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Medical Management of Islet Cell Carcinoma

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Neuroendocrine Tumors

Part of the book series: Current Clinical Oncology ((CCO))

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Abstract

Pancreatic endocrine tumors (PET), also often called islet cell tumors, are rare neoplasms thought to have an indolent natural course when compared to exocrine cancers of the pancreas. Their annual incidence is <1 per 100,000 person per year in the general population. Most tumors are sporadic but 15–30% can be part of multiple endocrine neoplasia type 1, von Hippel–Lindau’s disease, neurofibromatosis 1, or tuberous sclerosis (TSC1/2). Due the indolent nature and unspecific symptoms of the tumors, there is usually a delay in diagnosis, from 1 to 6 years, and, hence, they are metastastic and unresectable, when the diagnosis is finally made. The prognosis of patients with endocrine pancreatic tumors is difficult to predict because the criteria of malignancy have been ambiguous. Recently, two new classification systems have been developed. The WHO-classification divides PETs into three general categories: (1) well-differentiated endocrine tumors of benign behavior (confined to the pancreas, non-angioinvasive, no perineural invasion, <2 cm in diameter <2% Ki-67 positive cells) or uncertain behavior (confined to the pancreas and one or more of the following features: >2 cm in diameter, >2% Ki-67 positive cells, angioinvasion, perineural invasion), (2) well-differentiated endocrine carcinomas, low-grade malignant, with gross local invasion and/or metastases, and (3) poorly differentiated endocrine carcinomas, high-grade malignant. Recently, two tumor-node-metastasis (TNM) staging systems has been proposed, one by ENETS (Table 9.1), and another by the American Joint Committee on Cancer (AJCC) (Table 9.2), which will be used in the United States. Both the WHO and ENETS TNM-classifications are being adopted and validated by several groups.

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  1. Department of Medical Sciences, Uppsala University Hospital, Uppsala, Sweden

    Barbro Eriksson

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  1. Barbro Eriksson
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  1. MD Anderson Cancer Center, Department of Gastrointestinal, University of Texas, Holcombe Blvd. 1400, Houston, 77030-4009, Texas, USA

    James C. Yao

  2. São Paulo, Faculdade de Medicina da, Universidade de São Paulo,, Instituto do Cancer do Estado de, Ave Dr Arnaldo 251, São Paulo, 01246-000, São Paulo, Brazil

    Paulo M. Hoff

  3. Cancer do Estado de São Paulo, Faculdade, de Medicina da Universidade de São Paulo, Endocrine Neoplasia Unit, Instituto do, Av Dr Arnaldo 251, São Paulo, 01246-000, São Paulo, Brazil

    Ana O. Hoff

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Eriksson, B. (2011). Medical Management of Islet Cell Carcinoma. In: Yao, J., Hoff, P., Hoff, A. (eds) Neuroendocrine Tumors. Current Clinical Oncology. Humana Press. https://doi.org/10.1007/978-1-60327-997-0_9

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