Update of cartilaginous tumours according to the WHO classification 2020.

Cartilaginous tumours are a large and heterogeneous group of neoplasms characterised by the presence of a chondroid matrix, with lobular growth and arcuate, ring-like or popcorn-like calcification patterns. MRI shows hyperintensity in T2-weighted sequences and a lobulated or septal relief in postcontrast images. In the WHO 2020 classification, chondral tumours are classified as benign, intermediate or malignant. Despite technological advances, they continue to pose a challenge for both the radiologist and the pathologist, being the main difficulty the differentiation between benign and malignant tumours, which is why they require a multidisciplinary approach. This paper describes the main changes introduced in the 2020 update, describes the imaging characteristics of the main cartilaginous tumours and provides the radiological keys to differentiate between benign and malignant tumours.

Actualización de los tumores cartilaginosos según la clasificación de la OMS de 2020 / Update of cartilaginous tumours according to the WHO classification 2020

Los tumores cartilaginosos son un grupo amplio y heterogéneo de neoplasias caracterizadas por la presencia de una matriz condroide que presenta crecimiento lobular y patrones de calcificación en arcos y anillos o en palomitas de maíz. En RM destaca su hiperintensidad en las secuencias potenciadas en T2, y en las imágenes poscontraste, un relace lobulado o septal. En la clasificación de 2020 de la OMS, los tumores de estirpe condral se clasifican en benignos, intermedios o malignos. A pesar de los avances tecnológicos, siguen suponiendo un reto tanto para el radiólogo como para el patólogo, siendo la principal dificultad la diferenciación entre los tumores benignos y malignos, razón por la que requieren un abordaje multidisciplinar. Este trabajo recoge los principales cambios introducidos en la actualización de 2020, describe las características de imagen de los principales tumores cartilaginosos y proporciona las claves radiológicas para diferenciar entre tumores benignos y malignos.(AU)

Cartilaginous tumours are a large and heterogeneous group of neoplasms characterised by the presence of a chondroid matrix, with lobular growth and arcuate, ring-like or popcorn-like calcification patterns. MRI shows hyperintensity in T2-weighted sequences and a lobulated or septal relief in postcontrast images. In the WHO 2020 classification, chondral tumours are classified as benign, intermediate or malignant. Despite technological advances, they continue to pose a challenge for both the radiologist and the pathologist, being the main difficulty the differentiation between benign and malignant tumours, which is why they require a multidisciplinary approach. This paper describes the main changes introduced in the 2020 update, describes the imaging characteristics of the main cartilaginous tumours and provides the radiological keys to differentiate between benign and malignant tumours.(AU)

Encondroma osificado en fémur. Una localización atípica. A propósito de un caso / Ossified enchondroma in femur. An atypical location. Case study

Presentamos el caso de un hombre de 61 años en el que se halló de forma incidental un encondroma a nivel proximal de fémur. Tras seguimiento, se observó la estabilidad de la lesión. El encondroma es un tumor de origen cartilaginoso benigno. Se localiza principalmente en las falanges de las manos y los pies, aunque puede observarse en húmero o fémur. Por lo general, es una lesión asintomática y se halla de manera incidental. En las radiografías suelen verse lesiones redondeadas bien definidas con radiolucidez central. Se recomienda el seguimiento con radiografías seriadas para comprobar la progresión o estabilidad de la lesión y hacer su diagnóstico diferencial con el condrosarcoma.(AU)

We report the case of a 61-year-old man in whom an enchondroma was incidentally detected at the proximal level of the femur. After follow-up the lesion was stable. Enchondroma is a benign cartilaginous tumor. It is located mainly in the phalanges of the hands and feet, although it can be seen in the humerus or femur. It is usually an asymptomatic lesion, found incidentally. On x-rays, well-defined round lesions with central radiolucency are usually observed. Follow-up with serial x-rays is recommended to verify the lesion’s course or stability and make its differential diagnosis with chondrosarcoma.(AU)

Adelgazamiento cortical y área tumoral. ¿Predictores de fractura patológica en encondromas de falanges de manos? / Cortical thinning and tumoral area. Predictors of pathological fracture in enchondromas of phalanges of the hand?

