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Childhood Tumors
Thursday, October 31, 2024 9:06 PM

Wilms Tumor

Wilms' Nephroblastoma is one of the most common childhood malignancies

Typically presents in children under 5 years of age with a median age of 3YO

Associations:
• Beckwith Wiedemann Syndrome
• Part of WAGR Syndrome:
○ W – ilms Tumor
○ A – niridia
○ G – enitourinary Malformations
○ R – etardation
• Hemihypertrophy
• Around one-third of cases are associated with a loss of function mutation in
the
WT1 Gene on Chromosome 11

Features:
• Abdominal Mass (Most Common Presenting Features)
• Painless Hematuria
• Flank Pain
• Other Features include Anorexia and Fever

Refer children for possible Wilm's Tumor if they have an unexplained enlarged
abdominal mass within 48 Hours

Management:
• Nephrectomy
• Chemotherapy
• Radiotherapy if advanced disease
• Prognosis: Good

Retinoblastoma

What is it?
• Most common ocular malignancy found in children
• Average age of diagnosis is 18 months

Pathophysiology
• Autosomal Dominant
• Caused by a loss of function of the retinoblastoma tumor suppressor gene
on Chromosome 13
• Around 10% of cases are hereditary

Possible Features:
• Absence of red-reflex, replaced by a white pupil (LEUKOCORIA)
• Strabismus
• Visual Problems

Management:
• Depending on how advanced the tumor is, options can include
○ External Beam Radiation Therapy
○ Chemotherapy
○ Photocoagulation
• Enucleation is an option as well

Prognosis:
• >90% survival til adulthood

Acute Lymphoblastic Leukemia

What is it?
• The most common malignancy affecting children
• Peak incidence at around 2-5 years of age

Features:
• Features may be divided into those predictable by bone marrow failure:
○ Anemia: Lethargy and Pallor
○ Neutropenia: Frequent or Severe infections

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○ Thrombocytopenia: Easy Bruising, Petechiae
• Other Features:
○ Bone Pain
○ Splenomegaly
○ Hepatomegaly
○ Fever is present in up to 50% of new cases
○ Testicular Swelling

Types of Acute Lymphoblastic Leukemia:

• Common ALL – CD10 Present, Pre-B Phenotype
• T-Cell ALL
• B-Cell ALL

Poor Prognostic Factors

• Age < 2 Years or > 10 Years
• WBC > 20 x 10^9
• T or B Cell Surface Markers
• Non-Caucasian
• Male

Neck Masses in Children

Thyroglossal Cyst
• Located in the anterior triangle, usually in the midline and below
the hyoid
• Derived from remnants of the Thyroglossal Duct
• Thin walled and anechoic on USS

Branchial Cyst
• Six branchial arches separated by Branchial Clefts
• Incomplete obliteration of the branchial apparatus may result in cysts,
sinuses or fistulae
• Usually located anterior to the Sternocleidomastoid, near the angle of
the mandible
• Unless infected, the fluid of the cyst will be anechoic on USS

Dermoid Cyst
• Derived from pluripotent stem cells and are located in the midline
• Most commonly in a suprahyoid location
• They have heterogenous appearances on imaging and contain variable
amounts of calcium and fat

Thyroid Gland Mass

• True thyroid lesions are rare in children, and usually represent
thyroglossal cysts or tumors like lymphoma

Lymphatic Malformations
• Usually located posterior to the Sternocleidomastoid
• Cystic Hygroma results from occlusion of lymphatic channels
• The painless, fluid filled, lesions usually present prior to the age of 2
• They are often closely linked to surrounding structures and surgical
removal is difficult
• They are typically hypoechoic on USS

Infantile Hemangioma
• May present in either triangle of the neck
• Grows rapidly initially and then will often spontaneously regress
• Plain X-Rays will show a mass lesion --> Contains calcified Phleboliths
○ Phleboliths --> Small round lumps of calcium

Lymphadenopathy
• May be located in either triangle of the neck
• May be reactive / neoplastic
• Generalized lymphadenopathy

Brain Tumors in Children

What are they?

• Abnormal growths of cells in the brain that occur in children, they can be
benign or malignant.
• Can arise from the various structures within the brain:
○ Astrocytes -> Astrocytomas
○ Meninges -> Meningiomas
○ Cells of the Ventricular System -> Ependymomas
○ Pituitary Gland -> Craniopharyngiomas

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Etiology:
• Majority of Pediatric Brain Tumors, the Etiology is unclear.
• Believed to be a combination of genetic, environmental, and lifestyle factors
play a role in their development.

Classification:

Tumour Type Features

Medulloblastoma Most Common Malignant Brain Tumor in children
Typically arises in the cerebellum
Symptoms: Headache, Vomiting, Ataxia, and Cranial
Nerve Deficits
Pilocytic Astrocytoma Most common benign brain tumour in children
Often found in the cerebellum or optic pathway
Symptoms: Headache, Nausea, Visual Disturbances, and
Balance Issues
Ependymoma Arises from ependymal cells lining the ventricles or
central canal of the spinal cord.
Common locations: Posterior Fossa, and Spinal Cord
Symptoms: Hydrocephalus, Headache, Nausea, and
Balance Issues
Craniopharyngioma Benign tumour near the pituitary gland and
hypothalamus.
Symptoms: Endocrine Dysfunction, Vision Problems, and
Growth Delays

Signs and Symptoms:

• Clinical presentations vary with the specific tumor, but common symptoms
include:
○ Persistent headaches worse in the morning
○ Signs of Raised ICP such as nausea, vomiting, and altered
consciousness
○ Vision Changes (Blurring or Double Vision)
○ Seizure in an older child with no fever and no previous history of
seizures
○ Depending on the location of the tumour, patients may present with
focal neurological deficits.

Differentials to consider:
• Migraine – Chronic Headaches, often unilateral, accompanied by
photophobia, phonophobia, nausea, and/or vomiting
• Intracranial Hypertension – Headache, Vision Changes, and Signs of
Increased Intracranial Pressure.
• Epilepsy – Recurrent seizures, with or without a fever.
• Meningitis – Fever, Headache, Neck Stiffness, and Photophobia

Investigations:
• Child with newly abnormal cerebellar or neurologic function requires a very
urgent referral for a suspected brain cancer
• Key investigations for a suspected paediatric brain tumor include:
○ MRI or CT Imaging of the brain to visualize any space occupying
lesions and characterize their location, size and potential type
○ Lumbar Puncture may be necessary in some cases for obtaining
cerebrospinal fluid for examination
○ Biopsy: May be required to definitively diagnose the tumour type and
grade

Management:
• Depends on Site, Size, Type, and Stage
• Management often include:
○ Specialist input and MDT Approach
○ Steroids to reduce intracranial swelling
○ Anticonvulsants may be used to prevent seizures
○ Chemotherapy may be used to shrink tumour before surgery or to kill
remaining cancer cells after surgery
○ Radiotherapy – Used as a primary treatment adjuvant to surgery, or
for tumour that are not amenable to surgery
○ Surgical Intervention – Complete or Partial Removal of the Tumour.

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