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.

Primary aneurysmal bone cyst of hands and feet: A series of 14 cases.

Aneurysmal bone cyst (ABC) is a relatively rare, benign bone tumor that occurs exceptionally in the hands and feet. The objective of this article is to present clinical, radiological, histopathological features and management of a series of 14 primary ABC cases in these unusual locations. Eight cases occurred in hands and six in feet. We present the first reported subungual case to occur in the hand. The average age of the patients was 26 years (range 7 to 49), with half being over the age of twenty at diagnosis. The male to female ration was 9:5. In radiological terms, ABC appeared as an expansive lesion with internal septa and without cortical disruption. Twelve cases displayed the classic multicystic morphology and two cases were of the solid variant. "Blue bone" was detected in 50 % of the specimens. Treatment consisted of curettage, excision, or amputation in all cases. Recurrence rate was observed in 35 % of the cases, with the similar ABC morphology as the original samples. New therapeutic options have been proposed on their own or in combination with surgery to reduce local recurrence rates.

Giant thigh glomus tumor of uncertain malignant potential: Case report with pathologic-radiologic correlation.

INTRODUCTION: Glomus tumors (GT) are rare, benign tumors that arise from glomus bodies and usually develop in digital areas. Extradigital GT are exceptional and thigh location is infrequent. CASE REPORT: We report a case of a GT of the thigh in a 79-year-old male patient that measured 9.5 cm in maximum size. The GT lay above the muscular fascia without infiltrating it. Internal hypervascularity was seen by spectral Doppler ultrasound. Magnetic resonance image showed a heterogeneous mass with hyperintense and hypointense components and internal lobes with liquid-liquid levels. Histopathology revealed a monotonous round-cell proliferation with central nuclei without atypia or mitotic figures, around small-caliber vessels. These cells expressed smooth muscle actin and pericellular collagen IV. GT of uncertain malignant potential was diagnosed. The mass was completely removed. The patient did not experience local relapse nor distant metastasis. CONCLUSION: GT are rare soft tissue tumors whose diagnosis of unusual giant masses in uncommon locations may be delayed and misdiagnosed given the low suspicion.

Primary intraosseous alveolar soft part sarcoma: Report of two cases with radiologic-pathologic correlation.

Alveolar soft part sarcoma (ASPS) accounts for less than 1 % of all soft tissue sarcomas. ASPS presents a poor prognosis and develops frequent metastases, especially in the lungs, brain and bones. Current therapies, such as surgery, radiotherapy and chemotherapy, are not fully effective and other alternative treatments are currently being studied. ASPS is predominantly found in the deep soft tissues of the lower extremities. To our knowledge, only thirteen primary intraosseous ASPS have been reported in the literature. In this study, we report two new cases of this exceedingly rare entity. Both cases already had multiple metastases since diagnosis; one of them represents the first case of a primary bone ASPS in the ulna and is also the primary intraosseous ASPS with the longest reported case of survival, after having maintained long periods of stabilization despite not having received any systemic treatment.

Calcifying aponeurotic fibroma: Radiologic-pathologic analysis of ten cases and review of the literature.

Calcifying aponeurotic fibroma (CAF) is a very rare benign entity that principally affects the volar fascia, tendons, and aponeuroses of the hands and feet with a peak incidence of between 5 and 15 years, although there have been cases found for a wide age range and at various anatomical sites. We present ten CAF cases; consisting of eight children and two adults. CAF occurred in the extremities in nine of the cases and in the chest wall in one case. CAF ultrasound and radiological findings are nonspecific but may help orientate diagnosis. Magnetic resonance imaging should be performed when there are doubtful cases, when occurring in nontypical sites, and when there are cases of nontypical clinical presentation. Histologically, all cases showed two components, a fibromatosis-like component and a nodular component. Chondroid areas were present in five cases. Calcifications were observed in nine cases. ERG immunostaining showed the same patterns in all the cases; diffuse positivity in pericalcified areas, and patchy positivity in areas away from calcifications. CAF has distinctive histopathological features which should aid in the differential diagnoses with other entities.

