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1.
Acta Ortop Mex ; 35(2): 221-225, 2021.
Artigo em Espanhol | MEDLINE | ID: mdl-34731928

RESUMO

Mazabraud syndrome was first described in 1926 by Henschen, consisting of the association between bone fibrodysplasia and one or more intramuscular myxomas. The study paper conducted by Mazabraud et al. in 1967, described an association between the two pathologies. Later literature referred to this relationship with the eponym described above. In this report, we present the case of a 43-year-old female patient with a known diagnosis of bone fibrodysplasia and subsequent development of a right antecubital fossa mass, which was histologically confirmed as intramuscular myxoma. After the removal of the tumor, the literature was reviewed to find a possible relationship between myxomas and fibrous bone dysplasia, finding positive the association, referred to in the documents reviewed as Mazabraud syndrome.


Assuntos
Displasia Fibrosa Óssea , Displasia Fibrosa Poliostótica , Neoplasias Musculares , Mixoma , Adulto , Feminino , Humanos , Imageamento por Ressonância Magnética , Neoplasias Musculares/diagnóstico por imagem , Mixoma/diagnóstico , Mixoma/cirurgia , Síndrome
2.
Surg Pathol Clin ; 14(4): 707-721, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34742489

RESUMO

Fibrous and fibro-osseous tumors are some of the most common benign lesions involving bones. Although many of the histomorphologic features of these tumors overlap significantly, an interdisciplinary approach helps to consolidate the classification of these tumors. Herein, the clinical, radiologic, and pathologic features of lesions within these categories are described.


Assuntos
Neoplasias Ósseas , Fibroma Ossificante , Displasia Fibrosa Óssea , Neoplasias Ósseas/diagnóstico , Fibroma Ossificante/diagnóstico , Displasia Fibrosa Óssea/diagnóstico por imagem , Humanos
3.
Georgian Med News ; (316-317): 46-52, 2021.
Artigo em Russo | MEDLINE | ID: mdl-34511443

RESUMO

In a significant number of patients with fibrous dysplasia, among various orthopedic pathologies, bone pain, its deformation and pathological fractures, disorders of bone tissue and its metabolism are leading. Issues of correction of clinical and orthopedic manifestations of the disease and changes in the structural and functional state of bone tissue are insufficiently studied and need improvement The purpose of the study is to improve drug antiosteoporotic therapy for patients with fibrous dysplasia in order to reduce their pain, improve the condition of bone tissue and its metabolism. In the department of pediatric traumatology and orthopedics of the SI "ITO NAMSU" in the period from 2015 to 2020, 16 patients with FD were treated (with polyosal form - 6 patients, with Albright syndrome - 5, with Campanacci syndrome - 5), who have been used drug antiosteoporotic therapy, in particular with the use of antiresorbents (pamidronic acid preparations). The age of patients ranged from 6 to 28 years, 13 patients were children. Qualitative and quantitative assessment of the therapy results and reduction of pain was performed by applying the "VAS" scale, improving the condition of bone tissue and its metabolism by studying the mineral density of bone tissue and markers of bone metabolism. The article presents the rationale, general principles and features of antiosteoporotic therapy; indications and contraindications to it; calculations of doses and schemes of application of these or those drugs, including from group of bisphosphonates at a fibrous dysplasia. The presented drug therapy has been successfully tested in 16 patients with fibrous dysplasia. The effectiveness of the treatment is confirmed by the reduction or elimination of pain, stopping the progression of pathological bone disease of the lower limbs, improving structural condition of bone tissue and its metabolism. The results of drug therapy in patients with fibrous dysplasia indicates its effectiveness and appropriateness of use, which is shown by the relief of pain, improving the structural condition of bone tissue and its metabolism.


