Presentation1.pptx radio;ogical imaging of benign and malignant soft tissue tumours.

Radiological imaging of benign and malignant soft tissue tumours.
Dr/ ABD ALLAH NAZEER. MD.

INFANTILE (JUVENILE) HEMANGIOMA
Definition:
• Infantile (juvenile) hemangioma is a form of capillary hemangioma
which occurs during infancy.
Epidemiology:
• About 1 in every 200 live births. About 1/5 of cases are multiple.
Sites of involvement:
• Infantile hemangioma may be located on any body surface but is most
common in the region of the head and neck, particularly the parotid,
where follows distribution of cutaneous nerves and arteries.
Clinical findings:
• During early stage may resemble a common birthmark in that it is a flat,
red lesion that intensifies in color when infant strains or cries.
CT: On unenhanced CT, it may appear as an ill-defined mass of similar attenuation to muscle
may be identified. CT may also identify the presence of associated phlebolith.
MRI
Hemangiomas are typically well-defined, lobulated and heterogeneous with no features of local
invasion.
While many sequences show a rather heterogeneous signal mass certain signal characteristics
tend to dominate.
T1: overall signal is often intermediate to slightly high signal (relative to skeletal muscle)
some focal high signal areas may be present in a large proportion of lesions (up to 70%)
T2: high signal intensity tends to dominate on T2-weighted images
gradient echo: the presence of pheboliths may show blooming artefact

Orbital hemangioma. a) Axial CECT at the level of the mid orbit demonstrates a diffusely
enhancing hemangioma (arrow) of the right upper eyelid. b) 5-year-old male with left
proptosis diagnosed with orbit hemangioma, contrast CT examination shows extraconal,
uniformly enhancing lesion (arrow) along the medial aspect of the left orbit.

Orbital hemangioma. MR T2-weighted images a) axial b) coronal demonstrate a large hyperintense,
lobulated soft tissue lesion extending to orbit in the extra-conal space, lateral to the orbital cone
(arrows). c) Contrast-enhanced T1 image shows extensive near-homogeneous enhancement. Extent
of the lesion is better demonstrated following intravenous contrast (arrow).

ANGIOMATOSIS
Definition:
• Rare, benign but clinically extensive vascular lesion of soft.
tissue occurring in infants.
Epidemiology:
• These lesions probably begin to grow during intrauterine life when the limb
buds to form, grow proportionately with the fetus and involve large areas
of extremities and trunk.
• Can occurs in newborn and infants (when occur in the first year of life is
called diffuse neonatal angiomatosis)
• Involvement may be of two types: extensive vertical involvement of multiple
tissues (e.g., subcutis, muscle, and bone) extensive involvement of tissue of
the same type(e.g., multiple muscles).
• Frequently affects the lower extremities and buttock but may occur
throughout the body.
Clinical findings:
• Usually symptoms of diffuse swelling, sometimes associated with pain and
discoloration.
Imaging:
• CT scans appear as ill-defined nonhomogeneous masses that may resemble
sarcoma except for presence of dense areas corresponding to thick-walled vessels.
• Large presence of fat may appear as fatty tumors.

ANGIOMATOSIS OF THE THIGH MUSCLE

Massive juvenile angiomatosis of maxilla and mandible.

Encephalotrigeminal angiomatosis.

LYMPHANGIOMA
Definition:
• A benign, cavernous/cystic vascular lesion composed of dilated lymphatic
channels.
Epidemiology:
• Lymphangiomas are common pediatric lesions, most often present at birth
or during first year of life.
• Some cases may identified in Turners syndrome ( monosomy chromosome
X, 46 X) and may be found in abortuses.
• Cavernous/cystic lymphangioma of head and neck represents the most
common type and is also called cystic hygroma.
• Cystic lymphangiomas are mostly located in the neck, axilla and groin.
• Cavernous type occurs additionally in the oral cavity, upper trunk, limbs
and abdominal sites including mesentery and retroperitoneum.
Clinical features:
• Lesions rather present as circumscribed painless swelling, which are soft
and fluctuant in palpation, and can show displacement surrounding organs
at mediastinal or abdominal sites.
Imaging:
• USG show cystic nature, angiography show poor vascularization and
CT scan reveals multiple, homogenous, nonenhancing areas.

