Nirhale Dakshyani S, Parasnis Amit, Bora Charit, Gupta Ruchita, Aulakh Prabhjot
Department of General Surgery, Padmashree Dr. D. Y. Patil Medical Hospital and Research Centre, Sant Tukaram Nagar, Pimpri, Pune-18 India.
Indian J Surg. 2013 Jun;75(Suppl 1):12-4. doi: 10.1007/s12262-011-0300-z. Epub 2011 May 15.
The malignant peripheral nerve sheath tumour is the malignant counterpart to benign soft tissue tumours such as neurofibromas and schwannomas. Malignant Triton Tumour is a histological variant of malignant peripheral nerve sheath tumours with rhabdomyosarcomatous differentiation. They are known to develop in cases of neurofibromatosis type 1 (von Recklinghausen's disease). Malignant peripheral nerve sheath tumor is biologically an aggressive tumour for which the treatment of choice is surgery. Here we report a rare case of malignant peripheral nerve sheath tumour, not associated with neurofibromatosis type 1 and arising in the retroperitoneum.
恶性外周神经鞘瘤是神经纤维瘤和施万细胞瘤等良性软组织肿瘤的恶性对应物。恶性蝾螈瘤是恶性外周神经鞘瘤的一种组织学变体,具有横纹肌肉瘤分化。已知它们在1型神经纤维瘤病(冯·雷克林豪森病)患者中发生。恶性外周神经鞘瘤在生物学上是一种侵袭性肿瘤,其首选治疗方法是手术。在此,我们报告一例罕见的恶性外周神经鞘瘤病例,该病例与1型神经纤维瘤病无关,发生于腹膜后。