Inflammatory - PowerPoint Presentation

Slide1

Inflammatory Myofibroblastic Tumors In Children

Dr. Ibtisam Al ShuailiClinical Fellow, Pediatric ImagingAlberta Children HospitalCalgary, CanadaSlide2

DisclosureNo financial disclosure.Slide3

Introduction.

Pathogenesis.Clinical presentation. Imaging features. Management.

Conclusion.

OutlineSlide4

Introduction

Inflammatory myofibroblastic tumor (IMT) is a relatively new histopathologic term for an entity previously known as inflammatory pseudo-tumor, which is a rare pseudosarcomatous inflammatory lesion that occurs in the soft tissues of young adults.Slide5

Pathogenesis

Cause is unknown.Some causes are trauma, surgical inflammation, immune-autoimmune condition, and low-grade fibro-sarcoma with inflammatory cells.

Histologically

, an

IMT

contains cells

associated with

both acute and chronic inflammation

, including

lymphocytes and

plasma cells

, myofibroblastic spindle cells, and

collagen.

IMT can be differentiated from lymphoma because IMT

has both T and B cells

, in contradistinction to lymphoma, in which a clonal population of T or B cells is present.Slide6

Some pseudo-tumors are thought to

be secondary to infection. Various organisms have been implicated in pathologic specimens, including:

Mycoplasma

and nocardiae in lung

pseudo tumors,

Actinomycetes in

liver

pseudo tumors,

Epstein-Barr virus in

splenic and nodal

pseudo tumors,

Mycobacteria in

spindle cell

tumors.Slide7

Inflammatory Pseudotumor.

Lakshmana Das Narla, et al. Radio Graphics 2003;

23:719–729

Slide8

On ultrasound

image:Hypoechoic or hyperechoic.

Ill-defined or

well-circumscribed

borders.

Often have

increased

vascularity during

color or power Doppler examinations

.

Variable and nonspecific.

Possibly because of the amount of fibrosis and cellular infiltration.

Radiologic Features Of Inflammatory

PseudotumorSlide9

CT scan:

Lesions can be Low, equal, or high attenuation compared with the surrounding tissue.

Homogeneous

or

heterogeneous enhancement in post contrast.

MR images:

Lesion show low

signal intensity on both T1- and T2-weighted images, which may reflect the fibrotic nature of these lesions.

Delayed imaging often shows increasing enhancement due to the presence of fibrosis.Slide10

Lungs.

Mediastinum.Tracheobronchial tree.Cardiac.

Thoracic

Inflammatory Pseudotumor

Intraparenchymal

Pulmonary Inflammatory

Pseudotumors

.

C

.

Payá

Llorens

et al.

Vol

39.

Num

11. November 2003

 Slide11

Inflammatory Pseudotumor Of The Lung

Most common primary lung mass seen in children.50% of benign intrapulmonary tumors

seen in pediatric

patients.

No

sex

predilection.

The peak prevalence

is in the second decade of life.

Symptoms: Cough, fever

, dyspnea, and

hemoptysis.

Although

many

children are

asymptomatic, approximately 20%

have an

antecedent pulmonary insult, usually

infection.Slide12

Imaging Findings

On radiographs: Solitary, peripheral, sharply circumscribed, lobulated mass with an anatomic bias for the lower lobes.

On CT scans:

V

ariable

and

nonspecific appearance

, but most

commonly they demonstrate heterogeneous

attenuation and enhancement

.

On T1-weighted MRI

: Intermediate signal intensity.

On

T2-weighted

MRI

;

High

signal

intensity.Slide13

Calcification within the lesion occurs more frequently in children than in Adults.

It can have an amorphous, mixed, fine fleck-like pattern or be dystrophic.Can also be associated with atelectasis and pleural effusion, usually unilateral.

Inflammatory

Pseudotumor: The

Great Mimicker

Madhavi

Patnana

, et al.

AJR

2012; 198:W217–W227Slide14

Differential Diagnosis

Primary Or Secondary Neoplasm, Hamartoma, Chondroma,Hemangioma,

Granuloma,

Pulmonary Sequestration.Slide15

13 years female immigrant from Yugoslavia to Canada.Known to have scoliosis which worsened by time.Incidental findings of solitary lung nodule during the scoliosis assessment.

Resection of the nodule show inflammatory myofibroblastic tumor.Slide16

Mediastinal Inflammatory Pseudotumor

Rare in the mediastinum.Clinical presentation may be secondary to mass effect on adjacent structures including dyspnea, chest pain, or hemoptysis

.

CT may show mediastinal IPT encasing

various structures and show heterogeneous enhancement.Slide17
Slide18

Tracheobronchial Inflammatory Pseudotumor

Rare.Commonly seen in children and young adults.

Can

be asymptomatic,

or cause

cough, chest pain, dyspnea, hemoptysis

, post-obstructive

pneumonia, and

symptoms secondary

to airflow

obstruction.Slide19

Well-defined endoluminal tracheobronchial mass.

Heterogeneous lobulated or exophytic endoluminal mass.Calcification may be present.

Associated atelectasis, pleural effusion, or lymphadenopathy.

Imaging FindingsSlide20

Cardiovascular symptoms include chest pain, syncope, transient ischemic attack, and

arrhythmias.Cardiac MRI used to confirm the presence of a mass and obtain details about its size, location, and imaging appearances.

Rare, most commonly seen in children and young adults.

Common sites: right

atrium and right

ventricle.

Symptoms: fever, weight loss, anemia, thrombocytopenia, pallor, diaphoresis, and dyspnea.

