Oral manifestations of histiocytosis of langerhans cells: a pediatric case involving mandible bone / Manifestaciones orales de histiocitosis de células de Langerhans: un caso pediátrico relacionado con el hueso mandibular

Abstract Langerhans cells histiocytosis is a rare disease characterized by monoclonal proliferation and migration of special dendritic cells in a variety of organs, most commonly appears eosinophilic granuloma localized, often solitary, and bone lesions that occurs predominantly in pediatric patients. Although is most prevalent in children under the age of 15, this disorder presents in all ages and occurs at a rate of 2 to 5 cases per million per year. LCH is a complex entity; the clinic manifestations can mimic other common conditions and therefore a comprehensive evaluation is indicated. Since oral manifestations are frequent, the early diagnose of this pathology could be detected by dental professionals. The aim of this case report is to describe a case of LCH who initially was misdiagnosed and treated for a dental infection. This disease requires accurate histopathological diagnosis and timely treatment; hence it is necessary to raise awareness among dentists to avoid misdiagnose of oral manifestations of LCH.

Resumen La histiocitosis de las células de Langerhans es una enfermedad poco frecuente que se caracteriza por la proliferación monoclonal y la migración de células dendríticas especiales en una variedad de órganos; lo más común es que aparezca un granuloma eosinofílico localizado, a menudo solitario, así como lesiones óseas que se producen predominantemente en pacientes pediátricos. Aunque es más frecuente en los niños menores de 15 años, este trastorno se presenta en todas las edades y se produce a una tasa de 2 a 5 casos por millón al año. La HCL es una entidad compleja; las manifestaciones clínicas pueden imitar otras afecciones comunes y, por lo tanto, se indica una evaluación exhaustiva. Dado que las manifestaciones orales son frecuentes, el diagnóstico precoz de esta patología podría ser detectado por los profesionales de la odontología. El objetivo de este reporte de caso es describir un caso de HCL que inicialmente fue mal diagnosticado y tratado por una infección dental. Esta enfermedad requiere un diagnóstico histopatológico preciso y un tratamiento oportuno; por lo tanto, es necesario sensibilizar a los dentistas para evitar un diagnóstico erróneo de las manifestaciones orales de la HCL.

The diagnostic value of X-ray, CT and MRI multimodal imaging in the vertebral compression fractures caused by osteolytic metastases and osteoporosis / 中国医师进修杂志

Objective:To analyze the diagnostic value of X-ray, CT and MRI multimodal images in the vertebral compression fractures caused by osteolytic metastases and osteoporosis.Methods:The basic clinical data and X-ray, CT, MRI imaging data of 102 patients with vertebral compression fractures from January 2019 to May 2021 in Jiangnan Hospital (Xiaoshan Traditional Chinese Medicine) Affiliated to Zhejiang University of Traditional Chinese Medicine were retrospectively analyzed. Among them, vertebral compression fractures caused by osteolytic metastases was in 47 cases, and vertebral compression fractures caused by osteoporosis was in 55 cases.Results:The age and osteoporosis rate in patients with vertebral compression fractures caused by osteolytic metastases were significantly lower than those in patients with vertebral compression fractures caused by osteoporosis: (61.95 ± 11.84) years old vs. (72.37 ± 12.55) years old and 4.3% (2/47) vs. 83.6% (46/55), the body mass index and pain visual analogue score were significantly higher than those in patients with vertebral compression fractures caused by osteoporosis: (22.58 ± 3.85) kg/m 2 vs. (18.11 ± 2.79) kg/m 2 and (8.31 ± 2.91) scores vs. (7.02 ± 2.72) scores, and there were statistical differences ( P<0.05); there was no statistical difference in gender composition ( P>0.05). The rates of vertebral body wedge shape and double concave shape in patients with vertebral compression fractures caused by osteolytic metastases were significantly lower than those in patients with vertebral compression fractures caused by osteoporosis: 2.80% (3/107) vs. 60.82% (104/171) and 6.54% (7/107) vs. 29.82% (51/171), the rates of flat shape and posterior margin swelling were significantly higher than those in patients with vertebral compression fractures caused by osteoporosis: 75.70% (81/107) vs. 9.36% (16/171) and 14.95% (16/107) vs. 0, and there were statistical differences ( P<0.01); the rates of pedicle involvement and soft tissue mass in patients with vertebral compression fractures caused by osteolytic metastases were significantly higher than those in patients with vertebral compression fractures caused by osteoporosis: 68.09% (32/47) vs. 1.82% (1/55) and 46.81% (22/47) vs. 0, while the rate of linear image signal in vertebral body was significantly lower than that in patients with vertebral compression fractures caused by osteoporosis: 0 vs. 96.36% (53/47), and there were statistical difference ( P<0.01); there was statistical difference in MRI signals ( P<0.01), vertebral compression fractures caused by osteolytic metastases were mainly characterized by low T 1 high T 2 and low T 1 low T 2, while vertebral compression fractures caused by osteoporosis was mainly characterized by low T 1 high T 2; the rates of disc compression and widening in patients with vertebral compression fractures caused by osteolytic metastases were significantly lower than those in patients with vertebral compression fractures caused by osteoporosis: 4.26%(2/47) vs. 34.55% (19/55) and 2.13%(1/47) vs. 18.18% (10/55), and there were statistical differences ( P<0.01 or <0.05). The accuracies of multimodal imaging in the diagnosis of vertebral compression fractures caused by osteolytic metastases and osteoporosis were significantly higher than those of X-ray, CT and MRI (89.4% vs. 51.1%, 72.3%, 83.0%; 90.9% vs. 52.7%, 60.0%, 78.2%), and there were statistical differences( P<0.05). Conclusions:Multimodal imaging is helpful to improve the diagnostic accuracy of vertebral compression fractures caused by osteolytic metastases and osteoporosis, to reduce the clinical misdiagnosis rate, with important reference value for the differential diagnosis of the two diseases.