Lymphangioma T2 with fat suppression, transversal and coronal

Peripheral neurogenic tumour.
LIPOMATOSIS OF NERVE (FIBROLIPOMATOUS HAMARTOMA)
Definition:
• Lipomatosis of nerve is characterized by infiltration of the epineurium
by adipose and fibrous tissue.
• Tissue grows between and around nerve bundles causing enlargement
of involved nerve.
Epidemiology:
• Frequently first noted at birth or in early childhood.
• In the largest reported series patients ranged in ages 11 to 39 y/o. In some
cases it is associated with macrodactyly of the digits innervated by the
affected nerve.
• Females predominate when lipofibroma is accompanied by macrodactyly,
whereas males are more commonly affected when macrodactyly is absent.
• Medial nerve and its digital branches are most commonly affected followed
by the ulnar nerve.
Clinical features:
• Presents with a gradually enlarged mass in the affected area that may
be asymptomatic or associated with motor or sensory deficits.
• Patients with macrodactyly have symmetrical or asymmetrical enlargement
of affected finger(s) with enlargement of the involved bones.
Imaging:
• Fat around fascicles of enlarged nerve (usually median).

Fibroma of tendon sheath in a 63 year old male with a right upper limb lump. Colour Doppler
ultrasound study (a) shows a well-defined isoechoic ovoid-shape mass in the subcutaneous fat,
indenting the underlying muscle without invading it. The mass shows minimal internal vascularity. T1
axial (b) and corresponding T2 (F/S) (c) images show a homogenously hypointense lesion (arrow) in
the deep fascial plane of the arm which indents upon the triceps muscle. There is no evidence of
intramuscular invasion. On T1 post contrast image (d), the lesion shows minimal enhancement.

GIANT CELL TUMOUR OF TENDON SHEATH.

Fibrolipomatous hamartoma of the median nerve.

NEUROFIBROMA:
Definition:
• Well-demarcated intra-neural or diffusely infiltrative extra-neural tumor
consisting of mixture of cell types, including Schwann cells, perineural-like
cells, and fibroblasts; multiple and plexiform neurofibromas are typically
associated with neurofibromatosis type 1.
Epidemiology:
• Neurofibromas are common and occur either as sporadic solitary nodules
unrelated
to any apparent syndrome or as solitary, multiple or numerous lesions in
individuals with neurofibromatosis type 1 (NF1 loss of 17q gene region).
• All ages and both sexes are affected.
• Neurofibroma presents most commonly as a cutaneous nodule (localized
cutaneous neurofibroma) and less often as a circumscribed mass in a
peripheral nerve (localized intraneural neurofibroma) or as a plexiform
enlargement of a plexus or major nerve trunk.
Clinical features:
• Rarely painful, the tumors present as a mass.
• The presence of multiple neurofibromas is the hallmark of NF1, in which they
are associated with pigmented cutaneous macules (cafe-au-lait spots) as
well as 'freckling', often axillary in location.

Newborn girl with multifocal myofibromatosis with preferential involvement of metaphyses (arrows).

Multiple neurofibromas. A, Coronal short tau
inversion recovery magnetic resonance image
of the thighs shows multiple neurofibromas
distributed along the sciatic nerves of a young
woman first seen at the age of 16 with a
malignant peripheral nerve sheath
tumor. B, Transverse T2-weighted images show
multiple, discrete, high-intensity neurofibromas.
A characteristic target sign is present (arrow).
The larger lesion in the posterior left thigh,
which had shown recent growth, was a low-
grade malignant peripheral nerve sheath tumor.

Infantile myofibromatosis in a neonate. A, Ultrasound shows a solid, intramuscular
subscapular mass (arrows). B, Axial contrast-enhanced computed tomographic image
shows peripheral enhancement within the mass (arrows). C, Thigh lesion in the same
patient shows hyperintense signal on T1-weighted magnetic resonance imaging. D,T2-
weighted image with fat saturation demonstrates cystic changes with a thick, irregular
rind of soft tissue. E, Post-gadolinium T1-weighted fat-saturated images demonstrate
enhancement of the rind of soft tissue and central areas of non enhancement.

