Revised Classification of Neuroendocrine Tumors of the Lung, Pancreas and Gut

Capella, Carlo; Heitz, Philipp U.; Höfler, Heinz; Solcia, Enrico; Klöppel, G.

doi:10.1159/000201197

Cited by 183 publications

(172 citation statements)

References 58 publications

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“…Indeed, we mainly observed IGF2 in ␤-cells but sometimes also in non-␤-cells, as reported for fetal pancreases (41). We then chose an antibody against synaptophysin, a general endocrine marker (42), and not insulin for measuring the reference area in order to avoid restricting reference area to ␤-cells alone, contrary to Kassem et al (40). Furthermore, synaptophysin identifies endocrine cells, whatever their functional state, because its detection is independent of the presence of granules.…”

Section: Fig 3 Morphological Description Of Functional Characteristsupporting

confidence: 55%

The Focal Form of Persistent Hyperinsulinemic Hypoglycemia of Infancy

et al. 2003

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Paternal mutation of ATP-sensitive K؉ (K ATP ) channel genes and loss of heterozygosity (LOH) of the 11p15 region including the maternal alleles of ABCC8, IGF2, and CDKN1C characterize the focal form of persistent hyperinsulinemic hypoglycemia of infancy (FoPHHI). We aimed to understand the actual nature of FoPHHI in comparison with insulinoma. In FoPHHI, the lesion consists in clusters of ␤-cells surrounded by non-␤-cells. Compared with adjacent islets, proinsulin mRNA is similar and proinsulin production higher (P < 0.02), indicating regulation at a translational level, with slightly lower insulin stock and lower ABCC8 peptide labeling (P<0.05). Insulinomas, composed of ␤-cell nests or cords, have similar proinsulin mRNA compared with adjacent islets, highly variable proinsulin production, lower insulin stock (P < 0.02), and higher ABCC8 peptide labeling (P<0.05). Proinsulin mRNA is lower than in FoPHHI (P<0.001). Islets adjacent to FoPHHI appear to be resting, in contrast to those adjacent to insulinomas, evidencing intrapancreatic regulation of islet ␤-cell activity. IGF2 peptide is present inside and outside both lesions, but IGF2 mRNA is restricted to the lesions. The 11p15 LOH and absence of CDKN1C peptide staining are demonstrated in all FoPHHI but also in three of eight insulinomas. Despite some molecular similarities, FoPHHI is thus fundamentally different from insulinoma. Diabetes 52:784 -794, 2003

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Section: Fig 3 Morphological Description Of Functional Characteristsupporting

confidence: 55%

The Focal Form of Persistent Hyperinsulinemic Hypoglycemia of Infancy

et al. 2003

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“…Many other classifications and terminologies have been proposed over time on lung NET, whose detailed examination is beyond the scope of the current paper, by either introducing a concept of tumor grading 11,13,[46][47][48][49] , applying different thresholds to current defining criteria 50,51 or extending to these tumors the same defining criteria as those used in the gastroenteropancreatic tract 21,[52][53][54] . These different proposals, however, have not gained…”

Section: Diagnosing Lung Netmentioning

confidence: 99%

What clinicians are asking pathologists when dealing with lung neuroendocrine neoplasms?

Pelosi

Fabbri

Cossa

et al. 2015

Seminars in Diagnostic Pathology

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“…Over the last few years, a new classification of neuroendocrine gastroenteropancreatic tumours (GEPs) has been developed based on clinicopathologic features (Capella et al 1994, Kloppel & Heitz 1994, Rindi et al 1998. Tumours are termed functioning neuroendocrine tumours according to their leading clinical and endocrine profile, while those without plasma hormone elevation and lacking endocrine symptoms are labelled non-functioning neuroendocrine tumours (Taheri & Meeran 2002).…”

Section: Specific Syndromes Carcinoid and Other Gut Neuroendocrine Tumentioning

confidence: 99%

Neuroendocrine tumours.

Barakat¹,

Meeran²,

Bloom³

2004

Endocr Relat Cancer

209

111

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Neuroendocrine tumours are a heterogeneous group including, for example, carcinoid, gastroenteropancreatic neuroendocrine tumours, pituitary tumours, medullary carcinoma of the thyroid and phaeochromocytomas. They have attracted much attention in recent years, both because they are relatively easy to palliate and because they have indicated the chronic effect of the particular hormone elevated. As neuroendocrine phenotypes became better understood, the definition of neuroendocrine cells changed and is now accepted as referring to cells with neurotransmitter, neuromodulator or neuropeptide hormone production, dense-core secretory granules, and the absence of axons and synapses. Neuroendocrine markers, particularly chromogranin A, are invaluable diagnostically. Study of several neuroendocrine tumours has revealed a genetic etiology, and techniques such as genetic screening have allowed risk stratification and prevention of morbidity in patients carrying the particular mutation. Pharmacological therapy for these often slow-growing tumours, e.g. with somatostatin analogues, has dramatically improved symptom control, and radiolabelled somatostatin analogues offer targeted therapy for metastatic or inoperable disease. In this review, the diagnosis and management of patients with carcinoid, gut neuroendocrine tumours, multiple endocrine neoplasia types 1 and 2, and isolated phaeochromocytoma are evaluated.

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Revised Classification of Neuroendocrine Tumors of the Lung, Pancreas and Gut

Cited by 183 publications

References 58 publications

The Focal Form of Persistent Hyperinsulinemic Hypoglycemia of Infancy

The Focal Form of Persistent Hyperinsulinemic Hypoglycemia of Infancy

What clinicians are asking pathologists when dealing with lung neuroendocrine neoplasms?

Neuroendocrine tumours.

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