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婴幼儿胸壁间叶性错构瘤:5例患者的临床病理研究

Mesenchymal hamartoma of the chest wall in infants and children: a clinicopathological study of five patients.

作者信息

Ayala A G, Ro J Y, Bolio-Solis A, Hernandez-Batres F, Eftekhari F, Edeiken J

机构信息

Department of Pathology, University of Texas, M.D. Anderson Cancer Center, Houston 77030.

出版信息

Skeletal Radiol. 1993 Nov;22(8):569-76. doi: 10.1007/BF00197137.

Abstract

Mesenchymal hamartoma of chest-wall is a rare benign lesion that has varied histological characteristics and usually occurs during early infancy. We report the histological characteristics of mesenchymal hamartoma found in five patients aged respectively 25 days, 5 months, 8 months, and 4 and 8 years at presentation. Two patients presented with respiratory distress, two with an asymptomatic chest-wall mass, and one with a deformity of the left chest wall. Surgical resection was performed on four patients and a biopsy only in one patient. Overall, the tumors were well delineated, lobulated, tan to reddish in color, and on section showed blood-filled cystic spaces with interspersed small islands of cartilage and fibrous tissue. Histological analyses demonstrated mixtures of bone trabeculae with spindle-cell stroma, chondroblast-like proliferation, mature and immature hyaline cartilage, and aneurysmal bone cyst formation. All patients are alive and well without evidence of disease. Despite the alarming clinical and histologic presentation, mesenchymal hamartoma of the chest wall is a non-neoplastic benign lesion.

摘要

胸壁间叶性错构瘤是一种罕见的良性病变,具有多种组织学特征,通常发生在婴儿早期。我们报告了5例间叶性错构瘤的组织学特征,这5例患者就诊时年龄分别为25天、5个月、8个月以及4岁和8岁。2例患者表现为呼吸窘迫,2例有无症状胸壁肿块,1例有左胸壁畸形。4例患者接受了手术切除,仅1例患者进行了活检。总体而言,肿瘤边界清晰,呈分叶状,颜色为棕褐色至微红,切面可见充满血液的囊腔,其间散布着小的软骨岛和纤维组织。组织学分析显示骨小梁与梭形细胞基质混合存在,有成软骨细胞样增生、成熟和未成熟的透明软骨,以及动脉瘤样骨囊肿形成。所有患者均存活且状况良好,无疾病迹象。尽管胸壁间叶性错构瘤的临床和组织学表现令人担忧,但它是一种非肿瘤性良性病变。

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