MYOFIBROMA/MYOFIBROMATOSIS
Definition:
• Myofibroma (solitary) and myofibromatosis (multicentric) is benign
neoplasm composed of contractile myoid cells arranged around thin-walled
vessels.
• Myofibroma(tosis) forms a morphological continuum with
myopericytoma and so-called infantile hemangiopericytoma.
Epidemiology:
• May occur from newborn to elderly, however many cases are diagnosed
at birth or within the first two years of life.
• Myofibroma(tosis) is more common in males.
• The relative frequency of solitary versus multicentric forms in unclear.
• In adults, solitary lesions are more common.
• 50% of solitary myofibroma occur in the cutaneous/subcutaneous tissues
of head and neck region, followed by trunk, lower and upper extremities.
• Other 50% occur in skeletal muscle or aponeurosis, with a small number
involving bone, predominantly the skull.
• Myofibromatosis (multicentric) involves both soft tissue and bone and
frequently (10-20%) occurs in the deep soft tissue and visceral location.

NEUROTHECOMA
Definition:
• Also known as nerve sheath myxoma, dermal nerve sheath myxoma.
Epidemiology:
• Usually arise in childhood and early adult life.
• Situated in the dermis and subcutis and rare in deep soft tissue.
• Neurothecomas have predilection for upper portion of the body, such as the head,
neck, and shoulder.

Clinical features:
• Lesions may be of short or long standing duration.
• Cutaneous lesions have the appearance of purplish macules,
simulating vascular neoplasm.
• Subcutaneous lesions occur most often as painless, freely
movable masses while more deeply seated lesions may be
fixed.
• Visceral lesions may cause symptoms referable to the organs
that are involved.
Imaging:
• Soft tissue lesions varies, and can be well-circumscribed or
infiltrative, often with calcifications, either within or surrounding
the lesion.
• Bony lesions characteristically occur as multiple elongated
radiolucent lesions within the metaphyseal regions, sparing
the region adjacent to epiphysis.

Myofibromatosis of the head and neck.

FIBROMATOSIS COLLI
Definition:
• A benign, site-specific lesion that occurs in the distal
sternocleidomastoid muscle of infants.
• The mass results in fusiform thickening of the muscle and
cervico-fascial asymmetry due to its shortening (torticollis).
Epidemiology:
• Uncommon, occurs in about 0.4% of live births.
• There is no sex predilection.
• The majority of cases are diagnosed before 6 months of age.
• Typically affects the lower one third of the sternocleidomastoid
muscle.
Clinical features:
• The affected infants present with a smooth fusiform swelling of
the distal sternocleidomastoid muscle.
Imaging:
• uniform isoechoic mass confined to muscle.

INFANTILE FIBROMATOSIS, LIPOFIBROMATOSIS
Definition:
• Benign fibro-fatty tumor of childhood with predilection for distal
extremities.
Clinical features:
• Ill defined, slowly growing, painless mass in hands and feet and
rarely occurs in the thigh, trunk and head.
• This tumor has been described exclusively in children.
• Median age for surgery is 1 year.
• Male: female ratio 2:1.

Myofibromatosis of the diaphragm

Multicentric deep Fibromatosis

BENIGN FIBROUS HISTIOCYTOMA
Definition:
• A benign neoplasm composed of mixture of fibroblastic and
histiocytic cells arranged in sheets of short fascicles and
accompanied by inflammatory cells, foam cells and
siderophages, which may develop within subcutaneous tissue,
deep soft tissue or in parenchymal organs.
• When located in skin is also called dermatofibroma.
. CT scan shows a moderately irregular lytic area with an prominent
trabecular pattern and surrounding sclerotic bone. On MRI scans, there is
central low signal intensity with a surrounding rim of high signal on T1, and
more uniform but somewhat variegated high signal intensity on T2
sequences, with the surrounding sclerotic bone having low signal intensity.
On bone scan, the lesion has been reported to have no uptake, but the
author cannot confirm.
BENIGN FIBROHISTIOCYTIC TUMORS.

Dermatofibroma (Periungal Fibroma).

Benign mesenchymal lesion, compatible with solitary fibrous tumor

JUVENILE XANTHOGRANULOMA
Definition:
• Benign histiocytic subcutaneous lesion that usually occurs during childhood.
Epidemiology:
• JX usually develop during infancy and is characterized by one or more cutaneous
nodules and less often by additional lesions in deep soft tissue or organs.
• As a rule, those that develop after the age of 2 years or in adults are usually solitary
JUVENILE XANTHOGRANULOMA.

Juvenile Xanthogranuloma of the Heart and Liver: MRI, Sonographic, and CT Appearance.

MRI Findings of Juvenile Xanthogranuloma of the Spinal Cord.