[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.

La aplicación informática «SCAE» en el diagnóstico precoz del cáncer musculoesquelético en el área sanitaria del Hospital Universitario 12 de Octubre. Análisis de su efectividad y propuestas de mejora / The computer application «SCAE» in the early diagnosis of musculoskeletal cancer in the healthcare area of the Hospital Universitario 12 de Octubre. Analysis of its effectiveness and proposals for improvement

Introducción: La aplicación informática SCAE-SM (Solicitud de Cita en Atención Especializada-Sospecha de Malignidad) es una herramienta informática de la que disponen los médicos de Atención Primaria (AP) para la derivación de pacientes que deban ser valorados por el especialista en un plazo máximo de 2 semanas, cuando se sospeche una patología maligna. El objetivo de nuestro trabajo fue analizar la utilidad de esta herramienta y proponer áreas de mejora en la gestión de los pacientes con sospecha de malignidad musculoesquelética. Material y métodos: Se realizó un estudio descriptivo transversal de las 235 derivaciones recibidas en los años 2012-2017. Se analizó su procedencia, la información contenida en las solicitudes y la respuesta proporcionada por evaluadores históricos (facultativos traumatólogos sin formación específica oncológica). Para este estudio, se ha realizado una nueva valoración ciega de todas las solicitudes por 13 traumatólogos con distinto nivel de formación específica en oncología musculoesquelética (reevaluadores). Resultados: De entre todas las SCAE-SM, solo el 8,23% de los pacientes presentaron enfermedad maligna o benigna agresiva. Los reevaluadores más acertados en la adecuación del adelanto de cita fueron aquellos con formación oncológica moderada (5-10 años de experiencia). Durante el periodo de tiempo del estudio, de todos los pacientes tratados en la Unidad de Tumores, solo el 18,81% accedieron por el circuito SCAE-SM, transcurriendo un tiempo medio de espera de 18,11 días desde la derivación de AP. Conclusiones: La aplicación informática SCAE-SM como herramienta de gestión y adelanto de la asistencia a los pacientes con patología tumoral musculoesquelética maligna es útil, si bien el uso del circuito es inadecuado. Es necesario difundirlo y generalizarlo, así como implementar programas de formación oncológica básica tanto en el ámbito de la AP como de la Hospitalaria.(AU)

Introduction: The SCAE-SM (Request for an Appointment in Specialized Care-Suspicion of Malignancy) computer application is a tool available to Primary Care (PC) physicians for the referral of patients who should be evaluated by the specialist in a maximum period of 2 weeks when malignancy is suspected. The objective of our work was to analyze the usefulness of this tool and propose areas for improvement in the management of patients with suspected musculoskeletal malignancy. Material and methods: A descriptive cross-sectional study of 235 referrals received in the years 2012–2017 was carried out. Their origin, the information contained in the applications and the response provided by historical evaluators, without specific oncology training, were analyzed. For this study, a new blind assessment of all applications was carried out by 13 orthopedists with different levels of specific training in musculoskeletal oncology (re-evaluators). Results: Among all SCAE-SM, only 8.23% of patients had aggressive benign or malignant disease. The most successful re-evaluators in the adequacy of early appointment were those with moderate oncological training (5–10 years of experience). During the study, of all the patients treated in the Tumor Unit, only 18.81% accessed through the SCAE-SM circuit, with a mean waiting time of 18.11 days from the PC referral. Conclusions: The SCAE-SM computer application as tool for improve the management and advance care for patients with malignant musculoskeletal tumor pathology is useful, although the use of the circuit is inadequate. It is necessary to disseminate and generalize it, as well as to implement basic oncology training programs both in the field of PC and Hospital.(AU)