Introducción y objetivo: Los encondromas son los tumores primarios más comunes en las falanges de las manos. Cuando son descubiertos en pacientes asintomáticos, el cirujano debe decidir entre tratamiento quirúrgico o actitud expectante. Hasta la fecha, no se dispone de indicadores radiológicos establecidos que determinen el riesgo de fractura patológica. Pretendemos conocer la utilidad de dos parámetros de radiografía simple, adelgazamiento cortical y área ósea ocupada por tumor, como predictores de fractura patológica. Material y método: Estudio retrospectivo, observacional y analítico, de casos y controles, realizado entre 2003 y 2017 con 18 pacientes operados por encondromas en falanges de manos, 9 de ellos asintomáticos (grupo A, control), y otros 9 con fractura patológica como debut (grupo B, casos). Con el objetivo de identificar los parámetros radiológicos asociados con fractura, analizamos sus radiografías preoperatorias anteroposteriores y laterales. Cuantificamos el área de hueso ocupada por tumor y el grado de adelgazamiento cortical en ambos grupos. Resultados: No encontramos diferencias significativas respecto al área de hueso ocupada por tumor (p > 0.1). Sin embargo, en el grupo B evidenciamos, de manera estadísticamente significativa, un mayor grado de adelgazamiento cortical (p < 0.1).La probabilidad de desarrollar fractura patológica se incrementa en falanges con mayor adelgazamiento cortical, tanto en radiografías anteroposteriores [Odds Ratio (OR) 16, Intervalo de Confianza (IC) 90% 1.97-130.24] (p=0.01) como en laterales (OR 7, IC 90% 1.21-40.62) (p=0.03). Conclusiones: De nuestro estudio deducimos la asociación positiva entre adelgazamiento cortical y desarrollo de fractura patológica en falanges de manos afectadas por encondromas. (AU)

Background and objective: Enchondromas are the most common primary tumors involving phalanges of the hand. When they are identified in asymptomatic patients, surgeons must decide between surgery or watchful waiting. Till the date, there is no established radiological parameter for determining the risk of pathological fracture.Our study aims to determine the utility of two radiographic indicators, amount of cortical thinning and bone area occupied by tumor, as predictors of pathological fracture. Methods: Retrospective, observational and analytic, case-control study, conducted between 2003 and 2017. Eighteen patients with enchondromas of phalanges of the hand were operated on; 9 of them were asymptomatic (control, group A), and in the other 9 cases (group B), pathological fracture was the first presenting symptom of disease. In order to identify radiological parameters associated with pathological fracture, we analyzed their anteroposterior and lateral preoperative radiographs. We measured the bone area occupied by tumor, and the amount of cortical thinning, in both groups.Results: There was no statistically significant difference between the two groups with respect to bone area occupied by tumor (p > 0.1). Nevertheless, the amount of cortical thinning was significantly higher in group B (p < 0.1).The probability of developing a pathological fracture was increased in bones with greater cortical thinning. This was observed in anteroposterior radiographs [Odds Ratio (OR) 16, Confidence Interval (CI) 90% 1.97-130.24] (p=0.01) and in lateral radiographs (OR 7, CI 90% 1.21-40.62) (p=0.03). Conclusions: Our study suggests a positive association between cortical thinning and development of pathological fracture in hand phalanges affected by enchondromas. (AU)

Encondroma protuberans de falange en paciente pediátrico / Enchondroma protruberans of phalanx in pediatric patient

