Giant gastrointestinal stromal tumour of rare sarcomatoid epithelioid subtype: Case study and literature review

Lech, Gustaw; Korcz, Wojciech; Kowalczyk, Emilia; Guzel, Tomasz; Radoch, Marcin; Krasnodębski, Ireneusz W.

doi:10.3748/wjg.v21.i11.3388

Cited by 10 publications

(12 citation statements)

References 13 publications

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“…These findings appear to be similar to those described by Choi et al in their paper where response and prognosis of EGIST based on tumor size have been questioned [ 2 ]. The NCCN risk table and the NIH classification system have often been used to prognosticate and manage EGISTs; however, no significant data have been demonstrated to support their appropriateness [ 16 , 24 ].…”

Section: Discussionmentioning

confidence: 99%

Malignant Retroperitoneal Extra-Gastrointestinal Stromal Tumor: A Unique Entity

et al. 2016

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Extra-gastrointestinal stromal tumors (EGISTs) are a recently described group of tumors. A handful of less than 70 cases have been reported in English literature, so far, to the best of our knowledge. Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the alimentary canal. EGISTs are a unique entity, which require distinction from GISTs because, even though, they exhibit similar histology and immunohistochemistry to GISTs, they occur outside the gastrointestinal tract, i.e. in omentum, mesentery, retroperitoneum, etc. and have different behavior patterns as far as their prognosis and management are concerned. Retroperitoneal sub-group of EGISTs is extremely rare and we report such a case of primary malignant EGIST of the retroperitoneum which presented as a soft tissue mass on radiological evaluation. The tumor turned out to be a histopathological surprise, and could be distinctively labeled as EGIST only after morphological and immunohistochemical studies. It is imperative for radiologists, pathologists and oncologists, among other clinicians, to be able to recognize and understand the presentation of this group of tumors due to their rapid progression and poor prognosis, so that an early diagnosis and management may be able to improve the final disease outcome.

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Section: Discussionmentioning

confidence: 99%

Malignant Retroperitoneal Extra-Gastrointestinal Stromal Tumor: A Unique Entity

et al. 2016

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“…Our literature review confirmed that with early diagnosis, surgery is safe and feasible in these cases. R0 resection can provide good prognosis in these patients . In our sample, there were five patients with epithelioid cell GISTs; four patients had exon 11 mutation and the other had exon D842V mutation of PDGFRA 18.…”

Section: Discussionmentioning

confidence: 87%

Treatment options for giant gastrointestinal stromal tumours and related prognosis: a single‐centre retrospective study

Wang

Tao

Wang

et al. 2020

ANZ Journal of Surgery

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Background: Controversy persists about the best treatment for giant gastrointestinal stromal tumours (GISTs). This retrospective study aimed to investigate the different treatments for giant GISTs and the effect on outcomes. Methods: A total of 71 patients with GIST ≥10 cm in diameter were separated into four groups according to treatment received: emergency surgery group (n = 17), preoperative targeted (imatinib) therapy group (n = 12), palliative resection group (n = 17) and R0 resection group (n = 25). Baseline, intraoperative and post-operative findings were compared between the groups. Long-term follow-up was conducted to assess outcomes. Results: Preoperative gastrointestinal bleeding was significantly higher (P = 0.003) and haemoglobin level was significantly lower (P < 0.05) in the emergency surgery group than in the other groups. Mean tumour diameter was significantly more in the palliative resection group than in the other groups (P = 0.023). Overall survival was significantly higher in the R0 resection group and the preoperative targeted therapy group than in the other two groups (P < 0.05). Conclusion: In patients with giant GISTs, the best outcomes appear to be achieved with preoperative imatinib therapy plus surgery or R0 resection followed by imatinib therapy.© 2020 Royal Australasian College of Surgeons ANZ J Surg 90 (2020) 762-766ANZJSurg.com

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“…The spindled, epithelioid and mixed morphology GISTs are reported to be significantly more common in the small intestine, stomach, and stomach, respectively. [ 4 , 5 ] The growth of most GISTs is driven by oncogene mutations in either of two receptor tyrosine kinases: C-KIT (70%–80%) or platelet-derived growth factor receptor alpha (PDGFR-α) (8%–10%). [ 4 , 6 ] No mutants for both genes of GISTs are wild type.…”

Section: Discussionmentioning

confidence: 99%

The epithelioid gastrointestinal stromal tumor with pulmonary metastasis

Lin

Qiu

2020

Medicine

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Rationale: Available literature states that the histological subtype of the gastrointestinal stromal tumor (GIST) with pulmonary metastasis is often spindle cell type. To our knowledge, this is the first report of the GIST with pulmonary metastasis of very uncommon epithelioid subtype. Patient concerns: We report a 63-year-old male presenting with the symptom of bloodstained sputum without obvious inducement. The patient had no chest pain, low back pain, fatigue, fever or night sweats symptoms. Diagnoses: Combined chest digital radiography and the history of the patient who presented with the colon GIST of the epithelioid subtype two years ago that the mass may be a metastasis tumor. Combined with morphological and immunohistochemical staining results, a pathological diagnosis of the GIST with pulmonary metastasis was considered. Interventions: Right lobectomy and partial upper lobectomy were performed. Outcomes: The patient had not experienced any noticeable symptom and recurrent tumors at 6 months follow-up. Lessons: We report a rare case of the GIST with pulmonary metastasis of epithelioid subtype. This case is of great significance to the pathologist's clinical work. For pathologists, if an epithelioid tumor in the lung is found, it is necessary to check whether the gastrointestinal tract also has the tumor, which may be an epithelioid GIST with pulmonary metastasis.

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Giant gastrointestinal stromal tumour of rare sarcomatoid epithelioid subtype: Case study and literature review

Cited by 10 publications

References 13 publications

Malignant Retroperitoneal Extra-Gastrointestinal Stromal Tumor: A Unique Entity

Malignant Retroperitoneal Extra-Gastrointestinal Stromal Tumor: A Unique Entity

Treatment options for giant gastrointestinal stromal tumours and related prognosis: a single‐centre retrospective study

The epithelioid gastrointestinal stromal tumor with pulmonary metastasis

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