Oral Surg Oral Med Oral Pathol. Dec;68(6) Familial gigantiform cementoma: classification and presentation of a large pedigree. Young SK(1). Gigantiform cementoma is a rare, benign fibro-cemento-osseous disease of the jaws, seen most frequently in young girls. Radiographically, it typically presents. PDF | Familial gigantiform cementoma is an exceedingly rare but distinct subtype of cemento-osseous-fibrous lesion. Undocumented.

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National Center xementoma Biotechnology InformationU. EllisDerek G. Head Neck ; A clinicopathologic study of 44 cases and review of the literature with special emphasis on recurrence. ArmstrongPeter Dirks Journal of neurosurgery. Topics Discussed in This Paper.

Gigantiform cementoma is gigqntiform rare, autosomal dental tumor. Gigantiform cementoma in a child. The radiolucent peripheral margin showed cellular fibrous connective tissue with psammomatoid and trabecular bony deposits. In addition, FGC also carries a striking tendency toward more exuberant growth with cdmentoma jaw involvement. Open in a separate window. Microscopic examination showed a lobular calcified mass with a peripheral zone of fibropsammomatous tissue corresponding with the radiolucent margin.

F Two bony defects were identified in reconstructed pelvic CT images. Fibro-osseous lesions of the face and jaws. S50—S70 [ PubMed ]. E Technetium scintigraphy showed increased tracer uptaker in the chin area. In the family reported by Young et al. Furthermore, radiographic examinations of calvaria, pelvis, femoris, tibia, and fibula all revealed radiolucent areas signifying diffuse osteopenic gigzntiform losses.

Pediatr Blood Cancer ; For sake of differential diagnosis with hyperparathyroidism-jaw tumor HPT-JT syndrome, 4 several laboratory analyses, which included serum parathyroid hormone PTHphosphate levels, calcium levels, and alkaline phosphatase ALP activity, were undertaken accordingly.

Gigantiform cementoma – Wikipedia

Apart from that, it is still debatable about the timing of surgical intervention for FGC patients. Before referral, he had experienced 4 times of fractures in bilateral lower extremities within 4 years. By clicking accept or continuing to use the site, you agree to the terms outlined in our Privacy PolicyGgiantiform of Serviceand Dataset License. Pediatr Blood Cancer ; We might not hastily come to the conclusion that coexistence of FGC and polyostotic pathologic fractures is mere coincidental clinical manifestation.


This page was last edited on 11 Octoberat Since then, supplementation of calcium and vitamin D had been prescribed as a method to ameliorate the general calcium metabolism disorder. Besides, the informed consent was obtained from these patients. Retrieved from ” https: A radiolucent margin is present around most gigantiorm the lesion. Autosomal dominant gigantiform cementoma associated with bone fractures.

Familial gigantiform cementoma is an exceedingly rare but distinct subtype of cemento-osseous-fibrous lesion. Axial CT image showing the radiodense lobular mass surrounded by a radiolucent margin.

Gigantiform cementoma in a child

While the OMIM database is open to the public, users seeking information about a personal medical or genetic condition are urged to consult with a qualified physician for diagnosis and for answers to personal questions. The lesions had an early age of onset and developed slowly. We present an adolescent case with recurrent familial gigantiform cementomma who received surgical intervention in our hospital.

The sharp contrast of CT images between densely bony deposits in FGC lesion and the radiolucent low-density images confirmed our hypothesis that both maxilla and mandible of FGC shared unevenly and favorable distribution of calcium deposits in the general calcium metabolism of whole body.

RaubenheimerDavid A.

The skin over the lesion was freely movable and no nerve fall-out was noted. Misfortunes and troubles never come singly. Therefore, we agree with Noffke et al 14 and Finical 15 in resorting to a complete resection of FGC with curative purposes whenever feasible.


He surmised that this cementomw was merely because of genetic heterogeneity and not every case would develop such obvious concurrence of FGC and fractures. Histologically, the lesions were composed of benign, lobulated, calcified masses resembling cementum.

However, extragnathic presentations of FGC in patients have often been neglected, owing in part to rarity of this disease. Unfortunately, it is not free to produce.

Abstract The objective of this report is to present an ggigantiform case of a gigantiform cementoma manifesting with gross expansion of the maxilla in a 6-year-old black female.

Gabali Journal of oral and maxillofacial surgery….

Gigantiform cementoma

Undocumented radiographic changes and related bone metabolism disorder are herein hypothesized and discussed. His chief concern was dysmasesis and dysphagia caused by a protuberant mandible since 10 years old. A panoramic radiograph showed a well-defined round lobular radio-opacity in the left maxilla extending from the maxillary alveolus to the inferior orbital border and left maxillary tuberosity Figure 2.

Admittedly, not every FGC case will present such evident array of disorganized conditions. We need long-term secure funding to provide you the information that you need at your fingertips. To keep both practitioners and patients informed of the overall bony changes and corresponding risks of fractures, we contended that DEA should be routinely tested since initial clinic visit.