RMS: IRSG and SIOP classification, extended with new variants [12]

Prognosi
Group Subtype
s
Embryo
nal
RMS,
botryoid
Better type
I
prognosis Embryo
nal
RMS,
spindle-
cell type
Embryo
Intermedi
nal RMS
II ate
(remaini
prognosis
ng)
Worse Alveolar
III
prognosis RMS
RMS
with
rhabdoid
features
Embryo
Unclear nal RMS
IV
prognosis with
diffuse
anaplasi
a
Sclerosi
ng RMS

IRSG classification

Stage Characteristics
Localized disease A: Tumour confined to muscle or
completely resected organ of origin
I
(regional nodes not B: Tumour infiltrating outside organ
involved) of (muscle of) origin
 Prognosi
Group Subtype
s
A: Primary tumour grossly resected,
with microscopic residual disease
(negative findings in local nodes)
Localized or regional
B: Primary tumour and positive
II disease with total resection
nodes completely resected
of gross tumour
C: Primary tumour and positive
nodes resected, with evidence of
microscopic residual disease
Incomplete resection of tumour or biopsy, with gross residual
III
disease
IV Distant metastatic disease present at diagnosis

RMS
Ultrasound study shows a large left paratesticular mass compressing the left testicle. The
mass has heterogenous echotexture, with central hypoechoic ill-defined areas that may
represent necrosis.

Fig. 5

A 6-year-old boy with a mass in the left scrotum. US image shows an ill-defined
heterogeneous mass surrounding the testis (open arrow). The mass shows increased flow
(solid arrow). Histopathology: embryonal RMS

US is often the first imaging modality used in children with soft-tissue masses because it is
readily available, has high resolution, and can easily assess extent and vascularity of a mass.
One should not forget that most soft-tissue lesions are benign, can readily be diagnosed with
US, and do not need further diagnostic work-up or even treatment.

On US, RMS in general shows as a well-defined, slightly hypoechoic inhomogeneous mass

that can show significantly increased flow (Fig. 5). In the particular case of paratesticular
RMS, US is the imaging modality of choice, although CT of the abdomen is also necessary to
evaluate for retroperitoneal lymphadenopathy. In all other RMS locations additional imaging
using CT or MRI is mandatory.

US is also of use in image-guided biopsies. Recently Sebire and Roebuck [13] systematically
reviewed the pathological diagnosis of paediatric tumours from image-guided needle-core
biopsies. They concluded that image-guided biopsy material was sufficient to come to a
diagnosis in 94% (95%; CI 92–96%) of patients. Complications needing treatment, mostly
haemorrhage requiring transfusion, were reported in only 1% of patients. For image-guided
biopsies the material obtained should be transported fresh to the pathology department.
Fixation should not be performed as this precludes further cytogenetic studies.
Neuroblastoma

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 2.

Comparison of prenatal ultrasonography (US) findings of small neuroblastoma (A) and

extralobar pulmonary sequestration (B).

A. The axial US image of a 32-week fetus shows a round echogenic mass (arrow) at the right

adrenal gland. A part of adrenal gland is obliterated by the mass (arrowhead). B. The coronal

US images of a 32-week fetus show an echogenic mass (arrow) at the left subdiaphragmatic

area, mimicking neuroblastoma. Abutting left adrenal gland (AD) and kidney (LK) are intact.
 Figure 10a. NB in a 2½-year-old girl with an abdominal mass and hypertension. (a)
Longitudinal US scan of the left flank reveals a large, heterogeneous mass with multiple
anechoic areas, representing hemorrhage or cystic change (arrows). (b) Axial oral and IV
contrast-enhanced CT scan through the midabdomen reveals a large left flank mass, with
some rounded areas of low attenuation. (c) Photograph of the bisected gross specimen shows
a hemorrhagic mass with fluid-filled cavities, which at further inspection proved to be a
combination of hemorrhage and cystic change.
A 3 year old child presented with abdominal distention of 2 months duration. An ultrasound
was performed.
Caption: Magnified image of the right upper quadrant mass
Description: The internal characteristics of a section of the mass are displayed in this image.
Also noted are multiple, small rounded hypoechoic structures in close vicinity to the mass,
most likely representing metastatic nodes.

