Fibrous Dysplasia of the Parietal Bone with Focal Motor Seizures: A Case Report.

BACKGROUND Monostotic fibrous dysplasia is a benign proliferation of fibrous and osseous tissues that expand medullary bone to cause symptoms due to compression of adjacent organs and anatomical structures. Focal seizures are rarely the first sign of this kind of lesion. This report describes a young female patient with left-sided focal motor seizures associated with fibrous dysplasia presenting as a mass in the right parietal bone. CASE REPORT An 18-year-old female student with left-sided focal motor seizures presented with a mass in the right parietal bone. Computed tomography revealed an expansile mixed-density lesion on the right parietal bone, a relatively homogeneous ground-glass appearance in the outer circumferential portion, and a lucent eccentric area with thinned but sclerotic borders. Magnetic resonance imaging revealed a homogeneously hypointense signal on T1WI, a small hyperintense signal on T2WI, and avid enhancement signal intensity on post-contrast T1. Electroencephalogram showed inter-ictal epileptiform activities derived from the right fronto-central lobe. Surgical en bloc resection with a margin of normal bone and cranioplasty were performed. Histopathology showed features indicative of fibrous dysplasia, including osteoid trabeculae arranged haphazardly in a dense fibroblastic stroma, irregular trabeculae lacking conspicuous osteoblastic rimming, and intervening fibrous stroma containing cytologically bland spindle cells. The patient achieved seizure control and has remained neurologically intact. CONCLUSIONS This report has highlighted the importance of early diagnosis of fibrous dysplasia of bone to exclude primary bone malignancy or bone metastasis, to ensure rapid management and symptom control.

Craniofacial Fibrous Dysplasia: Experience at San José Hospital, Bogotá, Colombia.

INTRODUCTION: Fibrous dysplasia is a disorder in which normal bone is gradually replaced by immature fibro-osseous tissue, with an incidence of less than 7% of all benign bone tumors. The management of this disease is a challenge for plastic surgeons and neurosurgeons. GOAL: To describe the diagnostic, therapeutic, and outcome approach of patients with craniofacial fibrous dysplasia seen at the Plastic Surgery Service of the Hospital San José in Bogotá, Colombia. METHODS: This is a descriptive and retrospective case series study of patients diagnosed with monostotic and polyostotic fibrous dysplasia treated at the Plastic Surgery Department of Hospital San José during the period from January 1, 2010, to July 31, 2023. RESULTS: All (n=10) of the patients had monostotic craniofacial fibrous dysplasia. The most affected bones in patients with monostotic fibrous dysplasia were zone I bones (n=10, 100%), followed by zone II bones (n=2, 20%). Patients with zone I and II involvement manifested throbbing headaches associated with phosphenes and tinnitus (n=8, 80%) and pain during occlusion associated with edema in the affected cheek (n=5, 50%). Physical examination showed that patients with orbital wall involvement (zone I bone) had ocular dystopia (n=7, 70%).Regarding the treatment received by the patients, 90% (n=9) of the patients received surgical management as primary treatment, with orbitotomy, replacement, and/or remodeling of the roof and lateral wall of the orbit with bone graft, drilling, canthoplasty, ciliary suspension being the most frequently performed procedure (n=6, 60%). Of the patients, 20% (n=2) required reintervention. CONCLUSIONS: FD is a slowly progressive benign fibro-osseous disease that requires a timely, individualized, and multidisciplinary diagnosis and treatment to obtain favorable clinical and surgical results.The mainstay of treatment is surgery as a preventive measure since it is important to avoid future functional alterations that, depending on the location of the dysplasia, would cause a high risk of alteration of adjacent structures.

Frequency of spontaneous osteosarcomatous transformation of craniofacial fibrous dysplasia: a systematic review.

