Somatostatin Receptor Ligand Therapy-A Potential Therapy for Neurocytoma.

Document Type

Article

Publication Date

6-1-2019

Publication Title

The Journal of clinical endocrinology and metabolism

Keywords

Adolescent; Brain Neoplasms; Cavernous Sinus; Fluorodeoxyglucose F18; Humans; Male; Neurocytoma; Peptides, Cyclic; Receptors, Somatostatin; Sella Turcica; Somatostatin; Vasopressins

Abstract

CONTEXT: Neurocytoma (NC) is a rare, low-grade tumor of the central nervous system, with a 10-year survival rate of 90% and local control rate of 74%. However, 25% of NCs will be atypical, with an elevated Ki-67 labeling index >2%, and will exhibit a more aggressive course, with a 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 have been typically offered but have yielded inconsistent results.

CASE DESCRIPTION: We have described the case of a patient with a vasopressin-secreting atypical NC of the sellar and cavernous sinus region. After subtotal resection via endoscopic transsphenoidal surgery, the residual tumor showed increased fluorodeoxyglucose uptake and high somatostatin receptor (SSTR) expression on a 68Ga-DOTA-TATE positron emission tomography/CT scan. Somatostatin receptor ligand (SRL) therapy with lanreotide (120 mg every 28 days) was initiated. Four years later, the residual tumor was stable with decreased fluorodeoxyglucose tumor uptake. Immunocytochemical SSTR2 and SSTR5 expression >80% was further confirmed in a series of NC tissues.

CONCLUSIONS: To the best of our knowledge, we have described the first use of SRL therapy for an atypical NC. Our results support consideration of adjuvant SRL therapy for NC refractory to surgical removal. Our findings further raise the possibility of SSTR-directed peptide receptor radionuclide therapy as NC therapy.

Clinical Institute

Neurosciences (Brain & Spine)

Clinical Institute

Cancer

Department

Neurosciences

Department

Oncology

Department

Pathology & Laboratory Medicine

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