El encondroma protuberans (EP) es una forma rara de encondroma que muestra un crecimiento exofítico, fuera de los márgenes del hueso cortical. Se presenta el caso de una paciente de 4 años previamente sana que asistió en el servicio de urgencia con un aumento de volumen no doloroso en meñique izquierdo. La radiografía mostró una lesión radiolúcida expansiva en la falange proximal. Se complementa estudio con tomografía computarizada y resonancia magnética descubriéndose una lesión que comprometía el canal medular, con extensión más allá de la cortical y un aumento de intensidad de señal en secuencia T2 sugestiva de matriz condroide. El paciente fue sometido a escisión quirúrgica, curetaje e injerto óseo. El estudio histopatológico postquirúrgico confirmó el diagnóstico de encondroma. En este estudio se describió un caso de EP que involucra una falange de la mano. El estudio imagenológico, en particular las imágenes por resonancia magnética juegan un papel clave para un diagnóstico preciso previo al procedimiento quirúrgico.

Enchondroma protruberans (EP) is a rare form of enchondroma with exophytic growth outside the margins of cortical bone. We present the case of a previously healthy 4-year-old patient who attended the Emergency Department with painless enlargement of the left pinkie. The X-ray showed an expansive radiolucent lesion of the proximal phalanx. Further study with a computed tomography scan and magnetic resonance imaging revealed a lesion affecting the medullary cavity and extending beyond the cortical bone with an increase in signal strength in the T2 sequence suggestive of a chondroid matrix. The patient underwent surgical excision, curettage, and bone grafting. The post-surgical histopathological study confirmed the diagnosis of enchondroma. This study describes a case of EP involving a phalanx of the hand. The images, particularly those of the magnetic resonance imaging, played a key role in reaching an accurate diagnosis prior to surgery.

Enchondromas of the Hand: A 20-year Experience / Encondromas da mão: Uma experiência de 20 anos

Abstract Objective Enchondromas are the commonest tumors of the bones of the hand. Treatment approaches vary. The present article presents the characteristics of the tumors, diagnostic methods, and treatments. Methods We discuss the approach used in our institution, where we have treated 48 patients with enchondromas of the hand between 1996 and 2016. Our technique of treatment, which has remained the same over 2 decades, comprises the use of curettage, high-speed burr, and autologous bone graft (harvested with a minimally invasive technique, using a Craig biopsy needle). Results Pain and fractures were the most common symptoms leading the patients to consultation, at frequencies of 33.3% and 31.3%, respectively. A total of 27.1% of the cases were asymptomatic, and their lesions were discovered incidentally. The mean age was 34.4 years (SD = 12.9 years). Tumors were more frequently presented in the ulnar side of the hand, in the fifth ray (41.5%) and in the proximal bones (in the proximal phalanges [43.8%], and in the metacarpal [33.3%]). The size of the tumors ranged from 0.2 cm2 to 5.7 cm2, with a mean of 1.7 cm2 (standard deviation [SD] = 1.0 cm2) and were not associated with fracture (p = 0.291). Fracture was also not associated with any of the symptoms, neither with the age of the patients (p = 0.964). After the treatment, most patients achieved full range of motion (91.7%), with good integration of the bone graft. Three patients presented deficit in range of motion (6.3%) and the incidence of complications was also 6.3% (3 patients). At the end, after the needed surgical revisions, these three patients also recovered full function. They achieved full bone graft integration, regained full range of motion and returned to work. There was no tumor recurrence case during the follow-up period evaluated. For all cases, no donor site complications occurred. Conclusion Our method of treatment has consistently provided good outcomes, with only a few minor complications. Therapeutic level of evidence: IV.

