Primary intraosseous papillary intralymphatic angioendothelioma of the distal femoral epiphysis: a case report with literature review.

INTRODUCTION: Papillary intralymphatic angioendothelioma (PILA) is an exceptionally rare metastasizing soft tissue tumor. It tends to arise in the subcutaneous tissues of distal extremities in children. Only four intraosseous PILA cases have been reported until now in English language literature. CASE REPORT: We present a case of PILA arising in the distal femoral epiphysis of a 50-year-old female patient. It started as a relentless pain in her left knee. A plain radiography revealed a radiolucent area in the left internal femoral condyle. Computerized tomography revealed a 1-cm lytic lesion with a sclerotic rim. Magnetic resonance images showed a significant bone marrow edema signal focused on a 1-cm subchondral lesion suggestive of an intraarticular osteoid osteoma. Histologically, the tumor contained vascular channels covered by a single endothelial layer with intraluminal papillary endothelial structures lined with hobnail cells. Immunohistochemically, the cells were positive for ERG, CD31, and D2-40. The tumor underwent cryoablation and 6 months later, after local recurrence or tumor persistence, a wide tumor resection was referred. After 7 years of follow-up, the patient displayed neither local recurrence nor distant metastases. CONCLUSION: Primary intraosseous PILAs are exceedingly rare tumors that should be considered in the differential diagnosis of vascular bone tumors.

[Translated article] Oligometastases in oncological orthopaedic surgery. Our experience.

OBJECTIVE: To analyse the incidence and survival of patients with oligometastases (solitary and normal) when they are treated in centres that are experts in multidisciplinary approach to patients with sarcoma. MATERIAL AND METHOD: Retrospective analysis of 414 patients with bone metastases secondary to carcinomas at Hospital Universitario La Paz and Hospital MD Anderson Cancer Centre (Madrid) between May 2006 and May 2019. Metastases located in the pelvis and axial skeleton were excluded, analyzing a total of 28 patients who met the criterion for solitary metastases or oligometastases with normal criteria. The study survival estimate was carried out following the Kaplan-Meier statistical method. RESULTS: The survival of the patients following the oligometastases criteria (solitary and normal) was 53%. Breast cancer was the most prevalent and had a survival rate of more than 70%. The average age of the patients was 58 years old. DISCUSSION: Systemic treatments in cancer treatment have managed to improve disease-free survival curves and lead us to redirect on the paradigm for the treatment of oligometastases, stating that treatment should be carried out in the centres that are experts in the treatment of sarcomas. CONCLUSIONS: The choice of surgical treatment for patients with oligometastases in the strict sense (solitary) and normal should be evaluated by multidisciplinary teams according to the prognoses of the patient, anatomical location and histotype of the neoplasm.

Análisis de los costes de diagnóstico y tratamiento de los sarcomas de partes blandas en centros de referencia / Cost analysis of the diagnosis and treatment of soft tissue sarcomas in reference centres

Objetivo. Conocer los costes diagnósticos y terapéuticos en pacientes derivados a un centro de referencia ante la sospecha de un sarcoma de partes blandas. Material y métodos. Cohorte histórica de 48 pacientes consecutivos diagnosticados y tratados en nuestros centros, tanto previo a la realización de la biopsia como posteriormente a ella, entre los años 2009 y 2011 con el diagnóstico de sarcoma de partes blandas. Los costes por procedimiento son extraídos de la orden de precios públicos del año 2009. Se realizó un estudio comparativo de los datos mediante la prueba de U de Mann-Whitney-Wilcoxon. Resultados. El coste medio por paciente fue de 14.427,58 €. En los derivados previo a la biopsia el coste global medio fue de 11.818,67 € y en los derivados posteriormente fue de 16.456,74 € (p=0,0073). No existen diferencias significativas en el coste diagnóstico entre ambos grupos. Sin embargo, el coste del tratamiento medio por paciente fue mayor en el grupo B (p=0,0121). Discusión. La derivación a los centros de referencias con equipos multidisciplinares expertos en esta afección es un hecho común resaltado en muchos artículos en los que además se demuestra el ahorro económico que esto supone. Conclusiones. En este estudio podemos ver que además de la mejoría en el aspecto asistencial para el paciente cuando se deriva a un centro especializado previo a la biopsia existe un menor coste sanitario (AU)

Objective. To determine the diagnostic and treatment costs in patients referred to a reference centre with a suspected soft tissue sarcoma. Material and methods. The study consisted of a historic cohort of 48 consecutive patients who were diagnosed with soft tissue sarcomas and treated in our centres between the years 2009 and 2011, both prior to and after performing the biopsy. The cost per procedure was taken from the official list of prices published in the year 2009. A comparative study of the data was performed using the Mann-Whitney U and Wilcoxon tests. Results. The mean cost per patient was 14,427.58€. In those referred before the biopsy, the overall mean cost was 11,818.67€, and in those referred afterwards, it was 6,456.74€ (p=.0073). There were no significant differences in the diagnostic costs between the groups. However, the mean cosy of the treatment per patient was higher in the second group (p=.0121). Discussion. The referral to centres with experienced multidisciplinary teams in this disease is a common fact highlighted in many articles, where the financial savings have also been demonstrated. Conclusions. This study shows that, as well as improving the care aspect of the patients when they are referred to a specialist centre prior to the biopsy, there is also a lower health care cost (AU)

