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揭开胎中胎的神秘面纱:对其临床和病理特征的系统评价

Demystifying Fetus-in-fetu: A Systematic Review of Its Clinical and Pathological Attributes.

作者信息

Palo Seetu, Mangla Mishu, Gabbeta Spandana, Motwani Rohini

机构信息

Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Hyderabad, Telangana, India.

Department of Obstetrics and Gynaecology, All India Institute of Medical Sciences, Hyderabad, Telangana, India.

出版信息

J Indian Assoc Pediatr Surg. 2024 Sep-Oct;29(5):406-416. doi: 10.4103/jiaps.jiaps_67_24. Epub 2024 Sep 9.

Abstract

BACKGROUND

Fetus-in-fetu (FIF) is an exceedingly rare condition, characterized by a fetal-like or fetiform mass with a calcified vertebral axis surrounded by other organs or limbs. This systematic review was conducted to comprehensively analyze the clinicopathological attributes, management strategies, and prognosis of FIF to consolidate existing knowledge on FIF.

METHODOLOGY

Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, a comprehensive search was performed across various electronic databases, using the keywords "fetus-in-fetu," "fetus-ex-fetus," "homunculus," or "fetiform teratoma" to look for published articles until December 2023. Data extraction and analysis were carried out for cases meeting the defined criteria for FIF diagnosis, that is "presence of both/either the vertebral column and/or long bones of extremities" and "absence of immature elements."

RESULTS

A total of 249 case reports/case series comprising 241 single FIF cases and 33 multiple FIF cases were included for analysis. The majority of single FIF cases presented within the 1 year of life, with a slight male predominance. Retroperitoneal location was most common, and imaging modalities played a crucial role in preoperative diagnosis. Complete surgical removal of the mass with detailed histopathological examination is the cornerstone of treatment for FIF, with favorable outcomes in the majority of cases. Both mature and immature teratoma can rarely be found in association with FIF.

CONCLUSION

This comprehensive systematic review enhances understanding of FIF, emphasizing the importance of accurate diagnosis by diligent histopathological examination, appropriate management, and vigilant postoperative monitoring for favorable outcomes.

摘要

背景

胎中胎(FIF)是一种极为罕见的病症,其特征为具有钙化脊柱轴的胎儿样或胎儿状肿块,周围被其他器官或肢体环绕。本系统评价旨在全面分析胎中胎的临床病理特征、管理策略和预后,以巩固关于胎中胎的现有知识。

方法

按照系统评价和Meta分析的首选报告项目指南,在多个电子数据库中进行全面检索,使用关键词“胎中胎”“胎儿外胎儿”“小人儿”或“胎儿状畸胎瘤”查找截至2023年12月发表的文章。对符合胎中胎诊断既定标准的病例进行数据提取和分析,即“存在脊柱和/或四肢长骨中的两者/任一者”以及“不存在未成熟成分”。

结果

共纳入249篇病例报告/病例系列,包括241例单发胎中胎病例和33例多发胎中胎病例进行分析。大多数单发胎中胎病例在1岁内出现,男性略占优势。腹膜后位置最为常见,影像学检查在术前诊断中起关键作用。完整手术切除肿块并进行详细的组织病理学检查是胎中胎治疗的基石,大多数病例预后良好。成熟和未成熟畸胎瘤均很少与胎中胎相关联。

结论

这项全面的系统评价增进了对胎中胎的理解,强调了通过认真的组织病理学检查进行准确诊断、适当管理以及术后密切监测以获得良好预后的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c515/11521215/038c54c500db/JIAPS-29-406-g001.jpg

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