Hämorrhagischer Schock bei Doppelmalignom: Gastrointestinaler Stromatumor (GIST) und neuroendokrines Karzinom bei Morbus Recklinghausen

Kramer, Klaus; Siech, M.; Sträter, Jörn; Aschoff, AJ; Henne‐Bruns, Doris

doi:10.1055/s-2004-813810

Cited by 34 publications

(11 citation statements)

References 9 publications

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“…The incidence of GIST is very low, that is, 2 in 1,00,000 while jejunal GISTs are extremely rare, [3] accounting for 0.1–3% of all gastrointestinal (GI) tumors [5]. The most common site of presentation is stomach, but it can crop up anywhere in the digestive tract.…”

Section: Discussionmentioning

confidence: 99%

“…Two-thirds of GISTs occur in the stomach [1] while about one-fourth develop in the small intestine, usually in duodenum [2]. As per the literature, jejunal GISTs are the rarest type among all types of GIST [3]. The most common clinical manifestation for symptomatic GISTs is occult gastrointestinal (GI) bleeding from mucosal ulceration and pain abdomen [6].…”

Section: Discussionmentioning

confidence: 99%

“…GISTs are uncommon mesenchymal neoplasms of the alimentary tract. The incidence of GIST is very low (i.e., 2 in 1,00,000) while jejunal GIST is extremely rare accounting for 0.1–3% of all gastrointestinal (GI) tumors [3]. Usually they are asymptomatic but can present as abdominal pain, bleeding, or mechanical obstruction.…”

Section: Introductionmentioning

confidence: 99%

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The Inside Mystery of Jejunal Gastrointestinal Stromal Tumor: A Rare Case Report and Review of the Literature

Dhull

Kaushal

Dhankhar

et al. 2011

Case Reports in Oncological Medicine

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Gastrointestinal stromal tumors (GISTs) are malignant and rare form of soft tissue sarcoma of the digestive tract. The incidence of gastrointestinal stromal tumors is very low Kramer et al. 2005 Jejunal GISTs are extremely rare. Here we present a rare case of jejunal GIST with unusually large size at presentation. The patient presented with severe abdomen pain, exophytic growth, and dimorphic anemia. Surgical resection of the tumor was carried out, and operative findings revealed a 15 × 10 cm growth, arising from serosal surface of jejunum, at the antimesenteric surface. Diagnosis in this case was made by subjecting the resected specimen to immunohistochemical analysis. In view of large size of the resected tumor, and high-risk histopathological features, imatinib mesylate 400 mg once daily was given as adjuvant chemotherapy. Patient is asymptomatic without any evidence of tumor recurrence after six months of postoperative followup. Imatinib as such is recommended in metastatic, residual or recurrent cases of GISTs or which are surgically not removable; however, recent recommendations suggests the use of imatinib mesylate after radical surgery in high-risk cases, because it has shown a significant decrease in the recurrence rate, and the Food and Drug Administration (FDA) has also approved the use of imatinib as adjuvant therapy after complete resection of localized, primary GIST.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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The Inside Mystery of Jejunal Gastrointestinal Stromal Tumor: A Rare Case Report and Review of the Literature

Dhull

Kaushal

Dhankhar

et al. 2011

Case Reports in Oncological Medicine

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“…Gastrointestinal stromal tumors (GISTs), the most common mesenchymal tumors of the gastrointestinal tract, are observed in 3.9 % to 25% of NF1 patients [179].…”

Section: Other Tumorsmentioning

confidence: 99%

Developmental Abnormalities and Cancer Predisposition in Neurofibromatosis Type 1

Larizza

Gervasini

Natacci

et al. 2009

CMM

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Neurofibromatosis type 1 (NF1) is a developmental and cancer predisposing syndrome resulting from haploinsufficiency or alteration in neurofibromin, a multifunctional protein that acts in various signaling pathways affecting morphogenetic processes and cell proliferation. Neurofibromin deficiency deregulates Ras/Raf/MEK/ERK and Ras/PI3K/AKT/PKB/mTOR signaling networks and intersected pathways including the cAMP-dependent protein kinase A (PKA) and the Rho-cofillin which acts on actin cytoskeleton reorganization, cell motility and adhesion. As the neurofibromin-mediated pathways are associated with biological effects depending on the cell lineage, deregulation induced by NF1 mutation clearly has cell type-specific effects. This review summarizes our increasing knowledge of NF1 as a disease rooted in defective developmental mechanisms that can also influence the potential for malignant growth. The cardinal features of NF1 patients, at birth and during life involve the cardiovascular, connective/skeletal and central nervous systems, as they reflect the NF1 mutation sensitivity of cell lineages committed to specifying these systems during embryonic development. A switch to neoplastic transformation may also occur in both the prenatal and postnatal life in cancer initiating cells of defined lineages, with the cooperation of a genetically and epigenetically modified tumor microenvironment. We emphasize how much of our current knowledge of the pathomechanisms of NF1 clinical signs and cancer has come from engineered mouse models and in vitro primary cells and cell lines exposed to inhibitors of signaling molecules. Advances in our knowledge of the developmental defects primed by the loss neurofibromin should reveal further associations between given NF1 mutations and tissue-specific symptoms, thus improving the clinical management of the patients.

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“…Zumeist werden GIST als asymptomatische Zufallsbefunde im Rahmen einer endoskopischen Diagnostik erkannt. Größere Tumoren können hingegen bluten, durch die Raumforderung selbst auffallen oder auch metastasieren [4][5][6]. Bei Patienten mit Neurofibromatose vom Typ 1 gibt es einige biologische Besonderheiten der GIST.…”

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Multiple gastrointestinale Stromatumoren bei Neurofibromatose Typ 1

et al. 2007

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Hämorrhagischer Schock bei Doppelmalignom: Gastrointestinaler Stromatumor (GIST) und neuroendokrines Karzinom bei Morbus Recklinghausen

Cited by 34 publications

References 9 publications

The Inside Mystery of Jejunal Gastrointestinal Stromal Tumor: A Rare Case Report and Review of the Literature

The Inside Mystery of Jejunal Gastrointestinal Stromal Tumor: A Rare Case Report and Review of the Literature

Developmental Abnormalities and Cancer Predisposition in Neurofibromatosis Type 1

Multiple gastrointestinale Stromatumoren bei Neurofibromatose Typ 1

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