Multiple gastrointestinale Stromatumoren bei Neurofibromatose Typ 1

Zachäus, Markus; Plötner, Andreas; Weimann, Arved; Ruf, B.

doi:10.1007/s00063-007-1018-3

Cited by 4 publications

(3 citation statements)

References 32 publications

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“…An important aspect of GIST is their association with other malignancies; the literature of this continues to grow [28][29][30][31][32][33][34][35][36][37]. The reported malignancies include gastric carcinoma [28], colorectal adenocarcinoma [28,37], chronic lymphocytic leukemia [28], carcinoid of the pancreas [28], several gastrointestinal malignancies [29], cutaneous, uterine, and esophageal leimyomata [30,31], neurofibromatosis type 1 [32], gastric Mucosa Associated Lymphoid Tissue lymphoma [33,34], follicular carcinoma of thyroid [35], pulmonary chondroma and extra-adrenal paraganglioma (constituting Carney's triad) [36], and prostate carcinoma [38].…”

Section: Serendipitous Observations: Detection Of Synchronous and Metmentioning

confidence: 99%

FDG-PET and PET/CT in the clinical management of gastrointestinal stromal tumor

Basu

Mohandas²,

Peshwe³

et al. 2008

Nuclear Medicine Communications

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The management of gastrointestinal stromal tumors (GISTs) has been revolutionized in recent years by two major developments: the introduction of imatinib mesylate as a targeted therapeutic agent and the dramatic change in the tumor metabolic activity following successful therapy making in fluorodeoxyglucose (FDG)-PET as the modality of choice for monitoring therapeutic response. In the present communication, we have explored the current role of PET/computed tomography (CT) imaging in GIST on the basis of a brief overview of the published studies and our experience on the subject gained in a large tertiary care setting. There is now convincing evidence that serial PET study is more sensitive and reliable for determining treatment response to imatinib mesylate in patients of GIST, when compared with only conventional CT monitoring. This modality also appears to be of potential value in initial disease evaluation including prediction of malignant potential in recently diagnosed GIST and in selection of optimal dose of imatinib for therapy. The findings of detection of disease recurrence on discontinuing imatinib and acquired resistance to imatinib provide insight into the issue of therapeutic endpoint definition. On the basis of the experience gained in recent times, the future potential of this powerful modality in this setting is hypothesized.

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Section: Serendipitous Observations: Detection Of Synchronous and Metmentioning

confidence: 99%

FDG-PET and PET/CT in the clinical management of gastrointestinal stromal tumor

Basu

Mohandas²,

Peshwe³

et al. 2008

Nuclear Medicine Communications

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show abstract

“…11 As manifestações gastrintestinais de NF1 geralmente surgem depois do aparecimento das lesões cutâneas e os principais sintomas são: dor, dispepsia, hemorragia e obstrução. 6 O tratamento é baseado na ressecção do tumor com ampla margem de segurança e revisão do restante do trato gastrointestial. Quando não há alto risco de malignidade na analise histopatologica, a cirurgia pode ser curativa.…”

Section: Discussionunclassified

“…De acordo com estudos apenas 16% das intussuscepções intestinais em adultos são de origem idiopática. 5 Apesar da baixa ocorrência dos GITs na população, essa neoplasia aparece com certa frequência em pacientes portadores de neurofibromatose tipo 1, 6 dando preferência ao acometimento do intestino delgado. Na maioria, são achados incidentais, porém em alguns casos podem evoluir com complicações clinicas e cirúrgicas.…”

Section: Introductionunclassified

Intussuscepção intestinal decorrente de tumor de estroma gastrointestinal (GIST) em paciente com neurofibromatose tipo 1 / Intestinal intusception arising from gastrointestinal stroma tumor (GIST) in patient with neurofibromatosis type 1

Tayer¹,

Reis²,

Tayer³

et al. 2020

BJHR

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Patient with high-risk GIST not associated with c-KIT mutations

Izquierdo

Bonastre²

2012

Anti-Cancer Drugs

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A patient was diagnosed with neurofibromatosis type 1 (NF1) and gastrointestinal stromal tumour (GIST). This is not simply a coincidence; numerous molecular and genetic studies have established a close relationship between the two disorders, suggesting that GIST should be included in the clinical spectrum of NF1. In NF1 presenting GIST, the tumour usually develops in the small intestine. We present a 51-year-old woman diagnosed with NF1, who on follow-up showed an intra-abdominal mass. The pathological study of the mass after surgery led to the diagnosis of GIST with no mutations in exons 9, 11, 13 and 17 of the c-KIT gene or in exons 12, 14 and 18 of the platelet-derived growth factor receptor α gene. Imatinib was initiated as coadjuvant therapy with good tolerance, no toxicity and without evidence of relapse during follow-up.

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Multiple gastrointestinale Stromatumoren bei Neurofibromatose Typ 1

Abstract: Considering the distinct biology of GIST in patients with neurofibromatosis type 1, the recommendation for a generous endoscopic examination or a routine endoscopy in this population is discussed.

Cited by 4 publications

References 32 publications

FDG-PET and PET/CT in the clinical management of gastrointestinal stromal tumor

FDG-PET and PET/CT in the clinical management of gastrointestinal stromal tumor

Intussuscepção intestinal decorrente de tumor de estroma gastrointestinal (GIST) em paciente com neurofibromatose tipo 1 / Intestinal intusception arising from gastrointestinal stroma tumor (GIST) in patient with neurofibromatosis type 1

Patient with high-risk GIST not associated with c-KIT mutations

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