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孕晚期死产与成熟心肌细胞错构瘤(HMCM)相关。

Third Trimester Stillbirth Associated With Hamartoma of Mature Cardiac Myocytes (HMCM).

作者信息

Planas Silvia, Genero Mariona, Illa Miriam

机构信息

Department of Anatomical Pathology, SJD Barcelona Children's Hospital, Barcelona, Spain.

BCNatal | Fetal Medicine Research Center (Hospital Clínic and Hospital Sant Joan de Déu), University of Barcelona, Barcelona, Spain.

出版信息

Pediatr Dev Pathol. 2025 Jan-Feb;28(1):83-86. doi: 10.1177/10935266241286716. Epub 2024 Sep 28.

Abstract

Fetal primary cardiac tumors (FPCTs) are very rare. The majority of them correspond to cardiac rhabdomyomas, followed by other benign neoplasms or hamartomas. We describe the case of a third trimester female stillborn with an incidental autopsy finding of , a rare benign cardiac tumor previously unreported in the fetal or neonatal period. The intrauterine demise occurred at 32 + 6 weeks gestation after an uneventful pregnancy. The fetal autopsy revealed a structurally normal heart with a small subendocardial nodule just below the membranous septum. Microscopically, the nodule was well-demarcated from the surrounding penetrating bundle of the conduction axis and the adjacent left ventricular myocardium and consisted of disorganized mature cardiac myocytes in a haphazard arrangement with patchy mild interstitial fibrosis, consistent with HMCM. Awareness that HMCM can occur in the fetus is important in order to consider it among the differential diagnosis of FPCTs.

摘要

胎儿原发性心脏肿瘤(FPCTs)非常罕见。其中大多数为心脏横纹肌瘤,其次是其他良性肿瘤或错构瘤。我们描述了一例孕晚期女死胎的病例,尸检偶然发现了一种罕见的良性心脏肿瘤,该肿瘤在胎儿期或新生儿期以前未被报道过。此次宫内死亡发生在妊娠32 + 6周,孕期平顺。胎儿尸检显示心脏结构正常,在膜周部下方有一个小的心内膜下结节。显微镜下,该结节与周围传导轴的穿入束及相邻的左心室心肌界限清楚,由排列紊乱的成熟心肌细胞组成,呈杂乱排列,伴有散在的轻度间质纤维化,符合心脏错构瘤。认识到心脏错构瘤可发生于胎儿对于在FPCTs的鉴别诊断中考虑到它很重要。

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