Assuntos
Displasia Fibrosa Óssea , Preparações Farmacêuticas , Adolescente , Adulto , Osso e Ossos/diagnóstico por imagem , Criança , Difosfonatos/uso terapêutico , Displasia Fibrosa Óssea/tratamento farmacológico , Estado Funcional , Humanos , Adulto Jovem
4.
Pan Afr Med J ; 38: 385, 2021.
Artigo em Francês | MEDLINE | ID: mdl-34381529

RESUMO

Fibrous dysplasia is a genetic and rare bone disorder affecting the young subject. Its pathophysiology involves genetic mutations leading to defective skeletal development with fibrous and medullary proliferations. Cephalic extremity is involved in one third of cases. We here report a case of surgically treated fibrous dysplasia of the frontal and ethmoid sinuses. The study involved a 31-year-old man presenting with chronic holocranial headaches. Computed tomography (CT) scan showed voluminous bilateral hyperdense expansive process in the frontal and ethmoid sinuses. Surgery was performed on the basis of functional signs and imaging data. Anatomopathological examination of bone fragments confirmed the diagnosis of fibrous dysplasia. Fibrous dysplasia is rare and characterized by slow disease progression. In the absence of a consensus on therapy, surgery remains the treatment of choice for unifocal forms.


Assuntos
Seio Etmoidal/diagnóstico por imagem , Displasia Fibrosa Óssea/diagnóstico por imagem , Seio Frontal/diagnóstico por imagem , Adulto , Seio Etmoidal/patologia , Seio Etmoidal/cirurgia , Displasia Fibrosa Óssea/patologia , Displasia Fibrosa Óssea/cirurgia , Seio Frontal/patologia , Seio Frontal/cirurgia , Cefaleia/etiologia , Humanos , Masculino , Tomografia Computadorizada por Raios X
5.
Bone ; 153: 116157, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34425287

RESUMO

Fibrous dysplasia (FD) is a mosaic skeletal disorder in which the craniofacial bones are commonly affected. Normal structures are replaced by expansile, highly vascular, fibro-osseous tissue. The typical clinical course is a gradual, asymptomatic expansion of the osseous structures. However, in the periorbital region, even minor structural changes may cause functional impairment, such as diplopia and hyposmia. Furthermore, rapidly evolving secondary lesions, such as fluid-filled cysts, can sometimes develop. In the midface and periorbital regions, such acute change may be associated with severe pain, vision loss, and, signs of inflammation. Here we describe three patients with craniofacial FD who presented with recurrent episodes of periorbital inflammation mimicking orbital cellulitis. All presented with pain, edema, erythema, and warmth, with varying degrees of functional impairment. On imaging, all had cystic changes in the FD lesion, including two with aneurysmal bone cysts (ABCs). Two were initially diagnosed with periorbital cellulitis and treated with antibiotics; in two, the radiographic findings were misdiagnosed as osteomyelitis. Recurrent episodes were recognized as not infectious and effectively managed with corticosteroids. Given the vascular nature of FD and the association of ABCs, it is likely the findings in these patients represent inflammation associated with vascular leak in the relatively confined space of the tissues overlying the periorbital bones. Recognition of this entity can lead to more rapid and appropriate treatment.


Assuntos
Displasia Fibrosa Craniofacial , Displasia Fibrosa Óssea , Osteomielite , Osso e Ossos , Humanos , Inflamação
6.
J Contemp Dent Pract ; 22(3): 304-309, 2021 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-34210933

RESUMO

AIM: This report aims to provide the clinical and radiographic features of two symptomatic Indian patients with florid cemento-osseous dysplasia (FCOD), along with a discussion of the differential diagnosis, potential challenges, and therapeutic implications. BACKGROUND: FCOD is a rare, multifocal, periapical, and bilateral condition involving the premolar and molar region of the posterior mandible and sometimes the maxilla. CASE DESCRIPTION: The first patient is a 30-year-old female with a recent history of dental pain. The patient was otherwise healthy and the medical history was unremarkable. The second patient is a 50-year-old female with a history of orthodontic therapy. Radiographic evaluation using cone-beam computed tomography (CBCT) revealed bilateral involvement of the posterior mandible, sparing the entire maxilla in both patients Clinical significance: For a pathognomonic condition like FCOD, a radiology survey alone is often sufficient to arrive at the final diagnosis, and therefore surgical interventions should ideally be avoided.


