Parviz Sara, Zeinalkhani Fahimeh, Gity Masoumeh, Saligheh Rad Hamidreza, Fathi Kazerooni Anahita, Nili Fatemeh, Kamali Hakim Peyman, Zeinalkhani Hadise
Advanced Diagnostic and Interventional Radiology Research Center (ADIR), Tehran University of Medical Sciences, Tehran, Iran.
Department of Radiology, Medical Imaging Center, Tehran University of Medical Sciences, Tehran, Iran.
Med J Islam Repub Iran. 2024 Jul 16;38:81. doi: 10.47176/mjiri.38.81. eCollection 2024.
Primitive neuroectodermal tumors (PNET) are a family of poorly differentiated malignant neoplasms of neuroectodermal origin. According to the location of origin, PNETs could be further categorized as central or peripheral. Peripheral PNET (pPNET) is an uncommon type that accounts for 1% of all soft tissue sarcomas and occurs outside the central and sympathetic nervous systems. Ovarian PNET is a very rare tumor with a high mortality rate. We report a case of pPNET originating from the pelvic cavity of a young woman. Ultrasound and Magnetic Resonance Imaging (MRI) findings demonstrated the presence of a high-grade malignant ovarian tumor. On microscopic evaluation, the tumor was composed of solid nests and sheets of small rounded cells, and on Immunohistochemical (IHC) evaluation, the tumor cells showed intense cell-membranous immunoactivity for MIC2 protein (CD99). In the differential diagnosis of any invasive pelvic tumor in young women, pPNET should be considered.
原始神经外胚层肿瘤（PNET）是起源于神经外胚层的一类低分化恶性肿瘤。根据起源部位，PNET可进一步分为中枢性或外周性。外周原始神经外胚层肿瘤（pPNET）是一种罕见类型，占所有软组织肉瘤的1%，发生于中枢和交感神经系统之外。卵巢PNET是一种死亡率很高的非常罕见的肿瘤。我们报告一例起源于年轻女性盆腔的pPNET病例。超声和磁共振成像（MRI）检查结果显示存在高级别恶性卵巢肿瘤。显微镜评估显示，肿瘤由实性巢状和片状小圆形细胞组成，免疫组织化学（IHC）评估显示，肿瘤细胞对MIC2蛋白（CD99）表现出强烈的细胞膜免疫活性。在年轻女性任何侵袭性盆腔肿瘤的鉴别诊断中，都应考虑pPNET。
