Familial gigantiform cementoma with brittle bone disease, pathologic fractures, and osteosarcoma: A possible explanation of an ancient mystery

Rossbach, Hans‐Christoph; Letson, Douglas; Lacson, Atilano; Ruas, Ernesto J.; Salazar, Peter

doi:10.1002/pbc.20253

Cited by 40 publications

(40 citation statements)

References 31 publications

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“…1,2 Not all cases are familial and sporadic cases have been reported. 3 FGC has an equal gender distribution and although FGC appears to have a predilection for white people, a few cases have been reported in black people 4,5 and Jamaicans of African descent. 1 The early radiological features of FGC resemble the radiolucent and mixed radiolucent-radio-opaque appearance of the other more common OD types.…”

Section: Discussionmentioning

confidence: 99%

Gigantiform cementoma in a child

Noffke

Ngwenya²,

Nzima³

et al. 2012

Dentomaxillofacial Radiology

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The objective of this report is to present an unusual case of a gigantiform cementoma manifesting with gross expansion of the maxilla in a 6-year-old black female. No history of similar lesions in the family of the patient was obtained. Radiographic examination revealed a well-circumscribed lobular radio-opaque mass surrounded by a radiolucent margin. The macroscopic specimen showed lobular mineralized masses with a 0ginger root0-like appearance. Microscopic examination showed a lobular calcified mass with a peripheral zone of fibropsammomatous tissue corresponding with the radiolucent margin. The differential diagnoses, which include osteoma, ossifying fibroma and cementoblastoma, are discussed.

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Section: Discussionmentioning

confidence: 99%

Gigantiform cementoma in a child

Noffke

Ngwenya²,

Nzima³

et al. 2012

Dentomaxillofacial Radiology

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“…In a few familial cases of FCOD that have been reported in literature, the lesions were seen in younger subjects than non-familial cases, affecting both the genders and with an autosomal dominant inheritance (10,11). Some cases have been associated with bone fractures (10), abnormalities of the long bones and prepubertal pathologic fractures (11). In the case reported here the lesions of FCOD in patient's daughter develo-ped around the age of thirty years.…”

Section: Fcod Was First Described By Melrose Et Al (6) In 1976mentioning

confidence: 71%

“…The case reported here of FCOD in the mother and the daughter therefore makes it a rare presentation of FCOD in this population. In a few familial cases of FCOD that have been reported in literature, the lesions were seen in younger subjects than non-familial cases, affecting both the genders and with an autosomal dominant inheritance (10,11). Some cases have been associated with bone fractures (10), abnormalities of the long bones and prepubertal pathologic fractures (11).…”

Section: Fcod Was First Described By Melrose Et Al (6) In 1976mentioning

confidence: 99%

The use of hyperbaric oxygen therapy in actinomycotic osteomyelitis associated florid cemento osseous dysplasia: report of a familial case.

Mehkri¹,

Nagesh²,

Iyengar³

et al. 2010

J Clin Exp Dent

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Florid Cemento-Osseous Dysplasia (FCOD) is a well recognized fibro-osseous disease of the jaws commonly seen in the middle aged African women, although it may occur in the Caucasians and Asians. There is a reported incidence of less than 2% in the Indian population. The clinical and the radiographic features of FCOD may overlap with features of chronic diffuse scle-rosing osteomyelitis, which makes diagnosis of the disease difficult in symptomatic cases. Moreover the jaws may be susceptible to osteomyelitis, which may mask the underlying FCOD in some cases. The case reported here is that of a 56 year old Indian female patient with long standing FCOD invol-ving the maxilla and the mandible, who was asymptomatic till all her teeth were extracted for fabrica-tion of complete dentures. The patient experienced symptoms of pain, swelling and purulent discharge soon after the use of dentures and was treated by surgical removal of the affected bone. The same symptoms recurred in other areas of the jaw over a period of time and these areas were treated surgica-lly. The patient subsequently presented with pain in the mandibular left posterior region of ten days duration, and was diagnosed as FCOD with superimposed Actinomycotic Osteomyelitis based on his-topathology and Gram's stain, and was treated by surgical removal of the affected bone followed by hyperbaric oxygen therapy (HBO). The radiograph of the surgical site showed good amount of bone regeneration within six months of HBO therapy without recurrence of the FCOD in the same area till date. Key words: Hyperbaric oxygenation, florid cemento osseous dysplasia, osteomyelitis, panoramic radiography.Mehkri S, Nagesh KS, Iyengar AR, Rao SG. The use of hyperbaric oxygen therapy in actinomycotic osteomyelitis associated florid cemento osseous dysplasia: report of a familial case. J Clin Exp Dent. 2010;2 (3):e121-6.

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“…Gigantiform cementoma is an extremely rare form of ossifying fibroma, usually multifocal with tumors that are often massive. Lesions arise during childhood and progressively expand to cause facial deformity during early adult years (Young, Markowitz et al 1989;Rossbach, Letson et al 2005). …”

Section: Clinical and Imagiological Featuresmentioning

confidence: 99%

“…Among the few cases that have been reported, the gene appears to have a high level of penetrance with variable expressivity (Young, Markowitz et al 1989;Finical, Kane et al 1999;Abdelsayed, Eversole et al 2001;Rossbach, Letson et al 2005). …”

Section: Genomic Alterationsmentioning

confidence: 99%