Juvenile hyaline fibromatosis: a case report and literatures review / 口腔疾病防治

Objective@#To investigate the clinicopathological characteristics, imaging manifestations, genetic manifestations, diagnosis and treatment of juvenile hyaline fibromatosis.@*Methods @# A case of juvenile hyaline fibromatosis was reported, and the patient's clinical manifestations, imaging examinations, histopathological examinations, genetic changes and treatment were summarized and analyzed.@*Results @#Juvenile hyaloid fibromatosis is more common in infants and children. This patient had typical clinical and pathological manifestations, including posterior occipital masses, skin and subcutaneous nodules, gum hyperplasia, joint contractures, and joint osteolytic lesions. The histopathological lesions were characterized by the proliferation of spindle cells in the tissue accompanied by a large amount of amorphous transparent matrix. Genetic testing was performed to confirm an ANTXR2 gene mutation, consistent with the known genetic changes of juvenile hyaline fibromatosis. The 6-month follow-up of the patient showed that there was no obvious recurrence after resection of the gum and facial mass. In addition to surgery, the treatment of this disease requires multidisciplinary symptomatic treatment combined with rehabilitation and supportive treatment to achieve a better prognostic effect.@*Conclusion@# Juvenile hyaline fibromatosis is a rare nonneoplastic autosomal recessive genetic disease. Mutations in the ANTXR2 gene lead to disorders of collagen synthesis and metabolism in the tissues and further cause subcutaneous nodules, gingival hyperplasia, joint contractures and bone dissolution.

Mandible lymphoma: an aggressive osteolytic lesion

Background: Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-hodgkin's lymphoma. In oral cavity represents approximately 2% of all malignancies. Case presentation: This report describes a rare mandibular involvement of DLBCL. A 56 year-old man was referred for evaluation of left mandible pain. In the anamnesis, the patient informed to be treating tooth pain in lasting 6 months. On oral evaluation, an intense mobility of the left mandibular second molar and a swelling in posterior left mandible were observed. Computed tomography showed a large osteolytic lesion affecting both mandibular body and ramus. An incisional biopsy was performed and according to histopathological and imumnohistochemical features, DLBCL was diagnosed. The treatment consisted of 8 cycles of R-CHOP and adjuvant radiotherapy. He is asymptomatic after 6 years. Conclusion: This case showed a rare bone presentation of DLBCL and such tumor should be considered as differential diagnosis of osteolytic lesion of the mandible.

Mieloma múltiple: enfermedad ósea extensa en una paciente joven / Myeloma multiple: extensive bone disease in a young patient

