Plexiform Fibromyxoma A Distinctive Benign Gastric Antral Neoplasm Not to be Confused With a Myxoid GIST
Plexiform Fibromyxoma A Distinctive Benign Gastric Antral Neoplasm Not to be Confused With a Myxoid GIST
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DOI：
10.1097/pas.0b013e3181ae666a
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发表时间：
2009-11-01
影响因子：
5.6
通讯作者：
Lasota, Jerzy
中科院分区：
文献类型：
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作者：
Miettinen, Markku;Makhlouf, Hala R.;Lasota, Jerzy
胃间充质瘤绝大多数为胃肠道间质瘤(GIST)。一组罕见的非GIST包括粘液样间充质肿瘤。在这篇报告中，我们描述了12例特殊的胃肿瘤，这里命名为LIS丛状纤维粘液瘤。这些肿瘤发生在5名男性和7名女性，年龄在7到75岁(中位数，41岁)。所有肿瘤均位于胃窦，其中6例还侵犯胃外软组织或十二指肠球部。肿瘤直径3~15 cm，中位数5.5 cm。组织学上典型的是多个微小结节的丛状壁内生长，含有少细胞到中等细胞的粘液样到胶原和纤维粘液样的肿瘤成分。典型的表现为突出的、有时呈丛状的毛细血管图案。壁外成分包括浆膜下结节，有时更多的细胞，实实无丛的梭形细胞增殖。肿瘤细胞从椭圆形到梭形不等，异型性和有丝分裂活性有限&5/50高倍视野。常见的溃疡、黏膜侵犯和血管侵犯(4例)在这些肿瘤中无不良意义。免疫组织化学显示，肿瘤细胞对平滑肌肌动蛋白呈阳性反应，对CD10呈不同程度的阳性反应，对KIT、DOG1、CD34、结蛋白和S100蛋白均呈阴性反应。在所检查的3个病例中，没有出现KIT或血小板衍生生长因子受体α突变。随访9~20年(中位数19年)的4例患者无一例复发或转移。另有3名肿瘤状态不明的患者存活了14-25年。回顾大量的食道和肠道间充质肿瘤，没有发现类似的肿瘤。丛状纤维粘液瘤是一种独特的良性胃窦肿瘤，应与胃肠道间质瘤、神经鞘瘤及其他纤维粘液样肿瘤相鉴别。
A great majority of gastric mesenchymal tumors are gastrointestinal stromal tumor (GIST). A rare group of non-GISTs include myxoid mesenchymal neoplasms. In this report, we describe 12 cases of a distinctive gastric tumor, named here Lis plexiform fibromyxoma. These tumors occurred in 5 men and 7 women of ages 7 to 75 years (median, 41 y). All tumors were located in the gastric antrum and 6 of them also extended into extragastric soft tissues or into the duodenal bulb. The tumors measured from 3 to 15 cm (median, 5.5cm). Histologically typical was a plexiform intramural growth with multiple micronodules containing paucicellular to moderately cellular myxoid to collagenous and fibromyxoid neoplastic elements. A prominent, sometimes plexiform capillary pattern was typically present. Extramural components included subserosal nodules, and sometimes more cellular, solid nonplexiform spindle cell proliferation. The tumor cells varied from oval to spindled and had limited atypia and mitotic activity < 5/50 high-power fields. Frequent ulceration, mucosal invasion, and vascular invasion (4 cases) had no adverse significance in these tumors. Immunohistochemically, the tumor cells were positive for a smooth muscle actin, and variably for CD10, and were consistently negative for KIT, DOG1, CD34, desmin, and S100 protein. No KIT or platelet-derived growth factor receptor alpha Mutations were present in the 3 examined cases. None of the 4 patients who were followed from 9 to 20 years (median, 19 y) developed recurrences or metastases. Additional 3 patients survived 14 to 25 years with unknown tumor status. Review of large numbers of mesenchymal tumors in the esophagus and intestines did not reveal similar tumors. Plexiform fibromyxoma is a distinctive benign gastric antral neoplasm that should be separated from GIST, nerve sheath tumors, and other fibromyxoid neoplasms.