The computer application “SCAE” in the early diagnosis of musculoskeletal cancer in the healthcare area of the “Hospital Universitario 12 de Octubre”. Analysis of its effectiveness and proposals for improvement / La aplicación informática«SCAE» en el diagnóstico precoz del cáncer musculoesquelético en el área sanitaria del Hospital Universitario 12 de Octubre. Análisis de su efectividad y propuestas de mejora

Introduction: The SCAE-SM (Request for an Appointment in Specialized Care-Suspicion of Malignancy) computer application is a tool available to Primary Care (PC) physicians for the referral of patients who should be evaluated by the specialist in a maximum period of 2 weeks when malignancy is suspected. The objective of our work was to analyze the usefulness of this tool and propose areas for improvement in the management of patients with suspected musculoskeletal malignancy. Material and methods: A descriptive cross-sectional study of 235 referrals received in the years 2012–2017 was carried out. Their origin, the information contained in the applications and the response provided by historical evaluators, without specific oncology training, were analyzed. For this study, a new blind assessment of all applications was carried out by 13 orthopedists with different levels of specific training in musculoskeletal oncology (re-evaluators). Results: Among all SCAE-SM, only 8.23% of patients had aggressive benign or malignant disease. The most successful re-evaluators in the adequacy of early appointment were those with moderate oncological training (5–10 years of experience). During the study, of all the patients treated in the Tumor Unit, only 18.81% accessed through the SCAE-SM circuit, with a mean waiting time of 18.11 days from the PC referral. Conclusions: The SCAE-SM computer application as tool for improve the management and advance care for patients with malignant musculoskeletal tumor pathology is useful, although the use of the circuit is inadequate. It is necessary to disseminate and generalize it, as well as to implement basic oncology training programs both in the field of PC and Hospital.(AU)

Introducción: La aplicación informática SCAE-SM (Solicitud de Cita en Atención Especializada-Sospecha de Malignidad) es una herramienta informática de la que disponen los médicos de Atención Primaria (AP) para la derivación de pacientes que deban ser valorados por el especialista en un plazo máximo de 2 semanas, cuando se sospeche una patología maligna. El objetivo de nuestro trabajo fue analizar la utilidad de esta herramienta y proponer áreas de mejora en la gestión de los pacientes con sospecha de malignidad musculoesquelética. Material y métodos: Se realizó un estudio descriptivo transversal de las 235 derivaciones recibidas en los años 2012-2017. Se analizó su procedencia, la información contenida en las solicitudes y la respuesta proporcionada por evaluadores históricos (facultativos traumatólogos sin formación específica oncológica). Para este estudio, se ha realizado una nueva valoración ciega de todas las solicitudes por 13 traumatólogos con distinto nivel de formación específica en oncología musculoesquelética (reevaluadores). Resultados: De entre todas las SCAE-SM, solo el 8,23% de los pacientes presentaron enfermedad maligna o benigna agresiva. Los reevaluadores más acertados en la adecuación del adelanto de cita fueron aquellos con formación oncológica moderada (5-10 años de experiencia). Durante el periodo de tiempo del estudio, de todos los pacientes tratados en la Unidad de Tumores, solo el 18,81% accedieron por el circuito SCAE-SM, transcurriendo un tiempo medio de espera de 18,11 días desde la derivación de AP. Conclusiones: La aplicación informática SCAE-SM como herramienta de gestión y adelanto de la asistencia a los pacientes con patología tumoral musculoesquelética maligna es útil, si bien el uso del circuito es inadecuado. Es necesario difundirlo y generalizarlo, así como implementar programas de formación oncológica básica tanto en el ámbito de la AP como de la Hospitalaria.(AU)

SELNET clinical practice guidelines for bone sarcoma.

