Malignant pancreatic tumour within the spectrum of tuberous sclerosis complex in childhood

Verhoef, Senno; Diemen-Steenvoorde, R. van; Akkersdijk, W.L.; Bax, N. M. A.; Ariyürek, Yavuz; Hermans, C. J.; Nieuwenhuizen, Onno van; Nikkels, Peter G. J.; Lindhout, Dick; Halley, Dicky; Lips, K.; Ouweland, Ans M.W. van den

doi:10.1007/s004310051073

Cited by 103 publications

(64 citation statements)

References 11 publications

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“…Cases of PET in association with the tuberous sclerosis complex and type 1 neurofibromatosis have also been reported but are rare (Tan et al 1996, Verhoef et al 1999, Fujisawa et al 2002, Francalanci et al 2003.…”

Section: Pets Associated With Hereditary Syndromesmentioning

confidence: 99%

Pancreatic endocrine neoplasms: epidemiology and prognosis of pancreatic endocrine tumors

Halfdanarson¹,

Rubin²,

Farnell³

et al. 2008

Endocrine Related Cancer

343

233

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Pancreatic endocrine tumors (PETs) are uncommon tumors with an annual incidence !1 per 100 000 person-years in the general population. The PETs that produce hormones resulting in symptoms are designated as functional. The majority of PETs are non-functional. Of the functional tumors, insulinomas are the most common, followed by gastrinomas. The clinical course of patients with PETs is variable and depends on the extent of the disease and the treatment rendered. Patients with completely resected tumors generally have a good prognosis, and aggressive surgical therapy in patients with advanced disease may also prolong survival. The epidemiology, prognosis, and established and novel prognostic markers of PETs are reviewed.

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Section: Pets Associated With Hereditary Syndromesmentioning

confidence: 99%

Pancreatic endocrine neoplasms: epidemiology and prognosis of pancreatic endocrine tumors

Halfdanarson¹,

Rubin²,

Farnell³

et al. 2008

Endocrine Related Cancer

343

233

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“…A genetic etiology of NETs has been suggested in that NETs may occur as part of hereditary syndromes, such as multiple endocrine neoplasia type 1 (MEN-1; refs. 2, 3), von HippelLindau syndrome (3,4), neurofibromatosis type 1 (5,6), tuberous sclerosis (7)(8)(9), and nonpolyposis colon cancer (10). Of these, MEN-1 syndrome, an autosomal dominant disorder characterized by parathyroid hyperplasia, pituitary adenomas, and pancreatic tumors, is the one in which NETs most frequently occur.…”

mentioning

confidence: 99%

Family History of Cancer and Associated Risk of Developing Neuroendocrine Tumors: A Case-Control Study

Hassan

Phan

et al. 2008

Cancer Epidemiology, Biomarkers &Amp; Prevention

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Background: Carcinoids are rare neuroendocrine tumors (NET). Familial clusterings of NETs are rarely reported, except for a small proportion associated with multiple endocrine neoplasia syndrome type 1. We evaluated the effect of positive family history of NET as well as other cancers on the development of NETs arising at five different locations. Methods: We conducted a retrospective, hospital-based, case-control study involving 740 patients with histologically confirmed NETs and 924 healthy controls. Information on family history of cancer was collected, and unconditional logistic regression analysis was used to determine adjusted odds ratios (AOR) and 95% confidence intervals (CI). Results: The authors observed a significant relationship between first-degree relatives with cancers and the development of NETs arising at the small intesLeonetine, stomach, lung, and pancreas; AORs

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“…Malignant PETs have been described in children 48,49 and functional PETs have been reported to produce insulin 50,51 and gastrin. 52 Hamartin is highly expressed in normal islet cells, 53 so it is not difficult to imagine that loss of this tumor suppressor may have an etiologic role in these lesions.…”

Section: Tuberous Sclerosismentioning

confidence: 99%

Pancreatic endocrine tumors

Sylvia

2011

Modern Pathology

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The endocrine cells of the pancreas and their related cells throughout the gastrointestinal tract give rise to a variety of tumors that pose a diagnostic challenge. There has been progress in understanding their histogenesis, morphology, immunohistochemistry, molecular biology and classifications. This review will focus on nomenclature/terminology, classification, the role of immunohistochemistry, molecular advances, including genetic predisposition, and potential therapeutic targets to define the role of pathology in the application of prognostic and predictive markers for this disease.

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Malignant pancreatic tumour within the spectrum of tuberous sclerosis complex in childhood

Abstract: Pancreatic islet cell tumours have been mainly associated with multiple endocrine neoplasia syndrome type 1. In our case we demonstrate a direct relationship of this tumour to tuberous sclerosis complex, in the absence of further signs of multiple endocrine neoplasia syndrome type 1.

Cited by 103 publications

References 11 publications

Pancreatic endocrine neoplasms: epidemiology and prognosis of pancreatic endocrine tumors

Pancreatic endocrine neoplasms: epidemiology and prognosis of pancreatic endocrine tumors

Family History of Cancer and Associated Risk of Developing Neuroendocrine Tumors: A Case-Control Study

Pancreatic endocrine tumors

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