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一名绝经后患者的纯子宫体横纹肌肉瘤的管理:一例罕见病例报告及文献综述

Management of a pure uterine corpus rhabdomyosarcoma in a postmenopausal patient: A rare case report and review of the literature.

作者信息

Lugata John, Smith Caleigh, Mrosso Onesmo, Rwenyagila Doris, Shao Baraka, Mremi Alex

机构信息

Department of Obstetrics and Gynecology Kilimanjaro Christian Medical Centre Moshi Tanzania.

Faculty of Medicine Kilimanjaro Christian Medical University College Moshi Tanzania.

出版信息

Clin Case Rep. 2024 Mar 7;12(3):e8631. doi: 10.1002/ccr3.8631. eCollection 2024 Mar.

Abstract

KEY CLINICAL MESSAGE

Rhabdomyosarcoma of the female genital tract often involves the vagina and cervix. It usually occurs in infants and children. Such tumors are uncommon in the uterus, especially in adults. Treatment options are based on studies of younger individuals.

ABSTRACT

Rhabdomyosarcoma (RMS) is a malignant mesenchymal neoplasm with a tendency to differentiate into skeletal muscle cells. RMS is an aggressive tumor that tends to develop in children and younger patients. A vast majority of genital tract RMSs occur in the vagina and cervix. Such tumors rarely occur in adults. Usually, these tumors either occur as a component of a biphasic uterine tumor (carcinosarcoma or adenosarcoma) or can be a pure heterologous tumor. Pure uterine RMSs are extremely rare in adult patients and difficult to diagnose. Accurate diagnosis of these tumors depends on precise histopathological evaluation. The present report describes a rare case of embryonal RMS of the uterus in a postmenopausal female and explores the most recent literature. The aim is to strengthen the existing literature and aid clinicians in the management of similar cases. A 64-year-old postmenopausal female presented with a history of abdominal pain associated with abdominal distension, per vaginal bleeding, and foul-smelling discharge for 6 months. A transabdominal ultrasound revealed a bulky uterus with a well-circumscribed heterogeneous lesion. Histopathology confirmed the diagnosis of high-grade embryonal RMS within the corpus region of the uterus. A total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed, followed by adjuvant chemotherapy to prevent relapse of the disease. Six months after oncological care has passed, the patient remains symptoms-free without evidence of recurrence or metastasis.

摘要

关键临床信息

女性生殖道横纹肌肉瘤常累及阴道和宫颈。它通常发生于婴幼儿和儿童。此类肿瘤在子宫中罕见,尤其是在成年人中。治疗方案基于对较年轻个体的研究。

摘要

横纹肌肉瘤(RMS)是一种恶性间叶性肿瘤,有分化为骨骼肌细胞的倾向。RMS是一种侵袭性肿瘤,倾向于在儿童和较年轻患者中发生。绝大多数生殖道RMS发生于阴道和宫颈。此类肿瘤在成年人中很少见。通常,这些肿瘤要么作为双相性子宫肿瘤(癌肉瘤或腺肉瘤)的一个组成部分出现,要么可以是纯异源性肿瘤。纯子宫RMS在成年患者中极为罕见且难以诊断。这些肿瘤的准确诊断取决于精确的组织病理学评估。本报告描述了一例绝经后女性子宫胚胎性RMS的罕见病例,并探讨了最新文献。目的是充实现有文献并帮助临床医生处理类似病例。一名64岁绝经后女性,有6个月的腹痛伴腹胀、经阴道出血及恶臭分泌物病史。经腹超声显示子宫增大,有一个边界清楚的不均匀病变。组织病理学证实子宫体部为高级别胚胎性RMS。行全腹子宫切除术加双侧输卵管卵巢切除术,随后进行辅助化疗以预防疾病复发。肿瘤治疗6个月后,患者无症状,无复发或转移迹象。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4cf1/10918699/61db3f6e165d/CCR3-12-e8631-g003.jpg

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