Bone sarcoma are infrequent diseases, representing < 0.2% of all adult neoplasms. A multidisciplinary management within reference centers for sarcoma, with discussion of the diagnostic and therapeutic strategies within an expert multidisciplinary tumour board, is essential for these patients, given its heterogeneity and low frequency. This approach leads to an improvement in patient's outcome, as demonstrated in several studies. The Sarcoma European Latin-American Network (SELNET), aims to improve clinical outcome in sarcoma care, with a special focus in Latin-American countries. These Clinical Practice Guidelines (CPG) have been developed and agreed by a multidisciplinary expert group (including medical and radiation oncologist, surgical oncologist, orthopaedic surgeons, radiologist, pathologist, molecular biologist and representatives of patients advocacy groups) of the SELNET consortium, and are conceived to provide the standard approach to diagnosis, treatment and follow-up of bone sarcoma patients in the Latin-American context.

The computer application «SCAE¼ in the early diagnosis of musculoskeletal cancer in the healthcare area of the Hospital Universitario 12 de Octubre. Analysis of its effectiveness and proposals for improvement. / La aplicación informática «SCAE¼ en el diagnóstico precoz del cáncer musculoesquelético en el área sanitaria del Hospital Universitario 12 de Octubre. Análisis de su efectividad y propuestas de mejora.

INTRODUCTION: The SCAE-SM (Request for an Appointment in Specialized Care-Suspicion of Malignancy) computer application is a tool available to Primary Care (PC) physicians for the referral of patients who should be evaluated by the specialist in a maximum period of 2 weeks when malignancy is suspected. The objective of our work was to analyze the usefulness of this tool and propose areas for improvement in the management of patients with suspected musculoskeletal malignancy. MATERIAL AND METHODS: A descriptive cross-sectional study of 235 referrals received in the years 2012-2017 was carried out. Their origin, the information contained in the applications and the response provided by historical evaluators, without specific oncology training, were analyzed. For this study, a new blind assessment of all applications was carried out by 13 orthopedists with different levels of specific training in musculoskeletal oncology (re-evaluators). RESULTS: Among all SCAE-SM, only 8.23% of patients had aggressive benign or malignant disease. The most successful re-evaluators in the adequacy of early appointment were those with moderate oncological training (5-10 years of experience). During the study, of all the patients treated in the Tumor Unit, only 18.81% accessed through the SCAE-SM circuit, with a mean waiting time of 18.11 days from the PC referral. CONCLUSIONS: The SCAE-SM computer application as tool for improve the management and advance care for patients with malignant musculoskeletal tumor pathology is useful, although the use of the circuit is inadequate. It is necessary to disseminate and generalize it, as well as to implement basic oncology training programs both in the field of PC and Hospital.

[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.

[Percutaneous radiofrequency ablation of benign bone tumors: osteoid osteoma, osteoblastoma, and chondroblastoma]. / Tratamiento mediante radiofrecuencia percutánea de los tumores óseos benignos: osteoma osteoide, osteoblastoma y condroblastoma.

We report our experience in the computed tomography (CT)-guided percutaneous radiofrequency ablation of more than 100 benign bone tumors in the last eight years. We affirm that this should be the technique of choice in the vast majority of osteoid osteomas; it can also be applied as a definitive treatment in many cases of osteoblastomas or chondroblastomas as well as in less common benign bone tumors. CT-guided percutaneous radiofrequency ablation has proven highly efficacious and has resulted in very few complications; thus, patients tend to recover very quickly.

Late development of a malignant fibrous histiocytoma at the site of a giant cell tumor.

A case of late development of a high grade malignant fibrous histiocytoma at the site of a previously surgically treated giant cell tumor is reported. The patient initially was treated for a benign giant cell tumor of the lateral condyle of the distal femur by curettage, implant of auto and allograft bone, but no radiation. Eighteen years later, he noted progressively increasing pain and disability. Radiographs showed some change, but magnetic resonance imaging clearly disclosed a tumor arising at the site and extending outside the bone. After chemotherapy, the tumor was resected and histologically showed no evidence of a recurrent giant cell tumor, only a high grade malignant fibrous histiocytoma.