Displasia fibrosa del maxilar inferior: dilema sobre su tratamiento, a propósito de un caso clínico
Fibrous dysplasia (FD) is a rare bone disorder (7% of all benign bone tumors) difficult to diagnose, in which normal bone is replaced by fibrous tissue. It is etiologically related to a post-zygotic point mutation of the GNAS gene, for which it is necessary to rule out its association with McCune-Al...
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| Formato: | Artículo revista |
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Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
2022
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| Acceso en línea: | https://revistas.unc.edu.ar/index.php/med/article/view/39003 |
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I10-R327-article-39003 |
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| institution |
Universidad Nacional de Córdoba |
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I-10 |
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R-327 |
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Revista de la Facultad de Ciencias Médicas de Córdoba |
| format |
Artículo revista |
| topic |
fibrous dysplasia ground-glass lower jaw displasia fibrosa vidrio esmerilado maxilar inferior |
| spellingShingle |
fibrous dysplasia ground-glass lower jaw displasia fibrosa vidrio esmerilado maxilar inferior Ana María, Arja Meritano, Mónica Luque, Eugenia Martini, Carolina Ponzio, Marina Displasia fibrosa del maxilar inferior: dilema sobre su tratamiento, a propósito de un caso clínico |
| topic_facet |
fibrous dysplasia ground-glass lower jaw displasia fibrosa vidrio esmerilado maxilar inferior |
| author |
Ana María, Arja Meritano, Mónica Luque, Eugenia Martini, Carolina Ponzio, Marina |
| author_facet |
Ana María, Arja Meritano, Mónica Luque, Eugenia Martini, Carolina Ponzio, Marina |
| author_sort |
Ana María, Arja |
| title |
Displasia fibrosa del maxilar inferior: dilema sobre su tratamiento, a propósito de un caso clínico |
| title_short |
Displasia fibrosa del maxilar inferior: dilema sobre su tratamiento, a propósito de un caso clínico |
| title_full |
Displasia fibrosa del maxilar inferior: dilema sobre su tratamiento, a propósito de un caso clínico |
| title_fullStr |
Displasia fibrosa del maxilar inferior: dilema sobre su tratamiento, a propósito de un caso clínico |
| title_full_unstemmed |
Displasia fibrosa del maxilar inferior: dilema sobre su tratamiento, a propósito de un caso clínico |
| title_sort |
displasia fibrosa del maxilar inferior: dilema sobre su tratamiento, a propósito de un caso clínico |
| description |
Fibrous dysplasia (FD) is a rare bone disorder (7% of all benign bone tumors) difficult to diagnose, in which normal bone is replaced by fibrous tissue. It is etiologically related to a post-zygotic point mutation of the GNAS gene, for which it is necessary to rule out its association with McCune-Albright syndrome and hyperfunctioning endocrinopathies. This pathology can affect any bone, being less frequent a commitment of the lower jaw (LJFD) compared to the upper jaw, and its more commonly presented in children or adolescents. The diagnosis is based on clinical, radiological and histological findings. In addition to surgery, the use of bisphosphonates is a therapeutic option, with osteonecrosis of the jaw being a probable complication with the use of these drugs. Our objective is to describe the diagnosis, clinical evolution, follow-up and therapeutic management for a period of one year in a patient with DFMI, and to reconsider the available therapeutic options in the face of the probable appearance of complications.
This is a 39-year-old woman with an incidentally detected painless left mandibular lesion of 10x8 cm, visualized with tomography after which a biopsy was performed showing fibro-osseous proliferation, trabecular structures with a ground glass appearance, with the Chinese characteristic morphology immersed in a fibrous stroma, confirming bone fibrous dysplasia. Bone biochemical markers of formation and resorption were normal. The bone scintigraphy showed uptake only in the left mandibular region. A pituitary MRI showed a microadenoma interpreted as non-functioning with a normal hormonal profile. A conservative approach was adopted in the management of the patient, due to her stable medical condition and the absence of clinical and imaging changes in the one-year follow-up.
