Fracturas supracondíleas en pacientes pediátricos tratados quirúrgicamente con Técnica de Cruzada. Hospital Central San Cristóbal / Supracondylar humerus fractures in childrens surgically treated by Crossed Wiring Technique. San Cristóbal Central Hospital

Las fracturas supracondíleas en niños se asocian con algunas complicaciones. Su tratamiento estándar es la reducción cerrada y la fijación con alambres en distintas configuraciones. Se realizó un estudio observacional, analítico, prospectivo y longitudinal con pacientes pediátricos que ingresaron con fracturas supracondíleas de húmero tratados quirúrgicamente con alambres de Kirschner con "Técnica Cruzada" en el Hospital Central San Cristóbal enero a junio de 2022. Se incluyeron 30 pacientes. La edad promedio fue 6,3±2,2(3-10) años. La causa más frecuente del traumatismo fue la precipitación de altura en 63,3% de los casos. La posición del codo al momento del accidente fue en extensión en 90%. Todas las fracturas fueron cerradas. El tiempo promedio desde el accidente hasta su atención en emergencia fue de 8,5±13,4(2-72) horas. Los signos clínicos más frecuentes fueron dolor en 100,0%, limitación funcional en 96,7%, aumento de volumen 73,3% y deformidad 50,0%. Según la clasificación AO la más frecuente fue del tipo 13-M/3. 1 III en el 50% de los casos y según Gartland, las tipo IIIA en 53,3%. A las 4 semanas, 100,0% de las fracturas consolidaron, 13,3% presentó valgo y 6,7% varo en la radiografía anteroposterior. Mientras que, en la lateral, 33,3% antecurvatum. La media del ángulo de Baumann fue de 20,27±1,39 grados. La tasa de complicaciones fue de 16,66%, 2(6,7%) casos presentaron neuropraxia y 3(10,0%) granuloma. En conclusión, la Técnica Cruzada es segura en términos de reducción, funcionalidad y tasas de complicaciones en el seguimiento a medio plazo(AU)

Supracondylar fractures in children are associated with some complications. Its standard treatment is closed reduction and fixation with wires in different configurations. An observational, analytical, prospective and longitudinal study was made, with pediatric patients admitted with supracondylar humeral fractures surgically treated with Kirschner wires with the "Cross Technique" at the Hospital Central San Cristóbal from January to June 2022. 30 patients were included. The mean age was 6,3±2,2(3-10) years. The most frequent cause of trauma was high altitude precipitation in 63,3% of the cases. The position of the elbow at the time of the accident was 90% extended. All fractures were closed. The mean time from the accident to emergency care was 8,5±13,4(2-72) hours. The most frequent clinical signs were pain in 100,0%, functional limitation in 96.7%, volume increase in 73,3%, and deformity in 50,0%. According to the AO classification, the most frequent was type 13-M/3. 1 III in 50% of cases and according to Gartland, type IIIA in 53,3%. At 4 weeks, 100,0% of the patients consolidated, 13,3% presented valgus and 6,7% varus on the anteroposterior radiograph. While, on the side, 33,3% antecurvatum. The mean Baumann angle was 20,27 ± 1.39 degrees. The rate of complications was 16,66%, 2 (6,7%) cases presented neuropraxia and 3 (10,0%) granuloma. In conclusion, the Crossover Technique is safe in terms of reduction, functionality, and complication rates in medium-term follow-up(AU)

Effectiveness of Intra-Arterial Chemotherapy for the Treatment of Intraocular Retinoblastoma: Relevance of a Multidisciplinary Setting.

Purpose: We aim to report about effectiveness and safety in the context of our centers' setting in the management of retinoblastoma with intra-arterial chemotherapy (IAC) in a 5-year retrospective analysis of the Portuguese population. Patients and Methods: Retrospective analysis of consecutive cases of retinoblastoma selected to initiate IAC between 2015 and 2020, at the Portuguese National Reference Center. All included patients underwent complete ophthalmological evaluation under anesthesia with fundus photography. Diagnosis and classification of retinoblastoma was made according to the International Classification of Intraocular Retinoblastoma (ICRB). The patients were further divided into two groups: Group I for primary IAC and Group II for secondary IAC. Tumor recurrence or relapses, systemic metastasis and deaths were documented. Main efficacy outcome included ocular salvage and recurrence-free survival rates estimated using the Kaplan-Meier method. Results: Twenty-eight eyes (19 eyes included in Group I and 9 eyes included in Group II) were eligible and a total of 130 IAC procedures were performed, with a median number of sessions of 4 (range 1-8) for each treated eye, during a median follow-up of 21 months (range 4-64). Of the included eyes, 22 (78.6%) were preserved. An overall survival of 100% was achieved. Considering the preserved eyes, the overall median decimal visual acuity achieved at the last visit was 0.15 (range 0.02-0.8). Three patients had permanent adverse events related to IAC (cataract, vitreous hemorrhage and choroidal ischemia). Considering the survival analysis of recurrence, the mean survival without recurrence was 84.2% for Group I and 66.7% for Group II, and the mean survival without enucleation was 78.6% (no events in Group II). Conclusion: IAC has been shown to be an effective and safe treatment for children with intraocular retinoblastoma. This study demonstrates that IAC is effective even in moderate sample sizes, when a multidisciplinary approach is available.

