The Gist of Literature on Pediatric GIST

Kaemmer, D. A.; Otto, J.; Lassay, Lisa; Steinau, G.; Klink, C.; Junge, K.; Klinge, U.; Schumpèlick, V.

doi:10.1097/mph.0b013e3181923cd8

Cited by 43 publications

(38 citation statements)

References 23 publications

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“…Mass effect from large or inopportunely positioned epigastric tumors including hepatoblastoma, hepatocellular carcinoma, pancreaticoblastomas, and soft-tissue sarcomas of the mesentery or abdominal viscera [46] can also result in gastric outlet obstruction. Primary gastric mesenchymal tumors such as gastrointestinal stromal tumor (GIST) [47,48] and gastric teratomas [49,50] can also lead to obstruction (Figs. 9, 10 and 11).…”

Section: Neoplasmmentioning

confidence: 99%

Usual and unusual causes of pediatric gastric outlet obstruction

et al. 2012

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Gastric outlet obstruction in children encompasses a spectrum of disorders that extends beyond hypertrophic pyloric stenosis. Each condition can result in the clinical syndrome of persistent nonbilious vomiting, which can progress to dehydration and electrolyte imbalances. This paper reviews the spectrum of both the common and uncommon entities that cause partial or complete gastric outlet obstruction and their imaging appearances. The correct diagnosis of those with gastric outlet obstruction can be achieved by combining clinical presentation with appropriate imaging, leading to optimal and timely patient management.

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Section: Neoplasmmentioning

confidence: 99%

Usual and unusual causes of pediatric gastric outlet obstruction

et al. 2012

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“…GISTs are the most common mesenchymal neoplasm of the gastrointestinal tract occurring in the stomach (60–70%) (Corless and Heinrich, 2008; El-Rifai et al, 2000) and small intestine (25–35%) (Corless and Heinrich, 2008; El-Rifai et al, 2000; Miettinen et al, 2006); they occur rarely in the large intestine or colon (5–10%) (Huang et al, 2006) and esophagus (Gouveia et al, 2005), typically later in life with only a few cases in the pediatric population and young adults (Janeway and Pappo, 2012; Kaemmer et al, 2009). GISTs are considered to originate from the interstitial cells of Cajal , the pacemaker cells that regulate peristalsis in the digestive tract (Parkin and Chugh, 2011).…”

Section: Introductionmentioning

confidence: 99%

Carney triad, SDH-deficient tumors, and Sdhb+/− mice share abnormal mitochondria

Szarek¹,

Ball²,

Impériale³

et al. 2015

Endocrine-Related Cancer

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Carney Triad(CTr) describes the association of paragangliomas(PGL), pulmonary chondromas, and gastrointestinal(GI) stromal tumors(GISTs) with a variety of other lesions including pheochromocytomas and adrenocortical tumors. The gene(s) causing CTr remain(s) unknown. PGL and GISTs may be caused by loss-of-function mutations in succinate dehydrogenase (SDH) (a condition known as Carney-Stratakis syndrome (CSS)). Mitochondrial structure and function are abnormal in tissues carrying SDH defects but they have not been studied in CTr. For this study, we examined mitochondrial structure in human tumors and GI tissue(GIT) of mice with SDH deficiency. Tissues from 16 CTr tumors (n=12), and those with isolated GIST(n=1), with CSS caused by SDHC(n=1), SDHD(n=2) mutations were studied by electron microscopy (EM). GIT from mice with a heterozygous deletion in Sdhb (Sdhb+/−, n=4) were also studied by EM. CTr patients presented with mostly epithelioid GISTs that were characterized by plump cells containing a centrally located round nucleus and prominent nucleoli; these changes were almost identical to those seen in the GIST of patients with SDH. In tumor cells from patients, regardless of diagnosis or tumor type, cytoplasm contained increased mitochondria with “hypoxic” phenotype: mitochondria were devoid of cristae, exhibited structural abnormalities and of variable size. Occasionally, mitochondria were small/round, rarely thin, elongated with tubular cristae. Many mitochondria exhibited amorphous fluffy material with membranous whorls or cystic structures. Similar mitochondrial hypoxic phenotype was seen in Sdhb+/− mice. We conclude tissues from SDH-deficient tumors and mouse GIT and from CTr tumors shared identical abnormalities in mitochondrial structure and other features. Thus, the still elusive CTr defect(s) is(are) likely to affect mitochondrial function just like germline SDH-deficiency.

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“…The rarer peak is seen in teenManuscript accepted for publication September 11, 2013 a ager girls, 2.7 folds more often as compared to boys. Median age of presentation is 12.4 years in children [5]. Few congenital cases of the GISTs have also been reported in literature [6].…”

Section: Discussionmentioning

confidence: 99%

Sporadic Pediatric GIST With Novel Gene Mutation

Jabeen

2013

J Med Cases

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Advanced understanding of pathogenesis and tumor metabolism has increased the number of recently diagnosed gastrointestinal stromal tumors. We present a case of a young girl who attended our hospital with an episode of melena. Endoscopy revealed an irregular gastric lump with overlying ulceration. Following further investigation and biopsy, a gastrointestinal stromal tumor of the stomach was diagnosed and subsequently resected. Histopathology showed mixed type of epithelioid and spindle cells with exon 11 KIT mutations. Early diagnosis, prompt management, and long-term followup are the key prognostic determinants.

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The Gist of Literature on Pediatric GIST

Cited by 43 publications

References 23 publications

Usual and unusual causes of pediatric gastric outlet obstruction

Usual and unusual causes of pediatric gastric outlet obstruction

Carney triad, SDH-deficient tumors, and Sdhb+/− mice share abnormal mitochondria

Sporadic Pediatric GIST With Novel Gene Mutation

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