McCluggage W G, Bharucha H
Department of Histopathology, Royal Group of Hospitals, Belfast, UK.
Histopathology. 1995 Mar;26(3):247-54. doi: 10.1111/j.1365-2559.1995.tb01438.x.
Primary intrapulmonary tumours of nerve sheath origin are extremely rare. We describe the clinicopathological features of four such peripheral nerve sheath tumours, two benign and two malignant. Of the benign tumours, one was a typical schwannoma and the other an ancient or degenerated schwannoma. The typical schwannoma was endobronchial in origin, diagnosis being established by small bronchoscopic biopsy. The histological diagnosis of malignant peripheral nerve sheath tumour required immunohistochemical and/or ultrastructural evidence of nerve sheath differentiation. One malignant tumour arose in a patient with Von-Recklinhausen's disease. Both malignant tumours behaved aggressively with the development of multiple intrapulmonary metastases, despite the markedly different histological appearance of the two tumours. Flow cytometric analysis of nuclear DNA content was performed on the four tumours, all of which showed a diploid DNA profile.
原发性肺内神经鞘源性肿瘤极为罕见。我们描述了4例此类周围神经鞘肿瘤的临床病理特征，其中2例为良性，2例为恶性。在良性肿瘤中，1例为典型的神经鞘瘤，另1例为陈旧性或退化性神经鞘瘤。典型的神经鞘瘤起源于支气管内，通过小支气管镜活检得以确诊。恶性周围神经鞘瘤的组织学诊断需要免疫组化和/或超微结构证据来证明神经鞘分化。1例恶性肿瘤发生于患有冯·雷克林豪森病的患者。尽管这2例恶性肿瘤的组织学表现明显不同，但二者均表现出侵袭性，出现了多发肺内转移。对这4例肿瘤进行了核DNA含量的流式细胞术分析，所有肿瘤均显示二倍体DNA图谱。