OBJECTIVE: A systematic review was undertaken to examine the spontaneous of craniofacial fibrous dysplasia (FD) into osteosarcoma (OS). STUDY DESIGN: Five databases were searched, and data were evaluated in 2 subsets: (1) frequency analysis involving only cross-sectional studies that specifically examined patients for osteosarcomatous transformation, thereby allowing a calculation of the frequency of transformation of craniofacial FD, and (2) case analysis of case reports and case series of FD that underwent osteosarcomatous transformation. Quality assessment of the studies in the frequency and case analyses was performed. RESULTS: Seven cross-sectional studies involving 779 patients in the frequency analysis documented transformation of craniofacial FD into OS in 13 patients, for a frequency of 1.67%. Of the 18 separate cases of osteosarcomatous transformation in the case analysis, 11 (61.1%) occurred in females, 11 (61.1%) developed from monostotic FD, and 7 (38.9%) arose in the mandible, with 5 in the maxilla (27.8%) and 6 (33.3%) in other craniofacial sites. The mean age of the patients at the time of malignant diagnosis was 36.4 years, with an average of 11.3 years from FD diagnosis to the appearance of OS. The quality of the articles ranged from fair to excellent. CONCLUSIONS: The risk of osteosarcomatous transformation of craniofacial FD is low, but the possibility of transformation should be taken into consideration by clinicians, as this will guide treatment and follow-up regimens.

Displasia fibrosa monostótica / Monostotic fibrous displasia

Imágenes

Displasia fibrosa monostótica simulando proceso neoformativo intraoral en una paciente con sida. Primer caso reportado en esta población / MONOSTOTIC FIBROUS DYSPLASIA SIMULATING A NEOPROLIFERATIVE INTRAORAL NEOPLASM IN A FEMALE WITH AIDS. FIRST CASE REPORTED IN THIS POPULATION

Objetivo: Los pacientes con enfermedad por el virus de la inmunodeficiencia humana suelen presentar lesiones orales, hasta en un 50% de los casos con diagnóstico de sida. La displasia fibrosa es una lesión intra-ósea caracterizada por una alteración del crecimiento y diferenciación de los osteoblastos debida a una mutación genética. Clínicamente se caracteriza por presentar una tumoración de lento crecimiento con dolor, deformidad ósea y, en ocasiones, fracturas ante traumas mínimos. Caso clínico: Se presenta una paciente con sida y tuberculosis diseminada que desarrolló una lesión ósea tumoral , con compromiso de paladar y encía superior derecha, cuyo diagnóstico histopatológico fue de displasia fibrosa de paladar óseo y maxilar superior. Conclusión: La displasia fibrosa debe incluirse en el diagnóstico diferencial de las lesiones tumorales orales de los pacientes con enfermedad VIH/sida.

Aim: Patients with human immunodeficiency virus infection usually have oral lesions, including up to 50% of patients diagnosed with AIDS. Fibrous dysplasia is an intra-bone lesion, characterized by an alteration of the growth and differentiation of osteoblastes produced by a genetic mutation. Clinically it is characterized by presenting a tumor of slow growth with pain, bone deformity and sometimes fractures to minimal trauma. Clinical case: Here we describe a patient with AIDS and disseminated tuberculosis who developed a large tumor lesion that involve the hard palate and the maxilla. Final histopathological diagnosis was of fibrous dysplasia involving the hard palate and the upper maxilla. Conclusion: fibrous dysplasia should be included in the differential diagnosis of intraoral tumor lesions in HIV/AIDS patients.

Tratamiento multidisciplinario en un niño afectado por un quiste dentígero / Multidisciplinaty treatment in a child with dentigerous cyst

Se presenta el caso clínico de un paciente que a los 7 años de edad fue remitido a la Clínica Estomatológica Provincial Docente Mártires del Moncada de Santiago de Cuba por presentar un quiste dentígero y displasia fibrosa monostótica. Desde entonces, y durante casi 10 años, el paciente ha sido atendido por un equipo multidisciplinario odontopediátrico, que ha seguido su evolución y ha aplicado diferentes protocolos diagnósticos y terapéuticos; estos últimos han incluido intervenciones quirúrgicas, rehabilitaciones protésicas, quimioterapia, entre otros. Por la complejidad del caso y la poca frecuencia con que aparecen asociadas ambas entidades clínicas en la infancia, se decidió comunicar este artículo al gremio odontológico nacional y extranjero.

