Yousefi Zohreh, Sharifhi Nourieh, Hasanzadeh Malihe, Mottaghi Mansoureh, Bolandy Somayeh
Cancer Research Center, Ghaem Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran;
Department of Pathology, Ghaem Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran;
Iran J Med Sci. 2014 Jan;39(1):71-4.
The primitive neuroectodermal tumor (PNET) belongs to a group of highly malignant tumors and is composed of small round cells of a neuroectodermal origin. Categorized in the same tumor family as Ewing sarcoma, the PNET is most likely to occur in bones and soft tissues. However, a small number of PNET cases arising in the pelvis have been reported as well.
two cases in the ovary and one case in the broad ligament. The PNET often exhibits aggressive clinical behavior with worse outcomes than other small round cell tumors. The significant prognostic factors of the PNET include site of tumor, volume of neoplasm, and presence of metastasis. The treatment protocol is multimodal and includes local surgical treatment followed by chemotherapy. We herein describe three PNET cases as a rare entity in the pelvis. Pelvic PNETs should be included in the differential diagnosis of pelvic masses.
原始神经外胚层肿瘤(PNET)属于一组高度恶性肿瘤,由神经外胚层起源的小圆细胞组成。与尤因肉瘤归为同一肿瘤家族,PNET最常发生于骨骼和软组织。然而,也有少数发生于骨盆的PNET病例报道。
我们呈现三例盆腔PNET:两例发生于卵巢,一例发生于阔韧带。PNET通常表现出侵袭性临床行为,预后比其他小圆细胞肿瘤更差。PNET的重要预后因素包括肿瘤部位、肿瘤体积和转移情况。治疗方案是多模式的,包括局部手术治疗及随后的化疗。我们在此将三例PNET病例描述为骨盆中的罕见实体。盆腔PNET应纳入盆腔肿块的鉴别诊断。