Kaposi sarcoma in three pediatric liver transplantation recipients.

BACKGROUND: Kaposi sarcoma (KS) is an endothelial cell tumor, rare in children. It is 200 times more frequent after solid organ transplantation than in the general population. METHODS: We report three cases of pediatric patients who developed KS after liver transplantation (LT). RESULTS: Case 1, a 4-year-old boy undergoing LT due to familial intrahepatic cholestasis. Five months after LT, he presented with fever, dyspnea, and cough with enlarged lymph nodes and splenomegaly, anemia, thrombocytopenia, elevated liver enzymes, and positive EBV viral load. Lymph node biopsy diagnosed KS with an elevated HHV8 viral load. Case 2, a 4-year-old boy who underwent LT due to secondary biliary cirrhosis resulting from extrahepatic biliary atresia. Two years later, graft dysfunction was noticed with positive EBV viral load, thrombocytopenia, massive cervical lymph node enlargement, and splenomegaly. Lymph node biopsy diagnosed KS, Castleman's disease, and plasmablastic lymphoma related to HHV8 infection. Case 3, a 15-month-old girl, who received two LT due to biliary cirrhosis. Six months later, she presented with diarrhea, abdominal distension, anemia, thrombocytopenia, enlarged lymph nodes, splenomegaly, and positive CMV viral load. Axillary lymph node biopsy diagnosed KS and HHV8 infection was confirmed. In all three cases, tacrolimus was discontinued and, after diagnosis, sirolimus was started. All recovered without relapse and have a good graft function. CONCLUSIONS: Kaposi sarcoma is a rare disease post-LT in children. Recognizing keywords and early diagnosis is crucial for timely treatment and survival.

Aplicación clínica de la Impresión 3D en el manejo de fracturas abiertas de tibia: Nuevas fronteras en Traumatología. Estudio experimental / Clinical application of 3D Printing in open tibial fractures management: New frontiers of Orthopaedics. An experimental study

La Impresión 3D es una tecnología emergente utilizada cada vez más en medicina. En los países en vías de desarrollo, donde las fracturas por motocicletas y automóviles se encuentran en aumento, la disponibilidad de fijadores externos para el manejo de fracturas abiertas es un problema frecuente. La impresión 3D puede ser una alternativa económica e igualmente confiable a los dispositivos tradicionales elaborados con acero o titanio. El objetivo de este trabajo es mostrar la experiencia con el uso de Impresión 3D y su aplicación en el manejo clínico de fracturas abiertas diafisiarias de tibia. Se realizó un estudio pre-experimental y prospectivo. Se incluyeron 14 pacientes con fracturas de tibia AO/ASIF 42A, 42B y 42C tratados con un fijador externo con rótulas elaboradas con Impresión 3D como medida de Control de Daños en Ortopedia desde su ingreso hasta su resolución definitiva. Todos los pacientes fueron de sexo masculino, con un promedio de edad 23,16 años con 50% entre 20-23 años. Las fracturas fueron 42,85% tipo 42A, 37,71% 42B y 21,42% 42C. El 78,57% de las fracturas fueron ocasionadas por motocicletas: 57,14% grado II según Gustilo y Anderson, un 28,57% grado III y 14,28% grado I. El 37,71% eran politraumatizados. Ninguno de los pacientes presentó complicaciones como pérdida de la reducción, aflojamiento de las rótulas, ruptura o fatiga de las rótulas ni fatiga de la barra. La impresión 3D demostró ser una herramienta y alternativa útil en el manejo agudo de fracturas abiertas diafisiarias de tibia(AU)

