstructive jaundice

Clinical History

A 74-year-old male patient presented to our hospital with one month of jaundice, epigastric pain, fever and weight loss. Laboratory examinations revealed anaemia, elevated hepatic enzymes, elevated bilirubin profile that suggested obstructive jaundice and raised CA 19.9 levels.
He had a history of cholecystectomy for lithiasis 40 years before.

Imaging Findings

The patient presented to the hospital complaining of abdominal pain, fever, weight loss and an orange tint of his urine. Blood workup was consistent with obstructive jaundice and ultrasound examination revealed dilatation of the intrahepatic bile ducts and the common bile duct. He was diagnosed with cholangitis and an elective computed tomography (CT) was ordered.
The performed CT scan of the abdomen showed a circumferential, hypovascular and homogeneous wall thickening of the 2nd through the 4th portion of the duodenum of about 20 mm and mucosal folds effacement, suggesting infiltration by a neoplastic process. There were no signs of intestinal obstruction. This duodenal thickening caused biliary and pancreatic ducts dilatation. The circumferential mass did not invade other organs or vessels. Multiple periduodenal and para-aortic adenopathies were also noted.
The patient underwent an endoscopic ultrasound and biopsy samples were taken. The histological analysis reported B-cell lymphoma, although a definitive diagnosis was not reached.

Discussion

Extranodal lymphoma is classified as primary if involvement is confined to a single organ and immediately adjacent lymph nodes. Primary gastrointestinal (GI) lymphoma is an uncommon disease, accounting for approximately 20% of all extranodal lymphomas. The distal ileum is the most common site due to its greater amount of lymphoid tissue and just 0.8-2% of all primary GI lymphomas occur in the duodenum [1, 2]. Only 12% of all malignant duodenal neoplasms are duodenal lymphomas [3].
The recognized risk factors include immunodeficiency states, notably infection with human immunodeficiency virus, immunosuppression after solid organ transplantation, coeliac disease, inflammatory bowel disease and Helicobacter pylori infection [4].
The presenting symptoms are non-specific. Abdominal pain, anorexia, weight loss, nausea, vomiting and diarrhoea are the most frequent. Duodenal malignancies presenting with jaundice due to extrahepatic biliary obstruction occur in 43% of cases, but the exact prevalence of obstructive jaundice in duodenal lymphoma is unknown, although there are a few cases reported [5].
Unlike adenocarcinoma, which arises from the mucosa, lymphoma may originate in and extend along the submucosa or the deep mucosal layer, making the diagnosis by endoscopy sometimes difficult.
Computed tomography is easy to perform and allows a provisional diagnosis in patients without known disease. In patients with known disease, it provides important information for staging, evaluation of response to therapy, identification of recurrence or complications of therapy.
The CT appearance of duodenal lymphoma is variable: aneurysmal, constrictive, nodular and ulcerative infiltration. One of the most common presentations is a diffuse circumferential bulky mass in the duodenal wall, involving a relatively long segment with gradual tapering to a normal mucosa, often associated with regional adenopathies. The tumour may also ulcerate and perforate. Aneurysmal dilatation of the lumen may be seen and excludes other more common causes of duodenal wall neoplastic infiltration, namely adenocarcinoma. Obstruction and infiltration into adjacent structures is uncommon. An intraluminal, polypoid, homogeneous mass without wall thickening or lymphadenopathy has also been described [4, 6-8].
Regardless of the morphologic type, the lesions are usually homogeneous, iso or hypodense compared with the normal bowel wall, and hypovascular after contrast media administration [4, 6-8].
Although noninvasive imaging plays an important role, a definitive diagnosis always requires biopsy.
The modality of treatment depends on the stage of the disease, but many patients are treated with chemoradiation therapy alone. Bypass surgery is done in some cases to relieve the biliary and duodenal obstruction [4].

Differential Diagnosis List

Final Diagnosis

Duodenal lymphoma

URL:	https://www.eurorad.org/case/12773
DOI:	10.1594/EURORAD/CASE.12773
ISSN:	1563-4086