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

Abstract

Background: Little is known about management and prognosis for malignant head & neck paragangliomas. We reviewed records of these patients to determine optimal treatment strategies.

Methods: We 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.

Results: All 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.

Conclusions: Clinical benefit was derived from aggressive treatment. However, careful consideration of the risks of observation versus intensive therapy should be undertaken when managing these patients.

Figure 1

Representative magnetic resonance image revealing bone metastasis indicated by the white arrow.

Figure 2

Representative computed tomographic image revealing liver metastases in a PG patient.

Figure 3

The distribution of patients receiving radiation, chemotherapy and combination therapy.

Figure 4

Kaplan-Meier overall survival curves for patients based on age at diagnosis.

Figure 5

Kaplan-Meier overall survival curves over 10 years for patients with malignant head & neck paragangliomas.

Figure 6

Algorithmic overview of diagnostic and treatment options of patients with suspected local/regional and metastatic disease. CT = computed tomography, PET = positron emission tomography, MIBG = metaiodobenzylguanidine.