The case report of a seven years old child who was referred to Mártires del Moncada Provincial Teaching Stomatological Clinic in Santiago de Cuba, who had a dentigerous cyst and monostotic fibrous displasia is presented. Since then, and almost during 10 years, the patient has been assisted by a multidisciplinary odontopediatric team, which has followed his clinical course and has applied different diagnostic and therapeutic protocols, including surgical procedures, prosthetic rehabilitations, chemotherapy, among others. Due to the case complexity and the frequency with which both clinical entities are associated in childhood, it was decided to publish this work for the national anf foreing odontological community.

Malignant transformation of monostotic fibrous dysplasia into angiosarcoma in the proximal tibia.

The majority of bone angiosarcomas are primary tumors while secondary angiosarcomas arise after radiation therapy or bone infarctus. This article presents a case of malignant transformation of monostotic fibrous dysplasia into angiosarcoma. An 80-year-old female presented with pain on right cruris. Radiological examination revealed a lesion with lytic areas and destruction of cortical bone on right tibia. Gross and histopathological examination showed two areas with an abrupt transition. The solid component was composed of curved, immature bony trabeculae in a fibroblastic stroma. The other component involved epitheloid cells forming slit-like vascular spaces. The diagnosis of angiosarcoma and fibrous dysplasia was given. Malignant transformation of fibrous dysplasia into angiosarcoma is extremely rare; as this is the sixth case in the existing literature. Prognosis of fibrous dysplasia is generally good and less than 1% of the patients develop a malignant tumor. Therefore, patients with fibrous dysplasia should be offered a life-long follow-up.

Fibrous dysplasia limited to an ossicle.

Fibrous dysplasia is an unusual pathologic condition caused by abnormal bone metabolism. Temporal bone involvement is often seen, but it is uncommon to find fibrous dysplasia limited to the middle ear, especially originating in and confined to a single ossicle. Here we report a case of osteofibrous dysplasia limited exclusively to an ossicle (malleus) causing gradual conductive hearing loss, which recovered after eventual complete removal of the dysplastic area. The lesion showed firm attachment to adjacent structures and initial removal was not possible. This report provides information to help other otologic surgeons facing similar conditions.

Displasia fibrosa monostótica craneofacial / Craniofacial monostotic fibrous dysplasia

La displasia fibrosa es una enfermedad benigna del hueso, de base genética y de progresión lenta, que se caracteriza por el reemplazo progresivo del hueso normal por tejido fibrótico. El proceso puede afectar un solo hueso o múltiples huesos (displasia fibrosa monostótica o poliostótica); esta última puede estar asociada con enfermedades sindrómicas, como el síndrome de McCune-Albright. Objetivo: revisar el diagnóstico y tratamiento de los pacientes con displasia fibrosa craneofacial. Materiales y métodos: se realizó una revisión de artículos científicos en revistas indexadas en línea como técnica para la recolección de información relevante en bases de datos como SciELO, Google Académico, Scopus, PubMed y DynaMed, con artículos publicados en inglés y español. El período de revisión tuvo como límite el 1 de junio de 2020. Los términos de búsqueda fueron: "displasia fibrosa", "displasia fibrosa monostótica" y "displasia fibrosa craneofacial". Se obtuvieron 33 artículos. Conclusión: la displasia fibrosa monostótica craneofacial es una enfermedad rara que afecta principalmente la mandíbula, el hueso maxilar y el cornete inferior. Puede cursar asintomática o presentar deformidades faciales, dolor y trastornos visuales. Su diagnóstico es principalmente por estudios de imágenes y la biopsia. El tratamiento quirúrgico debe ser bien planeado en cada uno de los pacientes y enfocado a deformidades faciales, se deben evitar los trastornos visuales y es necesario cuando se maligniza la lesión.

