FAMILIAL GIGANTIFORM CEMENTOMA PDF

Affiliations 1 author 1. Medicine, 01 Mar , 95 9 :e DOI: Undocumented radiographic changes and related bone metabolism disorder are herein hypothesized and discussed. We present an adolescent case with recurrent familial gigantiform cementoma who received surgical intervention in our hospital. Apart from typical multiquadrant and expansile abnormalies involving both jaws, he also suffered from several times of fractures in lower extremity. Furthermore, radiographic examinations of calvaria, pelvis, femoris, tibia, and fibula all revealed radiolucent areas signifying diffuse osteopenic bone losses.

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Affiliations 1 author 1. Medicine, 01 Mar , 95 9 :e DOI: Undocumented radiographic changes and related bone metabolism disorder are herein hypothesized and discussed. We present an adolescent case with recurrent familial gigantiform cementoma who received surgical intervention in our hospital. Apart from typical multiquadrant and expansile abnormalies involving both jaws, he also suffered from several times of fractures in lower extremity. Furthermore, radiographic examinations of calvaria, pelvis, femoris, tibia, and fibula all revealed radiolucent areas signifying diffuse osteopenic bone losses.

Some of his consanguineous relatives bore the same burden of fractures during pubertal period. Considering these polyostotic conditions, a correlation of congenital bone metabolism disorder in cases with familial gigantiform cementoma, named "calcium steal disorder," was thus proposed.

Familial gigantiform cementoma is closely associated with "calcium steal disorder. Free full text Medicine Baltimore. Published online Mar 7. All rights reserved. This is an open access article distributed under the Creative Commons Attribution License 4. Go to: Abstract Familial gigantiform cementoma is an exceedingly rare but distinct subtype of cemento-osseous-fibrous lesion. According to the latest World Health Organization WHO classification of cemento-osseous dysplasias CODs , FGC is generally characterized by rapid osseous expansion involving all 4 jaw quadrants with predilection for young patients.

Besides, it follows an autosomal dominant inheritance pattern with divergent phenotypic expression. As far as our knowledge is concerned, this pathognomonic trait thus necessitates timely and appropriate surgical treatment to prevent these terrible clinical scenarios from spinning out of control.

However, extragnathic presentations of FGC in patients have often been neglected, owing in part to rarity of this disease. Among all the reports available, Rossbach et al 3 was the first to postulate the correlation of a brittle bone disorder with FGC. Nevertheless, in terms of his description, the progression of disease and related underlying causes has not been clarified.

Therefore, we present a case with a large FGC family history so as to sketch a more detailed portrait of such ailment. The ethical approval was granted by the Institutional Clinical Research Supervision Committee of our hospital.

Besides, the informed consent was obtained from these patients. His chief concern was dysmasesis and dysphagia caused by a protuberant mandible since 10 years old. Surgery with recontouring and shaving intent was initially attempted in a local institution about 7 months ago, but in vain.

The growth of the disfigured mandible had not yet been deterred, but instead, accelerated, accompanied by teeth loss and altered dietary patterns. As a direct consequence, physical deterioration was also found by his parents. B Preoperative panorex X-ray showed a characteristic radiographic feature of familial gigantiform cementoma FGC with well-circumscribed radiopaque areas involving all quadrants of the jaw, with mandible being more severely damaged.

C Lateral view of FGC. D Postoperative view of patient after bilateral mandibulectomy. E Postoperative panorex X-ray showed vascularized iliac bone flap for reconstruction. F 3-D computed tomography reconstruction of postoperative view. His previous surgical history was remarkable and extensive. Before referral, he had experienced 4 times of fractures in bilateral lower extremities within 4 years. All these fractures, which occurred in diaphyseal locations, were categorized to a minor-trauma or spontaneous causes owing to poor evidence of outside forces.

The radiographic evaluations of lower limb revealed decreased bone density and thinner- or void-cortex structures around fracture regions. Suspicious of similar osseous problems in other anatomic locations, we then recommended a pelvic computer tomography CT scan to the patient.

Based on findings garnered from CT, it was noteworthy that 2 big circular deficits could be easily detected on both sides of the iliac bones. Besides, his pelvis was also considered to be susceptible to fractures because both cortical and trabecular bones were undergoing a progeric or osteopenic conversion signifying an unexpected calcium loss. Loss of lamina dura, decreased skull bone density, and sporadic bony defects all served as convincing evidence of the serious osseous fragility.

To further identify the specific reasons for multiple fractures alongside these osseous changes previously described, whole-body dual-energy absorptiometry DEA measuring bone mineral density BMD was then offered under permission of the patient and his parents.

E Technetium scintigraphy showed increased tracer uptaker in the chin area. F Two bony defects were identified in reconstructed pelvic CT images. For sake of differential diagnosis with hyperparathyroidism-jaw tumor HPT-JT syndrome, 4 several laboratory analyses, which included serum parathyroid hormone PTH , phosphate levels, calcium levels, and alkaline phosphatase ALP activity, were undertaken accordingly. All these results came out with no marked aberrance.

The feasibility of using fibular flaps was ruled out in view of his unfortunate history of frequent lower-extremity fractures. During month follow-up afterwards, the young patient experienced another physical blow of minor-trauma fracture near the femur neck. Since then, supplementation of calcium and vitamin D had been prescribed as a method to ameliorate the general calcium metabolism disorder.

For the latest visit to our clinic 2 months ago, the outcome in the neomandible region was desirable, and in parallel, the recent DEA test result took a favorable turn as BMD had increased to Misfortunes and troubles never come singly.

Closer examination of his family pedigree verified our concern of a long-standing phenomenon of multiple fractures accompanying FGC in jaws. She was yet no exception given her own narratives of femur fracture during adolescence.

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LUDOVICO QUARONI PDF

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According to Young et al. In the affected family members, all 4 quadrants were involved. The lesions had an early age of onset and developed slowly. Cannon et al.

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