3D Printing is an emerging technology used more and more in medicine. In developing countries, where motorcycle and automobile fractures are on the rise, the availability of external fixators for the management of open fractures is a frequent problem. 3D printing can be a cheap and equally reliable alternative to traditional devices made of steel or titanium. The objective of this work is to show the experience with the use of 3D Printing and its application in the clinical management of open diaphyseal fractures of the tibia. A pre-experimental and prospective study was made. 14 patients with AO/ASIF tibia fractures 42A, 42B and 42C treated with an external fixator with 3D-printed ball-caps as a Damage Control measure in Orthopedics from admission to final resolution were included. All patients were male, with an average age of 23,16 years, 50% between 20-23 years. The fractures were 42,85% type 42A, 37,71% 42B and 21,42% 42C. 78,57% of the fractures were caused by motorcycles: 57,14% grade II according to Gustilo and Anderson, 28,57% grade III and 14.28% grade I. 37,71% were polytraumatized. None of the patients had complications such as loss of reduction, loosening of the patellas, rupture or fatigue of the patellas, or rod fatigue. 3D printing proved to be a useful tool and alternative in the acute management of open diaphyseal fractures of the tibia(AU)

Medición radiológica del extremo proximal del fémur / Radiological measurement of the femoral proximal end

El objetivo de este trabajo es determinar las mediciones radiológicas del extremo proximal del fémur en pacientes adultos cuyos casos fueron presentados en las discusiones clínico-radiológicas del servicio de Traumatología y Ortopedia del Hospital Central de San Cristóbal entre 2015 y 2021. Se realizó un estudio prospectivo, descriptivo, transversal, que incluyó los estudios radiológicos de cadera sana en proyección anteroposterior de pelvis centrada en pubis con rotación interna de 15 %, de 126 pacientes entre 20 y 95 años. Se midieron: ángulo cervicodiafisario, lateralización femoral, longitud y ancho del cuello femoral y diámetro de la circunferencia cefálica, mediante el sotfware MicroDicom DICOM 4.0.0. La media de ángulo cervicodiafisario fue 130,8 ± 4,5 grados, en 57 individuos (45,2 %) estuvo entre 127,4 y 133,3 grados (p = 0,001); la circunferencia cefálica media fue 42,0 ± 2,4 mm, en 60 personas (47,6 %) estuvo entre 40,2 y 45,7 mm (p = 0,001); la longitud cervical media fue 78,6 ± 16,4 mm, en 54 individuos (42,9 %) estuvo entre 69,4 y 92 mm (p = 0,001); el ancho cervical medio fue 75,9 ± 12,1 mm, en 64 casos, (50,8 %) estuvo entre 62,9 y 78,7 mm (p = 0,001). En 60 individuos (47,6 %) la lateralización del fémur estuvo entre 92,6 - 117,7 mm. Las medidas son independientes del sexo; a medida que aumenta la edad, el ángulo cervicodiafisario es menor (p= 0,021). Se realizaron tablas percentiladas de las medidas radiológicas del extremo proximal del fémur, según edad y sexo, que pueden servir de referencia en pacientes futuros(AU)

The objective of this work is to determine the radiological measurements of the proximal end of the femur in adult patients whose cases were presented in the clinical-radiological discussions of the Traumatology and Orthopedics service of the Central Hospital of San Cristóbal between 2015 and 2021. A prospective study was carried out, descriptive, crosssectional, which included radiological studies of a healthy hip in anteroposterior projection of the pelvis centered on the pubis with internal rotation of 15%, of 126 patients between 20 and 95 years of age. The following were measured: cervicodiaphyseal angle, femoral lateralization, length and width of the femoral neck, and diameter of the head circumference, using the MicroDicom DICOM 4.0.0 software. The mean neck-diaphyseal angle was 130.8 ± 4.5 degrees, in 57 individuals (45.2%) it was between 127.4 and 133.3 degrees (p = 0.001); the mean head circumference was 42.0 ± 2.4 mm, in 60 people (47.6%) it was between 40.2 and 45.7 mm (p = 0.001); the mean cervical length was 78.6 ± 16.4 mm, in 54 individuals (42.9 %) it was between 69.4 and 92 mm (p = 0.001); the mean cervical width was 75.9 ± 12.1 mm, in 64 cases (50.8%) it was between 62.9 and 78.7 mm (p= 0.001). In 60 individuals (47.6%), the lateralization of the femur was between 92.6 - 117.7 mm. The measurements are independent of sex; as age increases, the cervical shaft angle is less (p= 0.021). Percentage tables of the radiological measurements of the proximal end of the femur were made, according to age and sex, which can serve as a reference in future patients(AU)

Characterization of the Portuguese population diagnosed with retinoblastoma.

