Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, India.
Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, India.
Indian J Pathol Microbiol. 2021 Jan-Mar;64(1):158-160. doi: 10.4103/IJPM.IJPM_948_19.
Malignant peripheral nerve sheath tumor (MPNST) is a type of soft tissue sarcoma arising from peripheral nerves. They can be found in isolation, but about half of the cases are associated with neurofibromatosis type 1 (NF1), an autosomal dominant genetic disorder. They usually arise from the nerve plexus in extremities and trunk. MPNSTs arising from the viscera or internal organs are very rare. We hereby report a rare case of sporadic MPNST arising from pleural surface in a middle-aged male. The diagnosis of such a rare entity involved multimodal investigations with consideration of wide differential diagnosis.
恶性外周神经鞘瘤（MPNST）是一种起源于周围神经的软组织肉瘤。它们可以单独出现，但约一半的病例与神经纤维瘤病 1 型（NF1）有关，这是一种常染色体显性遗传疾病。它们通常起源于四肢和躯干的神经丛。起源于内脏或内部器官的 MPNST 非常罕见。我们在此报告一例罕见的中年男性胸膜表面散发性 MPNST。这种罕见实体的诊断涉及多模态检查，并考虑了广泛的鉴别诊断。
