El Malih Sara, Akammar Amal, El Mounssefe Ghada, Tahiri Laila, El Bouardi Nizar, Haloua Meriem, Alami Badreeddine, Lamrani Moulay Youssef Alaoui, Maaroufi Mustapha, Boubbou Meryem
Department of radiology Mother and child, University Hospital Hassan 2, Fez, Morocco.
Department of Pathology, University Hospital Hassan 2, Fez, Morocco.
Radiol Case Rep. 2023 Aug 31;18(11):3979-3983. doi: 10.1016/j.radcr.2023.07.074. eCollection 2023 Nov.
Malignant rhabdoid tumor is an uncommon pediatric tumor that often arises from the kidneys but can also develop in other soft tissues including the orbits, where it is known as atypical teratoid rhabdoid tumor. Orbital location is exceptional, with just a few reported cases. Imaging allows for an accurate diagnosis, but histological confirmation is required. The treatment is yet unclear but nearly often surgical, either with or without chemotherapy. In this report, we present a case of a neonate of 1-month-old with an orbital primary malignant rhabdoid tumor with synchronous cerebellar location. The patient was unable to undergo surgery, he had chemotherapy, and unfortunately died 1 week after following palliative treatment. We will illustrate this clinical experience and point out the importance of radiological imaging in the assessment of this uncommon condition.
恶性横纹肌样瘤是一种罕见的儿科肿瘤,通常起源于肾脏,但也可发生于包括眼眶在内的其他软组织,在眼眶处称为非典型畸胎样横纹肌样瘤。眼眶部位的情况较为特殊,仅有少数病例报道。影像学检查有助于准确诊断,但仍需组织学确诊。治疗方法尚不明确,但几乎都采用手术治疗,可联合或不联合化疗。在本报告中,我们介绍了一例1个月大的新生儿,患有眼眶原发性恶性横纹肌样瘤并伴有小脑同步发病。该患者无法接受手术,接受了化疗,不幸的是,在姑息治疗1周后死亡。我们将阐述这一临床经验,并指出放射影像学在评估这种罕见疾病中的重要性。