Carotid body tumor natural history, complications and prognosis
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If left untreated, patients with carotid body tumor may progress to develop cranial nerve dysfunction, airway obstruction, and malignant transformation of the tumor. Prognosis is generally good, and the 10-year survival rate of patients is approximately 50%.
Natural History, Complications, and Prognosis
- The symptoms of carotid body tumor usually develop in the fifth decade of life and start with symptoms such as asymptomatic mass in the anterior triangle of the neck.
- It may be clinically silent for years before presenting as a painless mass in the neck.
- The tumor can also present with a pulsating mass in the lateral side of the neck.
- This tumor is a slowly growing mass with the median growth rate of 1.0mm/year.
- The median time needed for the tumor to be doubled in 4.2 years.
- If left untreated, patients with carotid body tumor may progress to develop cranial nerve involvement, airway obstruction, and malignant transformation of the tumor.
- Carotid body tumor growth may cause local damage such as:
- Carotid body tumor may result in neurovascular complications peri and postoperatively.
- These complications include cranial nerve damage as well as carotid vessel hemorrhage.
- Neurovascular complication rate differs according to the Shamblin subtype and the size of the tumor.
- For type I and II, the peri and post-surgical complications are low and around 0% to 3% of the cases.
- For Type III, it may be as high as 7% to 35% of the cases.
- Carotid body tumor may transform into malignant form in almost 10% of the cases.
- There is no accepted guideline on the diagnosis of malignancy via histology of the tumor and it has been observed that pathological examination of the tumor is unable to differentiate between benign and malignant tumor. 
- Malignant carotid body tumor is usually diagnosed by the presence of:
- Prognosis is generally good after complete surgical removal of the tumor.
- In case of malignant metastasis, the follow-up must be done continuously, however, recurrence or metastasis may occur years later.
- The 10-year survival rate of patients with malignant paraganglioma is approximately 50%.
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