El mieloma múltiple (MM) es un tumor de proliferación clonal de plasmocitos en la médula ósea (MO). Hasta ahora no es curable1,2. Puede presentarse como una enfermedad indolente o con manifestaciones clínicas como insuficiencia renal, anemia y lesiones osteolíticas1. Se presenta el caso de una paciente femenina de 46 años, quien padecía dolor en la región del brazo izquierdo, acompañado por dolores óseos generalizados. Al examen físico se observó en el tercio proximal de la región humeral izquierda y hombro ipsilateral, gran tumoración que deformaba la anatomía local, indurada, inmóvil y dolorosa. Presentaba anemia severa (Hb. 6 g/dL), cuantificación de ß2 Microglobulina 4,23 mg/L (VR 0,80 ­ 3,0 mg/L) y rastreo óseo radiológico con múltiples lesiones líticas. En la muestra de médula ósea se encontró infiltración de 80 % de células plasmáticas mono- clonales kappa. Se le diagnosticó discrasia de células plasmáticas tipo MM monoclonal kappa sintomático, estadio II (ISS), con enfermedad ósea extensa y un gran plasmocitoma humeral izquier- do. Se indicó tratamiento de inducción de la remisión con el esquema VCD (bortezomib, ciclofosfamida y dexametasona). Adicionalmente ácido zoledrónico. Posteriormente se modificó a bortezomib, talidomida y prednisona. Luego del tratamiento antineoplásico, refirió acalmia completa del dolor con mejoría de la movilidad. Este caso clínico se trata de una presentación inusual de MM debido a la edad de la paciente y a la extensa enfermedad ósea. Llamó la atención la ausencia de niveles elevados de la cadena liviana kappa de las inmunoglobulinas libres en suero. Por la edad de la paciente y la ausencia de co-morbilidades significativas, es candidata para trasplante de células progenitoras hematopoyéticas (TCPH)(AU)

Multiple myeloma (MM) is a tumor of clonal proliferation of plasma cells in the bone marrow (BM). Until now it is not curable1,2. It can present as without symptoms or with clinical manifestations such as renal failure, anemia and osteolytic lesions1. We describe the case of a 46-year-old female patient, who complained of pain in her left arm, and, also, by generalized bone pain. On physical examination a large tumor was present in the proximal third of the left humeral region and ipsilateral shoulder, it was hard, painful and immo- bile. She had severe anemia (Hb 6 g / dL), quantification of ß2 Microglobulin 4.23 mg / L (VR 0.80 - 3.0 mg /L) and the radiological bone survey showed multiple lytic lesions. In the bone marrow sample, an infiltration of 80 % kappa monoclonal plasma cells was found. Her diagnosis was MM-type plasma cell dyscrasia, symptomatic kappa, stage II (ISS), with extensive bone disease and a large left humeral plasmacytoma. Remission induction therapy was indicated with the VCD scheme (bortezomib, cyclophosphamide and dexa- methasone). Additionally zoledronic acid was administered. Subsequently, it was modified to bortezomib, thalidomide and prednisone. After antineoplastic treatment, she referred pain relief with improvement of mobility. This clinical case is an unusual presentation of MM due to the age of the patient and extensive bone disease. The absence of high levels of the kappa light chain of free immunoglobulins in serum attracted attention. Due to the age of the patient and the absence of significant comorbidities, she is a candidate for trans- plantation of hematopoietic stem cells(AU)

Rare Langerhans cell histiocytosis in children: a case report

Langerhans cell histiocytosis (LCH) is a rare disease, formally known as histiocytosis X that is characterized by abnormal proliferation of histiocytes derived from bone marrow (Langerhans cells), joined with leucocytes, eosinophils, neutrophils, lymphocytes, plasma cells and giant multi-nucleated cells causing tissue destruction. One of the first signs of LCH is oral manifestation, in some cases, the oral cavity may be the only affected area. With the chance of oral lesion incidence in LCH being 77%.Initial symptoms are generally nonspecific, which can easily cause misdiagnoses.The purpose of reporting this case is to discuss the features of LCH clinically and radiographically and in the role of the dentist when diagnosing such lesions for a proper management.An 11-year-old boy reported a complaint of swelling in the left side of the lower jaw that is asymptomatic and had been gradually increasing in size for the past 6 months without any improvements. After preforming a biopsy and diagnosing the lesion as LCH, the patient was then treated with a dose of vinblastine (6 mg/m2 intravenous bolus) for 24 weeks as a total period. Two years follow up; the patient showed no sign of recurrence and is in good general condition. In conclusion, reporting this case serves as documentation of the proper route of clinical assessment and diagnosis of LCH with the best possible treatment as guidance.

A Case of Peripheral Neuropathy - A Presentation of Poems Syndrome with an Osteolytic Lesion