Resumo Objetivo Os encondromas são os tumores mais comuns dos ossos da mão, com uma grande variedade de abordagens terapêuticas. O presente artigo apresenta as características dos tumores, métodos diagnósticos e tratamentos. Métodos Discutimos a abordagem da nossa instituição, onde tratamos 48 pacientes com encondromas da mão, entre 1996 e 2016. Nossa técnica de tratamento, que permanece a mesma ao longo de duas décadas, compreende o uso de curetagem, esmeril de velocidade e enxerto ósseo autólogo (retirado com uma técnica minimamente invasiva, usando uma agulha de Craig). Resultados A dor e as fraturas foram os sintomas mais comuns, levando os pacientes à consulta, nas frequências de 33,3% e 31,3%, respectivamente. Um total de 27,1% dos casos era assintomático, e suas lesões foram descobertas incidentalmente. A média de idade foi de 34,4 anos (desvio padrão [DP] = 12,9 anos). Os tumores foram mais frequentemente encontrados no lado ulnar da mão, no quinto raio (41,5%), e nos ossos proximais (nas falanges proximais [43,8%] e no metacarpo [33,3%]). O tamanho do tumor variou de 0,2 cm2 a 5,7 cm2, com média de 1,7 cm2 (DP = 1,0 cm2) e não foi associado à fratura (p = 0,291). A fratura também não foi associada a nenhum dos sintomas, e nem à idade dos pacientes (p = 0,964). Após o tratamento, a maioria dos pacientes alcançou amplitude completa de movimento (91,7%), com boa integração do enxerto ósseo. Três pacientes apresentaram déficit no arco de movimento (6,3%) e a incidência de complicações também foi de 6,3% (3 pacientes). No final, após as revisões cirúrgicas necessárias, esses três pacientes também recuperaram a função completa. Eles tiveram a integração total do enxerto ósseo, recuperaram toda a amplitude de movimento e retornaram ao trabalho. Não houve nenhum caso de recorrência do tumor durante o período de acompanhamento avaliado. Em nenhum dos casos ocorreram complicações no local doador. Conclusão O nosso método de tratamento forneceu consistentemente bons resultados, com apenas algumas complicações menores. Nível terapêutico de evidência: IV.

Doble primario sincrónico: condrosarcoma y sarcoma fusocelular de alto grado asociado a síndrome de Maffucci / Synchronous primary double: condrosarcoma and sarcoma high grade fusocelular associated with Maffucci syndrome

Resumen: El síndrome de Maffucci se caracteriza por la presencia de múltiples encondromas y hemangiomas que pueden afectar tejidos blandos y otros órganos. El riesgo de transformación maligna de las lesiones es de 100% durante la vida del individuo, siendo el condrosarcoma el tumor maligno más frecuentemente asociado. Se presenta el caso de un hombre de 44 años de edad con diagnóstico de síndrome de Maffucci, el cual desarrolló un doble primario sincrónico: condrosarcoma y sarcoma fusocelular de alto grado multicéntrico de región escapular y tricipital, fue tratado con desarticulación interescapulotorácica, mostró progresión acelerada y enfermedad pulmonar. Existen otras neoplasias asociadas al síndrome de Maffucci tales como adenocarcinoma de páncreas, tumores mesenquimales de ovario, gliomas, astrocitomas y tumores de la pituitaria. Por lo que resulta muy interesante reportar la asociación infrecuente entre un sarcoma fusocelular y un condrosarcoma secundario en pacientes con síndrome de Maffucci. El seguimiento en este grupo de pacientes es complejo y se basa en la búsqueda intencionada de lesiones con crecimiento acelerado, prestando atención en lesiones de crecimiento progresivo, síntomas clínicos o datos radiológicos de malignidad.

Abstract: Maffucci syndrome is characterized by the presence of multiple enchondromes and hemangiomas that can affect soft tissues and other organs. The risk of malignant transformation of lesions is 100% during the life of the individual, with chondrosarcoma being the most frequently associated malignant tumor. We present the case of a 44-year-old man diagnosed with Maffucci syndrome who developed a synchronous double primary: chondrosarcoma and high-grade multicenter fusocellular sarcoma of scapular and tricipital region, was treated with disarticulation interscapule-thoracic, presented accelerated progression and lung disease. There are other neoplasms associated with Maffucci syndrome, such as pancreatic adenocarcinoma, mesenchymal ovarian tumors, gliomas, astrocytomas and pituitary tumors. It is therefore very interesting to report the uncommon association between fusocellular sarcoma and secondary chondrosarcoma in patients with Maffucci syndrome. Follow-up in this group of patients is complex and is based on the intentional search for accelerated growing lesions, paying attention to progressive growth injuries, clinical symptoms or radiological malignancy data.