Fibrous dysplasia is a slowly progressive, genetically based, benign bone disease characterized by progressive replacement of normal bone with fibrotic tissue. The process can affect a single bone or multiple bones (monostotic or polyostotic fibrous dysplasia); the latter may be associated with syndromic diseases, such as McCune-Albright syndrome. Objective to review the diagnosis and treatment of patients with fibrous craniofacial dysplasia. Materials and methods a review of scientific articles was carried out in online indexed journals as a technique for collecting relevant information in databases such as SciELO, Google Scholar, Scopus, PubMed, and DynaMed, with articles published in English and Spanish. The review period was limited to June 1, 2020. The search terms were: "fibrous dysplasia", "monostotic fibrous dysplasia", and "craniofacial fibrous dysplasia". Obtaining 33 articles. Conclusion craniofacial monostotic fibrous dysplasia is a rare disease, mainly affecting the mandible, the maxillary bone, and the inferior turbinate. It can be asymptomatic or present facial deformities, pain and visual disorders. Its diagnosis is mainly by imaging studies and biopsy. Surgical treatment must be well planned in each of the patients, focused on facial deformities, avoiding visual disorders and is necessary when the lesion is malignant.

Radiographic Follow-Up of Fibrous Dysplasia in 138 Patients.

OBJECTIVE. The purpose of this study was to explore the temporal changes in fibrous dysplasia (FD) at radiographic follow-up. MATERIALS AND METHODS. A total of 138 patients with FD who had undergone extremity radiography at least twice with a minimum 12-month interval between examinations were enrolled in this study. FD was monostotic in 99 patients and polyostotic in 39 patients. Patients were also classified according to skeletal maturity as follows: Patients 16 years old or younger were classified in the skeletally immature group (n = 34), and patients 17 years old or older were classified in the skeletally mature group (n = 104). We compared the initial and follow-up radiographs for the following findings: lesion size, opacity, sclerotic rim, calcification, and trabeculation. RESULTS. Of the 138 patients, radiographic follow-up showed no change in lesion size in 101 patients (73.2%), progression in 31 (22.5%), and regression in six (4.3%). FD in immature bones progressed more often than FD in mature bones (23/34 [67.6%] vs 8/104 [7.7%], respectively; p = 0.000), and polyostotic FD had a greater chance of regressing than monostotic FD (4/39 [10.3%] vs 2/99 [2.0%]; p = 0.032). A temporal change in FD lesion opacity was noticed in a minority of patients (19/138, 13.8%). Variable changes were observed in the sclerotic rim, calcification, and trabeculation. CONCLUSION. The radiographic follow-up of FD showed that approximately a quarter of lesions changed in size over time. Regardless of the change in lesion size, opacity and several morphologic features of FD changed during the follow-up period, which might reflect the histopathologic evolution of FD.

Kyphotic deformity of the lumbar spine due to a monostotic fibrous dysplasia of the second lumbar vertebra: a case report and its surgical management.

Monostotic fibrous dysplasia (MFD) of the lumbar spine represents an exceedingly rare lesion. A 26-year-old patient presented with a progressive osteolytic lesion of the vertebral body L2 and the diagnosis of MFD. A minimally invasive left-sided eXtreme Lateral Interbody Fusion (XLIF) approach with resection of the vertebral body L2 with placement of a mesh cage was performed. No complications were observed perioperatively and the symptoms rapidly improved. Minimally invasive piecemeal resection with a combined dorsolateral approach showed a favorable clinical and radiological outcome and seems to be a safe and reliable technique for MFD.

A case of presumed monostotic sphenoid fibrous dysplasia and abducens nerve palsy.

Monostotic fibrous dysplasia is a rare osteodystrophy that typically affects adolescents, manifests later in life, and may be associated with subclinical hormonal imbalances. It can lead to serious complications, including visual compromise. We report a case of presumed monostotic sphenoid fibrous dysplasia presenting with transient abducens nerve palsy and esotropia in an otherwise healthy infant. The mechanism of transient, noncompressive abducens nerve palsy is not clearly understood but has been theorized to be secondary to localized inflammation during a cycle of florid bone remodeling that includes cyst formation, microhemorrhage, and resorption.