Introduction: POEMS syndrome is a rare paraneoplasticsyndrome that can often be mistaken for other neurologicdisorders.Case Report: A 48-year-old gentleman presented withprogressive mixed motor and sensory neuropathy involvinglower limbs, with generalized hyperpigmentation andbilateral pitting pedal edema. Neurological examinationshowed symmetrical wasting involving both proximaland distal groups of muscles of lower limbs, decreasedpower (Grade 4/5) of the lower limbs with weakness beingmore severe distally, generalized areflexia, absence ofvibration sense and proprioception over the lower limbs andgraded sensory loss over the dorsum of both feet. Workupshowed a demyelinating sensorimotor polyneuropathy onnerve conduction velocity study and albuminocytologicaldissociation in CSF. After inconclusive initial evaluation, aPET CT was done which picked up an FDG avid lytic lesionin the L1 vertebra, and lymphadenopathy, and an MRI LSspine confirmed an expansile lesion with ""Mini brain sign"",indicative of plasmacytoma. Serum free light chain ratio andserum protein electrophoresis were normal. Bone marrowshowed no evidence of marrow involvement. Consideringthe constellation of findings of demyelinating sensorimotorpolyneuropathy, lymphadenopathy, peripheral edema anda solitary plasmacytoma, the possibility of a rare variant ofPOEMS syndrome with an osteolytic lesion was thought of.The lesion was biopsied and proven to be a plasmacytoma.The patient responded well to localized radiation therapy tothe lesion, along with intravenous steroids.Conclusion: A subacute symmetric sensorimotor neuropathymay be a presentation of POEMS syndrome. There can be rareinstances of a lytic lesion on radiography in POEMS"

A Case of SAPHO Syndrome Associated with Lytic Bone Lesions Resembling Metastases / 대한피부과학회지

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes a variety of inflammatory bone disorders associated with dermatologic pathology. A 57-year-old female presented with pustulosis on both hands that had persisted for several months. She also had lower back pain without trauma history. On physical examination, tenderness on her lower back and left anterior chest wall pain were found, and claudication was observed. Radiological studies including computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET)-CT showed endplate lytic changes in her spine, a focal hypermetabolic lesion in a left rib, and costochondral junction. These findings raised doubt on the presence of metastatic bone lesions, and there was no indication for primary cancer after a complete medical checkup. Palmoplantar pustulosis was well controlled by treatment with acitretin. The osteitis associated with SAPHO syndrome usually presents as osteosclerosis, while reports on osteolytic lesions are rare. We report herein a rare case of SAPHO syndrome associated with bone lesions resembling bone metastasis.

Peripheral primitive neuroectodermal tumor of the mandible: a case report and literature review / 口腔疾病防治

Objective@# To explore the clinical, imaging and pathological characteristics of mandibular peripheral primitive neuroectodermal tumors, and to review relevant literature to improve the understanding and diagnosis of pPNET in mandible.@* Methods@# The clinical and imaging features, pathological examination, treatment and prognosis of a case of mandibular pPNET diagnosed and treated at the First Affiliated Hospital of Xinjiang Medical University were observed, and analyzed a literature review. @* Results @#The patient′s clinical manifestations were an enlarged mass of the mandible, hard texture, unclear borders, involving loose teeth, and numbness of the lower lip; CT and MRI showed osteolytic and aggressive growth patterns. The mandibular tumor was resected and the mandibular partial truncated resection was performed on the titanium plate. Postoperative pathological sections showed small round cell tumors under HE staining and Vimentin and Fli-1 were positive, and the pathological diagnosis was pPNET. The patient did not undergo chemoradiotherapy after surgery and died of tumor recurrence after 9 months of follow-up. A review of the relevant literature revealed that pPNETs are a group of small round cell tumors, which are more common in children and adolescents. pPNETs have a high degree of malignancy, a short course of disease and fast metastasis. The main route of metastasis is through the blood circulation. Most patients die within 2 years, the 3-year survival rate is only 30%, and the 5-year survival rate is less than 10%. Imaging is generally nonspecific; therefore, pPNETs are easily misdiagnosed. The final tumor type is determined by pathological HE staining and immunohistochemical characteristics. Current treatment methods are mainly complete surgical resection combined with postoperative radiotherapy and chemotherapy, but it is critical to provide individualized treatment to patients when necessary.@*Conclusion@# pPNETs have a high degree of malignancy, easy recurrence and poor prognosis, so early diagnosis and treatment are extremely important.

Osteomielitis Crónica Multifocal Recurrente: presentación de caso / Chronic Recurrent Multifocal Osteomyelitis: presentation of case

Los síndromes autoinflamatorios son un grupo de enfermedades caracterizadas por episodios espontáneos, recurrentes o persistentes de inflamación multisistémica. En ellos no se evidencia una etiología infecciosa, neoplásica o autoinmune. Están causadas por alteraciones de la inmunidad innata, lo que ocasiona una desregulación del sistema inflamatorio a nivel del inflamosoma. Estos síndromes se subdividen en dos grandes grupos los síndromes hereditarios de fiebre periódica y las enfermedades autoinflamatorias persistentes, dentro de este último se ubican las enfermedades inflamatorias óseas donde se incluye la osteomielitis multifocal crónica recurrente. Presentamos un caso de una niña de 9 años que ingresa en nuestro hospital por un síndrome febril prolongado y después de múltiples investigaciones se concluye como una osteomielitis multifocal crónica recurrente.

