Gastrointestinal stromal tumor: a very rare cause of jejunoileal intussusception in a 6-year-old girl

Günaydın, Mithat; Bıçakçı, Ünal; Bozkurter, Asudan Tugce; Elli, Murat; Tander, Burak; Barış, Sancar; Rizalar, Rıza; Arıtürk, Ender; Bernay, Ferit

doi:10.1016/j.jpedsurg.2011.12.021

Cited by 7 publications

(18 citation statements)

References 11 publications

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“…Bowel obstruction due to intussusception in this context is extremely rare since GISTs tend to grow exophytically,17 not intraluminally, as in this case. A literature search revealed only a handful of case reports of a GIST presenting with pure jejunal18–21 or jejunoileal22 intussusception.…”

Section: Discussionmentioning

confidence: 99%

A jejunal GIST presenting with obscure gastrointestinal bleeding and small bowel obstruction secondary to intussusception

Sadeghi

Lanzon-Miller

2015

BMJ Case Reports

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SUMMARYA 68-year-old man with episodes of overt obscure gastrointestinal (GI) bleeding was investigated with multiple upper and lower GI endoscopies, CT enterography and capsule endoscopy, but no cause was found. He then presented acutely with small bowel obstruction. A laparotomy revealed complete small bowel obstruction secondary to jejunal intussusception over a 4 cm intraluminal polyp. Following resection and primary anastomosis, histology revealed that the polyp was a GI stromal tumour (GIST). This is an exceptionally uncommon presentation of a rare tumour. It is surprising that this tumour was not detected by CT enterography and not seen on capsule endoscopy. Immunohistochemistry and mutation analysis of the GIST suggested that it had a low risk of metastatic disease, but a high risk of recurrence. Staging CT scans did not reveal evidence of distal spread. The patient is currently receiving 3 years of chemotherapy with imatinib.

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Section: Discussionmentioning

confidence: 99%

A jejunal GIST presenting with obscure gastrointestinal bleeding and small bowel obstruction secondary to intussusception

Sadeghi

Lanzon-Miller

2015

BMJ Case Reports

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“…Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract (Gunaydin et al 2012 ; Benesch et al 2009 ; Durham et al 2004 ; Guhyun K et al 2013 ; Park et al 2006 ; Jagannathan et al 2012 ; Hayashi et al 2005 ) accounting for 6.5 to 14.5 per million per year (Benesch et al 2009 ). Men older than 40 years are predominantly affected (95% of all) (Gunaydin et al 2012 ; Park et al 2006 ; Jagannathan et al 2012 ), with a peak incidence in the sixth and seventh decades (Gunaydin et al 2012 ; Guhyun K et al 2013 ; Park et al 2006 ; Hayashi et al 2005 ; Tran et al 2013 ). Among children and adolescents GIST are very uncommon (Benesch et al 2009 ; Hayashi et al 2005 ) and have two peaks of incidence: less than 1-year-old and between 10 and 15 years old (Hayashi et al 2005 ).…”

Section: Discussion and Evaluationmentioning

confidence: 99%

“…Gastrointestinal stromal tumors (GISTs) are uncommon mesenchymal tumors of the gastrointestinal (GI) tract accounting for 0.1% to 3% of all GI malignancies (Gunaydin et al 2012 ). In children the true incidence is not available for the rarity of the pathology and the difficulty of the correct diagnosis (Benesch et al 2009 ).…”

Section: Introductionmentioning

confidence: 99%

“…Generally paediatric GIST has a female predisposition (Gunaydin et al 2012 ; Guhyun K et al 2013 ; Park et al 2006 ; Hayashi et al 2005 ; Tran et al 2013 ) and occurs in the stomach (Benesch et al 2009 ; Guhyun K et al 2013 ; Park et al 2006 ; Hayashi et al 2005 ; Tran et al 2013 ) or in the small intestines (Gunaydin et al 2012 ; Durham et al 2004 ; Hayashi et al 2005 ; Tran et al 2013 ). In up to 85% of paediatric cases c-kit/CD-117 or PDGFRA (Platelet-Derived Growth Factor Receptor Alpha) mutation is absent and it may result in decreased efficacy in target-based therapy with tyrosine kinase receptor inhibitors (Benesch et al 2009 ; Tran et al 2013 ).…”

