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Long-Term Follow-Up of a Patient with Primary Presacral Neuroendocrine Tumor: A Case Report with Literature Review

Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)

Mi Ra Kim, Hye-kyung Shim

South Korea Department of Otorhinolaryngology – Head and Neck Surgery, Haeundae Paik Hospital, Inje University College of Medicine, Busan, South Korea

Am J Case Rep 2019; 20:1969-1975

DOI: 10.12659/AJCR.921439

Available online:

Published: 2019-12-31


#921439

BACKGROUND: Primary neuroendocrine tumors (NETs) in the retroperitoneal space are extremely rare. We report the case of a patient diagnosed with primary presacral NET in the retroperitoneum that was initially suspected to be hepatic metastasis, who was followed up for more than 8 years.
CASE REPORT: A 78-year-old man was referred to our hospital for the treatment of a hepatic mass. Following resection, the patient was diagnosed with a grade 2 well-differentiated NET. A thorough evaluation to identify the primary tumor detected small well-demarcated presacral nodules on In-111 octreotide single-photon emission tomography/computed tomography (SPECT/CT). Metastases to other locations were not observed. Presacral nodules were difficult to remove using the surgical approach; therefore, we decided to follow up closely. After 4 years, the patient was diagnosed with recurrent hepatic metastasis and peritoneal seeding. Although combination therapy of everolimus and octreotide long-acting repeatable was administered, it was discontinued owing to disease progression. Baseline Ga-68 DOTATOC positron emission tomography-computed tomography revealed adequate avidity for the lesions observed on SPECT/CT; therefore, 5 cycles of peptide receptor radionuclide therapy (PRRT) were administered, after which stable disease was maintained.
CONCLUSIONS: We identified an extremely rare primary retroperitoneal NET on In-111 octreotide SPECT/CT. During long-term follow-up, although the patient presented with recurrent hepatic metastases and peritoneal seeding, PRRT was successful in stabilizing the disease.

Keywords: Diagnostic Techniques, Radioisotope, neuroendocrine tumors, Positron-Emission Tomography, Radionuclide Imaging, Retroperitoneal Neoplasms, Tomography, Emission-Computed, Single-Photon



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