Cardiac Inflammatory PseudotumorSlide21

Symptoms include pain, redness, edema, proptosis, ptosis, oculomotor deficits, diplopia, lid swelling, and (or) mass

.Third most common orbital disease, Graves disease and lymphoproliferative disease being first and second.

Benign orbital condition of unknown cause.

Considered a diagnosis of exclusion.

Accounts

for approximately 10% of orbital

masses.

The presentation can be acute or sub acute.

Orbital Inflammatory PseudotumorSlide22

CT and MRI show focal or diffuse enhancing abnormalities involving the orbit.

There may be associated fat infiltration of the retro bulbar fat or edema, bone destruction, and intracranial extension.MRI is considered the modality of choice for visualizing subtle inflammation of the nerves and muscles.Slide23

Abdominal Inflammatory PseudotumorHepatic Inflammatory Pseudotumor

Rare.Common in men.

Presenting symptoms abdominal

pain,

fever.

Biliary

obstruction or

portal hypertension

may also be

present.Slide24

Nonspecific.

Single or multifocal mass.On ultrasound: Lesions appear as hypo- or hyper-echoic

masses with

septations

.

Imaging FindingsSlide25

On unenhanced CT images

, the lesions are low in attenuation relative to the surrounding liver. Contrast-enhanced imaging shows various patterns of enhancement, including heterogeneous enhancement, homogeneous enhancement, enhancement of septa, peripheral enhancement with delayed central filling, and no enhancement or central necrosis.On MR images, these lesions are usually T1 hypo-intense and T2 hyper-intense with heterogeneous

enhancementSlide26

Biliary Inflammatory Pseudotumor

Rare. Has an association with biliary atresia and primary biliary cirrhosis.

In

some cases, IPT has been

seen in

conjunction with recurrent pyogenic

cholangitis mimicking

the

periductal

-infiltrating type

of

cholangiocarcinoma.Slide27

Symptoms: Patients presents with obstructive jaundice, fever, anemia, and pain.

Preoperatively, it is difficult to differentiate biliary IPT from cholangiocarcinoma, thus the diagnosis often is made at postsurgical pathologic examination.

Depending on

the degree of biliary obstruction and

portal hypertension

, perihilar IPT may

necessitate biliary

drainage, resection, or liver transplant.Slide28

Splenic Inflammatory PseudotumorRare.

Middle-aged to older persons and in both sexes.Patients can present with weight loss, fever, splenomegaly, and abdominal pain.Epstein-Barr virus infection has typically been associated with hepatic and

splenic

IPTs.Slide29

It appear as large, well-circumscribed single mass lesion

at imaging.Often mistaken for lymphoma.Preoperative diagnosis is difficult because

this lesion

can mimic other tumors.

The prognosis of

splenic IPT has been reported to be

favorable after splenectomy.Slide30

Gastrointestinal Inflammatory Pseudotumor

Uncommon.Involve the stomach in young girls.

The small

intestine, followed by the large intestine

, can

be

involved.

P

resent

with abdominal pain

but also

can have dysphagia, intestinal obstruction

, and

iron deficiency

anemia.

Appear as ulceration

, wall infiltration, and extraluminal

extension in imaging.Slide31

Mesenteric Inflammatory Pseudotumor

Uncommon.Tends to occur in children and young adults.

Systemic symptoms include fever, malaise

, weight

loss, and

pain.

Mesenteric lesions can

have well-circumscribed margins or

have an

infiltrative ill-defined border extending

to adjacent

bowel

loops.

There may be central calcification. Slide32

15 years female admitted to hospital in Dec 2014 with 4 months of severe aphthous ulcers and a 12- pound

 weight loss. MRE performed to rule out inflammatory bowel disease.

Pre and post gadolinium coronal images MR enterography.

Enhanced axial and sagittal CT abdomen reveal retroperitoneal mass.Slide33

Genitourinary Inflammatory Pseudotumor

Urinary Bladder IPT:Rare.Typically occurs in young adults.

When

seen in a child, it

must be

differentiated from

rhabdomyosarcoma because

management of the two lesions differs.Slide34

Presentation includes:

Gross hematuria and anemia, dysuria, increased urinary frequency, and urinary tract obstruction.Imaging findings includes: Polypoid enhancing intraluminal mass

and a submucosal mass with or

without perivesicular

fat

involvement.Slide35

Renal and adrenal

Extremely rare. Presents as flank pain and hematuria.Nonspecific imaging findings:

On Ultrasound

:

Hypoechoic

or

hyperechoic

mass with associated

vascularity.

On CT

: These

lesions may be

low in attenuation.

On MRI

: They are hypo-vascularSlide36

Management

Depends in the location.Surgical resection is curative in most cases.Adjuvant therapy can be added.

Some cases can be treated with observation and

nonsteroidal

anti-inflammatory drug.Slide37

Inflammatory

Pseudotumor: The Great MimickerMadhavi Patnana

, et al.

AJR

2012; 198:W217–W227Slide38

Conclusion

The imaging characteristics of IPT are nonspecific because the lesion has a wide range of imaging presentations, often mimicking other entities, including tumors. IPT can

occur throughout the body and should

be considered

preoperatively in the

differential diagnosis

.

Surgery

is generally required

for diagnosis

of IPT, but awareness of these

lesions may

prevent unnecessary radical

surgery in

some instances.Slide39

My Radiologist Staff Dr. Samarjeet Bhandal , ACH

AcknowledgmentSlide40