Malignant head and neck paragangliomas: Is there an optimal treatment strategy?

Abstract

BackgroundLittle is known about management and prognosis for malignant head & neck paragangliomas. We reviewed records of these patients to determine optimal treatment strategies.MethodsWe reviewed 113 cases of head & neck paragangliomas treated at our institution from 1970 to 2005. Nineteen patients were included in the study. All had primary surgical treatment at another institution. Metastatic disease was treated with radiation, chemotherapy, or both. Survival and complications were evaluated. P values were determined by Fischer's exact test.ResultsAll patients treated with chemotherapy and radiation age ≥ 40 years had disease progression. Of the patients < 40, two had stable disease; one had regression of disease with treatment. Patients without disease progression had better prognosis and were alive at last follow-up.ConclusionsClinical benefit was derived from aggressive treatment. However, careful consideration of the risks of observation versus intensive therapy should be undertaken when managing these patients.

Head and Neck: Paraganglioma: an overview

“…The median age at first diagnosis in the US National Cancer Data Base was 44 years (Lee et al, 2002). In another series, the mean age was 35 years at diagnosis with 53% of patients being older than 40 (Moskovic et al, 2010). The sex ratio was approximately 1:1 in the cohort reported by Lee et al (2004).…”

“…The sex ratio was approximately 1:1 in the cohort reported by Lee et al (2004). In the series reported by Moskovic et al (2010), 69% of the patients were males. Multicentric paragangliomas must be distinguished from true metastases.…”

“…Complete surgical resection is the treatment of choice when feasible (Lee et al, 2002;Moskovic et al, 2010). Tumors involving the base of the skull may require adjuvant radiation therapy.…”

Malignant carotid body tumor presenting with myelopathy: case report

“…Although no consistent benefit has been demonstrated, up to 92% can achieve stable disease. 13,15 Due to the small number of cases and the differences in groups receiving various treatment modalities, direct comparisons are difficult, and no proven treatment guidelines exist. Sunitinib, a potent inhibitor of multiple tyrosine kinase receptors, and an effective antiangiogenic drug, has been reported in some case series to benefit patients with sympathetic PG, 1 but its role in head and neck PGs remains to be elucidated.…”

“…External beam radiation therapy remains palliative in the treatment of spinal metastasis, with response more effective in younger patients. 13 Limited metastasis to isolated vertebrae may benefit from resection following embolization, and adds to the effectiveness of radiation therapy.…”