Autoinflammatory syndromes are a group of diseases characterized by spontaneous, recurrent or persistent episodes of multisystem inflammation. They do not show an infectious, neoplastic or autoimmune etiology. They are caused by alterations of the innate immunity, which causes a dysregulation of the inflammatory system at the level of the inflammasome. These syndromes are subdivided into two major groups, the hereditary syndromes of periodic fever and the persistent autoinflammatory diseases; within the latter are the inflammatory bone diseases which chronic recurrent multifocal osteomyelitis. We present a case of a 9-year-old girl who enters our hospital due to a prolonged febrile syndrome and after multiple investigations, it concludes as a Chronic Recurrent Multifocal Osteomyelitis.

Lesões líticas de alto risco e fraturas patológicas / High-risk lytic lesions and pathologic fractures

Introdução: As neoplasias ósseas, sejam primárias ou metastáticas, representam um grande risco à qualidade de vida do paciente; especialmente quando se tratam de lesões dolorosas e com risco de evoluírem a fraturas ósseas, principalmente as do tipo osteolíticas. Lesões osteolíticas podem ocorrer em diversas patologias ósseas não tumorais, como cisto ósseo simples, cisto aneurismático, hiperparatireoidismo, bem como por neoplasias, entre elas destacando-se Mieloma Múltiplo tumores metastáticos, principalmente, Câncer de Mama, Próstata, Pulmão e Rim, cujas principais complicações são as fraturas ósseas patológicas. Métodos: Foram revisados artigos disponíveis em plataformas indexadas online, bem como revisada a literatura disponível em livros textos, guidelines e base de dados nacionais e internacionais. Resultados: Fraturas patológicas podem acometer até 30% dos pacientes com doença metastática óssea, causando dor importante mesmo no período anterior à fratura propriamente dita (Fratura Iminente) ou quando ela já é inevitável. Visando avaliar de forma objetiva a indicação de intervenções profiláticas, Mirels propôs um escore de pontuação avaliando multifatorialmente as lesões ósseas e seus possíveis prognósticos para fraturas. Conclusão: Embora ainda seja considerada o padrão-ouro e relativamente eficiente, o sistema de pontuação de Mirels deve ser aplicado junto com a avaliação clínica e de exames de imagem do médico, como a melhor forma de antever a fratura e, se possível, tratá-la profilaticamente.

Introduction: Bone neoplasms, whether primary or metastatic, represent a great risk to the patient's quality of life; especially when they deal with painful lesions and risk of developing osteolytic fractures. Osteolytic lesions may occur as a result of several non-tumorous bone pathologies, such as simple bone cyst, aneurysmal cyst, hyperparathyroidism, as well as neoplasias, among them Metastatic Multiple Myeloma, Breast Cancer, Prostate, Lung, and Kidney and its main complications are the bone fractures. Methods: Articles available on online indexed platforms were reviewed, as well as the literature available in national and international text books, guidelines and databases. Results: Pathological fractures can affect up to 30% of patients with metastatic bone disease, causing important pain even in the period before the fracture itself (Imminent Fracture), when it is already inevitable. In order to objectively evaluate the functionality of prophylactic interventions, Mirels proposed a scoring score evaluating multifactorially the bone lesions and their possible prognoses. Conclusion: Although the gold standard is still considered to be relatively efficient, the Mirels scoring system should be applied in conjunction with clinical assessment and imaging of the physician in order to better anticipate the fracture and, if possible, treat prophylactically.

Histiocitosis de células de Langerhans con compromiso esternal aislado: Caso clínico / Langerhans cell histiocytosis with isolated sternum involvement: A clinical case

La histiocitosis de células de Langerhans es una entidad poco frecuente, que se caracteriza por la proliferación clonal de células dendríticas con amplia variabilidad clínica. En algunos casos, la enfermedad tiene afectación multisistémica con compromiso de vida y, en otros casos, de compromiso exclusivamente óseo, en los que la sobrevida es del 100%. Se presenta a una niña de 8 años que consultó al Servicio de Urgencias por dolor esternal de 4 días de evolución. Mediante estudios de imágenes, se observó una imagen lítica en el esternón. Se decidió la internación para el manejo del dolor y estudio. Se realizó una punción-aspiración con aguja fina, cuya inmunohistoquímica fue positiva para el marcador CD1a, que confirmó el diagnóstico de histiocitosis. La paciente recibió 40 mg/día de metilprednisolona, con buena evolución. Se decide exponer este caso por ser una forma de presentación atípica de una enfermedad poco prevalente, pero cuyo motivo de consulta amerita la sospecha diagnóstica.

