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胰腺孤立性纤维瘤:9 例分析并文献复习。

Solitary Fibrous Tumor of the Pancreas: Analysis of 9 Cases With Literature Review.

机构信息

Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, NY.

NewPath Diagnostics, Queens, NY.

出版信息

Am J Surg Pathol. 2023 Nov 1;47(11):1230-1242. doi: 10.1097/PAS.0000000000002108. Epub 2023 Aug 11.

Abstract

Solitary fibrous tumor (SFT) has been increasingly reported in various anatomic sites. However, it is still extremely rare in the pancreas. Herein, we present the first series of primary pancreatic SFTs. Nine cases of primary pancreatic SFTs were analyzed. The mean age was 60 years (36 to 76 y) with no sex predilection. Six tumors were in the head, 3 were in the tail. On imaging studies, tumors were described as a hypervascular mass, 2 revealed cystic areas, and 3 were favored to be neuroendocrine tumors. On biopsy, 2 cases were diagnosed as atypical spindle cell tumor; one was misdiagnosed as suspicious for sarcoma, and another case as metastatic renal cell carcinoma. Two were diagnosed as low-grade sarcoma and low-grade stromal tumor on frozen sections. Grossly, tumors were well-demarcated with a median size of 4 cm (0.9 to 15 cm). Microscopically, they were composed of ovoid to spindle tumor cells with no significant mitotic activity and were arranged in alternating hypercellular and hypocellular areas. Staghorn-like vessels and entrapped pancreatic parenchyma were also detected within all tumors. Tumor cells revealed diffuse/strong nuclear STAT6 expression in 7 of 8, CD34 in 7 of 9, and bcl-2 in 4 of 4 tested cases. One tested tumor harbored NAB2 - STAT6 fusion. Eight patients with available follow-up data were free of disease at a mean follow-up of 76 months (3 to 189 mo). SFT should be considered in the differential diagnoses of mesenchymal neoplasms of the pancreas. Immunohistochemical nuclear STAT6 expression is a characteristic feature of SFT. Primary pancreatic SFTs seem to have favorable biological behavior in our series.

摘要

孤立性纤维瘤(SFT)在各种解剖部位的报道越来越多。然而,它在胰腺中仍然极为罕见。在此,我们报告了首例原发性胰腺 SFT 系列病例。分析了 9 例原发性胰腺 SFT。平均年龄为 60 岁(36 至 76 岁),无性别倾向。6 个肿瘤位于头部,3 个位于尾部。在影像学研究中,肿瘤被描述为富血管性肿块,2 个显示囊性区域,3 个被认为是神经内分泌肿瘤。在活检中,2 例诊断为非典型梭形细胞瘤;1 例误诊为肉瘤,另 1 例误诊为转移性肾细胞癌。2 例在冷冻切片上诊断为低级别肉瘤和低级别间质瘤。大体上,肿瘤边界清楚,中位大小为 4cm(0.9 至 15cm)。显微镜下,由卵圆形至梭形肿瘤细胞组成,无明显有丝分裂活性,排列在交替的细胞丰富区和细胞稀少区。所有肿瘤内均检测到鹿角样血管和包裹的胰腺实质。在 7 例中的 8 例肿瘤细胞弥漫/强核 STAT6 表达、7 例中的 9 例 CD34 表达和 4 例中的 4 例 bcl-2 表达。1 例检测的肿瘤存在 NAB2-STAT6 融合。8 例有随访资料的患者平均随访 76 个月(3 至 189 个月)后无疾病。在胰腺间叶性肿瘤的鉴别诊断中应考虑 SFT。免疫组化核 STAT6 表达是 SFT 的特征性表现。在我们的系列中,原发性胰腺 SFT 似乎具有良好的生物学行为。

相似文献

1
Solitary Fibrous Tumor of the Pancreas: Analysis of 9 Cases With Literature Review.胰腺孤立性纤维瘤:9 例分析并文献复习。
Am J Surg Pathol. 2023 Nov 1;47(11):1230-1242. doi: 10.1097/PAS.0000000000002108. Epub 2023 Aug 11.

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