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胃丛状纤维黏液瘤:一种未得到充分认识的病变!

Plexiform fibromyxoma of the stomach: An underrecognized entity!

作者信息

Goyal Surbhi, Beso Patricia, Khullar Rahul, Sakhuja Puja

机构信息

Department of Pathology, Govind Ballabh Pant Institute of Post Graduate Medical Education and Research (GIPMER), New Delhi, India.

Department of Surgery, Govind Ballabh Pant Institute of Post Graduate Medical Education and Research (GIPMER), New Delhi, India.

出版信息

Indian J Pathol Microbiol. 2023 Apr-Jun;66(2):343-346. doi: 10.4103/IJPM.IJPM_480_20.

Abstract

Plexiform fibromyxoma (PF) is a recently described rare type of mesenchymal tumor of the stomach with only 123 cases reported in the literature. It is characterized by a peculiar plexiform growth pattern, myxoid stroma with arborizing microvasculature, and spindle-shaped myofibroblastic cells. We herein report a case of gastric PF in a 15-year-old boy, mimicking a gastrointestinal stromal tumor (GIST) due to overlapping clinicoradiological features. Distinct pathological and immunohistochemical features of PF do aid in distinction from GIST and other mesenchymal entities. Diagnosis is crucial as surgical resection is the mainstay of treatment unlike aggressive management in GIST. It is a benign entity with no local recurrence or distant metastasis reported so far, but confirmation of the same requires longitudinal observational studies with a larger sample size.

摘要

丛状纤维黏液瘤(PF)是一种最近才被描述的罕见的胃间充质肿瘤,文献中仅报道了123例。其特征为独特的丛状生长模式、伴有分支状微血管的黏液样间质以及梭形肌成纤维细胞。我们在此报告一例15岁男孩的胃PF病例,因其临床放射学特征重叠而酷似胃肠道间质瘤(GIST)。PF独特的病理和免疫组化特征确实有助于与GIST及其他间充质实体相鉴别。诊断至关重要,因为与GIST的积极治疗不同,手术切除是PF的主要治疗方法。它是一种良性实体,目前尚无局部复发或远处转移的报道,但要证实这一点需要进行更大样本量的纵向观察研究。

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