The Langerhans Cell Histiocytosis (LCH) is a rare condition, characterized by the proliferation of dendritic cells. Its clinical presentation is variable and ranges from an isolated skin or bone disease, mainly the skull, to a life-threatening multisystemic disease. This case is about a healthy 8-year-old girl with a history of four days of severe sternum pain and no other symptomatology. At the initial evaluation at the emergency department a chest X-ray (Figure 1) and thoracic computed tomography scan with 3-D reconstruction were performed and they showed the sternal osteolytic lesion (Figure 2). She was admitted to the hospital for further evaluation. The immunohistochemistry evaluation of the fine needle aspiration sample was positive for CD1a, confirming the diagnosis of histiocytosis. The patient received methylprednisolone 40 mg/day with clinical improvement.

Clinical analysis of aneurysmal bone cyst in jaw bone / 口腔疾病防治

Objective @#To investigate aneurysmal bone cyst (ABC) in oral and maxillofacial of clinical appearance, clinical treatment and prognosis.@* Methods @#From July 2003 to December 2015, 8 patients with aneurysmal bone cyst in jaw bone was treated and recorded with the general condition, clinical symptoms, imaging examinations, surgical treatment and prognosis.@*Results @#Among the 8 patients, there were 5 females and 3 males with the age from 5 to 48 years old. 5 cases happened in mandibular bone and 3 cases originated from the maxillary bone. All the patients were primary lesions and diagnosed by pathology. CT scan showed multilocular cystic expansive space occupying lesions, septa, residual bone crest and liquid-liquid form; enhanced imaging showed the tortuous and dilated small blood vessels. Cystic wall composed of spindle shaped fiber cells, tissues and cells, broken bone like multinucleated giant cells and chronic inflammatory cells form and a ribbon like structure, cysts separated by fibrous tissue containing bone and bone fragments, hemorrhage and hemosiderin deposition was observed which was confirmed under the light microscope. The main treatment of aneurysmal bone cyst was radical operation. After 1~6 years of follow-up, no patient was found recurrence. @* Conclusion@# The imaging findings of aneurysmal bone cyst in jaw bone was distintive, with diagnosis of the disease depended on pathology; the main treatment was radical operation with good prognosis.

Lesión osteolítica de calota por sífilis secundaria / Injury by skull osteolytic secundary syphilis

Bone involvement of syphilis can be observed in tertiary and congenital syphilis. It is infrequent during the secondary stage. The skull is the most affected bone in secondary syphilis, and its most frequent form of presentation is proliferative osteitis. If the skull is affected, headache is usual and can be as intense as in meningitis. Osteolyitic lesions may be seen in complimentary imaging studies, with a moth eaten aspect. These lesions raise concern over a number of differential diagnoses, among which are infectious, inflammatory and neoplastic diseases. The definitive diagnosis is made by bone biopsy of the compromised bone. Molecular techniques in the affected tissues increases diagnostic performance. There is no standardized treatment protocol for syphilis since there are no guidelines available. We report a case of a 19 year old female, presenting with a unique osteolytic lesion in the skull due to secondary syphilis.

El compromiso óseo de la sífilis se observa predominantemente en la sífilis terciaria y en la sífilis congénita, siendo infrecuente durante el estadio secundario. El hueso más afectado durante la sífilis secundaria es el cráneo, siendo la osteítis proliferativa la forma más frecuente de presentación. Cuando afecta la calota, la cefalea es habitual y puede ser tan intensa que se confunde con un proceso meníngeo. En las imágenes se observan lesiones líticas de aspecto apolillado, planteando el diagnóstico diferencial con otras patologías infecciosas, inflamatorias y neoplásicas. El diagnóstico definitivo se realiza por estudio histológico del hueso comprometido. Las técnicas de biología molecular en los tejidos afectados aumentan el rendimiento diagnóstico. No existen protocolos estandarizados para el tratamiento de la sífilis con compromiso óseo. Presentamos el caso clínico de una mujer de 19 años de edad, con una lesión osteolítica única de calota debida a una sífilis secundaria.

