1. Tumours of the head & neck in
childhood
Epidemiology
After trauma, cancer is the most common cause of death in childhood.
1. Approximately one-third of childhood malignancies are leukaemias
2. 25% are brain & spinal tumours,
3. 15% are embryonal (neuroblastoma, retinoblastoma, Wilm’s tumour & hepatoblastoma).
4. 11% are lymphomas.
5. The remainder are bone & soft tissue sarcomas.
It is thought that only around 5% of malignancies in childhood are inherited. The underlying
aetiology of the non-herited types remains unclear but certain chemotherapeutic agents ,
viruses(Epstein-Barr virus) & irradiation are known to play a role.other potential carcinogens are
suspected but not proven (pollution, parental exposure to toxins, electromagnetic fields).
Presentation & assessment
The most common presentation of a malignancy in the head & neck region in childhood is the
asymptomatic mass. Otalgia , rhinrrhoea, otorrhoea & nasal obstruction may be present in both
benign & malignant disease.
More worrying symptoms would be include stridor, dysphagia & haemoptysis.
Reactive lymphadenopathy in children are extremely common.
Investigation
FNAC has limited role in childhood tumour. With particular regard to cervical lymphadenopathy, the
most likely neoplastic diagnosis is lymphoma, excision biosy is the best method of confirming &
typing this histology.
USG, CT &MRI can all have their part to play in further assessment.PET scanning is also becoming
increasing used in the assessment of neoplastic lesions in the head & neck in the children.
1)Lymphoma
Cervical lymphadenopathy in childhood is common & although the huge majority of cases will be
due to reactive hyperplasia. It may necessary to exclude malignancy. Lymph nodes in the neck larger
than 2cm are unusual in childhood & systemic symptoms such as weight loss, fever & organomegaly
are usually indicators of serious pathology. FNAC has limited role but excision biopsy will provide the
diagnosis.

2. Hodgkin’s lymphoma
This is distinguished by morphologically by presence of Reed Sternberg cells which are large &
multinucleated with abundant cytoplasma. Rye classification are four types;
1. Lymphocyte predominant;
2. Mixed cellularity;
3. Lymphocyte depleted;
4. Nodular sclerosis.
Nodular sclerosis is the most common type found in children & young adults.
No definitive causal factors have been identified but there is an association with previous infection
with Epstein-Barr virus.
Hodgkin’s lymphoma most commonly presents with lymphadenopathy in the neck & 2/3 rd of all
children will have mediastinal lymphadenopathy at presentation. It is rarely occur under the age of
five & there is a male predominance.
Constitutional upsets such as fever, night sweats & weight loss are present in 25-30% & this is
associated with poor prognosis.
Biopsy confirmation would include chest x-ray routine blood test,( although abnormal result usually
nonspecific) staging scans (CT chest & MRI abdomen).
Recently PET scanning has become a routine part of staging & assessing effectiveness of treatment.
Bone marrow biopsy & bone scan are only indicated in children with more advanced disease.
Currently , the Ann Arbor staging classification is used for Hodgkin’s disease.
Non-Hodgkin’s lymphoma
Approximately 60% of paediatric lymphoma are Non-Hogkin’s lymphoma. Classification into three
main categories.
1. Lymphoblastic lymphoma(predominantly of T cell origin).
2. Small non-cleaved cell lymphoma( B cell origin);
3. Large cell lymphoma.
In some part of world, an extremely high number of these tumour are positive for Epstein-Barr
virus.(Burkitt’s lymphoma).
Diagnosis & staging
Full blood count, blood urea & electrolytes & liver function tests.
USG, CT scanning of chest, MRI of abdomen are used for staging.