GIANT CELL FIBROBLASTOMA
Definition:
• Painless nodule or mass in subcutis with the predilection for the back of the
thigh, inguinal region and chest wall in children younger than 5 years of age.
GIANT CELL FIBROBLASTOMA

PLANTAR FASCIA LESION / FIBROMATOSIS

BENIGN DESMOPLASTIC FIBROBLASTOMA.

PLEXIFORM FIBROHISTIOCYTIC TUMOR
Definition:
• Slowly growing plexiform fibrohistiocytic neoplasm of the deep
dermis, occurs almost exclusively in children and young adults.
Scalp Plexiform Schwannoma.

Plexiform fibrohistiocytic tumor in a 25 year old male who presented with hypophosphotemia,
osteomalacia and a lump in his sole. T1W (a) and T2W fat saturated (b)images show a
lobulated, infiltrating low signal intensity lesion in the subcutaneous plane of the sole which
shows minimal enhancement on T1W fat saturated post contrast image (c).

SPINDLE CELL TUMOUR WITH FIBROHISTIOCYTIC PATTERN.

Superficial plexiform neurofibroma . Deep plexiform neurofibroma.

Angiomatoid fibrous histiocytoma.

Lipomatous Tumour.
LIPOBLASTOMA/LIPOBLASTOMATOSIS
Definition:
• A lobulated, localized (lipoblastoma) or diffuse (Lipoblastomatosis) tumor,
resembling fetal adipose tissue.
Lipoblastoma is a rare, benign, encapsulated tumour arising from
embryonic white fat.
It occurs primarily in infancy and early childhood (more than 90%
before age 3). It most often occurs in the extremities and trunk,
although it can be seen in other areas.
The entity was originally described as an infiltrating anterior chest
wall mass.
Radiographic features
Imaging features are non-specific. Lipoblastoma is classically a
predominantly fatty mass with well-defined margins. However, it
may have myxoid components that predominate and that can also
be enhancing.

Lipoblastoma soft tissue mass showed predominantly lipomatous tissue with some part of non-fatty
component. The lesion was well-circumscribed, with compression of adjacent bones and muscles.

A 5-month-old patient with a lipoblastoma arising from the right axilla. A, Computed
tomographic section of the chest of shows a low-attenuation mass. B, On a coronal T1-weighted
magnetic resonance image, the inferior portion of the mass (arrow) is markedly hyperintense as
a result of adipose tissue. The low signal in this portion of the mass shown in short tau inversion
recovery (C) and fat-suppressed contrast-enhanced T1-weighted (D) images is also characteristic
of fat. Note exuberant enhancement of the superior half of the solid mass (D).

Desmoid tumours (DTs) are benign, non-inflammatory fibroblastic tumours with a
tendency to local invasion and recurrence but without metastasis.
Ultrasound
Desmoid tumours are the commonest neoplasms of the abdominal wall and typically
appear as homogeneously anechoic or hypoechoic masses. As such they should not be
confused with cystic lesions. They may render an appearance like muscles, may be
lobulated and may show vascularity on colour Doppler interrogation.
CT
most desmoid tumors are well circumscribed masses, although in some cases they may
appear more aggressive with ill-defined margins
most are relatively homogeneously or focally hyperattenuating when compared to
soft tissue on the non-contrast scan
most will demonstrate enhancement following administration of intravenous contrast
MRI
MRI, as is the case with other soft tissue tumours, is more sensitive to local tumour
extension. Their appearance is accounted for by their dense cellularity. Typical signal
characteristics include:
T1: low signal intensity
T2: low signal intensity
T1 C+ (Gd): may show homogeneous, inhomogeneous, or no significant enhancement.

Extra-abdominal desmoid tumor (Gardner fibroma) in a 5-year-old girl.
Axial contrast-enhanced computed tomographic image shows a large lenticular-
shaped mass originating in the anterior abdominal wall musculature.

Multicompartmental posterior thigh desmoid tumor in a 17-year-old boy.
A, T2-weighted fat-saturated axial magnetic resonance image demonstrates hyperintense
and hypointense components of the mass. Hyperintense areas likely represent cellular-
dominant components of the mass, whereas hypointense areas likely represent colloid
components. B, T1-weighted post-gadolinium coronal images demonstrate heterogeneous
enhancement of the mass.

