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Rare malignant insulinoma with multiple liver metastases derived from ectopic pancreas: 3-year follow-up and literature review

Authors Liu Q, Duan JH, Zheng YM, Luo J, Cai XP, Tan HY

Received 24 October 2017

Accepted for publication 14 March 2018

Published 3 April 2018 Volume 2018:11 Pages 1813—1819


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 3

Editor who approved publication: Dr Carlos E Vigil

Qing Liu,1 Jianghui Duan,2 Yumin Zheng,3 Jie Luo,4 Xiaopin Cai,5 Huangying Tan1

1Department of Integrative Oncology, China-Japan Friendship Hospital, Beijing, China; 2Department of Diagnostic Radiology, China-Japan Friendship Hospital, Beijing, China; 3Department of Nuclear Medicine, China-Japan Friendship Hospital, Beijing, China; 4Department of Pathology, China-Japan Friendship Hospital, Beijing, China; 5Department of Endocrinology, China-Japan Friendship Hospital, Beijing, China

Abstract: Here, we report the diagnosis and treatment of a very rare case of malignant insulinoma derived from ectopic pancreas. A middle-aged woman presented with a 6-year history of recurrent hypoglycemia with multiple lesions in liver. Admission workup revealed elevated serum insulin and C-peptide, as well as multiple lesions in the liver (largest being 4.3 cm), enlarged lymph nodes around the pancreas, and a lesion (of 3.5 cm) at the proximal jejunum, as shown by contrast computed tomography (CT). Liver biopsy showed the lesions to be well-differentiated neuroendocrine tumors, grade G1. 68Gallium-exendin-4 positron emission tomography/CT confirmed the origin as the lesion located at the jejunum. The combination treatment of everolimus plus long-acting octreotide relieved symptoms and achieved a partial tumor response. Maintenance treatment of the somatostatin analog (ie, octreotide) alone was administered. Three years of follow-up, up to the writing of this report, showed good survival, with the patient remaining asymptomatic and euglycemic without disease progression. This case shows that 68Gallium-exendin-4 positron emission tomography/CT is useful for locating insulinoma, especially for insulinoma derived from ectopic pancreas, and that everolimus plus octreotide with maintenance somatostatin analog alone is an effective drug strategy for treating inoperable malignant insulinoma.

Keywords: malignant insulinoma, ectopic pancreas, octreotide, everolimus, 68Gallium-exendin-4 positron emission tomography/computed tomography, 68Ga-exendin-4 PET/CT

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