Leiomiosarcoma primario de hueso en fémur distal metastásico a clavícula: reporte de un caso / Primary leiomyosarcoma of bone in the distal femur with clavicle metastasis: a case report

El leiomiosarcoma (LMS) es un tipo de tumor de células fusiformes de muy baja incidencia, que tiene un comportamiento agresivo, con alta tasa de mortalidad, por lo que el manejo debe ser quirúrgico, con una resección amplia de la lesión. No está claro el papel de la radio ni de la quimioterapia en su manejo. Presentamos a una paciente de 28 años que consultó por dolor de 2 meses en la rodilla derecha. Radiográficamente, se caracterizó como una lesión osteolítica pura en el fémur distal. La resonancia nuclear magnética (RNM) contrastada mostró áreas hipervasculares dentro del tumor. La gammagrafía mostró un marcado aumento en la captación de radiotrazadores. Se tomó una biopsia, con un informe de patología de LMS óseo bien diferenciado. Se trató con 3 ciclos de quimioterapia neoadyuvante preoperatoria con ifosfamida 1.000 mg/m2 en los días 1 a 3, además de doxorrubicina 70 mg/m2 , y resección quirúrgica de la lesión y salvamento de la extremidad con endoprótesis de rodilla. Una vez que se resecó la lesión, la paciente recibió quimioterapia adyuvante con 4 ciclos de gencitabina 1.000 mg/m2 entre los días 1 y 8, y doxetacel 70 mg/m2 el día 1. Durante los dos meses de seguimiento, la paciente presenóa una fractura en el tercio medio de la clavícula, compatible con una lesión patológica en radiografías y tomografía por emisión de positrones (TEP). La biopsia reveló una lesión metastásica de LMS óseo que fue tratada mediante resección quirúrgica de la clavícula. Este es un caso único, dado que, durante el seguimiento, recibió tratamiento adyuvante con quimioterapia y se evaluó con una TEP, con una evolución clínica satisfactoria y sin evidencia de nuevas lesiones

Leiomyosarcoma (LMS) is a type of spindle-cell tumor of very low incidence that tumor has an aggressive behavior, with high mortality rates; therefore, its management must be surgical, with a wide resection of the lesion. The role of radio and chemotherapy in its management is not clear. We present the case of a 28-year-old female patient who consulted for pain lasting 2 months in the right knee. Radiographically, it was characterized as a pure osteolytic lesion in the distal femur. Contrast magnetic resonance imaging (MRI) showed hypervascular areas within the tumor. The scintigraphy showed a marked increase in radiotracer uptake. A biopsy was taken, with a pathology report of well-differentiated osseous LMS. It was treated with 3 cycles of preoperative neoadjuvant chemotherapy with ifosfamide 1,000 mg/m2 in the first 3 days, as well as doxorubicin 70 mg/m2 , and surgical resection of the lesion and limb salvage with knee endoprosthesis. Once the lesion was resected, the patient underwent adjuvant chemotherapy, with 4 cycles of gencitabine 1,000 mg/m2 between days 1 and 8, and doxetacel 70 mg/m2 on day 1. During the 2-month follow-up, the patient presented a fracture in the middle third of the clavicle, which was compatible with a pathological lesion on radiographs and positron-emission tomography (PET) scans. The biopsy showed a metastatic lesion of bone LMS, which was treated by surgical resection of the clavicle. This is a unique case, given that, during the follow-up, the patient underwent adjuvant treatment with chemotherapy, and was evaluated with a PET scan, with a satisfactory clinical evolution and no evidence of new lesions.

Calcified fibrous pseudotumor with Castleman disease

Simultaneous calcified fibrous pseudotumor (CFT) and Castleman disease (CD) is an extremely rare association. CD is an uncommon lymphoproliferative disease that can arise in various sites of the body, while CFT is a rare type of benign fibrous lesion that frequently affects children and young adults, occurring as solitary or multiple lesions throughout the human body. Both entities are rare and exhibit typical and diverse histomorphological features. We report the case of a 15-year-old female patient, who, at the age of 13 had a biopsy performed at an external medical center; however, after 4 months the lesion had regrown. This lesion was removed with a surgical operation; however, it regrew 2 years later and was removed a third time. The results of the latter two biopsies were the same: CFT accompanying CD. The histologic examination of the excised lymph node and the surrounding tissue showed hyalinized fibrous tissue containing dystrophic and psammomatous calcification. In this case, the hyaline vascular type of CD was found to be intertwined with a CFT, which hampered the differentiation of whether both entities emerged within the lymph node or if the CFT developed from the soft tissue and then involved the lymph node. Future studies involving larger case series will provide a more precise insight, which should serve to resolve the current uncertainty.