Encondroma solitario de la mano: seguimiento a largo plazo después del tratamiento quirúrgico. serie de casos / Isolated enchondroma of the hand. long-term follow-up after surgical treatment. case series

Introducción: los encondromas solitarios son los tumores óseos más comunes de la mano. Aproximadamente el 40% compromete esta extremidad, con predilección por los rayos cubitales y las falanges proximales. El riesgo de transformación maligna a condrosarcoma es del 1% y el de recurrencia, del 2-15%. Se describen la experiencia y los resultados de un importante número de pacientes con un seguimiento a largo plazo. Materiales y Métodos: Estudio descriptivo, retrospectivo, de tipo serie de casos. Se seleccionaron pacientes con diagnóstico confirmado de encondroma solitario de la mano, que fueron manejados mediante resección con curetaje del tumor más aplicación de injertos autólogos de cresta ilíaca o radio. Después del tratamiento quirúrgico, se evaluaron la función, mediante la clasificación de Takigawa, los resultados radiográficos y la frecuencia de complicaciones o recidivas. Resultados: Se incluyeron 19 pacientes con un seguimiento posoperatorio promedio de 11 años. De acuerdo con la clasificación de Takigawa, la función fue excelente en 16 pacientes (84,2%) y buena en 3 (15,7%). Un paciente presentó una infección del sitio operatorio incisional superficial, con resolución completa. No hubo recidivas. Conclusiones: En los pacientes evaluados, los resultados funcionales y radiológicos fueron buenos después de la cirugía. Los encondromas plantean el riesgo de fracturas patológicas, recidiva y, en menor proporción, de transformación maligna; sin embargo, esto no ocurrió en ninguno de los casos. Se describe una opción de manejo quirúrgico, sencilla con buenos y excelentes resultados a largo plazo.

Introduction: Isolated enchondromas are the most common tumors of the hand. Approximately 40% affect this upper extremity, with preference for cubital rays and proximal phalanges. Risk of malignant transformation to chondrosarcoma is 1% and the risk of recurrence ranges from 2 to 15%. Our experience and results with a large number of patients with a long-term follow-up are described. Methods: Descriptive, retrospective, case series study. Patients with diagnosis of isolated enchondroma of the hand that were treated with tumor resection, curettage and iliac crest or radius autograft placement were included. After surgical treatment, hand function was assessed using the Takigawa classification, radiological results and frequency of complications or recurrence were evaluated. Results: Nineteen patients with a mean follow-up of 11 years were included. According to the Takigawa classification, function was excellent in 16 patients (84.2%) and good in 3 (15.7%). One patient had an incisional superficial surgical site infection that resolved completely. No cases of recurrence were identified. Conclusions: Good functional and radiological results were obtained after surgical treatment. Enchondromas can pose the risk of pathological fractures, recurrence and, in a lesser degree, malignant transformation; however, none of these complications were identified. The results of a simple surgical treatment option with good and excellent long-term results are described.

Encondroma solitario de fémur / Femoral solitary enchondroma

El encondroma solitario es un tumor cartilaginoso de naturaleza benigna, habitualmente asintomático, que se presenta entre la tercera y cuarta década de la vida. Se reporta a continuación un caso de una mujer de 60 años con encondroma solitario en fémur izquierdo, confirmando su diagnóstico mediante una resonancia nuclear magnética.

Solitary enchondroma is a benign cartilaginous tumor, usually asymptomatic, presents between the 3 and 4 decade. We report a 60 years old woman with solitary enchondroma in left femur, confirming the diagnosis with magnetic resonance imaging.