O Cripto-1 é um potencial biomarcador para o diagnóstico diferencial dos tumores ósseos benignos e malignos dos maxilares / Cripto-1 is a potential biomarker for the differential diagnosis of benign and malignant jaw bone tumors

O termo lesão fibro-óssea dos maxilares (LFOM) é uma designação inespecífica para um grupo de distúrbios caracterizados, morfologicamente, pela substituição do tecido ósseo por uma matriz de tecido conjuntivo fibrosa, a qual exibe neoformação de tecido ósseo com diferentes graus de mineralização. O diagnóstico preciso das LFOM não é fácil e só pode ser realizado após uma análise minuciosa dos aspectos clínicos, radiológicos e histológicos. No entanto, deve-se admitir que alguns casos desafiam a exatidão na emissão do diagnóstico. Considerando a diversidade do comportamento biológico das lesões e as pesquisas sobre a identificação de potenciais marcadores moleculares, o objetivo deste trabalho foi realizar uma análise imunohistoquímica do cripto-1 (CR-1) e da ß-catenina em uma série de casos diagnosticados microscopicamente como displasia fibrosa (DF) (n=30), fibroma ossificante central (FOC) (n=28) e osteossarcoma (OS) (n=5) armazenados nos arquivos do Serviço de Anatomia Patológica Oral de uma população brasileira. As expressões imuno-histoquímicas foram analisadas através de escore imunorreativo. Os dados obtidos foram inseridos em um arquivo do software Microsoft Excel® e, posteriormente, analisados no software Statistical Package for Social Science. Para todos os testes estatísticos utilizados, o nível de significância foi estabelecido em 5% (p<0,05). O CR-1 exibiu predominância de um padrão fortemente positivo para os casos de FOC e OS, e do padrão moderado para os casos de DF (p<0,001). A ß-catenina exibiu predominância do padrão negativo para os casos de FOC e DF, e do padrão fortemente positivo para os casos de OS (p=0,001). O teste de correlação de Spearman revelou correlação positiva entre os escores imunorreativos de CR-1 e ß-catenina. Os resultados desta pesquisa sugerem a participação do CR-1 na patogênese do FOC e OS, assim como o uso dessa proteína como potencial biomarcador molecular para o diagnóstico diferencial de LFOM (AU).

Fibro-osseous lesions of maxilar (FOLM) is a non-specific designation for a group of disorders characterized, morphologically, by replacement of bone tissue by a matrix of fibrous connective tissue, showing neoformation of bone tissue with varying degrees of mineralization. Precise diagnosis of FOLM is not easy, and requires careful analyisis of clinical, radiological and histological aspects. Even so, some cases still challenge accuracy in diagnosis. Considering the diversity of biological behaviour of the lesions and the research regarding identification of potential molecular markers, this study aims to perform immunohistochemical analysis of crypto-1 and ß-catenin in a series of cases diagnosed microscopically as fibrous dysplasia (FD) (n=30), central ossifying fibroma (COF) (n=28) and osteossarcoma (OS) (n=5), stored in archives of Oral Pathological Anatomy Service of a Brazilian population. Immunohistochemical expressions were analysed by imunorreactive score. All data obtained was inserted into a file of Microsoft Excel® software (Microsoft Corporation, USA) and then transferred to a database of SPSS® for Windows software (Statistical Package for Social Sciences; IBM, USA), version 20.0. For all statistical tests used, the significance level established was p ≤ 0.05. CR-1 showed a predominant pattern of strong positive in COF and OS cases, and a moderate positive in FD cases (p<0,001). ß-catenin showed a predominant negative pattern for COF and FD cases, and a predominant strong positive pattern for OS cases (p=0,001). Spearman correlation tests showed positive correlation of the imunoreative scores of CR-1 and ß-catenin. Those results suggests CR-1 could be involved in the pathogenesis of COF and OS, and this protein could be used as a potential molecular biomarker for diferential diagnosis of FOLM (AU).

Malignant transformation of fibrous dysplasia into angiosarcoma.