Osteosarcomas parostales: resultados infrecuentes / Parosteal osteosarcomas: unusual findings

Objetivo. Estudiar y evaluar los hallazgos poco frecuentes en la historia natural de los osteosarcomas parostales. Los osteosarcomas parostales son tumores bien diferenciados de bajo grado de malignidad. La desdiferenciación en una lesión más agresiva. Material y método. Se revisaron una serie de 7 osteosarcomas parostales tratados en el Hospital la Paz entre 2005 y 2009 (tres fueron osteosarcoma parostales desdiferenciados). Los autores analizaron las características clínicas y radiológicas, la histología, tratamiento y resultados en este grupo de pacientes. Resultados. La distribución por sexos fueron: 5 varones y dos mujeres. La edad media de los pacientes fue: 32,14 años; rango: 24-47 años. Tres tumores de 7 (42,8%) fueron osteosarcomas desdiferenciados. Los tres pacientes recibieron quimioterapia. Un paciente debutó con metástasis pulmonares. Ningún paciente falleció. Conclusiones. Es importante la comprensión que el fenómeno biológico de la desdiferenciación puede ocurrir en los osteosarcomas parostales. El pronóstico, tratamiento y supervivencia cambia radicalmente (AU)

Objective. To study and evaluate the unusual findings in the natural history of parosteal osteosarcomas. Parosteal osteosarcomas are well-differentiated tumours of low grade malignancy, but may dedifferentiate into a more aggressive lesion. Material and methods. We reviewed 7 parosteal osteosarcomas treated in La Paz Hospital between 2005 and 2009 (3 were dedifferentiated parosteal osteosarcomas). The authors analysed the clinical and radiological features, histology, treatment and outcomes in this patient group. Results. There were 5 men and 2 women, with a mean age of 32.14 years, range 24-47 years. Three of seven tumours (42.8%) were dedifferentiated osteosarcomas. All three patients received chemotherapy. One patient presented with pulmonary metastases. No patient died. Conclusions. It is important to understand that the biological phenomenon of dedifferentiation can occur in parosteal osteosarcomas. The prognosis, treatment and survival changes radically (AU)

[Parosteal osteosarcomas: unusual findings]. / Osteosarcomas parostales: resultados infrecuentes.

OBJECTIVE: To study and evaluate the unusual findings in the natural history of parosteal osteosarcomas. Parosteal osteosarcomas are well-differentiated tumours of low grade malignancy, but may dedifferentiate into a more aggressive lesion. MATERIAL AND METHODS: We reviewed 7 parosteal osteosarcomas treated in La Paz Hospital between 2005 and 2009 (3 were dedifferentiated parosteal osteosarcomas). The authors analysed the clinical and radiological features, histology, treatment and outcomes in this patient group. RESULTS: There were 5 men and 2 women, with a mean age of 32.14 years, range 24-47 years. Three of seven tumours (42.8%) were dedifferentiated osteosarcomas. All three patients received chemotherapy. One patient presented with pulmonary metastases. No patient died. CONCLUSIONS: It is important to understand that the biological phenomenon of dedifferentiation can occur in parosteal osteosarcomas. The prognosis, treatment and survival changes radically.

[Cost analysis of the diagnosis and treatment of soft tissue sarcomas in reference centres]. / Análisis de los costes de diagnóstico y tratamiento de los sarcomas de partes blandas en centros de referencia.

OBJECTIVE: To determine the diagnostic and treatment costs in patients referred to a reference centre with a suspected soft tissue sarcoma. MATERIAL AND METHODS: The study consisted of a historic cohort of 48 consecutive patients who were diagnosed with soft tissue sarcomas and treated in our centres between the years 2009 and 2011, both prior to and after performing the biopsy. The cost per procedure was taken from the official list of prices published in the year 2009. A comparative study of the data was performed using the Mann-Whitney U and Wilcoxon tests. RESULTS: The mean cost per patient was 14,427.58€. In those referred before the biopsy, the overall mean cost was 11,818.67€, and in those referred afterwards, it was 6,456.74€ (p=.0073). There were no significant differences in the diagnostic costs between the groups. However, the mean cosy of the treatment per patient was higher in the second group (p=.0121). DISCUSSION: The referral to centres with experienced multidisciplinary teams in this disease is a common fact highlighted in many articles, where the financial savings have also been demonstrated. CONCLUSIONS: This study shows that, as well as improving the care aspect of the patients when they are referred to a specialist centre prior to the biopsy, there is also a lower health care cost.