The purpose of this study is to characterize demographically and genetically the Portuguese population with retinoblastoma; to report the clinical stage at presentation and its impact on survival and ocular preservation rate and, finally, to assess the incidence of retinoblastoma in Portugal. Retrospective observational study including children consecutively diagnosed with retinoblastoma at the Portuguese National Referral Center of Intraocular Tumors, between October 2015 and October 2020. Twenty-eight children were diagnosed with retinoblastoma at our center, 15 hereditary from which 12 presented with bilateral retinoblastoma and 3 were unilateral. The overall mean age at diagnosis was 13.6 ± 11.1 months with hereditary retinoblastomas diagnosed slightly earlier at 9.6 ± 6.3 months. A familial history of retinoblastoma was found in only 4 (14.3%) of the cases. A pathogenic mutation in the RB1 gene was found in 13 (46.4%) of the children. The most frequent sign at referral was leukocoria in 71.4% of patients. Considering the ICRB classification of the tumors, 84.6% of non-hereditable hereditary retinoblastomas were referred to our center in advanced stages. In the group of hereditable retinoblastomas 86.7% presented with one of the eyes with advanced intraocular retinoblastoma. Fourteen children had one eye enucleated due to retinoblastoma. No deaths were registered during the study period. Considering the incidence analysis, we registered a year-of-birth controlled incidence analysis of 4.04 per 100.000 living births (IC 95% 1.59-6.49). This is the first characterization of the Portuguese Population diagnosed with Retinoblastoma in the National Reference Center.

Erdheim-Chester disease: a rare non-Langerhans histiocytosis.

A 3-year-old girl was admitted to our hospital with diabetes insipidus and a left eye proptosis. During investigation of diabetes insipidus, an extensive osteolytic mass, involving skull base and maxillo-facial bones, was revealed. Biopsy exhibited dense infiltrate of foamy histiocytes, which were positive for CD68 and CD163 and negative for CD1a and S100 confirming histopathological diagnosis of Erdheim-Chester disease. Treatment with dabrafenib was initiated, with good response and no side effects.

Current Practices on Diagnosis, Prevention and Treatment of Post-Transplant Lymphoproliferative Disorder in Pediatric Patients after Solid Organ Transplantation: Results of ERN TransplantChild Healthcare Working Group Survey.

(1) Background: Post-transplant lymphoproliferative disease (PTLD) is a significant complication of solid organ transplantation (SOT). However, there is lack of consensus in PTLD management. Our aim was to establish a present benchmark for comparison between international centers and between various organ transplant systems and modalities; (2) Methods: A cross-sectional questionnaire of relevant PTLD practices in pediatric transplantation was sent to multidisciplinary teams from 17 European center members of ERN TransplantChild to evaluate the centers' approach strategies for diagnosis and treatment and how current practices impact a cross-sectional series of PTLD cases; (3) Results: A total of 34 SOT programs from 13 European centers participated. The decision to start preemptive treatment and its guidance was based on both EBV viremia monitoring plus additional laboratory methods and clinical assessment (61%). Among treatment modalities the most common initial practice at diagnosis was to reduce the immunosuppression (61%). A total of 126 PTLD cases were reported during the period 2012-2016. According to their histopathological classification, monomorphic lesions were the most frequent (46%). Graft rejection after PTLD remission was 33%. Of the total cases diagnosed with PTLD, 88% survived; (4) Conclusions: There is still no consensus on prevention and treatment of PTLD, which implies the need to generate evidence. This might successively allow the development of clinical guidelines.

Treatment of Advanced Retinoblastoma in Children Evacuated from Low-Income Countries: Experience from a National Referral Center in Portugal.

OBJECTIVE: The aim of our study was to characterize the evacuated African patients diagnosed with retinoblastoma and referred to the Portuguese national referral center (Centro Hospital e Universitário de Coimbra, University of Coimbra), identifying inequalities in the stage of diagnosis and prognostic results. DESIGN: Retrospective observational study of evacuated African patients diagnosed with retinoblastoma and referred to the Portuguese National Referral Center (Centro Hospital e Universitário de Coimbra, University of Coimbra). RESULTS: The study included 15 patients between October 2015 and October 2020 from Angola, Cape Verde, Guinea-Bissau and São Tomé and Príncipe. Seven (46.7%) children presented bilateral retinoblastoma. The median age at the time of diagnosis was 20.9 (interquartile range, 16-41) months. The presenting symptoms were leukocoria (86.7%), strabism (53.3%) and buphthalmus (40%). In terms of tumor staging, five (33.3%) children presented with extraocular retinoblastoma and 10 (66.7%) children presented with intraocular retinoblastoma. At presentation, no pineal involvement was diagnosed but two (13.3%) children presented with central nervous system involvement at the time of the first observation. Children were treated with enucleation, exenteration, systemic chemotherapy, intra-arterial chemotherapy and/or supportive palliative care. During the follow-up period (mean 27.2 ± 18.2 months), the overall survival was 73.3%. CONCLUSION: A small proportion of African children are being referred to our center, when considering the expected incidence of retinoblastoma in these countries, and referred children arrive at advanced stages of the disease, compromising treatment outcomes. Considering retinoblastoma is now a curable disease, national and international interventions are required to attempt a better management of children born in low-income countries.

