Rare tumors of the neck

Rare tumours of the neck

Head and neck is a region of complex anatomy. Due to this there are many rare tumours which can occur in this region.

These tumours include:-

1. Neurogenic tumours and Paraganglioma – These arise from the vicinity of major nerves and blood vessels in the neck. It further includes :
2. Schwannoma – These arise from neuroectodermal sheath surrounding peripheral nerves.
3. Neurofibromas – These are benign, unencapsulated tumours that can arise anywhere in the bod.
4. Malignant peripheral nerve sheath tumours – These arise de novo or from a pre-existing neurofibroma (NF1)
5. Paraganglioma– These arise from neural cell aggregates that are present blood vessels and nerves. They are parasympathetic in origin.
6. Carotid body tumour (chemodectomas or nonchromaffinparagangliomas) – It is by far the most common paraganglioma in the neck region.
7. Lipoma / Liposarcoma of neck – Lipoma is benign tumour arising from fat cells in the body. In contrast liposarcoma is a rare malignant tumour that arises in an existing lipoma
8. Dermatofibrosarcomaprotuberans – DFSP is a low grade malignant tumour of fibrous origin most commonly occurring in head and neck region.
9. Sarcoma of the head and neck – Sarcomas are aggressive malignant tumours arising from musculoskeletal tissues
10. Hemangioma of face and neck –Hemangioma are benign tumours arising from the
11. Lymphangioma / cystic hygroma – Benign tumours that arise from lymphatic system in the neck. Cystic hygromas are common benign tumours in children.
12. Leiomyoma and synovial sarcoma – These arise commonly in the larynx and pharynx (throat);leiomyomas are benign tumours while synovial sarcomas are malignant.
13. Odontogenic tumours – These arise in the lower or upper jaw. This includes:-
14. Osteoma
15. Osteogenic sarcoma
16. Odontogenickeratocyst
17. Ameloblastoma mandible/maxilla
18. Dentigerous cyst

What are the possible treatments for rare head and neck tumours?

Surgery:

Surgical procedures are often used to remove the tumour. The extent of surgery depends on the size and location of the tumor. Types of surgery include:

• Open surgery: Most of the tumours are removed using an open approach as size of tumour is generally large at presentation. This means and incision will be made in the neck externally.
• Robotic surgery: for smaller sized tumours, robotic surgery is effective and ensures a scarless surgery.
• Reconstruction surgery: For some tumours, there may be a large loss of tissue at the surgical site. To resolve this tissue from other parts of the body may be harvested and used to reconstruct the area.

Ablative therapy:

Ablative therapy aims to reduce the size and, therefore, the symptoms of the tumour. It is tried in scenarios where a surgical resection is not possible.

Conclusion

Rare tumours present a unique clinical scenario to the head and neck surgeon. Treatment has to be personalised for each patient depending on age, sex and demographics.  Single stage reconstructive surgery allows better outcomes for the patient.