Fibrous dysplasia (FD) is generally considered to be a benign disease that affects the bones, but it has potential to become malignant over time, generally several decades after its initial diagnosis. Radiation therapy can induce malignant transformation of FD; however, reports have indicated a few cases of malignant transformation of FD in the absence of radiation therapy. Angiosarcoma is a particularly rare type of cancer in the oral region, which accounts for less than 1% of all soft-tissue sarcomas. Herein, we reported a case of a 62-year-old man with monostotic FD of the left maxilla of over 50 years' duration that underwent malignant transformation into an epithelioid-type angiosarcoma. To the best of our knowledge, this is the first report of such case.

The surgical management of monostotic fibrous dysplasia of the inferior turbinate.

Fibrous dysplasia is a non-neoplastic, sporadic, slowly progressing disease of the bone in which normal bone is replaced by abnormally overgrowing lesions. There are three different types of fibrous dysplasia: monostotic (affecting a single bone), poliostotic (affecting multiple bones) and syndromic, when it is associated with other diseases (such as McCune Albright syndrome). Fibrous dysplasia affects cranio-facial bones in 10% of the cases. However, its occurrence in the inferior turbinate is extremely rare. To the best of our knowledge, only four cases of monostotic form have been reported so far. Hereafter we describe a case of monostotic FD of the inferior turbinate surgically treated with a trans-nasal endoscopic partial maxillectomy type II.

Monostotic fibrous dysplasia at C7 treated with vertebroplasty: a case report and review of the literature.

BACKGROUND: Monostotic fibrous dysplasia (MFD) involving the spine is rare, and the treatment options are controversial. Surgery is needed when patients suffer from persistent pain, spinal cord compression/injury, and vertebral collapse/instability. Treatment methods include biopsy/observation, corpectomy with instrumented fusion, posterior fusion, vertebroplasty (VP), curettage and bone graft, and complete removal of the vertebra with a combined anterior and posterior fusion procedure. CASE PRESENTATION: The patient was a 56-year-old woman with a 2-year history of neck pain. No obvious abnormalities were detected on neurological or physical examination, and laboratory findings were all within normal limits. An imaging examination suggested a C7 vertebral bone tumor. The patient refused to continue conservative observation treatment and requested surgery. Open VP of the C7 vertebral body was carried out, and her postoperative neck pain was completely relieved. The postoperative pathological results supported the diagnosis of fibrous dysplasia, and the patient was ultimately diagnosed with MFD. At the 12-month follow-up visit, the patient reported no clinical symptoms, and no signs of tumor recurrence were detected. CONCLUSION: VP can relieve pain while stabilizing the spine. Thus, the surgical treatment of MFD vertebral lesions by VP is a valuable option.

Serie de Casos Clínicos: Displasia Osteofibrosa Craneofacial. Resolución Quirúrgica / Case Series: Craniofacial Fibrous Dysplasia. Surgical Treatment

INTRODUCCIÓN: La Displasia Fibrosa Ósea Craneofacial es una lesión ósea benigna en la que se da una sustitución de tejido óseo normal por tejido fibro-óseo. Desarrollada a partir de una mutación genética. Subordinada en variantes: monostósica y polistósica. Escasos casos son reportados sobre malignización de la patología. El Diagnóstico se realiza con la sospecha clínica y se confirma mediante exámenes de imagen y anatomopatológicos. CASO CLÍNICO: El presente trabajo presenta una serie de tres casos diagnosticados de Displasia Osteofibrosa Craneofacial en el Hospital José Carrasco Arteaga y Hospital del Río, Cuenca-Ecuador, en los que por el cuadro clínico de los pacientes se decidió tratamiento quirúrgico. EVOLUCIÓN: En todos los casos, la evolución fue favorable. Se logró resultados positivos, reducción de síntomas y mejoría estética general en todos los casos. CONCLUSIÓN: Se obtienen mejores resultados con los tratamientos quirúrgicos actuales (remodelado más congelamiento óseo), entre ellos: buenos resultados estéticos, mejor calidad de vida, menor riesgo de infecciones. En el presente trabajo los tres participantes sometidos a resolución quirúrgica señalaron que el tratamiento reflejó positivamente en el ámbito funcional, estético y emocional. (au)

