Gastrointestinal stromal tumors of the small intestine in pediatric populations: a case report and literature review

Shimomura, Manabu; Ikeda, Satoshi; Takakura, Yuji; Kawaguchi, Yasuo; Tokunaga, Masahito; Takeda, Haruka; Sumitani, Daisuke; Yoshimitsu, Masanori; Hinoi, Takao; Okajima, Masazumi; Ohdan, Hideki

doi:10.1007/s00383-010-2596-3

Cited by 8 publications

(3 citation statements)

References 22 publications

(10 reference statements)

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“…The features of pediatric GISTs of the small intestine have not yet been categorized and, to date, only 11 cases in patients younger than 18 years have been reported. GISTs of the small intestine were expected to show a better response to imatinib treatment than gastric GISTs because of the alterations in the c- kit gene [ 33 ].…”

Section: Discussionmentioning

confidence: 99%

Small intestinal gastrointestinal stromal tumor in a young adult woman: a case report and review of the literature

Manxhuka-Kërliu¹,

Sahatçiu-Meka²,

Kerliu³

et al. 2014

J Med Case Reports

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IntroductionGastrointestinal stromal tumor is the most common sarcoma of the gastrointestinal tract. We report a case of gastrointestinal stromal tumor in a small intestine, initially suspected for leiomyosarcoma given that gastrointestinal stromal tumors in young adult patients are limited due to their rarity.Case presentationA 30-year-old Caucasian ethnic Albanian woman from Kosovo presented with abdominal pain, nausea and vomiting. Subsequently, the tumor was detected in her small intestine, as an infiltrating mass approximately 10cm in diameter. The tumor was resected en bloc and duodenojejunal terminal-terminal anastomosis was performed. The tumor was a large, bulky, intramural mass, with fish-flesh to tan-brown appearance, as well as with foci of hemorrhage and necrosis. On histological examination the tumor showed transmural growth, deep infiltrative pattern and malignant feature, with mitotic count >5 per 50 high-power field, dense cellularity with plump spindle cells, and with eosinophilic cytoplasm within variably hyalinized and edematous stroma, skeinoid fibers (extracellular collagen globules) and foci of hemorrhage. In addition, the tumor was composed of areas with epithelioid morphology. The immunohistochemistry results showed high expression of proto-oncogene c-kit, CD117, CD34 and vimentin, whereas α-smooth muscle actin was focally positive. Desmin and S-100 protein were negative.ConclusionsGastrointestinal stromal tumor should be included in the differential diagnoses of intestinal mesenchymal tumors presenting as a single mass in young female adults. Given that gastrointestinal stromal tumors in young adults represent a more heterogeneous group than gastrointestinal stromal tumor in pediatric cases, more effort should be made to investigate its pathogenesis and potentially more specific treatment.

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Section: Discussionmentioning

confidence: 99%

Small intestinal gastrointestinal stromal tumor in a young adult woman: a case report and review of the literature

Manxhuka-Kërliu¹,

Sahatçiu-Meka²,

Kerliu³

et al. 2014

J Med Case Reports

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show abstract

“…Pediatrics GISTs are rare, with female predominance, had multifocal locations in GIT, and had a wild-type phenotype for the c-kit genes. GISTs of small intestine had not yet categorized due to few number of cases [11 case reports in patients <18 years of age were reported] [8] .…”

Section: Discussionmentioning

confidence: 99%

“…This suggests that these cases are similar to adult GISTs [11] . In pediatric populations, GISTs arising from small intestine show a better response than gastric GIST to imatinib treatment due to mutations in the c-kit gene [8] .…”

Section: Discussionmentioning

confidence: 99%

Pediatric Female with Adult Type Small Intestine Gastrointestinal Stromal Tumor [GIST]: A case report

Alsuwaigh

2019

International Journal of Medical Arts

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Background: Gastrointestinal stromal tumor [GIST] is commonest mesenchymal tumor, mainly affects adults. But, 1.5% to 2% GISTs were reported in pediatrics and adolescents. The treatment of GIST is managed as in adult because of lack of information about pediatric GIST. Aim of the work: Here we presented a case report of pediatric GIST. Current literature was reviewed and a summary was formulated to form a base for future optimal treatment of childhood GIST. Case summary: A 14 years Saudi female presented with vague abdominal pain and easy fatigability for 8 months. The pain located in lower abdomen, mild, waning, no aggravation or reliving factor and not radiating. Clinically, there was peri umbilical round, firm and not tender mass extending to right side of the abdomen till right hypochondrium, with no movement with respiratory movements. Investigations demonstrate severe microcytic hypochromic anemia. A computed tomography [CT] abdomen and pelvis showed evidence of soft tissue mass, about 13*11*12cm, of heterogeneous pattern and enhancement with area of hypodensity and cystic degeneration. There was necrosis of mesentery with some calcification seen inside the lesion suggestive of query GIST or Lymphoma. En block resection of the mass done with safety margin, biopsy from lymph node and side-to-side anastomosis done using linear stapler. The histopathological examination revealed ill and well defined fasicles of tumor cells that have spindle, oval and round shape nuclei invading mesentery and small bowel. Mitotic figurer 5-10/50 HPF, reactive lymph node. Immunohistochemistry showed diffused strong positive CD117 and CD34, negative S-100

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Pathology and Differential Diagnosis

Shehata

Kappa

2022

Pediatric Oncology

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Gastrointestinal stromal tumors of the small intestine in pediatric populations: a case report and literature review

Cited by 8 publications

References 22 publications

Small intestinal gastrointestinal stromal tumor in a young adult woman: a case report and review of the literature

Small intestinal gastrointestinal stromal tumor in a young adult woman: a case report and review of the literature

Pediatric Female with Adult Type Small Intestine Gastrointestinal Stromal Tumor [GIST]: A case report

Pathology and Differential Diagnosis

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