Brain tumor

Answer 1

Under spage of 15

Answer 2

During the 1st yr of life: Supratentorial tumors predominate most commonly: Choroid plexus complex tumors and teratomas.
In children 1-10 yr of age, Infratentorial tumors predominate, High incidence of juvenile pilocytic astrocytoma, and medulloblastoma.
After 10 yr of age, Supratentorial tumors again predominate, Diffuse astrocytomas most common.
Tumors of the optic pathway and hypothalamus region, the brainstem, and the pineal–midbrain region are more common in children and adolescents than in adults.

Question 3

Brain tumor… Brain tumor… - Pathophysiology

Brain tumors can cause symptoms by:

Answer 3

Brain tumor… - Pathophysiology
Brain tumors can cause symptoms by:
Impingement on normal tissue (usually cranial nerves) An increase in intracranial pressure (obstruction of CSF flow or by a
direct mass effect).
Tumors that obstruct the flow of CSF become quickly symptomatic.
 Lethargy, headache, irritability, anorexia
 Vomiting (particularly in the morning on awakening).,
 Poor school performance, and loss of developmental milestones
In young children with open cranial sutures, an increase in head circumference may occur.
The classic triad of headache, nausea, and vomiting as well as papilledema is associated with midline or infratentorial tumors.

Question 4

Post fosaa = crebellum

Answer 4

Ataxia 
Tremor 
Dysarthria 
Stiff neck 
Papilledema

Question 5

Brainstem

Answer 5

Brainstem primary
Extremity weakness
Cranial nerve signs
Double vision
Facial weakness
Swallowing dysfunction

Question 6

Supratentorial (cortical) lesions:

Answer 6

Supratentorial (cortical) lesions: may preceded with
Subtle changes in personality, mentation, and speech.
Supratentorial tumors are more commonly associated with:
 lateralized deficits (focal motor weakness, focal sensory changes, language disorders)
 Focal seizures, and reflex asymmetry.
Infants with supratentorial tumors may present with premature hand preference.

Answer 7

Suprasellar region tumors and third ventricular region tumors : may manifest initially as neuroendocrine deficits

Question 8

Infratentorial tumors.

Answer 8

Infratentorial tumors. Disorders of equilibrium, gait, and coordination Blurred vision, diplopia, and nystagmus Suprasellar region tumors and third ventricular region tumors : may manifest initially as neuroendocrine deficits
Tumors of the brainstem region may be associated with : Gaze palsy, multiple cranial nerve palsies, and upper motor neuron deficits Cerebellar tumors are associated with ataxia and diminished coordination.
Torticollis may occur in cases of cerebellar tonsil herniation

Question 9

Diencephalon

Answer 9

Diencephalon: (just above the brainstem. The hypothalamus and the thalamus.)
The diencephalic syndrome (Russell’s syndrome): Failure to thrive, emaciation despite normal caloric intake, and inappropriately normal or happy affect,

Question 10

Pineal region tumors

Answer 10

Pineal region tumors
Parinaud syndrome (Dorsal midbrain syndrome) With pineal region tumors
 Paresis of upward gaze,
 (Pseudo- Argyll Robertson pupil): Pupillary caliber reactive to accommodation but not to light
 Nystagmus to convergence and retraction
 Collier’s sign: Eyelid retraction.

Question 11

Nephroblastoma

Answer 11

Nephroblastoma
Wilms tumor is thought to arise from primitive, metanephric blastema, the precursor of a normal kidney.
The mean age at diagnosis is 3 to 3.5 years of age
 No gender predilection is apparent.
 A hereditary form: bilateral presentation and younger age at onset.
 Patients with beckwith- wiedemann syndrome and Denys-Drash syndrome
some other overgrowth syndromes are at increased risk for developing wilms
tumor
 Many congenital anomalies are associated with Wilms tumor, including sporadic aniridia, Hemihypertrophy, and genitourinary abnormalities.

Question 12

Wilms

Answer 12

Abdominal mass:
Discovered by their parents
With no symptoms
Associated symptoms may :
Abdominal pain
Fever
Hypertension
Hematuria

Question 13

Nephroblastoma

Answer 13

The definitive diagnosis of wilms tumor is made by histologic confirmation at the time of either surgical excision or biopsy.