Is cancer latency an outdated concept? Lessons from chronic myeloid leukemia.

Our concept of cancer latency, the interval from when a cancer starts until it is diagnosed, has changed dramatically. A prior widely-used definition was the interval between an exposure to a cancer-causing substance and cancer diagnosis. However, this definition does not accurately reflect current knowledge of how most cancers develop assuming, mostly incorrectly, one exposure is the sole cause of a cancer, ignoring the possibility the cancer being considered would have developed anyway but that the exposure accelerated cancer development and eliding the randomness in when a cancer is diagnosed. We show, using chronic myeloid leukaemia as a model, that defining cancer latency is not as simple as it once seemed. It is difficult or impossible to know at which event or mutation to start to clock to measure cancer latency. It is equally difficult to know when to stop the clock given the stochastic nature of when cancers are diagnosed. Importantly, even in genetically-identical twins with the same driver mutation intervals to develop cancer vary substantially. And we discuss other confonders. Clearly we need a new definition of cancer latency or we need to abandon the concept of cancer latency in the modern era of cancer biology.

Sacral Myeloid Sarcoma Manifesting as Radiculopathy in a Pediatric Patient: An Unusual Form of Myeloid Leukemia Relapse.

Myeloid sarcoma (MS), granulocytic sarcoma or chloroma, is defined as a localized extramedullary mass of blasts of granulocytic lineage with or without maturation, occurring outside the bone marrow. MS can be diagnosed concurrently with acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS). The authors report a case of sacral MS occurring as a relapse of myeloid leukemia in a 5-year-old girl who was taken to the emergency department with radiculopathy symptoms.

Fractura segmentaria abierta IIIa de húmero en paciente lactante: reporte de un caso / IIIa open segmental fracture of the humerus in an infant patient: a case report

Las fracturas diafisiarias de húmero constituyen solo el 3% de todas las fracturas en niños. Las fracturas abiertas de este segmento son poco frecuentes con muy pocos reportes en la literatura. Son el resultado de traumatismos de alta energía ocasionados por accidentes automovilísticos o precipitaciones de gran altura. Las fracturas abiertas en niños menores de 3 años, representa un verdadero reto y requieren un enfoque único y multidisciplinario. Las fracturas abiertas en los niños presentan desafíos especiales, donde deben considerarse las características especiales del hueso del niño que lo diferencian del hueso del adulto. La aplicación de los principios adecuados en el manejo de las fracturas abiertas, así como el establecimiento de protocolos específicos de tratamiento, son necesarios para garantizar una evolución adecuada de esas lesiones. Presentamos el caso de paciente lactante menor masculino, quien fue eyectado del asiento delantero de un vehículo rustico al volcarse éste por una pendiente, posterior a lo cual presenta fractura segmentaria abierta IIIa del húmero derecho. El paciente es llevado a quirófano de emergencia donde se realiza limpieza quirúrgica y la estabilización de la fractura con un mini fijador externo utilizado para el tratamiento de fracturas de muñeca en adultos. Presentamos su tratamiento, complicaciones y evolución a corto plazo.

The humeral shaft fractures accounts for only 3% of all fractures in children. Open fractures of this segment are rare with few reports in the literature. They result from high energy trauma caused by car accidents or precipitation from high altitude. Open fractures in children under 3 years represents a real challenge and requires a unique and multidisciplinary approach. We present male infant patient, who was ejected from the front seat of a rustic vehicle that overturned down a slope and presented an open segmental type IIIa fracture of the right humerus. The patient was taken to the operating room where an emergency surgical cleaning and stabilization of the fracture was performed using a mini external fixator currently utilized for treating adult wrist fractures. We present the treatment, complications and outcomes in the short term.

Enclavado Endomedular Trocantérico versus DHS por técnica mínimamente invasiva en el tratamiento de fracturas extracapsulares de fémur proximal / Intermediate Endomedullary Trochanteric versus DHS by minimally invasive technique in the treatment of extracapsular proximal femoral fractures