CT differentiation between vertebral multiple myeloma and osteolytic metastasis / 实用放射学杂志

Objective To explore the imaging differences of vertebral multiple myeloma(VMM)and vertebral osteolytic metastasis on CT. Methods Review the imaging signs of 32 patients with VMM and 52 patients with vertebral osteolytic metastasis,then record and finally statistical analysis was carried out.Results Compare to 1 67 vertebras involved in 52 cases of vertebral metastasis,the patents with VMM had 220 vertebras involved.The incidence of the multiple small circular type of bone destruction in VMM was 42.66%(93/218),which was higher than that in vertebral metastasis 0.00% (0/165)(χ2 =92.963,P =0.000).The incidence of the irregular shape type of bone destruction in VMM was 23.39% (5 1/218),while it was higher in patients with vertebral metastasis 45.45%(75/165)(χ2 =20.704,P=0.000).It was also found that the incidence of the fragmentary type of bone destruction and the involvement of the unilateral pedicle in VMM were lower than that in patients with vertebral metastasis 8.7% (19/218 )& 27.27% (45/165)(χ2 =23.238,P =0.000), 6.82% (1 5/220)& 1 7.96% (30/1 67)(χ2 =1 1.477,P =0.001).The incidence of the crest protrusion type of bone destruction in patients with VMM was 16.06% (35/218),however it was 9.10% (15/165)(χ2 =4.013,P =0.045)in vertebral osteolytic metastasis. Conclusion The imaging features of VMM and vertebral osteolytic metastasis had certain characteristic.And they can be differentiated from each other,combining with clinical traits.

NDRG2 Expression Decreases Tumor-Induced Osteoclast Differentiation by Down-regulating ICAM1 in Breast Cancer Cells

Bone matrix is properly maintained by osteoclasts and osteoblasts. In the tumor microenvironment, osteoclasts are increasingly differentiated by the various ligands and cytokines secreted from the metastasized cancer cells at the bone metastasis niche. The activated osteoclasts generate osteolytic lesions. For this reason, studies focusing on the differentiation of osteoclasts are important to reduce bone destruction by tumor metastasis. The N-myc downstream-regulated gene 2 (NDRG2) has been known to contribute to the suppression of tumor growth and metastasis, but the precise role of NDRG2 in osteoclast differentiation induced by cancer cells has not been elucidated. In this study, we demonstrate that NDRG2 expression in breast cancer cells has an inhibitory effect on osteoclast differentiation. RAW 264.7 cells, which are monocytic preosteoclast cells, treated with the conditioned media (CM) of murine breast cancer cells (4T1) expressing NDRG2 are less differentiated into the multinucleated osteoclast-like cells than those treated with the CM of 4T1-WT or 4T1-mock cells. Interestingly, 4T1 cells stably expressing NDRG2 showed a decreased mRNA and protein level of intercellular adhesion molecule 1 (ICAM1), which is known to enhance osteoclast maturation. Osteoclast differentiation was also reduced by ICAM1 knockdown in 4T1 cells. In addition, blocking the interaction between soluble ICAM1 and ICAM1 receptors significantly decreased osteoclastogenesis of RAW 264.7 cells in the tumor environment. Collectively, these results suggest that the reduction of ICAM1 expression by NDRG2 in breast cancer cells decreases osteoclast differentiation, and demonstrate that excessive bone resorption could be inhibited via ICAM1 down-regulation by NDRG2 expression.

Percutaneous Selective Vertebroplasty: State of the Art Management in Well-Confined Metastatic Vertebral Lesions

STUDY DESIGN: Prospective cohort study. PURPOSE: To evaluate the clinical and radiological results of percutaneous selective vertebroplasty (PSV) as first-line treatment options in the setting of well-confined spinal metastases. OVERVIEW OF LITERATURE: Recent technological advances combined with innovative interventional techniques enable an alternative less invasive treatment option for many patients with malignant vertebral body infiltration. Percutaneous vertebral augmentation procedures offer less invasive but effective pain relief to many patients with symptomatic spinal metastatic disease. METHODS: Eleven patients with 21 well-confined metastatic vertebral lesions that had been treated with PSV were included. Pain was evaluated one week, one month, 3 months and 6 months post-procedure using a 10-point visual analogue scale (VAS). A statistical analysis including repeated measures analysis of variance test was used to collectively indicate the presence of any significant differences between different time sequences. Medication usage and range of mobility were also evaluated. RESULTS: The 11 patients had an average age of 42 years and 54.5% were male. Highly significant improvements in VAS scores at rest and with activity (p<0.001) were evident. There was a significant decrease in rate of medication consumption post-procedure (p<0.05). CONCLUSIONS: PSV can be used successfully as the first-line treatment for well-confined metastatic vertebral lesions. It is also an effective method to decrease pain, increase mobility, and decrease narcotic administration in such patients.

