QUESTION 1

Which of the following is NOT a radiologic feature of idiopathic retroperitoneal

fibrosis?

 Enhancement after contrast administration.

 Absence of the normal psoas shadow on the radiograph.
 Fibrous tissue between the aorta and the vertebrae.
 Medial deviation of the ureters.
 Hypointense signal intensity on T1-weighted and variable signal intensity
on T2-weighted MR images.

Early idiopathic retroperitoneal fibrosis shows vascularity and enhances after contrast
administration. Long-standing fibrous plaques are relatively avascular and show minimal
enhancement. Option A is not the best response. Extensive retroperitoneal fibrous tissue
sometimes obscures the normal psoas shadow on a KUB (kidneys, ureters, and bladder)
examination. Option B is not the best response. In idiopathic retroperitoneal fibrosis,
fibrous tissue is usually not seen between the aorta and the underlying vertebrae, giving
a “taped-down” appearance to the aorta. Soft tissue between the aorta and the vertebrae
is typically seen in lymphoma or disseminated malignancy [1]. Option C is the best
response. Ureters are medially displaced in retroperitoneal fibrosis, which is frequently
apparent at the L4–L5 level. In disseminated malignancy, they are laterally displaced.
Option D is not the best response. The fibrous tissue appears hypointense on T1-
weighted imaging, whereas on T2-weighted imaging it is hyperintense during the early
active inflammation and becomes hypointense later during chronic disease. Option E is
not the best response.

QUESTION 2

Which of the following is NOT a cause of secondary retroperitoneal fibrosis?

 Lymphoma.
 Radiotherapy.
 Asbestosis.
 Prostate cancer.
 Penicillin.

One third of all retroperitoneal fibroses have some underlying secondary cause.
Lymphoma is the most common malignancy to cause retroperitoneal fibrosis. Colorectal,
breast, prostate, and bladder cancers are other causes. Radiation therapy usually causes
fibrosis in the field of irradiation. Asbestosis has also been associated with retroperitoneal
fibrosis. Options A, B, C, and D are not the best responses. Drugs such as methysergide,
pergolide, and methyldopa can cause retroperitoneal fibrosis. However, penicillin has not
been implicated. Option E is the best response.

QUESTION 3

Which of the following is TRUE of idiopathic retroperitoneal fibrosis?

  Patients with idiopathic retroperitoneal fibrosis usually do not have
constitutional symptoms.
 End-stage renal failure is the most common and severe late complication.
 Markers of autoimmune disease, such as antinuclear antibodies, are
negative in idiopathic retroperitoneal fibrosis.
 Acute phase reactants are not elevated in idiopathic retroperitoneal
fibrosis.
 In most patients with retroperitoneal fibrosis, constitutional symptoms such as
low-grade fever, weight loss, fatigue, nausea, and anorexia precede or coexist
with localized symptoms like abdominal pain, lower extremity edema, or scrotal
swelling. Option A is not the best response. The nonspecific nature of symptoms
often leads to a delay in diagnosis, resulting in advanced complications such as
renal failure due to ureteric obstruction [2]. Option B is the best
response. Patients with idiopathic retroperitoneal fibrosis often have some
manifestations of autoimmune disease. Antinuclear antibodies are present in up
to 60% of idiopathic retroperitoneal fibrosis patients. Option C is not the best
response. Acute phase reactants such as erythrocyte sedimentation rate and C-
reactive protein are also elevated in 80–100% of patients [3]. Option D is not the
best response

QUESTION 4

Which of the following is TRUE of retroperitoneal liposarcomas?

 The absence of identifiable fat on CT excludes the diagnosis of

retroperitoneal liposarcoma.
 Predominantly fatty liposarcomas with a minimal soft-tissue component
on CT are likely to be low-grade.
 The presence of calcification or ossification indicates low malignant
potential of the tumor.
 Secondary de-differentiated liposarcoma arising in a preexisting well-
differentiated liposarcoma is more common than de novo de-differentiated
retro peritoneal liposarcoma.

 Well-differentiated, de-differentiated, and myxoid liposarcomas usually have fat-

containing areas identifiable on imaging. However, pleomorphic and round cell
liposarcomas may not have any identifiable fat-containing areas, making their
differentiation from other retroperitoneal tumors difficult on CT or MRI. Option A is
not the best response. If a liposarcoma is composed predominantly of fat with
very little soft tissue, then it is likely to be low-grade. However, the reverse is not
true, and predominantly solid liposarcomas can be low-, intermediate-, or high-
grade [4, 5]. Option B is the best response. Calcification or ossification in a
liposarcoma often indicates de-differentiation and is considered to be a bad
prognostic indicator. Option C is not the best response. De-differentiated
liposarcomas can be primary and arise de novo or occur in long-standing, well-
differentiated liposarcomas. De novo de-differentiated liposarcomas are more
common than those that are secondary. Option D is not the best response.

QUESTION 5

Extraadrenal paragangliomas are NOT associated with which of the following?

  von Hippel-Lindau disease.
 Gastric leiomyosarcoma.
 Trisomy 21.
 Sipple's syndrome.

Paragangliomas are seen in various neurocutaneous syndromes such as

von Hippel-Lindau disease; tuberous sclerosis; Sturge-Weber syndrome;
and multiple endocrine neoplasia, type II (MEN IIA [Sipple's syndrome]
and MEN IIB). Options A and D are not the best responses. The Carney
triad consists of an association of extraadrenal paragangliomas, gastric
leiomyosarcoma, and pulmonary hamartoma. Option B is not the best
response. Trisomy 21 is associated with various anomalies but no
association with paragangliomas has been described. Option C is the
best response.

