Appendiceal GIST: report of an exceptional case and review of the litterature

Bouassida, Mounir; Chtourou, Mohamed Fadhel; Chalbi, Emna; F, Chebbi; Hamzaoui, Lamine; Sassi, Sadok; Charfi, Lamia; Mighri, Mohamed Mongi; Touinsi, Hassen; Sassi, Adok

doi:10.11604/pamj.2013.15.85.2430

Cited by 17 publications

(23 citation statements)

References 9 publications

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“…GISTs of the vermiform appendix constitute only 0.1% of all GISTs (1), with only 16 cases reported in the English literature to date (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16). The characteristics of these 17 GIST cases (including the present case) are listed in Table I.…”

Section: Discussionmentioning

confidence: 87%

Giant gastrointestinal stromal tumor of the vermiform appendix: A case report

Kaneko

Kawai

Murono

et al. 2017

Molecular and Clinical Oncology

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Abstract.Gastrointestinal stromal tumors (GISTs) of the vermiform appendix are rare, measuring <3 cm in 82.4% of the reported cases. Neoadjuvant therapy with the receptor tyrosine kinase inhibitor imatinib mesylate has the potential to improve resectability and organ preservation rates in locally advanced or metastatic/recurrent GISTs. We herein report the case of a 67-year-old male patient with an unusually large GIST (22 cm in diameter) of uncertain origin in the right lower abdominal quadrant, with a solitary peritoneal metastasis. Due to the size of this GIST and presence of metastatic disease, neoadjuvant therapy with imatinib (400 mg/day orally) was administered. Follow-up imaging studies revealed marked shrinkage of the primary and metastatic tumors. Subsequently, laparoscopic exploration revealed that the main tumor originated from the tip of the vermiform appendix, and that the peritoneal metastasis was located in the ascending mesocolon. The patient underwent laparoscopic appendectomy and excision of the peritoneal metastasis, without tumor rupture. Therefore, in appropriately selected patients, neoadjuvant imatinib for borderline resectable or oligometastatic GISTs may be a reasonable choice.

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Section: Discussionmentioning

confidence: 87%

Giant gastrointestinal stromal tumor of the vermiform appendix: A case report

Kaneko

Kawai

Murono

et al. 2017

Molecular and Clinical Oncology

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“…In patients with a history of GIST who present with abdominal symptoms, recurrent disease should be considered. Metastatic and primary GISTs may present similar to acute appendicitis [2, 3]; even perforation of GIST within Meckel's diverticulum has been described [10]. Nevertheless, spread of GIST both to the appendix and Meckel's diverticulum is a very rare condition with as yet undefined guidelines for surgical intervention.…”

Section: Discussionmentioning

confidence: 99%

“…Approximately, 60% of GISTs originate from the stomach with the small bowel being the second most common site affected. Primary appendiceal GIST is extremely rare [2, 3]. The CD117 gene is positive in the majority of GISTs.…”

Section: Introductionmentioning

confidence: 99%

Acute Right Lower Abdomen in a Patient with a History of Gastrointestinal Stromal Tumor

Bandyopadhyay

Bonatti

2019

Case Reports in Surgery

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Background. Gastrointestinal stromal tumor (GIST) is an uncommon tumor of the GI tract usually seen in elderly patients, often difficult to diagnose because of the unspecific symptoms such as abdominal pain and fullness. Recurrent GIST may have an even more obscure clinical presentation. Case Report. A 44-year-old female with a history of surgically treated GIST presented to the emergency room complaining of acute onset sharp RLQ pain, nausea, and vomiting. Clinically, she had RLQ tenderness with rebound, her WBC was elevated, and CT scan showed signs of appendicitis and also soft tissue masses suspicious for malignancy. After contemplating treatment options including antibiotics and further workup, it was recommended to proceed with surgery. Laparoscopy showed a thickened appendix with nodular infiltration and multiple mass-like lesions in the RLQ not amendable to minimal invasive resection. From a lower midline incision, an open appendectomy and excision of multiple masses in the terminal ileum and in the abdominal wall were done. Narrow-based Meckel’s diverticulum with multiple nodular lesions was also removed. Pathology identified appendicitis and serosal involvement of GIST in all specimens staining positive for CD68, CD117, and vimentin. The patient was started on imatinib and remained recurrence-free after 6 months. Conclusions. This case illustrates a rare presentation of acute symptomatic recurrent metastatic GIST. Our patient was unusually young, and GIST recurrence presented with acute RLQ pain suggestive for acute appendicitis and also involved Meckel’s diverticulum. Surgical debulking followed by imatinib seems to be a reasonable approach in such cases.

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“…The Chinese databases were China National Knowledge Infrastructure (CNKI) (seven cases), VIP (eight cases), WANFANG DATA (13 cases), while the foreign databases included PubMed (12 cases) and EMBASE (four cases). After data synthesis, 20 reports were filtered [ 8 – 27 ], which included a total of 24 cases. One case of PAST that was identified during autopsy was excluded.…”

Section: Methodsmentioning

confidence: 99%

Clinicopathological characteristics and prognosis of primary appendiceal stromal tumors

et al. 2018

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BackgroundGastrointestinal stromal tumors (GISTs) account for less than 1% of all gastrointestinal tumors. The biological behaviors of GISTs vary from benign to malignant. GISTs are common in the stomach (55.6%) and small intestine (31.8%), but rarely in the rectum, colon (6%), and other sites (5.5%). Currently, the majority of published reports of primary appendiceal stromal tumors (PASTs) are case reports or case series.MethodsThe PASTs described in this study were identified from a literature review (23 cases) and our center (one case). The relationship between PAST gross types and clinicopathological factors was analyzed and summarized. At the same time, the study also analyzed the related risk factors and survival of PASTs and GISTs.ResultsTwenty-four cases of PASTs were compared with 254 cases of GISTs from our center. The results showed that there was a significant difference between the two groups in tumor size (P < 0.001), histological type (P = 0.013), CD34 expression (P < 0.001), and DOG-1 expression (P < 0.001). Disease-free survival (DFS) analysis of 11 cases of PASTs and 227 cases of GISTs found that a comparison of 3-year and 5-year DFS was not statistically significant (P = 0.894 and P = 0.846, respectively). In the DFS multivariate analysis, tumor mucosal ulceration, tumor size, and NIH risk classification were independent prognostic factors in 3-year and 5-year DFS.ConclusionIn this study, there was no significance in the survival of patients with appendix and gastric stromal tumors, which we hypothesized to be associated with the low sample size and incomplete follow-up records. Based on this, we conclude that the prognosis of primary appendiceal stromal tumors may be better than gastric tumors, but this needs to be confirmed in further prospective studies.

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Appendiceal GIST: report of an exceptional case and review of the litterature

Cited by 17 publications

References 9 publications

Giant gastrointestinal stromal tumor of the vermiform appendix: A case report

Giant gastrointestinal stromal tumor of the vermiform appendix: A case report

Acute Right Lower Abdomen in a Patient with a History of Gastrointestinal Stromal Tumor

Clinicopathological characteristics and prognosis of primary appendiceal stromal tumors

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