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子宫颈罕见表现的炎性肌纤维母细胞瘤:一例报告

Inflammatory myofibroblastic tumour of an unusual presentation in the uterine cervix: a case report.

作者信息

López de Sa Alfonso, Pascual Alejandro, Garcia Santos Javier, Mendez Ramiro, Bellon Monica, Ramirez Mar, Matute Fatima, Del Arco Cristina, Manzano Aránzazu, Coronado Pluvio, Casado Antonio, Marquina Gloria

机构信息

Department of Medical Oncology, Hospital Clinico san Carlos, Department of Medicine, School of Medicine, Universidad Complutense de Madrid (UCM), IdISSC, Madrid, Spain.

Department of Pathology, Hospital Universitario Clinico San Carlos, Madrid, Spain.

出版信息

World J Surg Oncol. 2021 Nov 20;19(1):331. doi: 10.1186/s12957-021-02438-5.

Abstract

BACKGROUND

Inflammatory myofibroblastic tumour is an infrequent mesenchymal neoplasia of unknown aetiology and variable behaviour, ranging from rather benign lesions to locally aggressive and even metastatic disease. Its presence has been described in almost all organs; however, its location in the female genital tract has rarely been reported.

CASE PRESENTATION

We present the case of a 47-year-old female, who was studied in our institution for a recent medical history of several weeks of dyspareunia and abdominal pain. She underwent pertinent studies including ultrasonography and CT scan. Under suspicion of degenerated leiomyoma, a total hysterectomy was performed. Unexpectedly, the pathological study of the surgical specimen showed very few tumour cells with focal fusiform morphology surrounded by an abundant inflammatory infiltrate; a thorough immunohistochemistry study lead to myofibroblastic tumour of the cervix diagnosis. A PET-CT scan did not show metastatic disease. The patient did not undergo any adjuvant treatment, and she is currently on surveillance with no evidence of disease relapse.

CONCLUSIONS

Inflammatory myofibroblastic tumour remains a rare entity yet to be fully elucidated. The diagnosis is based on pathological study due to the lack of typical clinical manifestations and typical radiological images. Surgical resection is the most frequent treatment, whereas chemotherapy and radiotherapy are restricted to locally advanced or metastatic disease. Tirosine kinase inhibitor crizotinib has shown promising results especially in tumours harbouring ALK mutation.

摘要

背景

炎性肌纤维母细胞瘤是一种罕见的间叶性肿瘤,病因不明,行为多变,从相当良性的病变到局部侵袭性甚至转移性疾病。几乎在所有器官中都有其存在的描述;然而,其在女性生殖道中的位置鲜有报道。

病例介绍

我们报告一例47岁女性病例,该患者因近期数周性交困难和腹痛的病史在我院接受检查。她接受了包括超声检查和CT扫描在内的相关检查。因怀疑为退变的平滑肌瘤,遂行全子宫切除术。出乎意料的是,手术标本的病理研究显示肿瘤细胞极少,呈局灶性梭形形态,周围有大量炎性浸润;全面的免疫组织化学研究确诊为宫颈炎性肌纤维母细胞瘤。PET-CT扫描未显示转移性疾病。该患者未接受任何辅助治疗,目前正在接受监测,无疾病复发迹象。

结论

炎性肌纤维母细胞瘤仍然是一种罕见的疾病,尚未完全阐明。由于缺乏典型的临床表现和典型的影像学图像,诊断基于病理研究。手术切除是最常见的治疗方法,而化疗和放疗仅限于局部晚期或转移性疾病。酪氨酸激酶抑制剂克唑替尼已显示出有前景的结果,尤其是在携带ALK突变的肿瘤中。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8707/8606074/d7cd904568c8/12957_2021_2438_Fig1_HTML.jpg

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