Yadav Subhash C, Mittal Neha, Bal Munita, Qureshi Sajid
Department of Surgical Pathology, Tata Memorial Hospital, Mumbai, Maharashtra India.
Homi Bhabha National Institute, Mumbai, India.
J Maxillofac Oral Surg. 2022 Dec;21(4):1343-1347. doi: 10.1007/s12663-021-01521-4. Epub 2021 Feb 21.
Cemento-ossifying fibromas are benign fibro-osseous lesions characterized by deposition of cementum and fibrous tissue. Familial gigantiform cementoma (FGC) is an exceeding rare and distinct subtype of cemento-osseous-fibrous lesion. We hereby present a case of FGC in a young boy who was left to die due to the social stigma caused by the massive bony growth in the upper and lower jaw. The patient was somehow rescued by a non-governmental organization and underwent his surgical management at our hospital. On family screening, the mother also had similar smaller asymptomatic lesions in the jaw, but she refused further investigations and treatment. FGC is frequently associated with "calcium-steal phenomenon," which was also seen in our patient. Family screening is therefore necessary to identify asymptomatic patients in the family and follow them up with radiology and whole body dual-energy absorptiometry scans.
牙骨质骨化纤维瘤是一种良性纤维-骨病变,其特征是牙骨质和纤维组织的沉积。家族性巨大牙骨质瘤(FGC)是一种极其罕见且独特的牙骨质-骨-纤维病变亚型。我们在此报告一例FGC病例,该病例为一名年轻男孩,因其上下颌骨大量骨质生长所带来的社会耻辱感而被遗弃等死。该患者经一个非政府组织设法营救,并在我院接受了手术治疗。在家族筛查中,母亲的颌骨也有类似的较小无症状病变,但她拒绝进一步检查和治疗。FGC常与“钙窃取现象”相关,该现象在我们的患者中也有发现。因此,有必要进行家族筛查,以识别家族中的无症状患者,并通过放射学检查和全身双能吸收测定扫描对他们进行随访。