Fig. 1

Pheochromocytoma of the left adrenal gland (arrow). A. Conventional ultrasound in the B-

mode B. B-mode with harmonic imaging. Compared with image A, fewer artefacts – clearer
tumor margins and clearer contrast with surrounding structures
Fig. 2

Pheochromocytoma of the right adrenal gland (arrow). A. Conventional ultrasound in the B-

mode B. B-mode with spatial compound imaging. Compared with image A, fewer artefacts,
smoother image (lower image speckles) – clearer tumor margins and clearer contrast with
surrounding structures

limphoma
Figure 15

Transverse shear wave elastogram in a patient with carcinoma of the larynx shows a
metastatic lymph node in the internal jugular chain. The lymph node has relatively higher
stiffness values compared with the reactive lymph node in Fig. 14. The large circle measures
the overall stiffness of the lymph node, and the small circle measures the relatively harder
area within the node.
Lymphadenopathy is quite common, and it is many a time very difficult to differentiate
malignant lymphadenopathy from reactive lymphadenopthy.

The images of the case in discussion show some of the features that favor malignancy like
loss of central hilum, peripheral vasculature, non tapering vessels & high resistance spectral
waveform(R.I.=0.88).

Several other gray scale and color doppler features favor malignancy in a lymph node.

Gray scale parameters that favor malignancy

1. Size : larger – more likely malignant

2. Shape : Round , L/T < 2
3. Heterogenous echotexture
4. Loss of central fatty hilum / thinning of hilum
5. Eccentric / concentric thickening of cortex
6. Microcalcification
7. Necrosis – cystic / Coagulative
8. Ill defined capsular margins - invasion
Color Doppler / Power Doppler features that favor malignancy

1. Peripheral / mixed peripheral–central blood vessels

2. High resistance waveform
3. RI > 0.8, PI > 1.5
4. Abberent vessels – Displaced parent vessels, subcapsular vasculature, unperfused
areas, Non tapering vessels
5. The increase in resistivity in a malignant lymph node is attributed to increased
cellularity within an infiltrated lymph node. However, malignant lymph nodes with
necrotic change may show low resistance flow due to loss in the cellularity following
necrosis & this needs to be kept in mind while interpreting this sign.
6. when used in combination the above signs can help differentiate a malignant
lymphadenopathy from reactive lymphadenpathy and recommend an excision biopsy.

CT
CT

In order to assess pulmonary metastases from RMS, CT of the chest is a mandatory

examination. However, assessment of pulmonary involvement can sometimes be difficult.
Although criteria such as number and size of lesions, morphology (noncalcified, round and
well-defined) and location (inferior lobes, subpleural spaces, branching vessels) have been
applied in adult patients, none has shown 100% specificity. According to the EpSSG
guidelines for evaluating chest CT the following criteria with respect to the diagnosis of
pulmonary metastases should be applied: one pulmonary or one pleural nodule over 10 mm in
diameter, two or more well-defined nodules of 5–10 mm or five or more lesions >5 mm [3].
This comes with the assumption that there is no other medical explanation for these lesions.
When there is a high suspicion that a small lesion is metastatic, and appears to be the only
site of metastatic disease, biopsy may be performed. In the EpSSG protocol, lung biopsy is
not recommended.

With the introduction of multidetector CT (MDCT) the advantage of MRI of being a

multiplanar imaging modality has been overtaken, and by virtue of its underlying physics, CT
is superior to MRI in detecting osseous changes. The drawback of CT in children is the use of
ionizing radiation. Since the seminal papers of Paterson et al. [14] and Brenner et al. [15], we
should be aware of the theoretical risk of CT-induced cancer fatalities and take this into
consideration especially in the work-up of children with cancer, as they have already proven
their tendency to present with a malignancy early in life [14, 15].

