Shanmugasundaram S, Sachdeva Ashwani Kumar, Penumadu Prasanth, Srinivas B
Department of Surgical Oncology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.
Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.
J Cancer Res Ther. 2018 Jul-Sep;14(5):1145-1148. doi: 10.4103/0973-1482.203595.
Malignant peripheral nerve sheath tumor (MPNST) is a rare variety as sporadic tumor but more common when associated with neurofibromatosis, and its usual site of origin lies in the extremities. MPNST of thoracic cavity is a rare site of origin with only anecdotal reports in literature. Progression to a large size without distant metastases is even rarer owing to its high metastatic potential. We present a case of thoracic MPNST highlighting its rarity and the treatment options available to provide a best possible outcome.
恶性外周神经鞘膜瘤（MPNST）作为散发性肿瘤较为罕见，但与神经纤维瘤病相关时则更为常见，其通常起源于四肢。胸腔的MPNST是一种罕见的起源部位，文献中仅有零星报道。由于其高转移潜能，在无远处转移的情况下进展为大尺寸肿瘤的情况更为罕见。我们报告一例胸腔MPNST病例，突出其罕见性以及可供选择的治疗方案，以争取最佳治疗效果。
