Somatostatin-derived amyloidosis: a novel type of amyloidosis associated with well-differentiated somatostatin-producing neuroendocrine tumours

• Published in: Amyloid 2022-01, Vol.29 (1), p.58-63
• Main Authors: Van Treeck, Benjamin J., Dasari, Surendra, Kurtin, Paul J., Theis, Jason D., Nasr, Samih H., Zhang, Lizhi, Yasir, Saba, Graham, Rondell P., McPhail, Ellen D., Said, Samar
• Format: Article
• Language: English
• Subjects: Aged; Amyloid; Amyloid - metabolism; Amyloidosis - metabolism; Chromatography, Liquid; Female; Humans; Male; mass spectrometry; Middle Aged; Neuroendocrine Tumors; neuroendocrine tumour; Proteomics; Somatostatin; somatostatinoma; Tandem Mass Spectrometry
• ISSN: 1350-6129; 1744-2818
• DOI: 10.1080/13506129.2021.1979512

To report the clinicopathologic and proteomic characteristics of a novel form of amyloidosis derived from the precursor protein somatostatin. Cases were identified by searching the Mayo Clinic amyloid liquid chromatography and tandem mass spectrometry (LC-MS/MS) typing database from 1 January 2008 to 1 September 2020 for specimens with the amyloid signature proteins and abundant somatostatin, in the absence of other amyloid precursor proteins. All available medical records and pathologic materials were examined. Somatostatin-derived amyloid deposits were found in four patients, two females and two males, with a median age of 61.5 years (range 47-73 years). One patient also had neurofibromatosis-1. The amyloid in each case was associated with a well-differentiated, somatostatin-producing neuroendocrine tumour arising in the small bowel or pancreas. The amyloid deposits were Congo Red-positive and were readily identified by LC- MS/MS analysis. Somatostatin was present exclusively in somatostatin-associated amyloid cases (p