A2M:ALK重排对新生儿胎肺间质瘤的自发部分消退。

IF 1.3 4区 医学 Q3 PATHOLOGY Pediatric and Developmental Pathology Pub Date : 2024-03-01 Epub Date: 2023-10-11 DOI:10.1177/10935266231189929
Alfonso Tan-Garcia, York Tien Lee, Chik Hong Kuick, Shui Yen Soh, Kenneth Tou-En Chang, Khurshid Merchant
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引用次数: 0

摘要

新生儿原发性肺部肿块的鉴别诊断包括发育异常和先天性肺部肿瘤。胎儿肺间质瘤(FLIT)是一种罕见的良性间充质病变,在出生前或出生前3个月内出现 几个月大。FLIT是一种局限性实性囊性肿块,在20-24岁的小管期组织学上与胎儿肺相似 妊娠数周。它由未成熟的间充质细胞和不规则的气隙状结构组成。在所有已发表的病例中,只有1例确定了α2-巨球蛋白(A2M):间变性淋巴瘤激酶(ALK)融合,所有病例都在新生儿或婴儿期接受了手术切除。我们报告了第二例出生后诊断为A2M::ALK融合的FLIT新生儿,该新生儿在39岁时进行临时切除的保守治疗中部分自发消退 月龄,并提供文献综述。
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Spontaneous Partial Regression of Fetal Lung Interstitial Tumor With A2M::ALK Rearrangement in a Neonate.

The differential diagnosis for neonatal primary lung masses includes developmental anomalies and congenital lung tumors. Fetal lung interstitial tumor (FLIT) is a rare benign mesenchymal lesion which presents either antenatally or within the first 3 months of age. FLIT is a circumscribed solid-cystic mass which histologically resembles the fetal lung during the canalicular stage at 20-24 weeks of gestation. It is composed of immature mesenchymal cells expanding the interstitium and irregular airspace-like structures. Of all published cases, only 1 identified an α2-macroglobulin (A2M)::anaplastic lymphoma kinase (ALK) fusion and all cases underwent surgical resection in the neonatal or infancy period. We present the second case of FLIT with an A2M::ALK fusion diagnosed postnatally in a neonate which partially regressed spontaneously during conservative management with interim resection at 39 months of age, and provide a review of the literature.

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来源期刊
CiteScore
3.70
自引率
5.30%
发文量
59
审稿时长
6-12 weeks
期刊介绍: The Journal covers the spectrum of disorders of early development (including embryology, placentology, and teratology), gestational and perinatal diseases, and all diseases of childhood. Studies may be in any field of experimental, anatomic, or clinical pathology, including molecular pathology. Case reports are published only if they provide new insights into disease mechanisms or new information.
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