Ramineni Madhurya, Numbere Numbereye
Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, USA.
Cureus. 2024 Jun 14;16(6):e62366. doi: 10.7759/cureus.62366. eCollection 2024 Jun.
Solitary fibrous tumors (SFTs) are rare fibroblastic neoplasms with diverse biological behaviors and widespread distribution. Primary renal SFTs are uncommon, and their malignant variants, especially those that are CD34 negative, are even rarer. This study presents a case of malignant renal SFT in a 57-year-old female, focusing on its immunomorphological features. On gross examination, the tumor's large size (11.5 cm) was remarkable. Microscopic analysis showed high cellularity, diffuse sheets of moderately pleomorphic ovoid cells, prominent staghorn vessels, tumor cell necrosis, and a high mitotic count. Immunohistochemistry revealed strong positivity for STAT6, vimentin, and Bcl-2 and, notably, negativity for CD34. The presence of the gene fusion was confirmed through fluorescence in situ hybridization. This case emphasizes the need to consider SFT in the differential diagnosis of unusual renal tumors, even when CD34 is negative. The infrequency, morphological diversity, and resemblance to other tumors make diagnosing renal SFTs challenging. Accurate identification and classification as benign or malignant are crucial for proper clinical management and prognosis.
孤立性纤维瘤(SFTs)是一种罕见的成纤维细胞性肿瘤,具有多样的生物学行为且分布广泛。原发性肾孤立性纤维瘤并不常见,其恶性变体,尤其是那些CD34阴性的变体,更为罕见。本研究报告了一例57岁女性的恶性肾孤立性纤维瘤病例,重点关注其免疫形态学特征。大体检查显示,肿瘤体积较大(11.5 cm),十分显著。显微镜分析显示细胞密度高,弥漫性片状分布的中等多形性卵圆形细胞,鹿角状血管突出,肿瘤细胞坏死,有丝分裂计数高。免疫组化显示STAT6、波形蛋白和Bcl-2呈强阳性,值得注意的是,CD34呈阴性。通过荧光原位杂交证实了基因融合的存在。该病例强调,即使CD34阴性,在鉴别诊断不寻常的肾肿瘤时也需要考虑孤立性纤维瘤。其罕见性、形态多样性以及与其他肿瘤的相似性使得肾孤立性纤维瘤的诊断具有挑战性。准确识别并分类为良性或恶性对于正确的临床管理和预后至关重要。