A rare malignant tumor of corpus uterine: peripheral nerve sheath tumor (MPNST): Review and our experience

Tsikouras Panagiotis, M. Kouroupi, S. Zervoudis, G. Iatrakis, X. Athoulaki, A. Bothou, A. Chalkidou, S. Michalopoulos, M. Koutsogiannis, Pelagia Chloropoulou, A. Lazarou, Panagiotis Petsidis, Arsou Chalil Bouratzan, Ioannis Tsirkas, F. Gaitatzi, Natalia Sachnova, A. Giatromanolaki, N. Nikolettos
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Abstract

Soft tissue and visceral sarcomas are rare tumors which often develop in muscles, joints, nerves, fat, skin, or vessels. In a significant proportion, they appear in the abdomen and in the female reproductive system. Considering that early-stage sarcomas do not give symptoms, there are often late diagnosed in a quite advanced and/or metastatic stage. Malignant peripheral nerve sheath tumors (MPNSTs) are an uncommon type of malignant soft tissue sarcomas. In this report, we refer to a rare case of histologically confirmed MPNST arisen from corpus uterus in a 74 years old woman. The above-mentioned patient was admitted with abdominal pain and severe vaginal bleeding. Firstly, she underwent emergency endometrial ablation and removal of the tumor that was protruding from the endometrial cavity. Immediately after receiving histological diagnosis, abdominal hysterectomy was performed with bilateral oophorectomy. Considering that the available published literature has only a few reports of MPNST mainly arising from the uterine cervix, with no established treatment, our contribution could help the scientific community in adding data to MPNSTs arising from the uterine body.
一种罕见的子宫体恶性肿瘤:周围神经鞘瘤(MPNST):回顾和我们的经验
软组织和内脏肉瘤是罕见的肿瘤,通常发生在肌肉、关节、神经、脂肪、皮肤或血管中。在很大程度上,它们出现在腹部和女性生殖系统。考虑到早期肉瘤没有症状,通常在相当晚期和/或转移期才被诊断出来。恶性周围神经鞘肿瘤是一种少见的恶性软组织肉瘤。在此报告中,我们报告一例罕见的组织学证实的MPNST,发生于74岁的女性子宫。上述患者因腹痛和严重阴道出血入院。首先,她接受了紧急子宫内膜消融术并切除了从子宫内膜腔中突出的肿瘤。在接受组织学诊断后,立即行腹部子宫切除术和双侧卵巢切除术。考虑到目前已发表的文献中,主要发生于子宫颈的MPNST报道较少,且没有成熟的治疗方法,我们的贡献可以帮助科学界增加发生于子宫体的MPNST的数据。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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