Turin Sergey Y, Purnell Chad, Gosain Arun K
1 Division of Plastic and Reconstructive Surgery, Lurie Children's Hospital of Chicago, Northwestern Feinberg School of Medicine, Chicago, IL, USA.
Cleft Palate Craniofac J. 2019 Sep;56(8):1083-1088. doi: 10.1177/1055665619833294. Epub 2019 Feb 27.
Juvenile psammomatoid ossifying fibroma (JPOF) is a rare benign osseous tumor, usually presenting in the midface. There are many similarities in location, presentation, and radiographic appearance between fibrous dysplasia (FD) and JPOF. Awareness of this entity is important for craniofacial surgeons, as surgical timing and intraoperative management differ between these tumors. Findings that should raise suspicion of JPOF preoperatively include rapid growth, a shell of cortical bone surrounding the lesion, and clearly demarcated borders of the lesion on imaging, as opposed to a gradual transition between normal and abnormal bone. Definitive excision is the treatment of choice, and earlier surgery may provide better results by addressing the lesion at the smallest size possible. In contrast to FD, JPOF is not known to "burn out," so there is minimal benefit to be gained from delay. We present a summary of the evidence for diagnosis and treatment of JPOF as well our experience with JPOF in an 11-year-old female who was initially diagnosed with FD. We aim to draw attention to the similar presentations of these entities so the reader will be able to more accurately manage these patients.
青少年砂粒样骨化性纤维瘤（JPOF）是一种罕见的良性骨肿瘤，通常发生于面中部。骨纤维异常增殖症（FD）与JPOF在位置、表现及影像学特征上有许多相似之处。认识这一疾病实体对于颅面外科医生很重要，因为这些肿瘤在手术时机和术中处理方面存在差异。术前应怀疑JPOF的表现包括病变快速生长、病变周围有一层皮质骨壳以及影像学上病变边界清晰，这与正常骨和异常骨之间的逐渐移行不同。根治性切除是首选治疗方法，早期手术通过尽可能在病变最小的时候处理病变可能会取得更好的效果。与FD不同，JPOF不会“自行消退”，因此延迟治疗获益极小。我们总结了JPOF诊断和治疗的证据以及我们对一名最初被诊断为FD的11岁女性JPOF患者的治疗经验。我们旨在引起对这些疾病实体相似表现的关注，以便读者能够更准确地处理这些患者。