QUESTION 6

Which of the following is TRUE about extra-adrenal abdominal paragangliomas?

 Alpha blockade is essential before the administration of nonionic contrast

material for CT.
 Histopathologic examination is used to establish malignancy in
paragangliomas.
 Extraadrenal paragangliomas are less likely to be malignant than adrenal
pheochromocytomas.
 Renal hila and the inferior paraaortic region are the most common sites
for extraadrenal pheochromocytomas.

The use of high-molecular-weight iodinated contrast material has been associated with
the release of epinephrine or norepinephrine from pheochromocytomas and
paragangliomas, causing a sudden precipitous increase in blood pressure. In the past,
adrenergic α-blockade was routinely advised before contrast administration in these
patients. However, it has been shown that nonionic contrast material can be safely given
in patients with pheochromocytomas without any prior α-blockade [6]. Option A is not the
best response. Typical histologic markers of malignancy, such as nuclear atypia, number
of mitoses, and capsular or vascular invasion, do not consistently predict malignant
behavior. Conversely, benign features on histopathology do not ensure benign behavior
in the future. Malignancy is determined by the presence of documented metastasis to
sites that do not have paraganglion tissue, such as bone, lungs, lymph nodes, and liver.
Option B is not the best response. Extraadrenal paragangliomas are more likely to be
malignant than their adrenal counterparts, with malignancy rates ranging from 29% to
40%. Option C is not the best response. Paragangliomas can be found at every site
where healthy paraganglia occur, from the base of the skull to the urinary bladder. In the
abdomen, extraadrenal paragangliomas occur most frequently at the renal hila and in the
organs of Zuckerkandl, which are located in the abdominal paraaortic region near the
origin of the inferior mesenteric artery. Option is D the best response.

QUESTION 7

Which of the following is TRUE about imaging of extraadrenal paragangliomas?

 Low-attenuation regions and occasional calcification are common on CT.

  Paragangliomas enhance poorly on CT.
 131
I MIBG imaging is preferred over 123I MIBG imaging.
 18
F-FDG PET is highly specific for paragangliomas.
 Most paragangliomas have low-attenuation areas that represent cystic
degeneration or necrotic areas. Calcification is present in up to 20% of
paragangliomas. Option A is the best response. Paragangliomas are
hypervascular tumors and enhance briskly [7–9]. Option B is not the best
response. Although both 123I and 131I are used for functional imaging, 123I is
preferable because of its higher sensitivity, lower radiation dose, and better
image quality. Option C is not the best response. Most paragangliomas are
metabolically active and accumulate FDG. However, a variety of neoplastic and
nonneoplastic processes are also FDG PET-positive, resulting in low specificity.
Therefore, FDG PET is not recommended as a first-line imaging technique for
paragangliomas. Option D is not the best response.

QUESTION 8

The “floating aorta” sign is suggestive of which of the following?

 Lymphoma.
 Retroperitoneal sarcoma.
 Neurogenic tumors.
 Retroperitoneal hemorrhage.

Lymphoma is typically homogeneous, of soft-tissue attenuation, and mildly enhancing,

and it tends to surround adjacent vessels. Sometimes the aorta seems to be immersed in
the tumor, giving the floating aorta or “CT angiogram” sign. Option A is the best
response. This sign is characteristic of lymphoma and is not seen in other
retroperitoneal disorders such as sarcomas or neurogenic tumors. Retroperitoneal
hemorrhage is high-attenuation. Options B, C, and D are not the best responses.

QUESTION 9

Which of the following is TRUE of lymphoma?

 Spleen size correlates with splenic involvement.

 Hodgkin's disease tends to spread by involving contiguous lymph nodes.
 At the time of presentation, retroperitoneal lymph node involvement is more
common in Hodgkin's lymphoma than in non-Hodgkin's lymphoma (NHL).
 Extranodal involvement is more common in Hodgkin's disease than in NHL.

Correlation between spleen size and splenic involvement by Hodgkin's disease is

poor. In Hodgkin's disease, only one third of patients with splenomegaly have
splenic involvement, whereas one third of patients with a normal-sized spleen
have splenic involvement. Only massive splenomegaly correlates well with the
spread of Hodgkin's disease. Option A is not the best response. NHL may spread
hematogenously, so many different and widely separated lymph node chains can
be involved at the same time, whereas Hodgkin's disease tends to spread via the
lymphatics, involving contiguous lymph nodes. In patients with thoracic Hodgkin's
disease, the upper abdomen must be carefully evaluated for contiguous spread
of disease [10]. Option B is the best response. At the time of presentation,
retroperitoneal lymph node involvement is present in 55% of patients with NHL
but in only 25–35% of patients with Hodgkin's lymphoma. Option C is not the best
response. Extranodal involvement is more common in NHL than in Hodgkin's
 disease [11]. Option D is not the best response.

QUESTION 10

Which of the following is a known presentation of inferior vena cava (IVC)

leiomyosarcoma?

 Budd-Chiari syndrome.
 Nephrotic syndrome.
 Lower extremity edema.
 All of the above.
 Primary IVC leiomyosarcomas often present as a result of symptoms caused by
venous obstruction. Symptoms vary according to the level of the tumor. Infrarenal
tumors cause lower limb edema, tumors at the level of the renal hilum can cause
nephrotic syndrome, and tumors involving the upper third of the IVC may produce
Budd-Chiari syndrome [12, 13]. Options A, B, and C are all correct
responses. Option D is the best response.