Claviculectomía total como procedimiento de rescate: Reporte de un caso y revisión bibliográfica / Total claviculectomy as a rescue procedure. Case report and literature review

La seudoartrosis de clavícula es una causa infrecuente de síndrome del opérculo torácico. Se describe un caso de plexopatía braquial por seudoartrosis previamente infectada de clavícula. La paciente fue sometida a una claviculectomía total para aliviar los síntomas. Del análisis de la literatura citada, se concluye en que la claviculectomía total es una opción por considerar si se han agotado las opciones terapéuticas. Los resultados funcionales pueden ser satisfactorios cuando se la indica por infección, plexopatía o sarcoma. Nivel de Evidencia: IV

Clavicle non-union is an uncommon cause of thoracic outlet syndrome. A case of brachial plexopathy by previously infected clavicle pseudarthrosis is described. Total claviculectomy was performed for symptom relief. From literature analysis we conclude that total claviculectomy is an option to be taken into account when therapeutic options have exhausted. Functional results could be adequate when total claviculectomy is indicated in cases of infection, plexopathy or sarcoma. Level of Evidence: IV

Acortamiento relativo de clavícula en fracturas pediátricas: Su importancia en la decisión del tratamiento conservador / Relative clavicle shortening in pediatric fractures: Its importance when selecting conservative treatment

Antecedentes: Las fracturas de clavícula representan de 2 a 15% de las fracturas pediátricas. La literatura sugiere operar pacientes mayores de 9 años con acortamiento mayor o severa conminución en el trazo de fractura, pero nadie establece parámetros de acortamiento o angulación para el tratamiento conservador en niños. En pacientes pediátricos es importante tomar en cuenta el acortamiento relativo, es decir, aquel provocado por una fractura, ajustado a la longitud sana. Objetivo: Conocer la afectación del movimiento con relación al acortamiento relativo de clavícula en fracturas pediátricas tratadas de forma conservadora. Material y métodos: Estudio retrospectivo, longitudinal y observacional. Medimos radiográficamente el acortamiento relativo de la clavícula fracturada comparándola con el lado sano al momento de la fractura y una vez consolidada. El acortamiento se expresó en porcentajes. Las proyecciones utilizadas fueron: anteroposterior comparativa de hombros y panorámica con ambos hombros. Los pacientes se dividieron en 2 grupos: menores de 9 años y de 9 a 15 años. Resultados: Analizamos 94 fracturas, 31 mujeres y 63 hombres. No se presentó seudoartrosis o consolidación viciosa sintomática. El pronóstico fue bueno sin importar el porcentaje de acortamiento inicial. La edad y el acortamiento tienen una relación proporcional al periodo de rehabilitación y restitución de los arcos de movilidad sin molestias. Los rangos de valores registrados para acortamiento fueron de 9.5 a 28%. Conclusiones: Las indicaciones quirúrgicas por acortamiento para fracturas de clavícula pediátrica deben revisarse. Hemos observado buenos resultados a pesar del acortamiento mayor y desplazamiento total.

Background: Clavicle fractures represent 2-15% of pediatric fractures. The literature suggests operating on patients over 9 years of age with major shortening or severe comminution in the fracture line. However, no one establishes shortening or angulation parameters for conservative treatment in children. In pediatric patients it is important to take into account relative shortening, that is, the one caused by a fracture when compared with the length of the healthy clavicle. Objective: To know the effect of relative clavicle shortening on movement in pediatric fractures treated conservatively. Material and methods: Retrospective, longitudinal, observational study. We radiographically measured the relative shortening of the fractured clavicle comparing it with the healthy side at the time of the fracture and after fracture healing. Shortening was expressed in percentages. The following views were used: comparative anteroposterior view of the shoulders and panoramic view of both shoulders. Patients were divided into 2 groups: under 9 years and 9-15 years of age. Results: We analyzed 94 fractures; 31 in females and 63 in males. No pseudoarthrosis or symptomatic malunion occurred. The prognosis was good regardless of the initial shortening percentage. Age and shortening are proportionally related with the rehabilitation period and the restoration of painless ranges of motion. The value ranges recorded for shortening were 9.5 to 28%. Conclusions: The surgical indications for shortening resulting from pediatric clavicle fractures should be revised. We have observed good results despite major shortening and total displacement.

Imaging Findings Of Desmoplastic Fibroma Rarely Involving The Clavicle: Case Report

Desmoplastic fibroma of bone is a rare locally aggressive, but non-metastatic tumor. In this case report, we present a desmoplastic fibroma in an unusual location, the clavicle. Desmoplastic fibroma involving the clavicle is extremely rare, with only 2 reported cases before 1985. We report the imaging findings of a desmoplastic fibroma of the clavicle with a review of the relevant literature.

