Embryonal Tumours(3)
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Transcript of Embryonal Tumours(3)
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Ain Shams University
Faculty of Medicine
Pathology Department
2009/2010
Embryonic tumors
Names:
1- Ahmed Yaser 1520
2- Osama 1521
3- Eslam Mubarak Mohamed 1522
4- Asmaa Ahmed Mhmoud 1523
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Definition of Embryonic tumors
This broad group of childhood tumours crosses over the standard classification,
because embryonal tumours
They are characterised by the proliferation of tissue that is normally seen only in the
developing embryo. They are generally very rare after childhood, and occur most
commonly in the first few years of life. Several types of embryonal tumour have a
peak of incidence in the first year of life. Some types are occasionally found to be
present at birth.
They occur as:
1. Wilms tumour (nephroblastoma) in the kidney
2. retinoblastoma in the retina
3. hepatoblastoma in the liver
4. medulloblastoma in the cerebellum
5. neuroblastoma in the adrenal medulla
6. embryonal rhabdomyosarcoma in soft tissue
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Nephroblastoma "Wilms' tumour"
Definition:
Wilms' tumor is a rare kidney cancer that primarily affects children. Also known as
nephroblastoma, it's the most common malignant tumor of the kidneys in children.
The peak time of Wilms' tumor occurrence is around ages 3 to 4, and it occurs only
rarely after age 6.
Although Wilms' tumor can occur in both kidneys, it tends to affect just one kidney.
Wilms' tumor is believed to develop from immature kidney cells.
Improved imaging techniques help doctors to determine the extent of the cancer in
Wilms' tumor and to plan treatment. The outlook for most children with Wilms' tumor
is very good.
Gross picture:
synchronous or metachronous bilateral involvement in 510%
Usually:
o Solitary ,well circumscribed ,rounded ,soft consistency
Size variable (median 550 g)
Cut section:
o predominantly solid and pale gray or tan gray
o often cystic change, necrosis, and hemorrhage
Microscopic picture:
Three major components:
1. Blastematous areas:
o
extremely cellular& small round-to-oval primitive cellso cytoplasm usually scanty& sometimes oncocytoid appearance
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o growth pattern may be diffuse ,nodular ,cordlike (serpentine) or
basaloid (with peripheral palisading)
2. Mesenchymal elements:
usually a spindle cell fibroblast-like configuration may be differentiation toward various cell types, particularly smooth
and skeletal muscle
sometimes predominate almost to the exclusion of other components
3. Epithelial component:
embryonic tubular (and sometimes glomerular) structures that closely
recapitulate the appearance of normal developing metanephric tubules (and
glomeruli)
differentiation can be so pronounced that may be tumor analogs of
nearly all segments of normal nephron
these tubular structures can be small and round, simulating the rosettes
of neuroblastoma features favoring tubules over basal lamina are: A lumen, single cell
layer,distinct basal lamina &surrounding fibromyxoid stroma
exceptionally, marked hydropic changes in the tubular epithelium
o Papillonodular type grossly evident projections extend from the septa
into the cyst lumina &appearance on low power may be fibroadenoma like
o May be focal or extensive anaplastic features
o Possible additional features:ciliated, mucinous, squamous, or
transitional epithelium & renin-producing cells
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Diagnosis:
Several tests are used to confirm a Wilms tumor diagnosis and determine the stage of
the disease. Tests that might be used include:
Ultrasonography uses sound waves instead of X-rays to generate animage of the area doctors wish to view.
Computed tomography produces a detailed cross-sectional view of
an organ through X-rays
Magnetic resonance imaging (MRI) uses radio waves and strong
magnets to produce detailed pictures of the internal parts of the body.
X-rays are used to look for any metastasized areas, especially in the
lungs.
Bone scans use small amounts of radioactive material to highlight
areas of diseased bone.
Laboratory tests such as blood tests and urinalysis check the general
health of a patient and to detect any adverse side effects (such as low red or
white blood cell counts) of the treatment.
A physical examination. The doctor will look for possible signs of
Wilms' tumor.
Differential diagnosis: neuroblastoma - no triphasic patterns; has rosettes with no lumen, >1
cell layer, no distinct basal lamina, in contrast to Wilms immature tubules
which have a lumen, a single cell layer, distinct basal lamina and surrounding
fibromyxoid stroma perilobar nephrogenic rest - no fibrous capsule
renal cell carcinoma may resemble epithelial predominant Wilms
small blue cell tumors - if blastema predominates
Special Stains and Immunohistochemistry
Immunohistochemical profiles of various components mirror those of
their counterparts in the developing kidney, including highly specialized
compounds such as transport mediators
o
blastematous elements show only: focal positivity for vimentino epithelial elements react for :keratin ,EMA ,various lectins ,various
components of basement membrane
o mesenchymal elements show reactivity consonant with their
morphologic appearance such as: positivity for myogenin and desmin in
rhabdomyoblastomatous foci
o neural elements (when present) are reactive for: neuron-specific
enolase, glial fibrillary acidic protein, S-100 protein, Type I insulin-like
growth factor receptors:have been found & may be responsible for:
increased proliferation& inhibition of differentiation
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Retinoblastoma:
Definition:
Retinoblastoma (Rb) is a rapidly developing cancer which develops in the cells of the
retina, the light detecting tissue of the eye[1]. In the developed world, Rb has one of
the best cure rates of all childhood cancers (95-98%), with more than nine out of
every ten sufferers surviving into adulthood. Retinoblastoma is a very treatable
cancer.