3. 2)Rhabdomyasarcoma
Rhabdomyosarcoma account for up to 60% of all sarcomas in the paediatric population & 40% occur
in the head & neck region. Nearly half of these tumours occur in children under the age of five. The
prognosis of this tumour is extremely poor.
Histologically ,rhobdomyosarcoma resemble normal foetal skeletal muscle before innervations. Two
types are identified & these are embryonal( good prognosis) & alveolar (poor prognosis). Alveolar
type is found in older children & is associated with metastatic spread. It is less common than the
embryonal type.
Presentation
Rhabdomysarcoma of the head & neck occur most frequently in the orbit or parameningeal sites &
these include the paranasal sinuses, nose, nasophargeal & middle ear.
The most common presenting symptoms are pain & swelling.
1. Paranasal rhadomysarcoma may present with a gradual onset of nasal obstruction &
bloody nasal discharge.
2. Tumours within the ear may present with bloody discharge, persistent otalgia, despite
treatment. A polypoid mass may be seen in the ear canal or nasal cavity.
3. Metastases occur by both haematogenous & lymphatic spread.
Assessment
Assessment should include a through examination of the upper respiratory tract , head & neck
region including the cranial nerves. Flexible nasoendoscopy may be employed. CT or MRI may be
performed to evaluate the primary disease.
Staging of rhabdomyosarcoma
Group I Localised disease completely resected. No
regional nodes.
Confined to muscle or organ of origin.
Contiguous infiltration outside muscle or organ
of origin.
Group II Localised disease with microscopic disease.
Group III Incomplete resection or biopsy with gross
residual disease.
Group IV Metastasis disease present at onset.

4. Treatment
In general , the role of surgery today is to simply evaluate the extent of the lesion & biopsy of the
lesion. A debulking procedure may be helpful. Muiltiagent chemotherapy & radiotherapy are then
implemented as appropriate.
3)Medullary carcinoma of thyroid
The detection of MTC in younger children is usually made following screening in high risk individuals
who have a family history of MEN2. This is cinfirmed by elevated baseline levels of calcitonin or
screening for the Ret protooncogene on chromosome 10. If positive , the child should be considered
for prophylactic total thyroidectomy.
4)Neuroblastoma
Neuroblastoma is a common malignancy of early childhood & is the most common malignancy in
infants younger than one year. These tumours arise from undifferentiated sympathetic nervous
system precursor cells of neural crest origin. The adrenal gland is the most common site of origin &
additional sites include the sympathetic chain.
Neuroblastoma has a high tendency for lymphatic spread. Metastases to the head neck region are
common, but primary neuroblastoma in head & neck region is uncommon.
Presentation
Children with primary cervical neuroblastoma may present with a firm mass in the lateral neck,
occasionally associated with a Horner’s syndrome. Classical opthalmological manifestations include
proptosis & periorbital ecchymosis(usually secondary to intraorbital metastatic deposits.) bilateral
eye haematomas are a classical sign(recoon eyes).
Assessment
Biopsy, CT , bone scanning, uninary catecholamine levels should be measured ( 24 hour urine
collection) as they raised in over 90% of cases.
Treatment
Localized cervical neuroblastoma may be treated by curative surgery. Multiagent chemotherapy is
uasally indicated in patients if resection is incomplete.
5)Nasopharyngeal carcinoma
There is a bimodal age distribution of this disease with an early peak of 10 tp 20 years & second peak
between 40-60years. NPC is one of few malignant tumours in childhood that emerges from the
epithelium& there is an association with EBV. Male are twice as likely as female to develop NPC.
Undifferentiated NPC is a radiosensitive tumour & as such as is usually treated with external beam
radiotherapy. Such therapy is limited to the primary tumour & its regional metastasis.
Chemotherapy is required in patients with disseminated systemic disaease. There is now evidence
that combined chemotherapy & radiotherapy provides better disease –free survival as compared
with radiotherapy alone. Combined chemoradiotherapy is therefore becoming routine practice in
UK.

5. Best clinical practice
1. The vast majority of enlarged cervical lymph nodes in children are harmless. Imaging should be
considered before biopsy. Excision biopsy may be appropriate for:
-node >2cm
- supraclavicular area & / or fixed nodes;
-weight loss& / or unexplained fever;
-abnormal chest x-ray.
2. FNAC has limited role to play in the children than adult.
3. all children diagnosed with malignancy should be referred to a specialist centre.
4. children receiving chemotherapy should have central venous access & chemotherapy treatment.

6. Best clinical practice
1. The vast majority of enlarged cervical lymph nodes in children are harmless. Imaging should be
considered before biopsy. Excision biopsy may be appropriate for:
-node >2cm
- supraclavicular area & / or fixed nodes;
-weight loss& / or unexplained fever;
-abnormal chest x-ray.
2. FNAC has limited role to play in the children than adult.
3. all children diagnosed with malignancy should be referred to a specialist centre.
4. children receiving chemotherapy should have central venous access & chemotherapy treatment.