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眼眶成纤维细胞性网状细胞瘤:一例罕见疾病的病例报告及文献综述

Orbital fibroblastic reticular cell tumor: A case report and literature review for a rare disease.

作者信息

Yan Yu, Li Shuangle, Zhou Mi, Zhong Qin

机构信息

Department of Ophthalmology, Zigong First People's Hospital, Zigong, Sichuan Province, China.

Department of Ophthalmology, Mianyang Traditional Chinese Medicine Hospital, Mianyang, Sichuan Province, China.

出版信息

Medicine (Baltimore). 2025 Jun 6;104(23):e42807. doi: 10.1097/MD.0000000000042807.

Abstract

RATIONALE

This study investigates the clinical, imaging, and pathological features of fibroblastic reticular cell tumors (FRCTs) through a retrospective analysis of a patient with FRCT, along with a review of relevant literature.

PATIENT CONCERNS

A 49-year-old male was admitted to our hospital because of swelling and discomfort in the right eye, occasionally accompanied by double vision, for more than 3 months. Physical examination revealed an obliquely downward right eye, ptosis, and a palpable medium-hard tumor at the supraorbital rim of the orbit.

DIAGNOSES

An orbital B-type ultrasound, orbital computed tomography, and orbital contrast-enhanced magnetic resonance imaging were performed, and the findings suggested a diagnosis of right orbital hemangioma.

INTERVENTIONS

Following imaging studies, the tumor was surgically excised. Microscopic pathological examination revealed that the lesion was composed of lymphatic follicles and spindle cells. Immunohistochemistry revealed that: the tumor is mainly composed of spindle fibroblastic cells, accompanied by the formation of lymphoid follicles. Immunohistochemical staining shows that the lymphoid follicles express CD20 positively, while the Ki67 positive index of the spindle tumor cells is lower. Based on these findings, the pathologists believed that the lesion was consistent with an FRCT.

OUTCOMES

The patient refused subsequent treatment and was discharged. Postoperative imaging (computed tomography and magnetic resonance imaging) conducted at 4 and 24 weeks revealed no recurrence of the tumor.

LESSONS

FRCTs are exceedingly rare in clinical practice, This is the first case report of an orbital FRCT. The main clinical manifestation is a painless orbital mass, and the imaging findings are nonspecific; therefore, the diagnosis mainly depends on the pathology and immune phenotype of the tumor. Currently, there are no detailed data regarding the effects of postoperative adjuvant therapy. With more reports and studies on patients with FRCT, the diagnostic accuracy for this disease can be increased, and more accurate and personalized treatment plans can be developed.

摘要

原理

本研究通过对1例纤维母细胞性网状细胞瘤(FRCT)患者进行回顾性分析,并复习相关文献,探讨FRCT的临床、影像学及病理特征。

患者情况

一名49岁男性因右眼肿胀不适伴偶尔复视3个多月入院。体格检查发现右眼斜向下、上睑下垂,眼眶眶上缘可触及中等硬度肿块。

诊断

行眼眶B型超声、眼眶计算机断层扫描及眼眶增强磁共振成像检查,结果提示诊断为右眼眶血管瘤。

干预措施

影像学检查后,手术切除肿瘤。显微镜下病理检查显示病变由淋巴滤泡和梭形细胞组成。免疫组织化学显示:肿瘤主要由梭形纤维母细胞组成,伴有淋巴滤泡形成。免疫组织化学染色显示淋巴滤泡CD20阳性,梭形肿瘤细胞Ki67阳性指数较低。基于这些发现,病理学家认为该病变符合FRCT。

结果

患者拒绝后续治疗并出院。术后4周和24周进行的影像学检查(计算机断层扫描和磁共振成像)显示肿瘤无复发。

经验教训

FRCT在临床实践中极为罕见,这是首例眼眶FRCT病例报告。主要临床表现为无痛性眼眶肿块,影像学表现无特异性;因此,诊断主要依赖于肿瘤的病理及免疫表型。目前,关于术后辅助治疗效果尚无详细数据。随着对FRCT患者的更多报道和研究,可以提高该病的诊断准确性,并制定更准确、个性化的治疗方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5672/12150983/2fdf7599012a/medi-104-e42807-g001.jpg

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