Blastema (head of arrow) : Malignant small round blue cells primitive undifferentiated cells with round to oval hyperchromatic nuclei without prominent nucleoli and scant pink cytoplasm. Immature stroma (curved arrow) : spindle cells nondescript spindle stroma cells with fibroblasts-like features showing spindle nuclei and fibrillary cytoplasm.
Tubules (arrow): a true lumen lined by primitive tall columnar cells

Question 14

CT of wilms

Answer 14

Clow

Question 15

TREATMENT Wilms tumor

Answer 15

The timing of nephrectomy for unilateral, resectable Wilms tumor remains
controversial.  The North American approach is for immediate nephrectomy followed by
adjuvant chemotherapy,  Whereas the European approach is to make a diagnosis by imaging and sometimes
a biopsy, give neoadjuvant chemotherapy followed by surgery and then adjuvant
chemotherapy (plus radiation therapy, as indicated).
The overall survival is comparable regardless of the approach.
The Children’s Oncology Group protocols for Wilms tumor with favorable histologic findings generally include vincristine and actinomycin, with or without doxorubicin.
Radiation therapy is reserved for a minority of patients depending on stage, presence of metastases, response to therapy,

Question 16

Complications of wilms

Answer 16

Survivors of Wilms tumor are at risk for several late effects, including:
 Cardiomyopathy
 Scoliosis
 Hypertension and prehypertension,
 Renal and bladder insufficiency,
 Pulmonary dysfunction,
 Hepatic dysfunction,
 Infertility
 And second malignancies.
 Patients with bilateral wilms tumor are sometimes left with renal insufficiency or
failure.  Female survivors may have complicated pregnancies and deliveries.

Question 17

Neuroblastoma

Answer 17

Nuroblastoma
Neuroblastoma is derived from neural crest cells that form the adrenal medulla and the sympathetic nervous system.
Most common extracranial solid tumor of the childhood. ( in young children)

Question 18

Neuroblastoma … Sites

Answer 18

Neuroblastoma … Sites
In the abdomen:
Adrenal gland:       45%
Retroperitoneal sympathetic ganglia: 25%
Other sites of origin:
Paravertebral ganglia of the chest and neck.

Question 19

Clinical manifestations of Neuroblastoma

Answer 19

In an Infants younger than 1 yr of age also can present in unique fashion, termed stage 4S, with widespread subcutaneous tumor nodules, massive liver involvement, limited bone marrow disease,

Answer 20

Clinical manifestations of Neuroblastoma
Children with localized disease: Asymptomatic at diagnosis
Children with metastases often appear ill: fever, weight loss, and pain, cough, dyspnea, urinary retention, cytopenias, Bluish subcutaneous nodules,
The most common presentation is: abdominal pain or mass, in the flank and is hard, smooth, and nontender.
Paraspinal tumors may invade through the neural foramina and cause spinal cord compression.
Some tumors produce catecholamines that can cause increased sweating and hypertension, profound secretory diarrhea due to release vasoactive intestinal peptide,
In an Infants younger than 1 yr of age also can present in unique fashion, termed stage 4S, with widespread subcutaneous tumor nodules, massive liver involvement, limited bone marrow disease,

Question 21

Clinical manifestations of Neuroblastoma

Answer 21

Clinical manifestations of Neuroblastoma
Children with localized disease: Asymptomatic at diagnosis
Children with metastases often appear ill: fever, weight loss, and pain, cough, dyspnea, urinary retention, cytopenias, Bluish subcutaneous nodules,
The most common presentation is: abdominal pain or mass, in the flank and is hard, smooth, and nontender.
Paraspinal tumors may invade through the neural foramina and cause spinal cord compression.
Some tumors produce catecholamines that can cause increased sweating and hypertension, profound secretory diarrhea due to release vasoactive intestinal peptide,
In an Infants younger than 1 yr of age also can present in unique fashion, termed stage 4S, with widespread subcutaneous tumor nodules, massive liver involvement, limited bone marrow disease,
Clinical manifestations of Neuroblastoma
Horner syndrome (sympathetic):sometimes is seen with neck or apical masses / Miosis, Ptosis, Anhydrosis, Enophthalmous
Paraneoplastic syndromes:
• Including secretory diarrhea, profuse sweating. Paroxysmal
hypertension, Palpitations Flushing, Headache, Hypokalemia
Opsomyoclonus, are associated with neuroblastoma. Dancing eyes and dancing feet
Neuroblastoma may metastasize to liver, bone, bone marrow, and lymph nodes.
Periorbital ecchymoses is a sign of orbital bone involvement. Posterior mediastinal mass