Osteosarcoma of the Clavicle in an Adolescent Child.

Clavicle is an unusual site for any primary bone tumour, including osteogenic sarcoma. Although a rare site of affection, most clavicular tumours tend to be malignant. We present a case report of osteosarcoma of the clavicle in a twelve-year-old male child who presented with a huge swelling of the left clavicle for the last six months.

Pseudartrose congênita de clavícula / Congenital pseudarthrosis of the clavicle

A pseudartrose congênita de clavícula (PCC) é uma afecção rara, que representa um distúrbio na união dos núcleos de ossificação e que pode ser diagnosticada ao nascimento. É mais comum em meninas e do lado direito. Este trabalho tem por objetivo proceder a uma revisão bibliográfica sobre o tema, em pesquisa realizada nas bases de dados LILACS e MEDLINE. Foram encontrados 56 artigos publicados até a corrente data. Apesar de pouco frequente, a PCC não deve ser desconhecida ou mesmo esquecida, especialmente como diagnóstico diferencial com a fratura aguda da clavícula por distocia de parto e/ou tocotraumatismo. O diagnóstico é relativamente fácil e o tratamento pode ser apenas expectante ou até mesmo cirúrgico.

Congenital pseudarthrosis of the clavicle (PCC) is a rare affection, that can be diagnosed at birth and represent a disturbance of union of the ossification centers. It's more common in girls and in the right side. This study objectives to proceed a revision about the subject, that was searched in online database of LILACS and MEDLINE. We found 56 articles till present data. Besides be a bit infrequent, the PCC must not be missed or even forgotten, especially as differential diagnosis with acute fracture of the clavicle at birth by trauma in the childbirth. The diagnostic is relatively easy and the treatment can be just observation or even surgical.

Cytological Diagnosis of Ewing Sarcoma – Clavicle.

Ewing sarcoma is a rare malignant round cell tumor of the bone. It is the second most common primary malignant tumor of bone found in children. It commonly affects long bones, pelvis and ribs. An 11 year old boy presented with painful swelling at the medial end of left clavicle. X-ray revealed a diaphyseal lesion of the medial end of the left clavicle with destruction of bone and mottled appearance. FNAC was done and reported as Malignant round cell tumor suggestive of Ewing sarcoma. Histopathologically the diagnosis of Ewing sarcoma was confirmed with special stains. Ewing sarcoma affecting clavicle is uncommon. Clavicle can be often resected with no need of reconstruction in young children.

Doença de Gorham da escápula e clavícula: relato de caso e evolução de dois anos de uma afecção rara / Gorham's disease of scapula and clavicle: case report and two-year follow-up of a rare disorder

A doença de Gorham é uma rara desordem osteolítica, de etiologia controversa, que pode afetar qualquer osso. O substrato histopatológico é a substituição óssea por uma formação expansiva de natureza vascular agressiva, não neoplásica. Descrevemos os achados radiográficos, tomográficos e de ressonância magnética de um caso desta afecção acometendo a escápula esquerda e, dois anos depois, a clavícula ipsilateral.

Gorham's disease is a rare osteolytic disorder of still controversial etiology that may affect any bone. The histopathological substrate for such a condition is the replacement of normal bone by aggressive non-neoplastic expansile vascular tissue. The authors describe radiographic, computed tomography and magnetic resonance imaging findings in a case of this entity initially affecting the left scapula and, two years later, the ipsilateral clavicle.

Cleidocranial dysplasia- literature review and an evidence based clinical report

Cleidocranial dysplasia is an autosomal dominant disorder affecting the craniofacial region and clavicles along with other skeletal abnormalities. The Dental surgeon, is usually the first professional whom patients look for to solve the problem, since there is a delay in the eruption or an absence of permanent teeth. This peculiar case has been reported along with literature review to focus on the unusual findings so that the dental professionals become familiar with such features on confronting subjects with Cleidocranial Dysplasia

Pseudoartrose congênita da clavícula / Congenital pseudartrosis of the clavicle

A pseudoartrose congênita da clavícula é uma doença rara de etiologia desconhecida. Trata-se de uma patologia não familiar, em geral unilateral, com alta predominância pelo lado direito e pelo sexo masculino. Foi descrita inicialmente por Fitzwillians em 1910. Existem até o momento aproximadamente 200 relatos de caso na literatura. O diagnóstico diferencial inclui a pseudoartrose pós-traumática (tocotraumatismo), a disostose cleidocranial, a osteogênese imperfeita e a neurofibromatose. Os autores apresentam um paciente, masculino, de 28 dias de vida, com uma massa indolor situada no terço médio da clavícula direita.

