Solitary fibrous tumor of the pancreas in a patient with tumor duplicity: a case report.

Q4 Medicine
Ceskoslovenska patologie Pub Date : 2025-01-01
Jan Hrudka, Václav Eis, Radoslav Matěj
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引用次数: 0

Abstract

This case report describes a 71-year-old male patient in whom a solitary fibrous tumor (SFT) of the pancreatic tail was incidentally discovered during staging of chronic lymphocytic leukemia/small lymphocytic lymphoma (B-CLL/SLL). The patient also had a history of excised malignant melanoma. SFT is a mesenchymal neoplasm characterized by NAB2::STAT6 gene fusion, STAT6 and CD34 immunohistochemical positivity, and unclear biological behavior. In this case, the tumor was a firm, well-circumscribed spindle cell lesion without cytologic atypia, necrosis, or significant mitotic activity, showing strong diffuse STAT6 and CD34 expression. According to WHO classification criteria, it was classified as a low-risk SFT with respect to metastatic potential. The diagnosis of SFT is based on characteristic morphology and nuclear expression of STAT6, which helps distinguish it from a broad spectrum of CD34-positive mesenchymal lesions. The article discusses relevant differential diagnoses and highlights the molecular basis of SFT, including the prognostic implications of different NAB2::STAT6 fusion variants and the association of TERT promoter mutations with more aggressive behavior. Although pancreatic SFT is rare, similar cases have been reported in the literature. From a clinical standpoint, accurate risk stratification for recurrence or metastasis is essential. Several scoring systems have been proposed and validated, including the one adopted in the WHO classification, which considers tumor size, mitotic rate, necrosis, and patient age. In this case, the tumor was completely resected, and the patient has remained disease-free with no signs of SFT recurrence or B-CLL/SLL progression more than six months after surgery.

单发胰腺纤维性肿瘤伴肿瘤双重性1例报告。
本病例报告描述了一位71岁男性患者,他在慢性淋巴细胞白血病/小淋巴细胞淋巴瘤(B-CLL/SLL)分期期间偶然发现胰腺尾部孤立性纤维性肿瘤(SFT)。患者也有恶性黑色素瘤切除史。SFT是一种以NAB2::STAT6基因融合、STAT6与CD34免疫组化阳性、生物学行为不明确为特征的间充质肿瘤。在本例中,肿瘤是一个坚固的、界限清晰的梭形细胞病变,没有细胞学上的异型性、坏死或明显的有丝分裂活性,表现出强烈的弥漫性STAT6和CD34表达。根据世界卫生组织的分类标准,就转移潜力而言,它被归类为低风险SFT。SFT的诊断基于STAT6的特征形态学和核表达,这有助于将其与广泛的cd34阳性间质病变区分开来。本文讨论了相关的鉴别诊断,并强调了SFT的分子基础,包括不同NAB2::STAT6融合变异的预后意义以及TERT启动子突变与更具攻击性行为的关联。虽然胰腺SFT罕见,但文献中也有类似的病例报道。从临床角度来看,准确的复发或转移风险分层是必不可少的。已经提出并验证了几种评分系统,包括世界卫生组织分类中采用的评分系统,该评分系统考虑了肿瘤大小、有丝分裂率、坏死和患者年龄。在本例中,肿瘤被完全切除,患者在手术后6个多月没有SFT复发或B-CLL/SLL进展的迹象。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Ceskoslovenska patologie
Ceskoslovenska patologie Medicine-Medicine (all)
CiteScore
0.40
自引率
0.00%
发文量
17
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