SFE2002 Poster Presentations Endocrine tumours and neoplasia (17 abstracts)
Asymptomatic neuroendocrine pancreatic tumours associated with Multiple Endocrine Neoplasia type 1: what to do?
M Monteiro 1 , R Carvalho 1 , B Cavaco 5 , H Cardoso 1 , R Castro 2 , MJ Santos 3 , M Costa 4 , M Correia 4 & H Ramos 1
1Department of Endocrinology, Hospital Geral Santo Antonio, Porto, Portugal; 2Department of Nuclear Medicine, Hospital Geral Santo Antonio, Porto, Portugal; 3Department of Clinical Pathology, Hospital Geral Santo Antonio, Porto, Portugal; 4Department of Surgery, Hospital Geral Santo Antonio, Porto, Portugal; 5Department of Molecular Pathology, Instituto Portugues de Oncologia Francisco Gentil, Lisboa.
Background: The screening of patients and relatives for the presence of inactivating mutations of the MEN 1 gene, established quite accurately those for prospective detection of neoplasms. Active search for pancreatic lesions has increased the detection of neuroendocrine pancreatic tumours (NEPT) at early ages, most asymptomatic and with no signs of malignancy at diagnosis.
Case Report: We describe one kindred where five members were identified as carriers of the non-sense mutation W220X of the MEN1 gene. All developed primary hyperparathyroidism, which was managed with surgery. Three were found to have asymptomatic, biochemically non-functioning NEPT and without malignancy signs, on screening with magnetic resonance imaging and somatostatin receptor scintigraphy scan. One patient, aged 61, presents three NEPT smaller than 1 cm, two at the head and one at the tail of the pancreas. The other two patients, aged 31 and 39, both present with one tumour each, sized 1 cm, at the tail of the pancreas. Both patients and physicians, decided for an expectating position, with biochemical and image re-evaluation in one year and intervention in the case of evolution.
Discussion: Some data indicate that the NEPT of MEN1 are more indolent than their sporadic counterparts and other data correlate tumour size and type of mutation with the malignant potential of the tumours. However, the lack of consensus and the potential risks of a pancreatic surgery, difficult to accept for an asymptomatic patient, prevents physicians from being as aggressive as they would like to be in other to prevent malignancy.
Conclusion: Detection of asymptomatic NEPT with no signs of malignancy at diagnosis comprises a management dilemma since currently there are no means to foresee the propensity for malignant transformation and there is no consensus of how and when to perform surgery due to the absence of controlled studies.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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