Assuntos
Displasia Fibrosa Óssea , Osteomielite , Adulto , Diagnóstico Diferencial , Feminino , Displasia Fibrosa Óssea/diagnóstico , Humanos , Mandíbula/diagnóstico por imagem , Pessoa de Meia-Idade , Osteomielite/diagnóstico
7.
Indian J Dent Res ; 32(1): 134-136, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34269253

RESUMO

Cemento-osseous dysplasia is non-neoplastic, reactive fibro-osseous lesions that affect the tooth-bearing areas of the jaws. Osseous dysplasia is further divided into three subtypes: Periapical osseous dysplasia, focal osseous dysplasia, and florid osseous dysplasia. We hereby, present a case of florid cemento-osseous dysplasia occurring in a 40-year old dentulous Indian woman. The patient presented with lesions involving the mandibular right and left quadrant.


Assuntos
Cementoma , Displasia Fibrosa Óssea , Osteomielite , Adulto , Cementoma/diagnóstico por imagem , Feminino , Displasia Fibrosa Óssea/diagnóstico por imagem , Humanos , Mandíbula
8.
Int J Oral Sci ; 13(1): 21, 2021 06 30.
Artigo em Inglês | MEDLINE | ID: mdl-34188021

RESUMO

Ossifying fibroma (OF) and fibrous dysplasia (FD) are two fibro-osseous lesions with overlapping clinicopathological features, making diagnosis challenging. In this study, we applied a whole-genome shallow sequencing approach to facilitate differential diagnosis via precise profiling of copy number alterations (CNAs) using minute amounts of DNA extracted from morphologically correlated microdissected tissue samples. Freshly frozen tissue specimens from OF (n = 29) and FD (n = 28) patients were obtained for analysis. Lesion fibrous tissues and surrounding normal tissues were obtained by laser capture microdissection (LCM), with ~30-50 cells (5 000-10 000 µm2) per sample. We found that the rate of recurrent CNAs in OF cases was much higher (44.8%, 13 of 29) than that in FD cases (3.6%, 1 of 28). Sixty-nine percent (9 of 13) of the CNA-containing OF cases involved segmental amplifications and deletions on Chrs 7 and 12. We also identified eight CNA-associated genes (HILPDA, CALD1, C1GALT1, MICALL2, PHF14, AIMP2, MDM2, and CDK4) with amplified expression, which was consistent with the copy number changes. We further confirmed a jaw lesion with a previous uncertain diagnosis due to its ambiguous morphological features and the absence of GNAS mutation as OF based on the typical Chr 12 amplification pattern in its CNA profile. Moreover, analysis of a set of longitudinal samples collected from an individual with a cellular lesion in suspicion of OF at the first surgery, recurrence and the latest malignant transformation revealed identical CNA patterns at the three time points, suggesting that copy number profiling can be used as an important tool to identify borderline lesions or lesions with malignant potential. Overall, CNA profiling of fibro-osseous lesions can greatly improve differential diagnosis between OF and FD and help predict disease progression.


Assuntos
Fibroma Ossificante , Displasia Fibrosa Óssea , Variações do Número de Cópias de DNA , Diagnóstico Diferencial , Fibroma Ossificante/diagnóstico , Fibroma Ossificante/genética , Displasia Fibrosa Óssea/diagnóstico , Displasia Fibrosa Óssea/genética , Galactosiltransferases , Humanos , Arcada Osseodentária , Recidiva Local de Neoplasia , Proteínas Nucleares
9.
J Bone Miner Res ; 36(9): 1729-1738, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-34076303

