Laroia Shalini Thapar, Yadav Taruna, Rastogi Archana, Sarin Shiv
Department of Radiology, Institute of Liver and Biliary Sciences, Sector D-1, Vasant Kunj, New Delhi 110070, India.
Department of Hepato-Pathology, Institute of Liver and Biliary Sciences, Sector D-1, Vasant Kunj, New Delhi 110070, India.
World J Oncol. 2016 Jun;7(2-3):45-50. doi: 10.14740/wjon926w. Epub 2016 Jul 1.
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.
胃肠道外间质瘤（EGISTs）是一组最近才被描述的肿瘤。据我们所知，迄今为止，英文文献中报道的此类病例不足70例。胃肠道间质瘤（GISTs）是消化道最常见的间叶性肿瘤。EGISTs是一种独特的实体，需要与GISTs相区分，因为尽管它们在组织学和免疫组化方面与GISTs相似，但它们发生在胃肠道外，如大网膜、肠系膜、腹膜后等部位，并且就其预后和治疗而言具有不同的行为模式。EGISTs的腹膜后亚组极为罕见，我们报告了一例腹膜后原发性恶性EGIST，在影像学评估中表现为软组织肿块。该肿瘤在组织病理学上令人惊讶，只有在进行形态学和免疫组化研究后才能明确诊断为EGIST。由于这类肿瘤进展迅速且预后不良，放射科医生、病理科医生和肿瘤内科医生等临床医生必须能够识别和了解其表现，以便早期诊断和治疗能够改善最终的疾病结局。
