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Neuroendocrine tumours and genetic disorders 

Neuroendocrine tumours and genetic disorders
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date: 23 January 2020

Although Siegfried Oberndorfer is rightly credited with introducing the term karzinoide (carcinoma-like) in 1907, T. Langhans had in 1867 described a submucosal tumour that resembled poorly differentiated glandular tissue arranged in ‘nests’ with a rich, thick fibrous stroma (1). Thereafter in 1888, O. Lubarsch reported the post mortem identification of multiple ileal tumours, which he was reluctant to classify as ‘carcinomas’ due to a benign growth pattern appearance (2). In 1890, W.B. Ransom reported similar tumours at autopsy in the region of the ileocoecal valve with associated extensive hepatic tumours, but, in addition, emphasized the associated clinical symptoms, which included diarrhoea and wheezing (3). Nevertheless, despite these early descriptions, it remained for Oberndorfer to published his seminal paper Carcinoid Tumours of the Small Intestine in 1907 and recognize their unique nature; finally defining the lesions as a neoplasm distinct from carcinoma (Fig. 6.1.1) (4).

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