RESUMO

Denosumab (Dmab) treatment can benefit patients with fibrous dysplasia/McCune-Albright syndrome (FD/MAS) by suppressing the receptor activator of nuclear factor κB ligand (RANKL)-mediated increased bone resorption. However, limited data of two pediatric cases indicate that a rebound phenomenon may occur after withdrawal. Therefore we studied the safety of Dmab discontinuation in FD/MAS. Thirty-seven patients using Dmab, mostly after unsuccessful bisphosphonate (BP) treatment, were included. Health records were screened for pain scores, side effects, and bone turnover markers (BTMs) (calcium, alkaline phosphatase [ALP], procollagen 1 N-terminal propeptide [P1NP], and ß-crosslaps [B-CTX, also termed ß-C-terminal telopeptide]) during treatment, and for BTMs and clinical rebound effects after withdrawal. BTM levels after withdrawal were compared to pretreatment values. Data were calculated as median (interquartile range [IQR]). BTMs normalized in two-thirds of patients and pain scores decreased significantly during treatment (p = 0.002). One patient (2.7%) developed osteonecrosis of the jaw. Sixteen patients discontinued Dmab treatment after a median of 1.6 years (IQR 1.0 years) because of insufficient effect on pain (n = 10, 63%), side effects (n = 4, 25%), or other reasons (n = 4, 25%). Follow-up posttreatment was 3.2 (2.8) years, wherein no fractures, pain flares, or lesion progression occurred. Calcium remained normal in all but one patient, who had a mild asymptomatic hypercalcemia (2.73 mmol/L) 5 months after discontinuation. ALP passed pretreatment levels in five of 11 patients (46%), increased most after 6 months by 18 (43) U/L, and returned to baseline levels thereafter. P1NP exceeded pretreatment levels in four of nine patients (44%), CTX in eight of nine patients (89%). P1NP rose most after 3 months and stabilized thereafter. CTX showed the highest relative elevation. Patients with high pretreatment levels responding well to Dmab seemed to have the highest rebound. These results suggest beneficial effects of Dmab on pain and BTMs, and show a biochemical but asymptomatic rebound phenomenon after withdrawal in adults with FD/MAS, mainly in case of high pretreatment levels, good response, and multiple injections. Further studies on the safety of Dmab and withdrawal are needed and ongoing. © 2021 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR).


Assuntos
Displasia Fibrosa Óssea , Displasia Fibrosa Poliostótica , Adulto , Osso e Ossos , Criança , Denosumab/efeitos adversos , Difosfonatos , Displasia Fibrosa Óssea/tratamento farmacológico , Displasia Fibrosa Poliostótica/tratamento farmacológico , Humanos
10.
J Craniofac Surg ; 32(8): e705-e708, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33935141

RESUMO

ABSTRACT: Fibrous dysplasia (FD), a benign fibro-osseous bone tumor commonly involving the craniofacial region, presents a wide spectrum of radiographic patterns. When FD lesions are suspected, various imaging modalities and histopathologic analyses may be required to reach a firm diagnosis. Here, the authors report the case of a 10-year-old male patient presenting with polyostotic type of FD with follow-up data for the next 9 years. The patient showed involvement of cranial bones and bilateral femurs, with skin pigmentation and no endocrine disease, consistent with the diagnosis of Jaffe Lichtenstein disease. The craniofacial radiographic features obtained with computed tomography and cone-beam computerized tomography are documented in this article.


Assuntos
Displasia Fibrosa Óssea , Displasia Fibrosa Poliostótica , Criança , Tomografia Computadorizada de Feixe Cônico , Displasia Fibrosa Óssea/diagnóstico por imagem , Displasia Fibrosa Poliostótica/diagnóstico por imagem , Humanos , Masculino , Crânio/diagnóstico por imagem , Tomografia Computadorizada por Raios X
11.
Pan Afr Med J ; 38: 106, 2021.
Artigo em Francês | MEDLINE | ID: mdl-33912276

RESUMO

Cemento-osseous dysplasia is a benign fibro-osseous lesion affecting the alveolar bone. It is classified into three forms: periapical, focal or florid dysplasia. It is often asymptomatic and fortuitously discovered during a routine radiological examination. However, it may become symptomatic after superinfection, after patient's exposure to oral bacterial flora. We here report a case of florid cemento-osseous dysplasia associated with actinomycosis of bone in a 53-year-old Tunisian woman. This superinfection has been rarely reported in the literature; Boolean Searching on PubMed for the keywords "cemento-osseous dysplasia AND actinomyces" displays a single article (Smith et al. 2011). The treatment of actinomycosis infection often requires long-term antibiotic therapy, sometimes associated with surgical debridement, as in the case of this patient who underwent piezosurgery to treat dysplasia and necrotic bone.


