Gupta Rakesh Kumar, Saran Ravindra Kumar, Ghuliani Deepak, Garg Lalit, Das Abhijit
Department of Pathology, GB Pant Institute of Postgraduate Medical Education and Research, New Delhi, India.
Department of Surgery, Maulana Azad Medical College, New Delhi, India.
Indian J Med Paediatr Oncol. 2017 Jul-Sep;38(3):387-390. doi: 10.4103/ijmpo.ijmpo_113_17.
Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue tumors commonly arising from nerve roots in the extremities with a predilection in neurofibromatosis (NF) patients. MPNSTs are known to behave aggressively, with worse prognosis in nonresectable cases, and a 5-year survival of about 52% and 15% in resectable and unresectable cases, respectively. Although cytological diagnosis in a known case of NF-1 is possible, however, in the absence of any associated syndrome in a sporadic case, it is very challenging to accurately diagnose this tumor. Till date, to the best of our knowledge, only three cases of epithelioid MPNST correctly diagnosed on cytological examination have been described. We are presenting another case of epithelioid MPNST in a 40-year-old patient with stigmata of NF-1 since childhood, diagnosed on fine-needle aspiration cytology from the left flank mass with subsequent histopathology from left inguinal lymph node metastasis.
恶性外周神经鞘瘤（MPNSTs）是一种罕见的软组织肿瘤，通常起源于四肢神经根，在神经纤维瘤病（NF）患者中更易发生。已知MPNSTs具有侵袭性，不可切除病例的预后较差，可切除和不可切除病例的5年生存率分别约为52%和15%。虽然在已知的NF-1病例中进行细胞学诊断是可能的，然而，在散发性病例中缺乏任何相关综合征的情况下，准确诊断这种肿瘤非常具有挑战性。迄今为止，据我们所知，仅有3例经细胞学检查正确诊断的上皮样MPNST病例被描述。我们在此报告另一例上皮样MPNST病例，患者为一名40岁男性，自幼有NF-1的体征，通过对左侧腰部肿块进行细针穿刺细胞学检查并结合左腹股沟淋巴结转移灶的后续组织病理学检查得以确诊。
