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伴有骨分化和基质钙化的胃胃肠道间质瘤:一例报告并文献复习

Gastric gastrointestinal stromal tumor with osseous differentiation and stromal calcification: A case report and review of literature.

作者信息

Al-Maghrabi Haneen, Meliti Abdelrazak

机构信息

Anatomic pathology department, King Faisal Specialist Hospital & Research Centre, Jeddah, Saudi Arabia.

出版信息

SAGE Open Med Case Rep. 2017 Dec 21;5:2050313X17746310. doi: 10.1177/2050313X17746310. eCollection 2017.

Abstract

Gastrointestinal stromal tumors are the most common primary mesenchymal tumors of the gastrointestinal tract accounting for 0.1%-3.0% of all gastrointestinal malignancies. The stomach is the most common site (60%) followed by the small bowel (30%-35%) particularly jejunum and ileum, colorectum (5%) and rarely affect esophagus and appendix. Most gastrointestinal stromal tumors arise sporadically, however, less commonly they develop in association with various clinical syndromes like Carney triad, Carney-Stratakis syndrome, familial gastrointestinal stromal tumor syndrome and neurofibromatosis type1 (NF1). We report a 65-year-old male patient presented with gastric mass (7.5 × 4.5 × 3.5 cm) arising from the posterior gastric wall. Histologic examination revealed neoplastic proliferation of spindled and epithelioid cells with focal plexiform pattern and low mitotic activity 3/50 HPF. No evidence of cytological atypia, abnormal mitosis or necrosis. Interestingly enough, there were focal areas of mature bone formation/osseous differentiation associated with calcification. The tumor cells were strongly positive for CD117, DOG1 with focal immunoreactivity against CD34. The morphologic features and the immunoprofile were diagnostic of gastrointestinal stromal tumor. Herein, we present a rare case of gastric gastrointestinal stromal tumor with mature bone formation, osseous metaplasia and calcification. To the best of our knowledge, this is the second case report of gastric gastrointestinal stromal tumor with osseous differentiation and mature bone formation.

摘要

胃肠道间质瘤是胃肠道最常见的原发性间叶组织肿瘤,占所有胃肠道恶性肿瘤的0.1%-3.0%。胃是最常见的发病部位(60%),其次是小肠(30%-35%),尤其是空肠和回肠,结直肠(5%),很少累及食管和阑尾。大多数胃肠道间质瘤为散发性,然而,较少情况下它们与各种临床综合征相关,如卡尼三联征、卡尼-斯特拉塔基斯综合征、家族性胃肠道间质瘤综合征和1型神经纤维瘤病(NF1)。我们报告一例65岁男性患者,其胃后壁出现一个胃肿物(7.5×4.5×3.5cm)。组织学检查显示梭形细胞和上皮样细胞呈肿瘤性增生,具有局灶性丛状结构,有丝分裂活性低,50个高倍视野中有3个。无细胞学异型性、异常有丝分裂或坏死的证据。有趣的是,存在与钙化相关的局灶性成熟骨形成/骨分化区域。肿瘤细胞CD117、DOG1呈强阳性,对CD34有局灶性免疫反应。形态学特征和免疫表型可诊断为胃肠道间质瘤。在此,我们报告一例罕见的伴有成熟骨形成、骨化生和钙化的胃胃肠道间质瘤。据我们所知,这是第二例伴有骨分化和成熟骨形成的胃胃肠道间质瘤病例报告。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a590/5753916/d237fedd89fa/10.1177_2050313X17746310-fig1.jpg

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