Assuntos
Actinomicose/diagnóstico , Displasia Fibrosa Óssea/diagnóstico , Osteomielite/diagnóstico , Piezocirurgia/métodos , Actinomyces/isolamento & purificação , Actinomicose/microbiologia , Actinomicose/cirurgia , Feminino , Displasia Fibrosa Óssea/microbiologia , Displasia Fibrosa Óssea/cirurgia , Humanos , Pessoa de Meia-Idade , Osteomielite/microbiologia , Osteomielite/cirurgia , Radiografia , Superinfecção/diagnóstico , Superinfecção/microbiologia
12.
Acta Orthop Traumatol Turc ; 55(2): 184-188, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33847584

RESUMO

Focal fibrocartilaginous dysplasia (FFCD) is a rare disease that can cause angular deformities of long bones. The common pathologic finding is a thick fibrotic band extending from epiphysis to metaphysis on one side of the bone. The tethering effect of the fibrotic band around the growth plate is thought to be the main etiology for the development and progression of the deformity. FFCD mostly affects the proximal tibia and the distal femur. The literature contains different treatment options. Here, we present the case of a 20-month-old girl with FFCD on the medial side of the distal femur causing varus deformity. Our treatment protocol included excision of the fibrotic band from the medial side and application of a two-hole plate for guided growth on the lateral side of the distal femur. Deformity correction was achieved rapidly with no complications. A literature review is also presented along with pathologic and magnetic resonance imaging findings.


Assuntos
Coxa Vara , Fêmur , Displasia Fibrosa Óssea , Coxa Vara/diagnóstico , Coxa Vara/etiologia , Coxa Vara/prevenção & controle , Feminino , Fêmur/diagnóstico por imagem , Fêmur/patologia , Fêmur/cirurgia , Displasia Fibrosa Óssea/complicações , Displasia Fibrosa Óssea/diagnóstico , Displasia Fibrosa Óssea/cirurgia , Fixação Interna de Fraturas/métodos , Humanos , Lactente , Imageamento por Ressonância Magnética/métodos , Radiografia/métodos , Resultado do Tratamento
14.
Clin Nucl Med ; 46(9): 774-775, 2021 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-33883497

RESUMO

ABSTRACT: 68Ga-labeled fibroblast activation protein inhibitor (68Ga-FAPI) PET imaging has been introduced for detecting many primary and metastatic tumors. However, false-positive uptakes have been reported in some benign lesions. Here, we presented a 68Ga-FAPI-avid lesion in the left temporal bone in a 41-year-old man with a history of signet ring cell gastric adenocarcinoma. The osseous lesion was finally distinguished as fibrous dysplasia according to the clinical and imaging findings. This case suggests that 68Ga-FAPI may have false-positive uptakes in bone benign lesions, which should be paid attention to in the diagnosis of bone lesions in patients with malignant tumors.


Assuntos
Carcinoma de Células em Anel de Sinete , Displasia Fibrosa Óssea , Quinolinas , Adulto , Displasia Fibrosa Óssea/diagnóstico por imagem , Humanos , Masculino , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Tomografia por Emissão de Pósitrons
15.
Semin Ultrasound CT MR ; 42(2): 150-163, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33814102

RESUMO

We will provide an imaging-focused discussion of 3 benign bone tumors that do not fit in the categories of cartilaginous tumors or osteoid tumors. We have chosen giant cell tumor of bone, unicameral bone cyst, and fibrous dysplasia. All 3 of these entities are common enough that one does not have to be a musculoskeletal radiologist in a cancer hospital to encounter them occasionally, but none of them should be seen frequently.


Assuntos
Neoplasias Ósseas , Displasia Fibrosa Óssea , Neoplasias de Tecidos Moles , Neoplasias Ósseas/diagnóstico por imagem , Diagnóstico Diferencial , Diagnóstico por Imagem , Displasia Fibrosa Óssea/diagnóstico por imagem , Humanos
16.
Neurosurg Rev ; 44(6): 3459-3469, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33751282