Question 22

Laboratory/imaging studies

Neuroblastoma

Answer 22

A complete blood count Plain films of abdomen: Calcification within abdominal neuroblastoma tumors Urinary Catecholamines (Vanillylmandelic acid; Homovanillic acid) in 90% of neuroblastomas light microscopic, electron microscopic, and immunohistologic examination.
(CT) scan of the chest, abdomen, and pelvis Bone scan Meta iodo benzyl guanidine scan (iobenguane scan and MIBG scan) used to find neuroendocrine tumors, such as neuroblastomas and pheochromocytomas Bilateral bone marrow aspiration and biopsies

Question 23

Neuroblastoma

Answer 23

Bone marrow aspiration

Rosettes of cells surrounding an inner mass of fibrillary material are characteristic of neuroblastoma.

Question 24

Complications

Neuroblastoma

Answer 24

Complications
Spinal cord compression
Children with the opsomyoclonus syndrome may suffer from developmental delay or mental retardation.
Chemotherapy and radiation complications: such as ototoxicity, nephrotoxicity, growth problems, and second malignancies. Neuroblastoma is remarkable for its broad spectrum of clinical prognosis ranging from spontaneous regression to rapid progression and metastasis resulting in death. (1) neurotrophin deprivation, (2) loss of telomerase activity, (3) humoral or cellular immunity and (4) alterations in epigenetic regulation and possibly other mechanisms.
Neuroblastoma may metastasize to multiple organs, including the liver, bone, bone marrow, and lymph nodes.

Question 25

Retinoblastoma

Answer 25

Retinoblastoma:
Is the most common primary malignant intraocular tumor of childhood. there is no gender or race predilection.
Autosomal dominant The average age at diagnosis is 15 mo for bilateral cases, compared with 27 mo for unilateral cases. It is unusual for a child to present with a retinoblastoma after 3 yr of age. Rarely, the tumor is discovered at birth, during adolescence, or even in early adulthood.

Question 26

The clinical manifestations of retinoblastoma:

Answer 26

The initial sign in the majority of patients is: A white pupillary refex (leukocoria). Leukocoria results because of the refection of light of the white tumor. red refex is not present at a routine newborn or well-child examination or in a fash photograph The second most frequent initial sign is: strabismus.
Less-frequent presenting signs is: pseudohypopyon (tumor cells layered inferiorly in front of the iris) caused by tumor seeding in the anterior chamber of the eye, Hyphema (blood layered in front of the iris) secondary to iris neovascularization, vitreous hemorrhage, and signs of orbit

 Decreased vision
 Orbital inflammation
 Pupil irregularity can occur with advancing disease.
 Pain can occur if secondary glaucoma is present.
Only approximately 10% of retinoblastoma cases are detected by routine ophthalmologic screening in the context of a positive family history.

Question 27

retinoblastoma

Answer 27

Indirect ophthalmoscopy with slit-lamp evaluation can detect retinoblastoma tumors, but a complete evaluation requires an examination under general anesthesia by an experienced ophthalmologist to obtain complete visualization of both eyes, which also facilitates photographing and mapping of the tumors. Orbital ultrasonography, CT, or MRI is used to evaluate the extent of intraocular disease and extraocular spread.
Diagnosis
CT or ultrasonography may help to confirm the diagnosis and demonstrate calcification within the mass.
MRI may better detect the presence of an associated pineoblastoma (trilateral retinoblastoma). (Occasionally (~60%), a pineal area (primitive neuroectodermal) tumor is detected in a child with hereditary and bilateral retinoblastoma
MRI allows for better evaluation of optic nerve involvement. A definitive diagnosis occasionally cannot be made, and removal of the eye must be considered to avoid the possibility of lethal metastasis of the tumor.

Question 28

Treatment

Answer 28

Most unilateral disease presents with a solitary, large tumor. Enucleation is performed if there is no potential for the salvage of useful vision.
With bilateral disease: Chemoreduction (systemic chemotherapy ) in combination with focal therapy (laser photocoagulation or cryotherapy) has replaced the traditional approach of enucleation of the more severely affected eye and irradiation of the remaining eye.
If feasible, small tumors: Can be treated with focal therapy with careful follow-up for recurrence or new tumor growth.

Larger tumors: Often respond to multiagent chemotherapy, including carboplatin, vincristine, and etoposide

If this approach fails, external-beam irradiation
Patients treated with radiation tend to develop brain tumors and sarcomas of the head and neck. Secondary cataracts can also develop from radiation. Enucleation may be required for unresponsive or recurrent tumors.