Delayed and Long-Lasting Response to 177Lu-DOTATATE in a Head and Neck Paraganglioma: Case Report and Literature Review

Abstract

Introduction: Malignant paragangliomas (M-PGL) are a group of neuroendocrine tumors that originate from chromaffin cells. The most common location for PGL is the head and neck, which comprise 65–70% of all PGL, and the M-PGL accounts for 0.6% of all head and neck cancers. It is a rare tumor, with an incidence of 2–8 per million. Diagnosing PGL can be challenging, and treatment for metastatic disease is usually not curative. Case Presentation: A 66-year-old woman was diagnosed with left cervical pain and laterocervical mass in March 2015. Octreotide scintigraphy showed intense uptake in the cervical mass, two pulmonary micronodules of 4–5 mm, and another lesion in the lumbar region (L3–L4). The final diagnosis was malignant nonsecretory PGL with adjacent tissue involvement and distant metastases. After three different treatments with minimal symptomatic improvement, 177Lu-DOTATATE was requested off-label. With a dose of 7,400 MBq until January 2018, the patient showed remarkable symptomatic pain improvement and a decrease in tumor size. Conclusion: We believe that our case report provides relevant information that can be considered in similar cases. First, the patient tripled the expected survival in such a clinical setting, and this benefit seems to rely on 177Lu-DOTATATE treatment. Second, we documented an early symptomatic response to this treatment but a long-term delayed volumetric radiographic response.

Keywords

• paraganglioma
• head and neck
• mutation in succinate dehydrogenase subunit b
• 177lu-dotate
• case report