We conclude that the LJFD is a bone pathology of rare presentation and even more so in adulthood. Despite the favorable evolution of this case, we emphasize the need for strict clinical and radiological follow-up to detect possible local complications, malignant transformation or the appearance of new lesions. in these situations, a choice should be made between surgical therapy or antiresorptive pharmacological treatment, without ignoring the paradoxical but infrequent possibility of the appearance of osteonecrosis of the IM described in patients treated with these drugs. |
| publisher |
Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología |
| publishDate |
2022 |
| url |
https://revistas.unc.edu.ar/index.php/med/article/view/39003 |
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I10-R327-article-390032024-04-15T16:14:45Z Displasia fibrosa del maxilar inferior: dilema sobre su tratamiento, a propósito de un caso clínico Ana María, Arja Meritano, Mónica Luque, Eugenia Martini, Carolina Ponzio, Marina fibrous dysplasia ground-glass lower jaw displasia fibrosa vidrio esmerilado maxilar inferior Fibrous dysplasia (FD) is a rare bone disorder (7% of all benign bone tumors) difficult to diagnose, in which normal bone is replaced by fibrous tissue. It is etiologically related to a post-zygotic point mutation of the GNAS gene, for which it is necessary to rule out its association with McCune-Albright syndrome and hyperfunctioning endocrinopathies. This pathology can affect any bone, being less frequent a commitment of the lower jaw (LJFD) compared to the upper jaw, and its more commonly presented in children or adolescents. The diagnosis is based on clinical, radiological and histological findings. In addition to surgery, the use of bisphosphonates is a therapeutic option, with osteonecrosis of the jaw being a probable complication with the use of these drugs. Our objective is to describe the diagnosis, clinical evolution, follow-up and therapeutic management for a period of one year in a patient with DFMI, and to reconsider the available therapeutic options in the face of the probable appearance of complications. This is a 39-year-old woman with an incidentally detected painless left mandibular lesion of 10x8 cm, visualized with tomography after which a biopsy was performed showing fibro-osseous proliferation, trabecular structures with a ground glass appearance, with the Chinese characteristic morphology immersed in a fibrous stroma, confirming bone fibrous dysplasia. Bone biochemical markers of formation and resorption were normal. The bone scintigraphy showed uptake only in the left mandibular region. A pituitary MRI showed a microadenoma interpreted as non-functioning with a normal hormonal profile. A conservative approach was adopted in the management of the patient, due to her stable medical condition and the absence of clinical and imaging changes in the one-year follow-up. We conclude that the LJFD is a bone pathology of rare presentation and even more so in adulthood. Despite the favorable evolution of this case, we emphasize the need for strict clinical and radiological follow-up to detect possible local complications, malignant transformation or the appearance of new lesions. in these situations, a choice should be made between surgical therapy or antiresorptive pharmacological treatment, without ignoring the paradoxical but infrequent possibility of the appearance of osteonecrosis of the IM described in patients treated with these drugs. La displasia fibrosa (DF) es un trastorno óseo poco común (7% de todos los tumores óseos benignos) y de difícil diagnóstico, en el cual el hueso normal es reemplazado por tejido fibroso. Se relaciona etiológicamente con una mutación puntual post-cigótica del gen GNAS por lo cual es necesario descartar su asociación con el sindrome de McCune-Albright y con endocrinopatías hiperfuncionantes. Puede afectar cualquier hueso, siendo menos frecuente el compromiso del maxilar inferior (DFMI) con respecto al superior, presentándose más comunmente en niños o adolescentes. El diagnóstico es clínico, radiológico e histológico. Además de la cirugía, el uso de bifosfonatos es otra opción terapéutica, siendo la osteonecrosis de mandíbula, una probable complicación del uso de estas drogas. Nuestro objetivo es describir diagnóstico, evolución clínica, seguimiento y manejo terapéutico durante un año de una paciente con DFMI, y replantear las opciones terapéuticas disponibles ante la probable aparición de complicaciones. Se trata de una mujer de 39 años con lesión mandibular izquierda de detección incidental, indolora, de 10x8 cm, visualizada con tomografía luego de la cual se efectúa biopsia que evidenció proliferación fibroósea, estructuras trabeculares de apariencia de vidrio esmerilado, con morfología de carácter chino inmersas en un estroma fibroso, confirmando displasia fibrosa ósea. Los marcadores bioquímicos óseos de formación y resorción eran normales. El centellograma corporal mostró captación sólo en la región mandibular izquierda. Una resonancia hipofisaria mostró microadenoma interpretado como no funcionante con perfil hormonal normal. Se adoptó una conducta conservadora en el manejo de la paciente, debido a su condición médica estable y a la ausencia de cambios clínicos e imagenológicos en el seguimiento durante un año. Concluímos que la DFMI es una patología ósea de rara presentación y más aún en edad adulta. A pesar de la favorable evolución de este caso, remarcamos la necesidad del seguimiento clínico y radiológico estrictos para la detección de posibles complicaciones locales, transformación maligna o aparición de nuevas lesiones. Ante tal posibilidad, se deberá optar entre terapia quirúrgica o tratamiento farmacológico antiresortivo, sin desconocer la paradójica aunque poco frecuente posibilidad de aparición de osteonecrosis del MI descripta en pacientes tratados con dichas drogas. Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2022-10-26 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion texto texto texto https://revistas.unc.edu.ar/index.php/med/article/view/39003 Revista de la Facultad de Ciencias Médicas de Córdoba.; Vol. 79 No. Suplemento JIC XXIII (2022): Suplemento JIC XXIII Revista de la Facultad de Ciencias Médicas de Córdoba; Vol. 79 Núm. Suplemento JIC XXIII (2022): Suplemento JIC XXIII Revista da Faculdade de Ciências Médicas de Córdoba; v. 79 n. Suplemento JIC XXIII (2022): Suplemento JIC XXIII 1853-0605 0014-6722 http://creativecommons.org/licenses/by-nc/4.0 |