RESUMO

Calvarial lesions are rare and can present as a variety of different diseases. The lesions can be palpable on the skin and cause local pain and paraesthesia and, depending on the location, neurological deficits can also occur. This research aims to present an overview of typical imaging features as well as neurosurgical management. We examined the charts of patients who underwent surgery on a calvarial lesion in our department between 2004 and 2017 (n=133). Retrospectively, the pre-, intra-, and postoperative data were analyzed with morphological and histological findings and compared with each other. Pain, swelling, cosmetically disturbing, and neurological deficits were the main complaints. Seventy-seven lesions were limited to the bone, while another 56 lesions showed an infiltrating growth in the adjacent tissue. Depending on the clinical signs and suspected diagnosis, a biopsy, a partial removal, or a complete resection was performed. Histiocytosis (n=20), meningiomas (n=20), metastases (n=19), and osteomas (n=16) were the most common lesions. Fibrous dysplasia (n=6) and intraosseous hemangioma (n=9) were less common; other lesions were present only in isolated cases. Imaging features may suggest the lesion to be benign or malignant, but the diagnosis can be only confirmed by histological examination. The surgical strategy depends on the complaints, location of the lesion, and suspected diagnosis. Adjuvant treatment should be initiated according to the histological findings.


Assuntos
Displasia Fibrosa Óssea , Neoplasias Meníngeas , Meningioma , Humanos , Estudos Retrospectivos , Crânio/diagnóstico por imagem , Crânio/cirurgia
17.
J Pediatr Orthop ; 41(4): 227-235, 2021 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-33655901

RESUMO

BACKGROUND: Primary benign osseous tumors and tumor-like lesions at the sacrum are rare in the pediatric population and exact surgical strategy is still unclear. In this study, we evaluate the outcome for pediatric patients with benign tumors and tumor-like lesions at the sacrum who were receiving surgical treatment according to our proposed surgical strategy and classification. METHODS: We analyzed 49 pediatric patients with sacral benign tumors or tumor-like lesions aged 18 years and below from 2005 to 2018. There were 23 men and 26 women with a mean age of 14.0±3.8 years. Nineteen patients had giant cell tumors (GCTs), 9 aneurysmal bone cysts, 5 osteoblastomas, 5 neurogenic tumors, 3 hemangiomas, 3 teratomas, 2 Langerhans cell histiocytosis, 1 chondroblastoma, 1 fibrous dysplasia, and 1 GCT of tendon sheath. We proposed our surgical plan and surgical classification for pediatric patients with sacral benign tumors or tumor-like lesions. RESULTS: The mean follow-up duration was 6.2 years (range, 1.0 to 18.9 y). GCTs (39%, 19/49) and primary aneurysmal bone cysts (18%, 9/49) are the top 2 common histologic types. Preoperative selective arterial embolization (SAE) was performed in 12 cases and 24 patients received intraoperative aortic balloon occlusion (ABO) as the preoperative surgical plan. Furthermore, according to tumor location at the sacrum, we classified surgical excision of sacral benign tumors and tumor-like lesions into 3 types. Fourteen cases were classified as type I, 27 as type II, 3 as type III, and 5 patients with neurogenic tumors cannot be classified into this surgical classification. Ten patients had wound complications. Two had femoral artery thrombosis because of ABO application. One had mechanical failure. Rate of local recurrence was 16%. Seven patients with GCTs and 1 with neurogenic tumor had local recurrence. No patient died of disease at the last follow-up. For the assessment of neurological function, the rate of neurological dysfunction was 12% (6/49). Four cases had urinary incontinence, 3 fecal incontinence, and 3 had bowel obstruction. Next, univariate analysis for influence of preoperative SAE and intraoperative ABO on complications demonstrated that both of them exerted no significant influence on the occurrence of oncological and nononcological complications. CONCLUSIONS: The proposed surgical strategy can provide an excellent therapeutic effect for pediatric benign tumors and tumor-like lesions at the sacrum. Preoperative SAE and intraoperative ABO can safeguard pediatric patients with high vascularity of benign tumor at the sacrum during the operation. LEVEL OF EVIDENCE: Level IV.