Section: Introductionmentioning

confidence: 99%

“…Guidelines for the management of paediatric GIST are not clear and often derives from the adult literature (Benesch et al 2009 ; Hayashi et al 2005 ), however it is widely accepted that surgery is the first-line treatment (Gunaydin et al 2012 ; Benesch et al 2009 ; Hayashi et al 2005 ) and gross resection with negative microscopic margins may lead to full remission of the pathology (Gunaydin et al 2012 ; Benesch et al 2009 ; Durham et al 2004 ; Hayashi et al 2005 ; Tran et al 2013 ). Despite a high rate of metastases, tumor recurrence and limited response to tyrosine kinase inhibitors, paediatric GIST have a relatively benign clinical course (Gunaydin et al 2012 ; Benesch et al 2009 ; Jagannathan et al 2012 ; Tran et al 2013 ).…”

Section: Introductionmentioning

confidence: 99%

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Laparoscopic resection of a rare gastrointestinal stromal tumor in children

et al. 2015

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IntroductionGastrointestinal stromal tumors (GIST) are uncommon mesenchymal tumors of the gastrointestinal (GI) tract, accounting for 0.1% to 3% of all GI malignancies. Paediatric GIST have an annual incidence of 0.02 per million children, have a female predisposition, are usually located in the stomach (50–60%) and in up to 85% of cases CD117-cKit or PDGFRA mutation is absent, resulting in a decreased efficacy of the target therapy.Case descriptionWe report the case of an incidentally diagnosed gastric GIST in a 14-year-old boy with multiple malformations. Genetic tests and Kariotype resulted negative.Recently, an abdominal US visualized an hypoechoic heterogeneous abdominal mass. The common tumor markers resulted negative and the abdominal CT-scan confirmed the presence of a solid round lesion (42×36mm) in contact with the stomach and the pancreas. Laparoscopy allowed the recognition and the removal of the nodular mass at the posterior margin of the stomach. The histopathologic and the molecular biology findings were consistent with a kit-wilde type GIST. Surgical margins were microscopically free of tumor cells.These results justify the decision not to add other surgical or medical therapy. However, for high risk of recurrence and metastasis, a close follow-up was started.Discussion and evaluationGIST are asymptomatic in 10% to 30% of patients or present nonspecific symptoms and signs. These tumors present usually irregular, lobulated and ulcerated. CT-scan of the abdomen and pelvis or magnetic resonance imaging (MRI) are mandatory in the diagnostic work-up. The final diagnosis is based on histology and immunohistochemistry. Surgery is the first-line treatment in patients with localized disease.ConclusionGuidelines for the management of pediatric GIST are not presently available for the paucity of reports and data. However it is widely accepted that surgery is the first-line treatment and gross resection with negative microscopic margins can be considered therapeutic and lead to full remission of the pathology. Laparoscopy is a safe surgical approach for the exploration of the abdominal cavity, the evaluation of the disease and the complete removal of the tumor.

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Incidental GIST after appendectomy in a pediatric patient: a first instance and review of pediatric patients with CD117 confirmed GISTs

et al. 2013

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A 7-year-old boy underwent uncomplicated laparoscopic appendectomy for acute appendicitis. Incidentally, he was found to have a spindle cell tumor with CD117 immunopositivity, consistent with gastrointestinal stromal tumor (GIST) in the appendix. Although commonly reported in adults, pediatric GISTs are rare gastrointestinal malignancies that occur in only 1.4-2.7% of children and adolescents. Due to the paucity of reports, data are insufficient to adequately characterize tumor behavior, recurrence, and survival. We present the first case of pediatric GIST in the appendix. In addition, a review of the literature for CD117 confirmed pediatric GISTs was conducted to summarize its clinical features and current treatment options.

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Gastrointestinal stromal tumor: a very rare cause of jejunoileal intussusception in a 6-year-old girl

Cited by 7 publications

References 11 publications

A jejunal GIST presenting with obscure gastrointestinal bleeding and small bowel obstruction secondary to intussusception

A jejunal GIST presenting with obscure gastrointestinal bleeding and small bowel obstruction secondary to intussusception

Laparoscopic resection of a rare gastrointestinal stromal tumor in children

Incidental GIST after appendectomy in a pediatric patient: a first instance and review of pediatric patients with CD117 confirmed GISTs

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