Miettinen Markku, Paal Edina, Lasota Jerzy, Sobin Leslie H
Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Am J Surg Pathol. 2002 Mar;26(3):301-11. doi: 10.1097/00000478-200203000-00003.
Glomus tumors usually occur in the peripheral soft tissues, but similar tumors have also been reported in the stomach and occasionally in the intestines. However, the relationship of these tumors to peripheral glomus tumors and gastrointestinal stromal tumors has not been fully clarified because previous series of gastrointestinal glomus tumors predate availability of immunohistochemistry. This clinicopathologic study examined 32 gastrointestinal glomus tumors. All but one of the tumors were located in the stomach and the remaining tumor was from the cecum. The tumors occurred with a strong female predominance (23 females and 9 males) and a median age of 55 years (range 19-90 years). The gastric tumors typically presented with gastrointestinal bleeding or ulcer-like symptoms, and 14 tumors had mucosal ulceration. Five tumors were incidental findings. The tumor sizes varied from 1.1 to 7 cm (median 2 cm), and most were located in the antrum. Histologically, the tumors typically had a solid pattern of sharply demarcated, round glomus cells with prominent, mildly dilated pericytoma-like vessels. Vascular invasion and focal atypia were relatively common (seen in 11 and 13 cases, respectively), and low mitotic activity (1-4 per 50 high power fields), was seen in 10 cases. Immunohistochemically, all tumors were positive for alpha-smooth muscle actin and calponin, and nearly all had a net-like pericellular laminin and collagen type IV positivity. All tumors were negative for desmin and S-100 protein. Three tumors had focal synaptophysin positivity, but none was positive for chromogranin. All tumors lacked KIT expression and the GIST-specific mutations in the c-kit gene. Follow-up revealed one patient death of metastatic disease to liver at 50 months; this tumor had 1 mitosis per 50 high power fields, but had spindle cell foci, mild atypia, and vascular invasion. Thirteen patients were well and alive after long-term follow-up. Gastrointestinal glomus tumors occur almost exclusively in the stomach, and they have a good overall prognosis, but a small, unpredictable potential for malignant behavior exists. These tumors are phenotypically similar to peripheral glomus tumors and differ from epithelioid GISTs.
血管球瘤通常发生于外周软组织，但胃内也有类似肿瘤的报道，偶尔也可见于肠道。然而，由于既往关于胃肠道血管球瘤的系列研究早于免疫组化技术的应用，这些肿瘤与外周血管球瘤及胃肠道间质瘤的关系尚未完全阐明。本临床病理研究对32例胃肠道血管球瘤进行了检查。除1例肿瘤位于盲肠外，其余肿瘤均位于胃。肿瘤发生以女性为主（23例女性和9例男性），中位年龄55岁（范围19 - 90岁）。胃肿瘤通常表现为胃肠道出血或溃疡样症状，14例肿瘤有黏膜溃疡形成。5例肿瘤为偶然发现。肿瘤大小从1.1至7 cm不等（中位值2 cm），多数位于胃窦部。组织学上，肿瘤通常呈实性结构，边界清晰，圆形的血管球细胞，伴有显著、轻度扩张的类周细胞瘤样血管。血管侵犯和局灶性异型性相对常见（分别见于11例和13例），10例可见低核分裂活性（每50个高倍视野1 - 4个核分裂象）。免疫组化方面，所有肿瘤α - 平滑肌肌动蛋白和钙调蛋白均呈阳性，几乎所有肿瘤细胞周围层粘连蛋白和IV型胶原呈网状阳性。所有肿瘤结蛋白和S - 100蛋白均为阴性。3例肿瘤局灶性突触素呈阳性，但嗜铬粒蛋白均为阴性。所有肿瘤均无KIT表达及c - kit基因的胃肠道间质瘤特异性突变。随访发现1例患者在50个月时死于肝转移；该肿瘤每50个高倍视野有1个核分裂象，但有梭形细胞灶、轻度异型性及血管侵犯。13例患者经长期随访情况良好且存活。胃肠道血管球瘤几乎仅发生于胃，总体预后良好，但存在小的、不可预测的恶性行为潜能。这些肿瘤在表型上与外周血管球瘤相似，与上皮样胃肠道间质瘤不同。