Congenital pseudartrosis of the clavicle is a rare disease of unknown etiology. It is a non-familial disorder which is usually unilateral, with a high prevalence for the right side and males. It was first reported by Fitzwillians in 1910. There are up to now about 200 case reports in the literature. The differential diagnosis includes post-traumatic pseudartrosis (birth trauma), cleidocranial disostosis, imperfect osteogenesis, and neurofibromatosis. The authors report the case of a 28-day old male patient with a painless mass at the medium third of the right clavicle.

[Primary synovialosarcoma: a case of unusual location]

Report a new case of primary Synovi alosarcoma. We report a case of a 16 year old girl, who presented with a tumefaction on the right clavicle evolving or 3 months. Various explorations carried out, in particular thoracic scanner and imagery by magnetic resonance, showed a multilocular cystic formation of the seat and the clavicle reaching the mediastinum. A pathological examination of the part [piece] highlighted a biphasic synovialosarcoma of grade III. This diagnosis was validated by the immunohistochemistry study and the detection of transcribed specific fusion of the synovialosarcoma, SYT-SSXI. The unusual locations of the primitive SS must be known because the diversity of their microscopic aspects may cause confusion in the diagnosis. The immunohistochemistry, and more recently the cytogenetic studies, helps to solve these problems of differential diagnosis

Free fibular graft in treatment of congenital pseudarthrosis of the clavicle [case report]

Congenital pseudarthrosis of the clavicle is a rare entity, a little more than one hundred cases have been reported in the English literature [1, 2, 3]. This article describe a 7 year-old boy presented with a painless lump in the right clavicular region. Clinical, radiological and histological studies proved to be congenital pseudarthrosis of the clavicle. A well- developed right cervical rib was observed. The case was treated by excision of the pseudarthrosis, a free fibular graft and fixation with a controured 1/3 tubular. AO plate. Solid union was achieved at 5 months postoperatively. Histological examination of the excised ends of the pseudarthrosis showed enchondral ossWcation which supports the hyposthesis that the pseudarthrosis resultsfromfailedfusion of two ossification centers. The final result was satisfactory from the functional and cosmetic points of view after five years of follow-up and there were no signs of complications

Etude epidemiologique; clinique et therapeutique des fractures de la clavicule chez l'enfant de (0 a 15 ans) dans le service d'orthopedie et traumatologie de l'hopital Gabriel Toure (HGT)

Nous avons rapporte les resultats d'une etude de 60 cas de fractures de la clavicule chez l'enfant (0 - 15 ans) dans le service de chirurgie orthopedique et traumatologique de l'Hopital Gabriel TOURE de Bamako sur une periode de 9 mois. Les garcons sont plus touches que les filles. Les accidents de la voie publique constituent l'etiologie la plus frequente. Les fractures tiers moyen de la clavicule sont les plus retrouvees. Le traitement orthopedique a donne de tres bons resultats. Aucun traitement chirurgical n'a ete effectue

Punção aspirativa por agulha fina de cisto ósseo aneurismático: relato de caso / Fine needle aspiration cytology of aneurysmal bone cyst: case report

O diagnóstico de tumores ósseos deve ser realizado baseado na correlação dos dados clínicos, radiológicos e anatomopatológicos. Antes do tratamento, é freqüentemente necessário um diagnóstico morfológico. A citologia pode ajudar a estabelecer um diagnóstico presuntivo antes da cirurgia. É um método tecnicamente simples e rápido, que fornece valiosas informações suplementares aos dados clínico-radiológicos. Esse trabalho relata um caso de cisto ósseo aneurismático diagnosticado por punção aspirativa por agulha fina. As vantagens e limitações do método são discutidas.

Cleidectomía total / Total claviculectomy

La clavícula cumple cinco funciones: 1) actuar como puntal; 2) sostener las inserciones musculares y ligamentos; 3) proteger el paquete vasculo-nervioso; 4) transmitir la fuerza de sostén del trapecio y 5) contribuir al aspecto estético del cuello. A pesar de ello, es posible efectuar la cleidectomía total sin alterar la función del hombro si se realiza una buena reparación de los dos grupos musculares que en ella se insertan, trapecio con deltoides y esternocleidomastoideo con pectoral mayor. Las secuelas que dejan son mínimas. Los estudios realizados muestran una buena función del hombro, quedando sólo una leve debilidad en la abducción y la flexión. Se describe la técnica quirúrgica