Las fracturas extracapsulares de cadera están asociadas a traumatismos de baja energía en ancianos y a traumatismos de alta energía, como los arrollamientos o heridas por arma de fuego, en pacientes jóvenes. El objetivo de este estudio fue comparar los resultados del tratamiento de las fracturas extracapsulares de cadera con dos métodos quirúrgicos. Se realizó un estudio experimental de tipo ensayo clínico controlado, conducido en un centro de IV nivel, diseñado para comparar la efectividad del enclavado endomedular trocantérico y el tornillo deslizante de cadera por técnica MIO en el tratamiento de fracturas extracapsulares de cadera. Se incluyeron pacientes de ambos géneros, mayores de 16 años. El tiempo promedio de seguimiento fue de 8,2 meses. Se incluyeron 120 pacientes, 60 para cada grupo. 65% de sexo femenino y 35% masculino. La edad promedio fue de 79,39 años. El tipo de fractura más frecuente fue la 31A2.1 en 34,16% y la 31A1.2 con 21,66%. El mecanismo de trauma más frecuente fue la caída simple en 85%. El enclavado endomedular trocantérico mostró mejores resultados en cuanto a sangrado operatorio, tiempo exposición al fluoroscopio, duración de la cirugía, y resultados en la escala de Harris y Montoya al final de la valoración (p<0,05). La tasa de complicaciones fue de 24,16%. El enclavado endomedular trocantérico demostró ser superior al tornillo deslizante de cadera por técnica MIO en el tratamiento de estas fracturas(AU)

Extracapsular hip fractures are associated with low-energy trauma in elderly and high-energy trauma, such as coils or gunshot wounds in young patients. The aim of this study was to compare the results of treatment of extracapsular hip fractures with two surgical methods. A controlled clinical trialexperimental study, conducted at a level IV center was made. Designed to compare the effectiveness of proximal femoral nail and sliding hip screw for MIO technique in the treatment of extracapsular hip fractures. Patients of both genders, aged 16 years, were included. The average follow-up time was 8.2 months. We study 120 patients, 60 for each group. 65% were female and 35% male. The average age was 79.39 years. The 31A2.1 and 31A1.2 type of fracture was the most common in 34.16% and 21.66% respectively. The most common mechanism of trauma was simply falling by 85%. The proximal femoral nail showed better results in terms of operative bleeding, fluoroscopy exposure time, duration of surgery, and results on the scale of Harris and Montoya at the end of following (p <0.05). The complication rate was 24.16%. The trochanteric nailing proved superior to the sliding hip screw for MIO technique in the treatment of these fractures(AU)

Angiolipoma intraóseo del calcáneo. Reporte de un caso y revisión de la literatura / Calcaneous intraosseous angiolipoma. A Case Report and literatura review

Los lipomas intraóseos son considerados como los tumores óseos primarios benignos más raros por su muy poca frecuencia. Su incidencia es menor del 1 por 1.000 entre los tumores primarios y para 2002 se habían publicado menos de 100 casos a nivel mundial. El angiolipoma, es una variante histológica del lipoma aún menos frecuente, representando sólo del 5 al 17% de todos los lipomas intraóseos. Generalmente se trata de una lesión asintomática, un hallazgo radiológico en exámenes realizados por otras razones suele ser la forma de su diagnóstico. Sin embargo, algunos lipomas pueden ser sintomáticos. Presentamos el caso de paciente femenino de 36 años quien posterior a presentar dolor del tobillo derecho se diagnostica una lesión en el calcáneo, que posterior a su análisis anatomopatológico resultó ser un angiolipoma intraóseo. Presentamos su tratamiento con médula ósea autóloga y su evolución(AU)

Intraosseous lipomas are considered the rarest and a very infrequently benign primary bone tumors. Its incidence is less than 1 per 1,000 between primary tumors and by 2002 had published fewer than 100 cases worldwide. The angiolipoma, is a histological variant of lipoma even rarer, representing only 5 to 17% of all intraosseous lipomas. This is usually an asymptomatic lesion, radiological finding in tests performed for other reasons is usually the way to diagnosis. However, some may be symptomatic lipomas. We report the case of a female patient aged 36 who after presenting pain in the right calcaneus, which after pathologic analysis proved diagnosed intraosseous angiolipoma. We present treatment with autologous bone marrow and evolution(AU)

Reconstrucción del húmero con aloinjerto masivo tras resección en bloque por sarcoma de Ewing. Evolución a largo plazo. A propósito de un caso / Reconstruction of the humerus with massive allograft after block resection by Ewing's sarcoma. Long-term evolution. A case report

El Sarcoma de Ewing es un tumor óseo primitivo maligno que fue descrito por Ewing en 1921. Representa aproximadamente el 5% de todas las neoplasias malignas del hueso. Tiene el pronóstico menos favorable de todas las neoplasias malignas del hueso. Desde finales de los años 70 se han desarrollado técnicas de salvataje, con la finalidad de preservar la vida, limitar el daño, mantener la funcionalidad en la medida de lo posible y optimizar la calidad de vida evitando al máximo las complicaciones. La reconstrucción del húmero, posterior a resecciones amplias, es un reto. Presentamos el caso de paciente masculino de 7 años, portador de Sarcoma de Ewing en el húmero el cual fue tratado con resección en bloque extensa del húmero y reconstrucción con aloinjerto masivo de tibia. Presentamos su manejo y evolución a largo plazo(AU)

