The authors present the clinical case of a malignant paraganglioma of the parapharyngeal region: diagnosis, surgery and treatment with radiotherapy, MIBG-I131, chemotherapy and octreotid. 63 year old woman was referred to our department with a diagnosis of left parapharyngeal glomic tumour to which she previously refused treatment. She complained of sialorrhea, cough, dysphonia, difficulty in articulating words, vomiting and repeated syncopal episodes during the past year. She presented a history of hypertension, anxiety, and a pacemaker for symptomatic bradicardia. Physical exam revealed paralysis of the left vocal cord; slight atrophy of left hemitongue; an area of protrusion of the soft palate on the left; neck palpation on the left side elicited episodes of cough. Cervical CT scan revealed a 5.8 cm × 3.3 cm × 2.7 cm expansive lesion of the left parapharyngeal region with heterogeneous contrast enhancement. AngioCT showed displacement of internal carotid artery medially and anteriorly. Arteriography revealed a highly vascularized tumour, irrigated by the external carotid artery. Scintigraphy MIGB-I123 demonstrated anomalous uptake on the lesion. Urinary and plasma catecholamines and metanephrines presented normal values. Surgery consisted of a left functional neck dissection (levels I–III) to facilitate the resection of the tumour originating on the left hypoglossal nerve, extending from the carotid bifurcation to the cranial base. Pathology confirmed a paraganglioma with vascular invasion and a metastasis in one of eight ganglia. The patient underwent adjuvant radiotherapy, adjuvant MIBG-I131 and palliative chemotherapy. Ten months after adjuvant radiotherapy the patient presented metastatic disease and local recurrence. MIBG-I131 and palliative chemotherapy were performed with remission, however fourteen months latter presented disseminated disease. We present a case of malignant paraganglioma with significant semiology. Functional neck dissection became determinant in the early diagnosis of malignancy. MIBG-I131 and palliative chemotherapy showed satisfactory immediate results although recurrence occurred. paraganglioma,parapharyngeal, hypoglossal.
Malignant paraganglioma of the parapharyngeal region – clinical case
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