INTERMEDIATE MALIGNANT TUMOURS INFANTILE FIBROSARCOMA
Definition:
• Infantile fibrosarcoma (IFS) is histologically identical to classic fibrosarcoma
of adults, but carries a much more favorable prognosis.
• It occurs in infants and young children, metastasizes rarely, and has similar
history of Fibromatosis.
• IFS is morphologically and genetically related to congenital mesoblastic nephroma.
Infantile fibrosarcoma.

Infantile fibrosarcoma with rhabdoid elements in a 1-week-old boy. A, Anteroposterior
radiograph of the foot demonstrates gracile metatarsal bones and a large soft tissue
density mass. T1 sagittal (B) and T1 short-axis (C) magnetic resonance image demonstrate
a large multi-compartment soft tissue mass enveloping metatarsal bones (arrows).

Hemorrhage into an infantile fibrosarcoma.

MALIGNANT TUMOURS.
RHABDOMYOSARCOMA
EMBRYONAL RHABDOMYOSARCOMA
Definition:
• A primitive, malignant soft tissue sarcoma with phenotypic and biological
features of embryonic skeletal muscle.
CT
soft tissue density
some enhancement with contrast
adjacent bony destruction seen in over 20% of cases.
MRI
Signal characteristics include:
T1
low to intermediate intensity, isointense to adjacent muscle
areas of hemorrhage are common in alveolar and pleomorphic subtypes
T2
hyperintense
prominent flow voids may be seen particularly in extremity lesions.
T1 C+ (Gd): shows considerable enhancement.

Rhabdomyosarcoma of the hand in an 11-year-old boy. A, T2-weighted fat-saturated magnetic
resonance imaging (MRI) demonstrates a multilobulated, multicompartment mass with
secondary involvement of the second metacarpal bone. Note that the mass has a sharp interface
with the second metacarpal cortex, an indication that the mass abuts and secondarily involves
the bone rather than arising primarily from the bone. B, Post-gadolinium T1-weighted fat-
saturated coronal MRI demonstrates diffuse tumoral enhancement of the mass.

Two cases of embryonal rhabdomyosarcoma.

Rhabdomyosarcoma in a 6-week-old girl with Beckwith-
Wiedemann syndrome who presented with a right thigh mass.

EMBRYONAL RHABDOMYOSARCOMA, BOTRYOID TYPE.
Epidemiology:
• Botryoid rhabdomyosarcoma accounts for approximately 6%
of all rhabdomyosarcoma.
• Mostly young children
They occur principally beneath a mucous membrane. Recognised sites of
occurrence include:
vagina
urinary bladder
Cervix
nasopharynx
biliary tract - rhabdomyosarcoma of biliary tracts
With vaginal and urinary bladder locations being the most common.
MRI
Lesions can be large.
Signal characteristics are similar to rhabdomyosarcoma i.e:
T1: low to intermediate
T2: high

Botryoid-type of embryonal rhabdomyosarcoma of renal pelvis in a young woman

ALVEOLAR RHABDOMYOSARCOMA
Definition: is a type of rhabdomyosarcoma characterized by its appearance,
which is similar to the alveoli of the lungs.
• Primitive, malignant , round cell neoplasm that cytologically resembles
lymphoma and show partial skeletal muscle differentiation.
Non-contrast computed tomography of head showing a large mass without any intracranial
extension. The diagnosis was post-auricular congenital alveolar rhabdomyosarcoma.

Alveolar rhabdomyosarcoma with patent ductus arteriosus.

Multiple cases of alveolar rhabdomyosarcoma.

Synovial sarcomas are relatively common intermediate-to-high grade malignant soft
tissue tumours, often with an initial indolent course.
CT
CT is non-specific, similar to ultrasound. It appears as a soft tissue mass of heterogeneous
density and enhancement. CT is of course more sensitive to calcifications that either
radiographs or MRI.
MRI
MRI is the modality of choice to locally stage the tumour, although again imaging findings
are not pathognomonic. The mass is usually large and variably well-defined (smaller
lesions tend to be better circumscribed).
T1 :iso- (slightly hyper-) intense to muscle
heterogeneous
T2 : mostly hyperintense
markedly heterogeneous appearance of synovial cell sarcomas on fluid sensitive
sequences result in so called "triple sign" which is due to areas of necrosis and cystic
degeneration with very high signal, relatively high signal soft tissue components, and
areas of low signal intensity due to dystrophic calcifications and fibrotic bands
due to high tendency of lesions to bleed, there might be areas of fluid-fluid
levels known as "bowl of grapes" are seen in up to 10-25% of cases
T1 C + (Gd)
enhancement is usually prominent and can be diffuse (40%) heterogeneous (40%) or
peripheral (20%).