Ewing´s Sarcoma is a malignant bone tumor that was first describe by Ewing in 1921. Represents about 5% of all malignant neoplasms of bone. Have the least favorable prognosis of all malignancies of bone. Since the late 70s salvage techniques have been developed, in order to preserve life, limit damage, maintain functionality as far as possible and optimize quality of life posible with minimal complications. Therefore, the reconstruction of the posterior humeral wide resections still challenging. We present the case of 7 years old male patient, with Ewing´s Sarcoma in the humerus which was treated with wide resection and reconstruction humeral block with massive tibia bone allograft. We present handling and long-term evolution(AU)

Osteosarcoma de fémur en un adulto y manejo con artroplastia no convencional. Reporte de un caso / Femur osteosarcoma in an adult and management with non-conventional arthroplasty. Report of a case

El osteosarcoma es el tumor óseo maligno primario más frecuente. Se caracteriza por la producción de osteoide tumoral (trabéculas óseas inmaduras) por parte de las células neoplásicas. Su mayor incidencia se da en la adolescencia y después de los 65 años. Son más comunes en varones y personas de raza negra. A pesar de su baja incidencia este tipo de lesión requiere una atención multidisciplinaria, diagnostico precoz, y tratamientos oportunos para aumentar la sobrevida y mantener la calidad de vida del paciente. En la actualidad y con los adelantos quirúrgicos-reconstructivos, la resección tumoral con cirugía de preservación del miembro es el tratamiento estándar para esta patología. Presentamos el caso de paciente masculino de 25 años quien presentó un osteosarcoma del fémur distal derecho. Fue tratado con quimioterapia adyuvante y resección en bloque y artroplastia total no convencional de la rodilla. Presentamos los resultados de su tratamiento y su evolución a largo plazo(AU)

Osteosarcoma is the most common primary malignant bone tumor. It's characterized by the production of tumor osteoid (immature bone trabeculae) by the neoplastic cells. His greatest incidence occurs in adolescence and after age 65. They are more common in males and blacks individuals. The low incidence of this type of injury, requires a multidisciplinary care, early diagnosis and appropriate treatments to increase survival and maintain quality of life of patients. Today, with the reconstructive surgical advances, tumor resection with limb-sparing surgery is the standard treatment for this condition. We present a 25 years old male patient who presented an osteosarcoma of the right distal femur. He was treated with adjuvant chemotherapy and en bloc resection and unconventional Total Knee Arthroplasty. We present the results of their treatment and long-term evolution(AU)

[Hodgkin's Lymphoma and Autoimmunity: Is There a Relationship?]. / Linfoma de Hodgkin e Autoimunidade: Existirá uma Relação?

INTRODUCTION: The relationship between lymphomas and autoimmune diseases has been reported as bi-directional, however there is a few data in pediatric population. The aim of this work is to evaluate the prevalence of autoimmune diseases in children and adolescents with Hodgkin's lymphoma followed in a Pediatric Oncology Unit. MATERIAL AND METHODS: By reviewing Hodgkin's lymphomas data from the past 16 years (collected prospectively), an apparently large incidence of autoimmune diseases, mostly in female patients, was noted. We decided to do this retrospective study with an update on follow-up. Data analyzed: age, gender, autoimmune disease, temporal relation with lymphoma, lymphoma's stage, histologic subtype and treatment. RESULTS: Fifty-two cases were included, 7 (13.5%) of which, all female, had an autoimmune disease diagnosed previously, simultaneously or after lymphoma. Autoimmune diseases were: juvenile idiopathic arthritis, inflammatory bowel disease, Behçet's disease, autoimmune hepatitis, systemic erythematosus lupus, Hashimoto's thyroiditis and idiopathic thrombocytopenic purpura. The diagnosis was made after lymphoma in 4 patients, before in 2 and simultaneously in 1 patient. All cases, except the one with simultaneous diagnosis, are out of treatment and without oncologic disease relapse. No deaths were registered. DISCUSSION: There was an important prevalence of autoimmune diseases in girls with Hodgkin's lymphoma. We present data and discuss the possible causes based on a literature review. CONCLUSIONS: This relationship should be invoked, requiring more studies, especially in pediatric age.

