First line treatment with 177Lu-Dotatate in a patient with miliary liver metastases and carcinoid syndrome from a midgut neuroendocrine tumor

A. Diamantopoulos*^a (Mr), V. Antonopoulou^a (Mrs), G. Kyriakopoulos^b (Mr), M. Papachristou^c (Dr), SE. Papadimitriou^c (Mrs), P. Stavrou^c (Mr), I. Datseris^c (Dr), DA. Vassiliadi^a (Dr), S. Tsagarakis^a (Dr)

^a Department of Endocrinology, Diabetes and Metabolism, National Expertise Center for Rare Endocrine Disorders and member of the Endo-ERN, “Evangelismos” General Hospital of Athens, Athens, GREECE ; ^b Pathology Department, “Evangelismos” General Hospital of Athens, Athens, GREECE ; ^c Nuclear Medicine and PET/CT Department, “Evangelismos” General Hospital of Athens, Athens, GREECE

* aris_diamad@yahoo.gr

Introduction

Surgery is the only curable treatment for neuroendocrine tumors (NETs). However, many patients are diagnosed at an advanced, inoperative stage, aggravating their prognosis. Endocrine syndromes may impair both quality of life and survival. The NETTER-1 study established ¹⁷⁷Lu-Dotatate as an effective therapeutic tool for patients with well-differentiated, metastatic midgut NETs. Herein we present the outcome of¹⁷⁷Lu-Dotatate as first-line treatment in a 21-year-old male patient with a clinically and structurally advanced metastatic midgut NET.

Observation

The patient presented with carcinoid heart disease, flushing, chronic diarrhea and multiple liver metastases (>30 lesions, affecting c.70% of the liver). He had excessively high serotonin and 5-ΗΙΑΑ levels. Liver biopsy showed metastasis from Grade2 NET. ⁶⁸Ga-DOTApeptide PET/CT showed uptake from the liver metastases and other abdominal lesions (Krenning3). He was started on Lanreotide and Telotristat, with partial biochemical control. The patient received Lutetium-177(¹⁷⁷Lu)-Dotatate at a dose of 7355MBq every 8 weeks. Lanreotide and Telotristat were continued.

PRRT was very well tolerated with mild adverse effects, mainly during the infusion of the amino-acid solution. After completion of treatment, we noted grade 1-2 myelosuppression, which improved 1 year after the last infusion. With regards to response, the patient is biochemically controlled and reports significantly reduced frequency of flushing and complete recession of diarrhea. According to RECIST criteria decrease of size and number of liver metastases was noted and liver involvement reduced from 70% to 55%, 17 months after the last infusion. Carcinoid heart disease improved and pro-BNP levels returned to normal. Due to gall bladder stones, a consequence of lanreotide treatment, he underwent an uneventful cholecystectomy and resection of primary tumor, located in the small intestine.

Discussion

Among other conventional therapies, PRRT has recently been shown to be effective, with regards to improved PFS and quality of life rate, significantly higher response, and a relative safe option when surgery is not feasible. In our case, treatment with 177Lu-Dotatate and somatostatin analogue was well-tolerated and resulted to PR that is sustained during a 19-month period, with biochemical normalization, clinical improvement and improvement of parameters of carcinoid heart disease.

The author has declared no conflict of interest.