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静脉内平滑肌瘤病的临床病理特征和临床结局:病例系列研究。

Clinicopathologic features and clinical outcomes of intravenous leiomyomatosis of the uterus: A case series.

机构信息

Department of Obstetrics and Gynecology, West China Second Hospital, Sichuan University.

Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Ministry of Education.

出版信息

Medicine (Baltimore). 2021 Jan 8;100(1):e24228. doi: 10.1097/MD.0000000000024228.

DOI:10.1097/MD.0000000000024228
PMID:33429819
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7793403/
Abstract

RATIONALE

Intravenous leiomyomatosis (IVL) is a rare and special type of smooth muscle tumor originating in the uterus. It is classified as a benign disease according to its histological features but shows the behavioral characteristics of a malignant tumor. It is easily misdiagnosed and recurrent. The purpose of this study was to retrospectively analyze clinicopathological data of 25 cases of IVL in order to enhance clinicians' understanding of this rare disease.

PATIENT CONCERNS

We screened and identified 25 cases of IVL at our hospital from October 2013 to January 2020. Five patients had tumors.

DIAGNOSES

The diagnosis in each case was pathologically confirmed after surgical treatment.

INTERVENTIONS

All patients were managed surgically. Although the surgical procedures were different, the surgical approach was geared towards achieving complete excision. Three patients received hormonal therapy with gonadotropinreleasing hormone agonists after surgery.

OUTCOMES

We retrospectively reviewed all medical records and analyzed the clinicopathologic features and clinical outcomes of this disease as well as the correlations between the clinical features and risk of recurrence. Neither the symptoms nor the preoperative imaging results were suggestive of IVL in any of the cases. Except for two patients who were lost to follow-up, twenty-three patients who were followed up are still alive. Three patients experienced a recurrence.

LESSONS

The clinical manifestations and ultrasound images of IVL in the early stages are not typical; thus, IVL is easily misdiagnosed as uterine leiomyoma. Radiologists, pathologists, and surgeons should have a thorough understanding of IVL and a high index of vigilance for IVL in clinical practice. Surgery should always be aimed at achieving complete tumor excision. Patients with large lesions (≥7 cm) and lesions extending to the broad ligament may have an increased risk of recurrence. Early detection, diagnosis, and treatment are very important; once the diagnosis is confirmed, regular follow-ups are crucial.

摘要

背景

静脉内平滑肌瘤病(IVL)是一种罕见且特殊的子宫平滑肌肿瘤,被归类为良性疾病,但具有恶性肿瘤的行为特征,易误诊且易复发。本研究回顾性分析 25 例 IVL 的临床病理资料,旨在提高临床医生对这种罕见疾病的认识。

患者关注

我们从 2013 年 10 月至 2020 年 1 月在我院筛选并确诊了 25 例 IVL 患者,其中 5 例有肿瘤。

诊断

每例患者均经手术治疗后病理证实。

干预

所有患者均接受手术治疗,尽管手术方式不同,但手术目的均为实现肿瘤完全切除。术后 3 例患者接受促性腺激素释放激素激动剂的激素治疗。

结果

我们回顾性分析了所有病历,分析了该疾病的临床病理特征和临床结局以及临床特征与复发风险的相关性。在任何病例中,症状和术前影像学结果均不提示 IVL。除 2 例失访患者外,23 例随访患者均存活。3 例患者复发。

教训

IVL 早期临床表现和超声图像不典型,易误诊为子宫肌瘤。放射科医生、病理科医生和外科医生应充分了解 IVL,并在临床实践中对 IVL 保持高度警惕。手术应始终以实现肿瘤完全切除为目标。对于病灶较大(≥7cm)和延伸至阔韧带的病灶患者,复发风险可能增加。早期发现、诊断和治疗非常重要;一旦确诊,定期随访至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f881/7793403/953f6e68a650/medi-100-e24228-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f881/7793403/5e7810a84ec0/medi-100-e24228-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f881/7793403/2e24a6c7fa7a/medi-100-e24228-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f881/7793403/953f6e68a650/medi-100-e24228-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f881/7793403/5e7810a84ec0/medi-100-e24228-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f881/7793403/2e24a6c7fa7a/medi-100-e24228-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f881/7793403/953f6e68a650/medi-100-e24228-g003.jpg

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