Introdução: A relação entre linfomas e doenças autoimunes é descrita na literatura como bidirecional, existindo poucos dados em idade pediátrica. Este trabalho tem como objetivo avaliar a prevalência de doenças autoimunes em crianças e adolescentes com linfoma de Hodgkin seguidos num Serviço de Oncologia Pediátrica. Material e Métodos: Ao rever a casuística do Serviço de linfomas de Hodgkin nos últimos 16 anos (dados colhidos prospetivamente), constatou-se uma incidência aparentemente elevada de doenças autoimunes nas raparigas pelo que se realizou um estudo retrospetivo, com atualização do seguimento fora de tratamento, relativamente à existência de doenças autoimunes. Avaliaram-se: idade, sexo, tipo de doença autoimune, relação temporal com o linfoma, estádio e grupo histológico do linfoma e terapêutica efetuada. Resultados: Incluíram-se 52 casos de linfoma de Hodgkin, dos quais sete (13,5%), todos do sexo feminino, tiveram uma doença autoimune diagnosticada previamente, em simult'neo ou posteriormente ao linfoma. As doenças autoimunes encontradas foram: artrite idiopática juvenil, doença inflamatória intestinal, doença de Behçet, hepatite autoimune, lúpus eritematoso sistémico, tiroidite de Hashimoto e púrpura trombocitopénica idiopática. Em quatro doentes o diagnóstico foi posterior ao linfoma, em dois, prévio, e num simult'neo. Todos os casos, exceto o diagnóstico simult'neo, estão fora de tratamento e sem recidiva da doença oncológica. Não se verificaram óbitos. Discussão: Verificou-se uma importante prevalência de doenças autoimunes nas raparigas com linfoma de Hodgkin. Apresentamos os dados e discutimos possíveis causas desta relação com base numa revisão bibliográfica. Conclusões: Esta associação deve ser evocada, sendo necessário mais estudos, sobretudo em idade pediátrica.

Nueva clasificación para la rodilla flotante en adultos / A new classification for Floating knee in adults

La clasificación de Fraser para la rodilla flotante, ha sido desde su publicación, la más difundida y utilizada a nivel mundial para estadificar esta lesión. En la rodilla flotante, el compromiso intraarticular se ha relacionado con mayor tasa de complicaciones y con los resultados funcionales más pobres. Sin embargo, la clasificación de Fraser, no toma en cuenta el compromiso de los tejidos blandos, lo cual también es importante para determinar el tratamiento, complicaciones y pronóstico. Otra lesión asociada a la rodilla flotante y que ha sido ampliamente documentada, es el compromiso de los ligamentos de la rodilla y las fracturas de rótula. Otros sistemas de clasificación propuestos posteriormente a la clasificación de Fraser, no toman en cuenta estos y otros elementos. Consideramos que una clasificación para la rodilla flotante, debe tomar en cuenta cuatro aspectos fundamentales para su diagnótico, tratamiento y pronóstico: morfología de la lesión, compromiso articular, compromiso de las partes blandas y compromiso de los ligamentos de la rodilla y afectación de la rótula. Con esta investigación proponemos un nuevo sistema de clasificación para la rodilla flotante que toma en cuenta estas variables, y que además, es simple y proporciona importante información para su diagnóstico, tratamiento y pronóstico(AU)

The Fraser classification for floating knee has been since its publication, the most widespread and worldwide used to stage this injury. In the floating knee, intra-articular commitment has been linked to higher rates of complications and poorer functional outcomes. However, the classification of Fraser, does not take into account the commitment of the soft tissues, which is also important in determining treatment, complications and prognosis. Another lesion associated with floating knee and has been widely documented, is the commitment of the ligaments of the knee and patella fractures. Other classification systems subsequently proposed to Fraser classification, do not take into account these and other elements. We think that a classification for floating knee should include four key factor for diagnosis, treatment and prognosis: lesion morphology, joint involvement, commitment soft tissue and commitment of knee ligament and patellar involvement. With this research we propose a new classification system for floating knee that takes into account these variables, and furthermore, is simple and provides important information for diagnosis, treatment and prognosis(AU)

Anaplastic cutaneous lymphoma mimicking an infection.

We present a case of a 17-year-old boy who presented with a skin lesion with extension to the soft tissues of the left thigh. On ultrasound, a homogeneous and hypoechoic expansile formation in the subcutaneous tissue was found, measuring 6.5 × 5 × 3.5 cm, with scarce vascularization. Computed tomography showed a low attenuating neoformation with surrounding edema. An inflammatory disorder was the first diagnosis, but the absence of improvement with antibiotics led us to perform magnetic resonance imaging that showed a high signal lesion on T2-weighted imaging and low intensity signal on T1-weighted imaging and surrounding contrast uptake. Positron emission tomography and computed tomography showed uptake of 18F-fluorodeoxyglucose by the lesion. The final diagnosis was anaplastic cutaneous lymphoma.