Introduction

Teratomas are tumour masses arising from uncontrolled growth of cells derived from the three primary germ layers: the endoderm, mesoderm, or ectoderm. They are typically benign (non-cancerous) and painless.

Cervical teratomas are benign masses located in the neck region. The precise location can vary between individuals; however, the most common sites are the anterior neck, including the submandibular and thyroid regions. Teratomas may also develop along the lateral aspects of the neck or elsewhere within the upper respiratory tract.

Prevalence

Cervical teratomas are typically congenital, developing during the embryonic stage, with a reported prevalence of approximately 1 in 20,000 births.¹ They affect males and females equally,² and fewer than 50 cases of cervical teratoma have been diagnosed after childhood.

Aetiology and Pathophysiology

The exact cause of cervical teratoma remains unknown. The underlying mechanism involves uncontrolled division of germ cells,³ whereby cells continue to proliferate without regulation, likely due to a genetic malfunction in the cell growth regulatory pathway. Although these growths are benign, they may undergo malignant transformation as the child ages.

Features and Characteristics of Cervical Teratoma

The characteristics of teratomas differ depending on their type:

Mature Teratoma

Mature teratomas are generally benign with a relatively low risk of malignancy. They are well differentiated and have a cystic appearance, typically presenting as fluid-filled masses.

Immature Teratoma

Immature teratomas are solid masses with a higher risk of malignancy. They are poorly differentiated.

Complications

Cervical teratomas can give rise to a range of serious complications, particularly in foetuses and neonates:

• Airway obstruction: Tumour mass may obstruct the respiratory tract, leading to breathing and swallowing difficulties
• Respiratory failure: Severe airway obstruction can result in cyanosis and respiratory failure in foetuses and neonates
• Hypoxia and tissue ischaemia: Prolonged oxygen deficiency causes hypoxia, which can progress to tissue ischaemia; the brain and heart are particularly vulnerable, and encephalopathy and acidosis may also occur
• Impaired lung development: During the foetal stage, the lungs are immature, and the presence of a teratoma can cause neck hyperextension, disturbing fluid dynamics and impairing the foetus's ability to swallow amniotic fluid (polyhydramnios), which may lead to premature birth and respiratory failure

Diagnosis

The diagnosis of cervical teratoma is typically established through evaluation of abnormalities identified during routine pregnancy ultrasound. Findings such as masses and fluid accumulation may raise clinical suspicion, which can be further investigated using Magnetic Resonance Imaging (MRI) for more detailed visualisation.

Fine needle aspiration (FNA) may be performed to obtain tissue samples for histological examination. Histological findings typically include assessment of cell differentiation and calcification patterns.

Laboratory tumour markers, including Alpha-fetoprotein (AFP) and beta-human chorionic gonadotrophin (β-hCG), are used to evaluate the risk of malignancy.

Treatment

The treatment of cervical teratoma requires a multidisciplinary approach involving a team of specialists with clearly defined roles. Multidisciplinary management involves coordinated teamwork for patient care and survival, encompassing evaluation, planning, and execution of procedures and treatment strategies. Core team members include:

• Gynaecologist
• Obstetrician
• Radiologist
• Paediatric specialist
• Paediatric surgeon
• Anaesthesiologist
• ENT specialist
• Pathologist
• Oncologist
• Paediatric ICU supportive staff
• Nursing staff
• Pharmacist
• Psychologist

Treatment strategies are determined based on the diagnosis, the stage of presentation (foetal, perinatal, or neonatal), and the severity of airway obstruction.

Foetal Stage

If a cervical teratoma is identified during routine pregnancy ultrasound, immediate action is taken to prevent further complications. Foetal MRI is performed to assess the extent of teratoma growth, airway obstruction, and fluid accumulation (polyhydramnios). Parents are counselled regarding possible risks, surgical options, and management strategies. Many cases are not detected on routine ultrasound, particularly during early pregnancy.

Perinatal Stage

If airway obstruction at birth is anticipated, an Ex Utero Intrapartum Treatment (EXIT) procedure⁴ is performed. A caesarean section is carried out, and only the foetal head and neck are delivered while the placenta remains intact to maintain oxygenation. Endotracheal intubation is then performed. Once the infant's condition is stabilised and adequate ventilation is achieved, the placenta is delivered and birth is completed. This procedure requires highly qualified and experienced personnel.

Neonatal Stage

Following birth, once the infant has been stabilised, surgical resection of the teratoma is performed. Further supportive care is provided as required.

Postnatal Stage

In some cases, teratoma growth is very slow and goes undetected during pregnancy. Even when present, if the mass is not large enough to obstruct the airway, diagnosis may be delayed. Once growth occurs and obstruction develops, surgical intervention becomes necessary.

Supportive Therapies

Supportive therapies may be employed to sustain the patient until surgery is performed. These include:

• Endotracheal intubation for respiratory support
• Orogastric or nasogastric tubes for feeding
• Positional adjustments or neck extension to ease breathing and swallowing
• Prophylactic medications such as corticosteroids and antibiotics to prevent infection and inflammation

It should be noted that supportive therapies, including physiotherapy or medications, are not alternatives to surgery. They are solely intended to stabilise the patient prior to the surgical procedure.

Complications of Teratoma Surgery

Neonates and infants have incompletely developed immune systems, which places them at increased risk of bacteraemia and sepsis. Surgical risks also include damage to key vascular structures (carotid arteries, jugular veins, and thyroid vessels) and nerves (including the laryngeal nerve, facial nerve, accessory nerve, and hypoglossal nerve). There is also a risk of tumour recurrence if any residual tissue remains following resection.

Follow-up

Regular follow-up is essential for patient safety for a minimum of two years following surgery. The following parameters should be monitored to identify and manage potential complications:

• Ultrasound or MRI if incomplete resection is suspected
• Chest X-ray if respiratory problems persist
• Full blood count (FBC) to screen for infection
• Electrolytes
• Thyroid function tests
• Tumour markers (AFP, β-hCG) in cases where malignancy is suspected.⁵ Given reported cases of post-surgical malignancy, ongoing monitoring of these markers is strongly advisable
• ENT follow-up for airway assessment
• Speech therapy for swallowing difficulties
• Physiotherapy if neck muscle weakness is present

FAQs

Are teratomas cancer?

Teratomas are not initially cancerous; however, if left untreated, they may progress to malignancy.

Are teratomas curable?

Surgical removal is the only definitive treatment, with a survival rate of over 80%. If the tumour has metastasised, chemotherapy may be initiated.

What is the size of a cervical teratoma?

The size and location of teratomas vary between individuals. A small teratoma may range from approximately 2 to 10 cm in diameter.

Summary

Cervical teratoma is a rare congenital disorder characterised by uncontrolled cell division leading to the formation of a tumour mass anywhere along the upper respiratory tract, most commonly in the cervical region of the neck. Early diagnosis is achievable through routine pregnancy ultrasound, followed by detailed MRI assessment. The size and extent of the tumour are evaluated to determine the degree of airway obstruction and the need for surgical intervention. Surgical resection remains the only definitive treatment, with a reported survival rate of approximately 80%. A multidisciplinary team approach is essential across all stages of management to optimise outcomes for both the foetus and the neonate.