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| Discussion |
A 62-year-old man presented with pain in the abdomen radiating to the back and dyspepsia since one month. There was no history of any other major illness. On examination, there was a well defined palpable mass in the epigastrium associated with mild tenderness. Routine blood investigations were normal. Serum amylase was normal. Serum lipase level was increased (1531 IU/L). Serum levels of CA 19-9 (tumor marker for pancreatic carcinoma) were normal.
RADIOLOGICAL FINDINGS:
Ultrasonographic
findings:
There was a large, well-defined, hypoechoic mass (approximately
12x10x10 cm in size ) arising from the body of the pancreas.
CT
findings (Figs 1-5):
A soft tissue density mass was noted in the upper
abdomen - arising from the body of the pancreas and extending anteriorly and cranially-
causing encasement of the celiac artery and its branches and occupying most of
the lesser sac. The mass showed minimal homogenous enhancement after intravenous
contrast administration. There was loss of fat planes between the mass and the
stomach and the left lobe of the liver. There was no calcification seen in the
mass. Incidentally noted were a right renal cyst and a simple cyst in the right
lobe of the liver. A few calcified granulomas were also seen in spleen.
Fig.
1 | Fig.
2 | |
Fig.
3 | Fig.
4 | |
Fig.5 | ||
MR findings: (Figs 6-9) On T1W images, a well defined homogenously hypointense mass was seen in the upper abdomen, arising from the body of the pancreas. The lesion appeared hyperintense on T2W images .The mass was seen invading the anterior structures including the posterior wall of stomach and the left lobe of the liver. The pancreatic duct was not dilated. The tail of the pancreas was normal. The mass showed encasement of vessels including the celiac artery and its branches and the splenic vein. The liver and spleen were of normal size.
Fig.6 | Fig.7 | ||
Fig.8 | Fig.
9 | ||
USG
guided biopsy:
An
ultrasound guided Tru-Cut biopsy was performed under local anesthesia. .Histopathological
evaluation revealed sheets of small round cells of apparent lymphoid origin. The
cells showed hyperchromatic nuclei and scanty cytoplasm. These features were suggestive
of low grade malignant lymphoma (NHL).
FINAL DIAGNOSIS: Pancreatic lymphoma
DISCUSSION:
In lymphoma, primary involvement
of the pancreas is uncommon, representing 2% to 5% of cases of extranodal lymphomas,
comprising less than 0.5% of pancreatic tumours. Many of these cases occur in
patients who are immunocompromised hosts - particularly patients infected with
HIV.
Lymphoma, predominantly the non Hodgkin B cell subtype, involves the pancreas secondarily in approximately 30% of patients with widespread disease. It usually spreads to the pancreas by direct extension from peripancreatic lymphadenopathy.
To distinguish PPL (primary pancreatic lymphoma) from secondary involvement of the pancreas by non-Hodgkin's lymphoma, Behrns' clinical and diagnostic criteria of PPL include:
1.
Mass predominantly within the pancreas with grossly involved lymph nodes confined
to the peripancreatic region
2. No palpable superficial lymphadenopathy
3.
No hepatic or Splenic involvement
4. No mediastinal nodal enlargement on chest
radiograph
5. Normal white cell count.
AGE: The disease mainly affects middle age and elderly patients
SEX: Male to female ratio is around 1.4: 1
PRESENTING SYMPTOMS are non-specific, typically including abdominal pain, weight loss, nausea and vomiting, but also jaundice, acute pancreatitis, and small bowel obstruction.
RADIOLOGICAL FINDINGS:
USG
Findings:
Sonography reveals a homogeneous, sonolucent, or complex mass.
These masses are usually echo-poor and may mimic cystic lesions. Transabdominal
sonography allows the detection of enlarged peripancreatic and periaortic lymph
nodes and dilatation of the common bile and pancreatic ducts. Doppler waveform
scanning, provide information about the patency of the major peripancreatic vessels,
the celiac and superior mesenteric arteries, and the portal, superior mesenteric,
and splenic veins.