Progresión de plasmocitoma óseo a mieloma múltiple y reporte de un caso / Progression of plasmacytoma of bone to multiple mieloma and a case report

El plasmocitoma óseo solitario y el mieloma múltiple son dos gammapatías monoclonales que pertenecen al mismo grupo de alteraciones neoplásicas de células plasmáticas. En ocasiones el plasmocitoma precede al mieloma. La localización esternal de un plasmocitoma es excepcional. Se reporta el caso de una persona de sexo masculino de 75 años, con dolor moderado en la región dorsal acompañado de disnea de medianos esfuerzos. La tomografía reveló lesiones osteolíticas localizadas en el tercio inferior del esternón, con características radiológicas de un plasmocitoma óseo solitario, además se observan lesiones en la 5ta. costilla izquierda y escápulas. La biopsia de médula ósea evidenció una plasmocitosis medular del 45 % y la inmunoelectroforesis, una concentración de 4210 mg/dl de Inmunoglobulina G. Se hace el diagnóstico de mieloma múltiple y se analizan las alteraciones que lo hicieron evolucionar hasta ese punto con el fin de tener presente la posibilidad de la evolución a mieloma múltiple en aquellos pacientes con plasmocitoma óseo. Se expone el siguiente caso con el objetivo de mejorar la calidad de vida de los pacientes a través del diagnóstico oportuno del plasmocitoma óseo, logrando así evitar la evolución a mieloma múltiple, y resultar en una mayor supervivencia.

Solitary plasmacytoma of bone and multiple myeloma are two monoclonal gammapathies that belong to the same group of neoplastic alterations of plasma cells. The plasmacytoma occasionally precedes myeloma. The sternal location of plasmocytoma is rare. This is the case of a man aged 75 years, with moderate pain in the dorsal region and short of breath when making medium efforts. The axial tomography showed osteolytic lesions in the lower third of sternum with radiological characteristics of a solitary plasmocytoma of bone in addition to lesions in the 5th left rib and scapulas. The bone marrow biopsy revealed 45% medullary plasmocytosis whereas immunoelectrophoresis showed Ig G concentration of 4210 mg/dl. Multiple myeloma was diagnosed and an analysis was made on the alterations leading to this result, with the purpose of bearing in mind the possibility of progression to a multiple myeloma in those patients with plasmocytoma of bone. The following case was presented in order to improve the quality of life of patients through timely diagnosis of plasmocytoma of bone and to avoid progression to multiple myeloma, thus achieving higher survival rates.

Osteofibrous dysplasia of the proximal tibia: an illustrative case report.

A 5 years old girl presented 3 years back with pain and swelling over the upper right leg for 2 years duration. X-ray, CT and MRI revealed osteolytic well defined lesion 2.5 × 2 cm in the meta-diaphyseal region of the proximal tibia with pathological fracture of anterolateral cortex. Child underwent thorough curettage of the lesion and the defect was filled with allograft (iliac crest graft) from mother which was harvested in an adjacent operation theatre. The limb was protected with a plaster splint for a period of 3 months. The graft gradually consolidated and new bone formation was apparent by 6 months. Remodelling of the medullary canal occurred at 1 year follow up. Child has been followed up to 3 years (till date) and there is no evidence of recurrence. The case illustrates that osteofibrous dyplasia can be effectively treated by curettage and replacement of defect by allograft from parents.

Ultrasound-guided percutaneous 125I seed implantation for the treatment of osteolytic metastases:clinical analysis of 18 cases / 介入放射学杂志

Objective To assess the effect of ultrasound-guided percutaneous 125I seed implantation for the treatment of osteolytic metastases. Methods From February 2011 to December 2013, a total of 18 patients with advanced cancer complicated by osteolytic metastases received ultrasound-guided percutaneous implantation of 125I seeds. According to visual analog score (VAS) the pain was estimated before and 3 days as well as one month after the treatment. One month after the treatment follow-up CT scan was performed in all patients to check the tumor size and the distribution of 125I seeds, and the therapy was repeated if necessary. The efficacy was evaluated according to Response Evaluation Criteria in Solid Tumors (RECIST). Results A total of 33 times of procedure were carried out in the 18 patients. Three days after the treatment, local pain was obviously relieved in all cases. Compared with preoperative VAS, the postoperative VAS was significantly improved (P=0.000). The pain was well controlled even one month after the treatment (P=0.000). One month after the last treatment the complete remission (CR) rate was 0% (n=0), partial remission (PR) rate 61.1% (n=11), stable disease (SD) 33.3% (n=6) and progress disease (PD) 5.6% (n=1), and local control rate (CR+PR) was 61.1%. No severe brachytheray-related complications occurred. Conclusion Ultrasound-guided percutaneous 125I seed implantation brachytherapy is technically simple and repeatable, it can safely and effectively guide the performance of percutaneous 125I seed implantation for the treatment of osteolytic metastases. Even in the circumstance of not using TPS, this technique can also obtain satisfactory local control rate and significant pain relief.