Retrospective Analysis of the Effectiveness of Somatostatin Analogs in the Treatment of Metastatic Pheochromocytoma/Paraganglioma. A Clinical Case on the efficacy of long-acting release octreotide

Introduction. Pheochromocytoma (PC) and paraganglioma (PG) are neuroendocrine neoplasms and are characterized by a high frequency of somatostatin receptor (SSTR) expression. The efficacy of somatostatin analogues in PC/PG remains poorly studied, despite the lack of evidence base, these drugs are included in the international NCCN recommendations. The aim of this study was to investigate the effectiveness of somatostatin analogues in the treatment of PC/PG.

Materials and methods. This retrospective single-center study included patients older than 18 years with positive expression of SSTR 2A и/или SSTR 5 or with accumulation of 68Ga-DOTATATE who received somatostatin analogs (octreotide-depo or lanreotide) at the first line of treatment for metastatic PC/PG from April 2020 to September 2023.

Results. The study included 8 patients, 6 male (75 %) and 2 female (25 %). All patients had positive SSTR 2A expression and accumulation on 68Ga-DOTATATE PET. Half patients (N=4, 50 %) had pheochromocytoma and half (N=4, 50 %) paraganglioma. SDHB mutation was detected in 1 (12.5 %) patient. Four patients (50 %) had disease progression on follow-up before starting therapy with somatostatin analogues. No objective response was achieved (0 %). However, all patients had disease control ≥ 6 months. Two (33.3 %) of 6 had a ≥ 50 % decrease in metanephrine/ normetanephrine secretion. Median PFS was 21.57 months. (95 % Confidence Interval 14.95–28.19).

Conclusion. Based on our findings, we recommend considering somatostatin analogs as a first-line treatment option for patients with indolent disease course and low tumor burden who demonstrate progression during active surveillance. The impact of long-acting somatostatin analogs on catecholamine secretion warrants further investigation in a prospective cohort.

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