Somatostatin Receptor Ligand Therapy-A Novel Therapy for Neurocytoma.

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The Journal of clinical endocrinology and metabolism


Context: Neurocytoma (NC) is a rare low grade tumor of the central nervous systems with a 10-year survival of 90% and local control rate of 74%. However 25% of NCs are atypical with an elevated Ki-67 labeling index >2%, and exhibit a more aggressive course with high propensity for local recurrence and/or craniospinal dissemination. Although no standard treatment regimen exists for these atypical cases, adjuvant stereotactic or conventional radiotherapy and/or chemotherapy are typically offered but yield inconsistent results.

Case Description: We describe a patient with a vasopressin-secreting atypical neurocytoma of the sellar and cavernous sinus region. Following subtotal resection via endoscopic transsphenoidal surgery, the residual tumor showed increased FDG uptake and high somatostatin receptor expression on a 68Ga-DOTA-TATE PET/CT scan. Somatostatin receptor ligand (SRL) therapy with lanreotide (120 mg Q28d) was initiated, and 4 years later the residual tumor remains stable with decreased FDG tumor uptake. Immunocytochemical somatostatin receptor 2 (SSTR2) and SSTR5 expression was further confirmed in > 80% in a series of neurocytoma tissues.

Conclusions: We describe the first use of SRL therapy in atypical NC and our results support consideration of adjuvant SRL therapy in NC refractory to surgical removal. Our findings further raise the possibility of SSTR-directed peptide receptor radionuclide therapy (PRRT) as NC therapy.

Clinical Institute

Neurosciences (Brain & Spine)

Clinical Institute