Shinohara Yuki, Chijiiwa Yoshiro, Nishio Jun
Section of Orthopaedic Surgery, Department of Medicine, Fukuoka Dental College, Fukuoka, Japan.
Cancer Diagn Progn. 2024 Nov 3;4(6):825-827. doi: 10.21873/cdp.10403. eCollection 2024 Nov-Dec.
BACKGROUND/AIM: Desmoplastic fibroblastoma, also known as collagenous fibroma, is a rare benign mesenchymal neoplasm that primarily arises in the subcutaneous tissue of upper extremities and limb girdles. The knee is an uncommon location for desmoplastic fibroblastoma. Recent studies have demonstrated the presence of immunoreactivity for FOS like 1 (FOSL1) and rearrangements of FOSL1.
A 70-year-old woman presented with a 1-year history of a palpable mass in the medial aspect of the right knee. Physical examination revealed a 4-cm, elastic hard, mobile, nontender mass. Magnetic resonance imaging (MRI) showed a well-defined mass with prominent low signal intensity on all pulse sequences. Contrast-enhanced MRI demonstrated mild internal enhancement with rim enhancement. After an open biopsy, the lesion was successfully treated by complete excision. Histologically, the tumor was composed of bland spindle or stellate-shaped cells embedded in an abundant collagenous stroma. Immunohistochemically, the tumor cells showed diffuse nuclear positivity for FOSL1. These findings were consistent with a diagnosis of desmoplastic fibroblastoma. The patient was asymptomatic and there was no evidence of local recurrence eight months after surgery.
Desmoplastic fibroblastoma is a distinctive benign soft-tissue tumor with FOSL1 immunoreactivity and should be clearly distinguished from more biologically aggressive mesenchymal neoplasms.
背景/目的:促纤维增生性成纤维细胞瘤,也称为胶原纤维瘤,是一种罕见的良性间叶性肿瘤,主要发生于上肢和肢带的皮下组织。膝关节是促纤维增生性成纤维细胞瘤的罕见发病部位。最近的研究表明存在FOS样蛋白1(FOSL1)免疫反应性以及FOSL1重排。
一名70岁女性,右膝内侧可触及肿块1年。体格检查发现一个4厘米大小、质地硬且有弹性、可活动、无压痛的肿块。磁共振成像(MRI)显示一个边界清晰的肿块,在所有脉冲序列上均表现为明显的低信号强度。增强MRI显示内部轻度强化,边缘强化。开放活检后,通过完整切除成功治疗了该病变。组织学上,肿瘤由嵌入丰富胶原基质中的温和梭形或星状细胞组成。免疫组化显示肿瘤细胞FOSL1弥漫性核阳性。这些发现符合促纤维增生性成纤维细胞瘤的诊断。患者无症状,术后8个月无局部复发迹象。
促纤维增生性成纤维细胞瘤是一种具有FOSL1免疫反应性的独特良性软组织肿瘤,应与生物学行为更具侵袭性的间叶性肿瘤明确区分。