Gross pictures-:In the gross pathology of the eye, we should comment of the following:
-The size of the tumour-The location of the tumour (endophytic or exophytic; single or multiple; any vitreal
seedings(
-Any spread outside the globe (especially of the optic nerve. In optic nerve
involvement, there may be abnormal optic nerve enlargement or tumour surrounding
the nerve. Extraocular spread is of great prognostic value
Microscopic pictures:Under the microscope, retinoblastoma contains deep blue cells with little cytoplasm.
As the tumour cells usually outgrow their blood supplies, necrosis and haemorrhage
are common within the tumour
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Diagnosis:-
Retinoblastoma can be diagnosed by an eye examination, supported by imaging
studies. There are no blood tests available to diagnose retinoblastoma.
The following tests and procedures may be used:
History: The doctor will first seek to know if there is a family history of
retinoblastoma.
A physical examination of the person will be carried to check for general signs of
disease.
An eye examination will be carried out on the person by dilating the pupils with
medicated drops to look at the retina through the lens and pupil. In the case of
children, this exam may be done under anesthesia.
Ultrasound examination may be carried
CT scan: This procedure is also called computerized tomography. Detailed pictures
of the inside of the eye can be taken, from different angles, to define the extent of the
tumor
Magnetic resonance imaging : This procedure, that uses a magnet, radio waves,
and a compute, is also used to make detailed pictures of the eye. This procedure is
also called nuclear magnetic resonance imaging (NMRI).
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A chest x-ray, bone marrow biopsy and/or a CT scan of the chest, may be carried
out. A biopsy is usually not required to diagnose retinoblastoma.
If the disease is extensive, a lumbar puncture is carried out to examine the
cerebrospinal fluid for the degree of damage
Hepatoblastoma
Definition:
Hepatoblastoma is the most common malignant liver tumor in early childhood. Mostpatients present before the age of 3 years with an enlarging asymptomatic abdominal
mass. Some patients have fever, pain, anorexia, and weight loss
Gross picture
On gross inspection, the epithelial type tends to be homogenous, while mixed
epithelial-mesenchymal tumors demonstrate a more variegated appearance with areas
of osteoid, cartilage, calcification, fibrosis, necrosis, and hemorrhage. The anaplastic
variant frequently contains a large focus of central necrosis. Microscopic vascular
invasion may be seen beyond an apparently encapsulated tumor
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Microscopic picture
Hepatoblastoma can be classified into a range of subgroups, although the prognosticsignificance of these are unclear : 75% are epithelial (fetal and embryonal types) and
the remainder are mixed epithelial or mesenchymal (figure 1). At the present time, no
stratification is made on the basis of pathology ; however, patients with pure fetal
histology, particulary those with small localised tumours may have a better outcome.
Diagnosis
In addition to a complete medical history and physical examination, diagnostic
procedures for hepatoblastoma may include:
Biopsy a sample of tissue is removed from the tumor and examined under a
microscope.
Complete blood count (CBC) a measurement of size, number and maturity
of different blood cells in a specific volume of blood.
Additional blood tests may include blood chemistries, evaluation of liver
and kidney functions and genetic studies.
Multiple imaging studies, including:
o Computed tomography scanuses a combination of X-rays and
computer technology to produce cross-sectional images, both
horizontally and vertically, of the body. A CT scan shows detailed
images of any part of the body, including the bones, muscles, fat and
organs. CT scans are more detailed than general X-rays.
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o Magnetic resonance imaging (MRI) uses a combination of large
magnets, radio frequencies and a computer to produce detailed images of
organs and structures within the body.
o X-ray uses invisible electromagnetic energy beams to produce
images of internal tissues, bones and organs onto film.
o Ultrasounduses high-frequency sound waves and a computer to
create images of blood vessels, tissues and organs. Ultrasounds are used
to view internal organs as they function and to assess blood flow through
various vessels.
o Bone scans pictures or X-rays taken of the bone after a dye has been
injected that is absorbed by bone tissue. These are used to detect tumors
and bone abnormalities.
Alpha-fetoprotein test alpha-fetoprotein levels in the blood
Recurrent the disease has returned after it has been treated. It may come
back in the liver or in another part of the body.
References
www.cancer.gov
http://medsavailable.com
http://www.pathconsultddx.com
http://peir2.path.uab.edu
http://www.wikipedia.org
http://emedicine.medscape.com
http://www.md.ucl.ac.be/pedihepa/Liver%20tumours.htm
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http://www.cancer.gov/http://medsavailable.com/http://www.pathconsultddx.com/http://peir2.path.uab.edu/http://www.wikipedia.org/http://emedicine.medscape.com/http://www.md.ucl.ac.be/pedihepa/Liver%20tumours.htmhttp://www.cancer.gov/http://medsavailable.com/http://www.pathconsultddx.com/http://peir2.path.uab.edu/http://www.wikipedia.org/http://emedicine.medscape.com/http://www.md.ucl.ac.be/pedihepa/Liver%20tumours.htm -
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