Synovial sarcoma adjacent to the Achilles tendon. A, Transverse T1-weighted magnetic
resonance imaging (MRI) shows mixed intermediate and low signal in the mass. The
Achilles tendon (arrow) is shown. B, A transverse fat-saturated T2-weighted MRI at the
same level shows a fluid-fluid level typical of hemorrhage.

Synovial sarcoma in a 17-year-old girl. T2-weighted fat-saturated axial magnetic resonance
imaging demonstrates a cystic mass with solid soft tissue elements posteriorly (arrow).

RHABDOID TUMOR
Definition:
• Malignant tumour of infants and children, characterized by neoplastic cells with
large nuclei, prominent nucleoli, and abundant, eccentric cytoplasm with variably
prominent eosinophilic, cytoplasmic "inclusions".
Rhabdoid tumor (RT) is an aggressive pediatric soft tissue sarcoma that arises in the kidney,
the liver, the peripheral nerves and all miscellaneous soft-parts throughout the body. RT
involving the central nervous system (CNS) is called atypical teratoid rhabdoid tumor (ATRT|).
CT brain
often isodense to gray matter
may demonstrate heterogeneous enhancement
calcification is common
may show associated obstructive hydrocephalus
MRI brain
Can show necrosis, multiple foci of cyst formation and sometimes hemorrhage:
T1: iso- to slightly hyperintense to grey matter (hemorrhagic areas can be more hyperintense)
T2: generally hyperintense (hemorrhagic areas can be hypointense)
T1 C+ (Gd): heterogeneous enhancement
MR spectroscopy
Cho: elevated
NAA: decreased

Malignant Rhabdoid of right kidney.

NUEUROBLASTOMA
Definition:
• Neuroblastomas are derived from neuroblasts that arise in the neural crest
and normally migrate downward to form ganglion cells and chromaffin tissue.
Ultrasound
Neuroblastoma on ultrasound demonstrates a heterogeneous mass with internal
vascularity. Often there are areas of necrosis that appear as regions of low
echogenicity. Calcification may or may not be evident on ultrasound.
CT: On CT, the tumour typically is heterogeneous with calcifications seen in
80-90% of cases . Areas of necrosis are of low attenuation.
MRI
MRI is superior to all other modalities in assessing the organ of origin, intracranial
or intraspinal disease and bone marrow disease.
T1: heterogeneous and iso to hypointense
T2
heterogeneous and hyperintense
cystic/necrotic areas very high intensity
signal voids may be evident
C+ (Gd): variable and heterogeneous enhancement

Neuroblastoma.
A transverse T2-weighted magnetic resonance image of the pelvis of a 2-year-old girl
with stage IV neuroblastoma shows a large metastasis in the right gluteal muscle.

Neuroblastoma with metastasis.

WILMS TUMOR
Definition:
• Triphasic tumor, with undifferentiated blastoma, fibroblast-like
stroma and epithelium also called nephroblastoma,
• Usually seen in children.
CT
Wilms tumours are heterogeneous soft-tissue density masses with
infrequent areas of calcification (~15%) and fat-density regions.
Enhancement is also patchy, and allows for better delineation of the
relationship between the mass and kidney. 20% of cases have lung
metastases at time of diagnosis.
MRI
MRI is probably the most accurate modality in assessing for IVC
involvement. These tumours appear heterogeneous on all sequences
and frequently contain blood products, but typically are:
T1: hypointense
T1 C+ (Gd): heterogeneous enhancement
T2: hyperintense.

RETINOBLASTOMA
Definition:
• Most common small blue cell intraocular tumor of children.
Epidemiology:
• May be congenital, and not be recognized until 6 months of life. Incidence
of 1 per 20,000 live births.
• Represent 60% sporadic, and 40% familial (autosomal dominant) cases.
• Sites of involvement: eye. About 30% of cases are bilateral. CT
CT demonstrates a contrast-enhancing retrolental mass that is usually calcified.
A dense vitreous due to hemorrhage is also common.
MRI
MRI is the modality of choice for pre-treatment staging on retinoblastoma.
T1: intermediate signal intensity, hyperintense c.f. vitreous.
T2: hypointense c.f. vitreous
T1 C+ (Gd)
the mass usually enhances relatively homogeneously when small
larger tumours often have areas of necrosis. the tumour shows restricted diffusion on
DWI at high b values. It exhibits low ADC values in contrast to the high intensity of the
vitreous in the ADC maps
ADC map can be used to differentiate viable and necrotic tumour.
DWI is valuable in evaluating the response to eye-preservation treatment

Intraocular tumor - retinoblastoma

USG and MRI showing calcifications in Retinoblastoma.

