Paraganglioma is a rare endocrine tumour, which develops in the extra-adrenal paraganglion tissue. Moreover, their occurrence is very common in carotid bifurcation where they are called carotid body tumours. However, most paragangliomas are benign, aggressive locally, and infiltrative tumours. Statistically, distant metastasis is seen in 10% of the patients, 10% have multiple or bilateral tumours (usually carotid) and 10% have a family history. Additionally, malignant transformation tumours of the carotid body are reported in 6% of cases.
Case Presentation
This article is about a case where a 64-year-old Caucasian woman presented with a giant gliomic tumour in her neck. Moreover, she had a history of undergoing an unsuccessful surgery to remove the mass, twenty years ago. The patient also complained that her mass is becoming bigger drastically, almost double the previous size.
The doctors did an angio magnetic resonance imaging, which revealed a 9cm paratracheal mass on the left cervical side. Furthermore, it laterally displaced the sternocleidomastoid muscle and 2m of the trachea.
Since a change was seen in the behaviour of the tumour, the team of maxillofacial surgeons at the hospital decided to remove it surgically through embolization. They gently dissected the tumour from the carotid, and it was removed from the bifurcation without any complications. Furthermore, two small nodes that adhered tightly to the internal carotid adventitia and posterior torn hole were left in place. This decision was taken to prevent any fatal complications.
Diagnosis Confirmation of Paraganglioma
The last biopsy conducted confirmed the diagnosis of carotid body paraganglioma. Moreover, a Ki-67 expression of 19% was also seen. However, due to the aggressive growth behaviour of the tumour and high Ki-67 expression, the patient was further referred for CyberKnife for the remaining treatment of the nodes.
Conclusion
The management of cervical paragangliomas is very challenging. However, the likeliness of tumour control surgically is high. Moreover, radiotherapy is also beneficial but there is no best treatment option available currently. The case presented is a combination of both to allow complete recovery and reduce fatality risk.