Assuntos
Oclusão com Balão , Cistos Ósseos Aneurismáticos/cirurgia , Tumor de Células Gigantes do Osso/cirurgia , Recidiva Local de Neoplasia , Osteoblastoma/cirurgia , Neoplasias da Coluna Vertebral/cirurgia , Adolescente , Aorta , Cistos Ósseos Aneurismáticos/patologia , Criança , Pré-Escolar , Condroblastoma/patologia , Condroblastoma/cirurgia , Incontinência Fecal/etiologia , Feminino , Displasia Fibrosa Óssea/cirurgia , Seguimentos , Tumor de Células Gigantes do Osso/patologia , Hemangioma/cirurgia , Histiocitose de Células de Langerhans/cirurgia , Humanos , Obstrução Intestinal/etiologia , Masculino , Recidiva Local de Neoplasia/patologia , Doenças do Sistema Nervoso/etiologia , Complicações Pós-Operatórias/etiologia , Sacro , Neoplasias da Coluna Vertebral/complicações , Neoplasias da Coluna Vertebral/patologia , Neoplasias da Coluna Vertebral/terapia , Teratoma/cirurgia , Resultado do Tratamento , Incontinência Urinária/etiologia
18.
Osteoporos Int ; 32(9): 1889-1893, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-33772327

RESUMO

Denosumab has been advocated as a potential treatment for the rare skeletal disorder fibrous dysplasia (FD); however, there is limited data to support safety and efficacy, particularly after drug discontinuation. We report a case of successful treatment of aggressive craniofacial FD with denosumab, highlighting novel insights into the duration of efficacy, surrogate treatment markers, and discontinuation effects. A 13-year-old girl presented with persistent pain and expansion of a maxillary FD lesion, which was not responsive to repeated surgical procedures or bisphosphonates. Pre-treatment biopsy showed high RANKL expression and localization with proliferation markers. Denosumab therapy was associated with improved pain, decreased bone turnover markers, and increased lesion density on computed tomography scan. During 3.5 years of treatment, the patient developed increased non-lesional bone density, and after denosumab discontinuation, she developed hypercalcemia managed with bisphosphonates. Pain relief and lesion stability continued for 2 years following treatment, and symptom recurrence coincided with increased bone turnover markers and decreased lesion density back to pre-treatment levels. This case highlights the importance of considering the duration of efficacy when treating patients with FD and other nonresectable skeletal neoplasms that require long-term management.


Assuntos
Displasia Fibrosa Craniofacial , Displasia Fibrosa Óssea , Hipercalcemia , Adolescente , Denosumab/uso terapêutico , Difosfonatos , Feminino , Displasia Fibrosa Óssea/diagnóstico por imagem , Displasia Fibrosa Óssea/tratamento farmacológico , Humanos
19.
Ned Tijdschr Geneeskd ; 1652021 02 25.
Artigo em Holandês | MEDLINE | ID: mdl-33651517

RESUMO

A 78-year-old man was evaluated at the outpatient orthopaedic clinic with progressive pain in the right groin. X-ray revealed osteoarthritis of the right hip and fibrous dysplasia of the proximal femur. Total hip arthroplasty was performed using a cemented long-stem femoral component spanning the entire lesion.


Assuntos
Artroplastia de Quadril , Fêmur/patologia , Displasia Fibrosa Óssea/patologia , Virilha/patologia , Prótese de Quadril , Osteoartrite/patologia , Dor/diagnóstico , Idoso , Displasia Fibrosa Óssea/diagnóstico , Displasia Fibrosa Óssea/terapia , Quadril/patologia , Quadril/cirurgia , Articulação do Quadril/patologia , Articulação do Quadril/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Osteoartrite/diagnóstico , Osteoartrite/terapia , Dor/etiologia , Dor/cirurgia , Radiografia
20.
Pediatr Med Chir ; 43(1)2021 Mar 19.
Artigo em Inglês | MEDLINE | ID: mdl-33739058

RESUMO

Fibrous Dysplasia (FD) is a non-malignant condition caused by post-zygotic, activating mutations of the GNAS gene that results in inhibition of the differentiation and proliferation of bone-forming stromal cells and leads to the replacement of normal bone and marrow by fibrous tissue and woven bone. The clinical behavior and progression of FD is variable. The management of this condition is difficult and in every case is strictly individualized. We report a case of frontal fibrous dysplasia in a 6month's old boy who underwent a successfully resection of the lesion with an excellent cosmetic effect.


Assuntos
Displasia Fibrosa Óssea , Displasia Fibrosa Óssea/diagnóstico por imagem , Displasia Fibrosa Óssea/genética , Humanos , Lactente , Masculino , Mutação
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