MRI

MRI

With its superior ability to depict soft-tissue changes, MRI is the primary imaging modality
in RMS [16]. Although imaging protocols should be tailored for each individual patient, they
should at least consist of axial T1-W and T2-W images (for anatomic detail and assessment
of neurovascular structures), T1-W images perpendicular to the axial plane, and imaging after
gadolinium administration. It is important that at least two series should be identical, one
before and one after contrast agent administration, to be able to discern enhancement.
Contrast-enhanced series are mandatory and ideally be performed with fat saturation.
The imaging characteristics of RMS are relatively nonspecific. Like most soft-tissue tumours
they have intermediate signal intensity on T1-W images (Fig. 6). On T2-W images they tend
to be of intermediate-to-high signal intensity. If the tumour contains a high number of septa it
may have a lobular shape. RMS in general show strong enhancement on postcontrast imaging
(Fig. 6). In very rare instances the tumour may show a predominantly cystic appearance
(Fig. 7). Dynamic series are useful in order to assess tumour vascularity, and to differentiate
between postchemotherapy/surgery residual disease and fibrosis.

CT lymphoma
Parenchymal lung involvement occurs in 1/3 of patients with Hodgkin Almost all have

associated hilar or mediastinal adenopathy Most common manifestation Present in 90-99%

Commonly multiple lymph node groups involved Anterior mediastinal and retrosternal nodes

commonly involved Confined to anterior mediastinum in 40% 20% with mediastinal nodes

have hilar lymphadenopathy also Hilar lymph nodes involved bilaterally in 50%

Bronchovascular form (most common type of involvement) Coarse reticulonodular pattern

contiguous with mediastinum from direct extension from mediastinal nodes along lymphatics

Nodular parenchymal lesions Miliary nodules Endobronchial involvement Lobar atelectasis

secondary to endobronchial obstruction (rare) Atelectasis is very uncommon and almost always

due to an endobronchial lesion Cavitation secondary to necrosis (rare) Subpleural form

Circumscribed subpleural masses Pleural effusion from lymphatic obstruction About 1/3 have

pleural effusions Effusion usually does not contain malignant cells Pneumonic form Diffuse

nonsegmental infiltrate (pneumonic type) Massive lobar infiltrates (30%) Homogeneous

confluent infiltrates with shaggy borders Contain air bronchogram Nodular form Multiple

nodules <1 cm in diameter Extraparenchymal manifestations in the chest Hilar adenopathy is

usually bilateral but asymmetric Anterior mediastinal nodes commonly involved They may

calcify after radiation therapy

CT Neuroblastoma

On CT, the tumor typically is heterogeneous with calcifications seen in 80-90% of cases 2.
Areas of necrosis are of low attenuation.

The tumor morphology is often helpful, with the mass seen insinuating itself beneath the
aorta and lifting it off the vertebral column. It tends to encase vessels and may lead to
compression. Adjacent organs are usually displaced, although in more aggressive tumors
direct invasion of the psoas muscle or kidney can be seen. In the latter, it can make
distinguishing neuroblastoma from Wilms tumor difficult (see neuroblastoma vs. Wilms
tumor).

Lymph node enlargement is often present.

MRI neuroblastoma

MRI is superior to all other modalities in assessing the organ of origin, intracranial or
intraspinal disease and bone marrow disease 2.

 T1: heterogeneous and iso to hypointense

 T2
o heterogeneous and hyperintense
o cystic/necrotic areas very high intensity
o signal voids may be evident
 C+ (Gd): variable and heterogeneous enhancement

MRI lymphoma
Imaging characteristics will depend on the location and subtype of lymphoma. CT is the
workhorse of imaging in lymphoma and plays a crucial role in staging (see main article:
lymphoma staging). US and MRI are also used; for example, when assessing cervical lymph
nodes (US) or CNS lymphoma (MRI). FDG-PET is used for staging and re-staging of
lymphoma.