CT
Findings:
CT is the most common imaging technique used in the detection
and characterization of primary pancreatic lymphoma.
Pancreatic
lymphomas generally appear as homogeneous soft tissue mass, showing little enhancement
after intravenous contrast administration. Intrinsic involvement of the pancreas
may be difficult to differentiate from lymphoma affecting the peripancreatic lymph
nodes.
Two distinct CT patterns have been described, including focal and circumscribed
single or multiple masses and diffuse enlargement of the gland by an infiltrating
tumor. The latter appearance can be associated with involvement of the peripancreatic
fat and mimic acute pancreatitis on CT.
Encasement of the peripancreatic vessels
may occur, but dilatation of the pancreatic duct is uncommon, despite the presence
of bulky tumor, a helpful distinguishing feature from adenocarcinoma.
The presence
of associated lymphadenopathy below the level of the renal veins also favors the
diagnosis of lymphoma.
MR
Imaging :
Two different morphologic patterns of pancreatic involvement
are seen on MR imaging that are similar to the CT appearance.
The
well-circumscribed tumoral type appears as a low-signal-intensity homogeneous
mass within the pancreas on T1-weighted images with subtle enhancement after IV
administration of gadolinium-containing contrast medium. On T2-weighted images,
a tumoral mass shows a more heterogeneous character with a low to intermediate
signal amplitude slightly higher than that of the residual gland but much lower
than the signal intensity of fluid.
The diffuse infiltrating type of pancreatic
involvement shows similar characteristics of low signal intensity on unenhanced
T1- and T2-weighted images, with mild to moderate enhancement after gadolinium
injection. In the diffuse infiltrating type, enhancement is predominately homogeneous
but may include small foci of little or no gadolinium uptake. Bile and pancreatic
ductal dilatation can be easily assessed with MR imaging using MR cholangiopancreatography
.
ERCP and
Percutaneous Transhepatic Choledochography
Unlike pancreatic adenocarcinoma,
moderate to severe dilatation of Wirsung's duct is apparently rare in pancreatic
lymphoma because Wirsung's duct is either normal, displaced or simply narrowed
in patients with pancreatic lymphoma.
Bile duct dilatation from obstruction
is seen more often because jaundice occurs in 42% of patients with non-Hodgkin's
lymphoma primarily involving the pancreas.
Diagnosis,
Treatment, and Prognosis:
As the prognosis of a pancreatic lymphoma is
favorable, its differentiation from a carcinoma is crucial. The correlation of
USG , CT and MR findings may result in a correct diagnosis. However, if doubt
exists, biopsy may reveal the true nature of the mass.
Percutaneous(usg guided)
or endoscopic core biopsy should be performed to establish the diagnosis. In most
patients, the diagnosis can be established without surgery; this fact is a major
reason to look for findings suggestive of pancreatic lymphoma.
Summary
When the radiologist is faced with a well-circumscribed tumoral mass in
the pancreas, knowing when to direct the patient toward non-surgical biopsy instead
of surgical biopsy is critical. Lymphoma does not require surgical staging or
a palliative Whipple's procedure before chemotherapy or radiation therapy. In
patients with primary pancreatic lymphoma, no marked pancreatic ductal dilatation
is present even with ductal invasion. Adenocarcinoma commonly dilates the more
distal pancreatic duct when more proximal ductal invasion has taken place. Lymph
node involvement below the level of the renal veins is another finding not seen
with adenocarcinoma. Clinical and imaging findings are otherwise not specific
in the differentiation of pancreatic lymphoma and pancreatic cancer, but a bulky
homogeneous tumoral mass without alteration of Wirsung's duct or the peripancreatic
vessels should suggest the diagnosis. In patients with diffuse infiltration of
the pancreatic gland without clinical signs of pancreatitis, the radiologist should
be alert to the possibility of pancreatic lymphoma.