BACKGROUND: Craniofacial Bone Fibrous Dysplasia is a benign bone lesion where normal bone tissue is replaced with fibrous tissue. Developed from a genetic mutation. Subordinated in variants: monostotic and polyistotic. Few cases are reported on malignancy of the pathology. The diagnosis is determined with clinical suspicion and confirmed by imaging and pathology tests. CASE REPORT: In this paper, we present a series of three cases diagnosed with Craniofacial Fibrous Dysplasia at José Carrasco Arteaga Hospital and Hospital del Río, Cuenca-Ecuador. All of patients were treated with surgery, because of the clinical features. EVOLUTION: In all the cases, the patient evolution was favorable. Reduction of symptoms and general aesthetic improvement were achieved in all cases. CONCLUSION: Better results are obtained with the current surgical treatments (bone remodeling plus bone freezing), among them: good aesthetic results, better quality of life, lower risk of infections. In the present paper, the three patients treated with the surgical procedure indicated that the treatment reflected positively in the functional, aesthetic and emotional aspects.(au)

A Rare Case of Radiculopathy: Monostotic Fibrous Dysplasia of the Sacrum.

Fibrous dysplasia is a bone disease characterized by an osteoblastic dysfunction resulting in a fibrous replacement of the normal medullary bone. We describe the case of a 33-year-old who presented with low back pain irradiating to her right leg. Both the computed tomography scan and magnetic resonance imaging showed an osteolytic, multicystic lesion of the right hemi-sacrum with invasion of the right S1 foramen. She underwent foraminotomy and curettage of the lesion. Histological diagnosis was fibrous dysplasia, without features of malignant transformation. Three years after surgery the patient is asymptomatic and imaging is stable. This is the fifth known case of monostotic fibrous dysplasia involving the sacrum, a rare entity that must be considered in the differential diagnosis when approaching patients with sacral lesions.

A displasia fibrosa é uma doença óssea caracterizada por uma disfunção osteoblástica que resulta na substituição do osso medular normal por tecido fibroso. Descrevemos o caso de uma doente de 33 anos que se apresentou com lombalgia e ciatalgia direita. A tomografia computorizada e ressonância magnética lombares revelaram uma lesão osteolítica e multiquística no hemissacro direito com invasão do foramen de S1. A doente foi submetida a foraminotomia e curetagem da lesão. O diagnóstico histológico foi de displasia fibrosa, sem sinais de transformação maligna. Três anos após a cirurgia, a doente está assintomática e os estudos de imagem apresentam estabilidade da lesão. Este é o quinto caso conhecido na literatura de displasia fibrosa monostótica com atingimento do sacro, uma entidade rara que deve ser considerada no diagnóstico diferencial de doentes com lesões do sacro.

Surgical Treatment of Polyostotic Craniomaxillofacial Fibrous Dysplasia Associated With Acromegalia: The McCune - Albright Syndrome.

INTRODUCTION: The McCune-Albright syndrome (MAS) is a complex congenital disorder caused by the embryonic post-zygotic somatic activating mutations in the GNAS1 gene. In such syndrome, phenotypes are heterogeneous and comprised polyostotic/monostotic fibrous dysplasia, café au lait macules, and hyperfunctioning endocrinopathies as the excess growth hormone. Likewise, acromegaly, as a manifestation of the endocrine hyperfunction, is unusual and affects about 20% of patients with MAS. CASE PRESENTATION: This research study describes a case of a 31-year-old female subject presenting polyostotic fibrous dysplasia with severe facial involvement, along with acromegaly and the MAS. The case was satisfactorily managed by surgical re-alignment and presented no clinical signs of relapse in a 12-year follow-up period. Finally, a literature review was conducted to discuss the standard protocols and the controversies when treating such cases. CONCLUSION: Patients with craniomaxillofacial fibrous dysplasia associated with acromegaly may present significant facial deformities that can be satisfactorily treated by cosmetic treatment, especially in patients with psychological problems and severe social acceptance.