LEIOMYOSARCOMA OF SOFT TISSUE
Definition:
Malignant tumour composed of cells with smooth muscle features.
Epidemiology:
leiomyosarcoma usually occurs in middle-aged or older persons very rare
in infantile or young children
represent significant percentage of retroperitoneal sarcomas and is the
predominant sarcoma arising from larger blood vessels.
CT
generally heterogeneous
commonly demonstrate central low attenuation representing necrosis
calcification exceedingly rare
MRI
May frequently demonstrate cystic foci within. Signal characteristics
include
T1: isointense to muscle
T2 non-fat suppressed: intermediate to hypointense to neighboring fat
T2 FS: predominantly hyperintense.

(Left) This MRI clearly shows a large, soft tissue sarcoma in the inner
thigh. (Right) The same tumor as shown in a cross-section CT scan.

Low grade fibrosarcoma in a 49 year old female with rapidly increasing wrist lump. MRI reveals a relatively well circumscribed
ovoid mass in the ulnar aspect of the right distal forearm, located primarily in the subcutaneous plane. It lies in close proximity
to the flexor carpi ulnaris tendon (blue arrow), with no clear fat plane seen between the two. This lesion is predominantly of low
signal intensity on T1W (a) and T2W (b) images with heterogeneously bright T2 signal areas (orange arrow). On T1 fat saturated
post contrast image (c), the lesion appears very vascular and shows mildly heterogeneous enhancement. Dark linear strands are
also seen on T1, T2W and post contrast images which suggest fibrous nature of this lesion. No blooming was seen on the gradient
images (not shown). On ultrasound (d), the lesion appears isoechoic. The flexor carpi ulnaris tendon lies deep to it (arrow).

Recurrent Myxofibrosarcoma in a 80 year old man with a recurrent soft tissue mass (arrows) in
the expected location of the biceps muscle. The mass is of intermediate signal intensity on
T1W image (a) and of high signal intensity on T2W fat-saturated image (b). The mass appears
to have slightly irregular margins with tail-like configuration (orange arrow). T1 post contrast
image (c) shows avid enhancement. The tumour abuts underlying brachialis muscle and is
closely related to the neurovascular structures (brachial vessels, median nerve and basilic vein)
(blue oval). The patient later underwent right scapulothoracic amputation.

Soft tissue lymphoma is a rare manifestation of lymphoma.
Clinical presentation
Focal swelling and/or pain along with B-type symptoms. Any muscle can be
involved but most commonly:
thigh
trunk
upper limb
leg
Pathology
Muscle lymphoma is typically the non-Hodgkin type. It can manifest in two ways:
metastatic spread from primary disease (e.g. nodal, osseous)
primary lesion (i.e. primary muscle lymphoma), which is less common
Radiographic appearance
MRI
Muscle enlargement with focal mass or diffuse abnormal signal:
T1: iso- or hyperintense to normal muscle
T2: intermediate signal intensity to fat and muscle
Gad (C+): diffuse (more common), thick peripheral and/or septal
marginal enhancement.

Large-cell anaplastic lymphoma of T-cell origin

Soft Tissue intra-muscular Lymphoma.

Primary giant lymphoma of the right thigh.

Intra-muscular non-Hodgkin's lymphoma.

Malignant peripheral nerve sheath tumours (MPNSTs)
are forms of peripheral nerve sheath tumours and comprise of
malignant forms of neurofibromas and schwannomas.
Epidemiology
They are estimated to account for 5-10% of all soft-tissue sarcomas.
They typically present in adults. There is no recognised gender
predilection and clinical presentation varies dependant on location.
MRI
T1: usually isointense to muscle; heterogeneous signal on T1 (if
present) may useful in differentiating from a neurofibroma
T2: can have low signal due to high collagen content
Scintigraphy
Gallium67 scintigraphy may show higher uptake than that of
a neurofibroma.

Malignant peripheral nerve sheath tumour.

Malignant Peripheral Nerve Sheath Tumor.

Presentation1.pptx radio;ogical imaging of benign and malignant soft tissue tumours.

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