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具有不寻常特征的胰腺孤立性纤维瘤:一例报告

Solitary Fibrous Tumor of Pancreas With Unusual Features: A Case Report.

作者信息

Afzal Anoshia, Maldonado-Vital Manuel, Khan Shahbaz, Farooque Umar, Luo Wenyi

机构信息

Pathology, University of Oklahoma Health Sciences Center, Oklahoma City, USA.

Neurology, Dow University of Health Sciences, Karachi, PAK.

出版信息

Cureus. 2020 Oct 6;12(10):e10833. doi: 10.7759/cureus.10833.

Abstract

Solitary fibrous tumor (SFT) is an uncommon fibroblastic neoplasm that is most commonly associated with the pleura but has also been reported in almost all anatomic sites. Although the majority of SFTs are benign, few cases follow a malignant clinical course and may recur and/or metastasize after several years of their original occurrence. Only 16 cases of pancreatic SFTs are reported so far, and only one has metastasized to lung and subcutis. Pancreatic SFT resembles more common neuroendocrine tumor and gastrointestinal stromal tumor (GIST) radiographically and is at times almost indistinguishable from GIST histologically. Diagnosis of SFTs particularly, if attempted on biopsied specimens, can be very challenging due to its rare occurrence and nondescript morphology. It is imperative to understand the pathological spectrum of this entity to avoid misdiagnosis. We report a case of pancreatic SFT in a 43-year-old male with some unusual morphologic and immunohistochemical features including pseudoangiomatous growth pattern, a hypercellular area demonstrating nuclear pleomorphism, and only focal positivity for cluster of differentiation (CD)34. These atypical features can pose even more diagnostic challenge by causing additional confusion with other malignancies like dedifferentiated liposarcoma and vascular tumors. The potential diagnostic pitfalls are discussed.

摘要

孤立性纤维性肿瘤(SFT)是一种罕见的成纤维细胞肿瘤,最常与胸膜相关,但几乎在所有解剖部位均有报道。尽管大多数SFT是良性的,但少数病例呈恶性临床过程,可能在原发后数年复发和/或转移。迄今为止,仅报道了16例胰腺SFT,其中仅1例转移至肺和皮下组织。胰腺SFT在影像学上与更常见的神经内分泌肿瘤和胃肠道间质瘤(GIST)相似,有时在组织学上几乎与GIST无法区分。由于SFT罕见且形态不典型,因此对其进行诊断,尤其是通过活检标本进行诊断,极具挑战性。了解该实体的病理谱以避免误诊至关重要。我们报告了1例43岁男性的胰腺SFT,其具有一些不寻常的形态学和免疫组化特征,包括假血管瘤样生长模式、显示核多形性的高细胞区以及仅局灶性分化簇(CD)34阳性。这些非典型特征会与去分化脂肪肉瘤和血管肿瘤等其他恶性肿瘤造成更多混淆,从而带来更大的诊断挑战。本文讨论了潜在的诊断陷阱。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8908/7647364/1eeff7a1c8d1/cureus-0012-00000010833-i01.jpg

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