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多形性肺错构瘤:一种明显独特的肺错构瘤变体。

Pleomorphic pulmonary hamartoma: an apparently unique variant of pulmonary hamartoma.

作者信息

Gisser S D, Young I

出版信息

Hum Pathol. 1979 Jul;10(4):393-403. doi: 10.1016/s0046-8177(79)80045-3.

Abstract

Pulmonary hamartomas of limited variety have been described. Most present as asymptomatic coin lesions in adults and consist of mesenchymal tissue, usually cartilage, in combination with irregular spaces lined by epithelium. Another form is found in the neonate and involves large portions or all of a lung. This is associated with a developmental aberration and is best described as a "congenital adenomatoid malformation". An apparently unique noninvasive tumor mass was resected from the lung of a middle aged man where it was associated with anomalous lung segmentation and the bronchial and blood supply to the lung. The tumor appeared to be undifferentiated by light microscopic criteria. Neurilemoma, leiomyoma, chordoid tumor, mixed tumor, and neurogenic sarcoma all entered the differential diagnosis. Ultrastructural examination demonstrated a highly complex and unique organization. Such a lesion, apparently developmental in this case, should be recognized and carefully distinguished from the malignant mesenchymal, neurogenic, and teratomatous lesions with which it may be confused. Electron microscopy may be helpful in this regard.

摘要

已描述过种类有限的肺错构瘤。大多数表现为成人无症状的钱币状病灶,由间充质组织(通常为软骨)与上皮衬里的不规则间隙组成。另一种形式见于新生儿,累及肺的大部分或全部。这与发育异常有关,最好描述为“先天性腺瘤样畸形”。从一名中年男性的肺中切除了一个明显独特的非侵袭性肿瘤块,该肿瘤与异常的肺段划分以及肺的支气管和血液供应有关。根据光镜标准,该肿瘤似乎未分化。神经鞘瘤、平滑肌瘤、脊索样肿瘤、混合瘤和神经源性肉瘤均列入鉴别诊断范围。超微结构检查显示出高度复杂且独特的结构。这种病变在本例中显然是发育性的,应予以识别,并与可能与之混淆的恶性间充质、神经源性和畸胎瘤性病变仔细区分。在这方面,电子显微镜检查可能会有所帮助。

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