Update in Imaging Evaluation of Bone and Soft Tissue Sarcomas / Atualização na avaliação por imagens dos sarcomas ósseos e das partes moles

Abstract The evolution in imaging evaluation of musculoskeletal sarcomas contributed to a significant improvement in the prognosis and survival of patients with these neoplasms. The precise characterization of these lesions, using the most appropriate imaging modalities to each clinical condition presented, is of paramount importance in the design of the therapeutic approach to be instituted, with a direct impact on clinical outcomes. The present article seeks to update the reader regarding imaging methodologies in the context of local and systemic evaluation of bone sarcomas and soft tissues.

Resumo A evolução na avaliação por imagens dos sarcomas musculoesqueléticos contribuiu para melhora significativa no prognóstico e na sobrevida dos portadores destas neoplasias. A caracterização precisa destas lesões, mediante utilização das modalidades de imagem mais adequadas a cada condição clínica apresentada, é de suma importância no delineamento da abordagem terapêutica a ser instituída, com impacto direto sobre os desfechos clínicos. O presente artigo busca atualizar o leitor a propósito das metodologias de imagem no contexto da avaliação local e sistêmica dos sarcomas ósseos e das partes moles.

Condrosarcoma de la pelvis en paciente joven / Pelvic chondrosarcoma in young patient

Introducción. Los sarcomas de la pelvis ósea constituyen un subgrupo muy pequeño dentro de la nosología tumoral ósea. El Condrosarcoma, frecuente a partir de la cuarta década de la vida, es la segunda neoplasia ósea más frecuente de los tumores óseos primarios malignos y su localización pélvica constituye un verdadero reto para los cirujanos actuantes. Objetivo. Reportar un caso de Condrosarcoma pélvico en edad temprana y revisar la literatura acerca del tema. Caso clínico. Paciente femenina de 19 años con antecedentes de salud que acude a consulta por dificultad al caminar y aumento de volumen en cadera derecha y dolor asociado. Luego de estudios radiológicos correspondientes, se interviene quirúrgicamente con diagnóstico presuntivo de Condrosarcoma que se corrobora a posteriori por histopatología. Conclusiones. El Condrosarcoma pélvico es una lesión de difícil abordaje quirúrgico. Su pronóstico depende de la posibilidad de exéresis amplia y del grado histológico del tumor. Para su tratamiento curativo se requiere habitualmente de intervenciones extensas, en su mayoría, mutilantes. Su escasa respuesta a la radioterapia y la quimioterapia hacen de la cirugía el arma fundamental para su resolución. (AU)

Introduction. Sarcomas of the bony pelvis constitute a very small subgroup within bone tumor nosology. Chondrosarcoma, common from the fourth decade of life, is the second most frequent bone neoplasm of malignant primary bone tumors and its pelvic locationconstitutes a real challenge for acting surgeons. Objective. To report a case of pelvic hondrosarcoma at an early age and to review the literature on the subject. Clinical case. 19-year-old female patient with a medical history who came to the clinic due to difficulty walking and increased volume in the right hip and associated pain. After corresponding radiological studies, he underwent surgery with a presumptive diagnosis of hondrosarcoma that is corroborated by histopathology. Conclusions. Pelvic chondrosarcoma is a lesion with a difficult surgical approach. Its prognosis depends on the possibility of extensive excision and the histological grade of the tumor. Its curative treatment usually requires extensive interventions, mostly mutilating. Its poor response to radiotherapy and chemotherapy make surgery the fundamental weapon for its resolution. (AU)

Osteoblastoma in the Distal Humerus of a Cat.

A 7-year-old neutered male domestic shorthair cat was presented with chronic lameness in the right forelimb. A cystic bony lesion was identified in the distal right humerus and amputation was performed. The epiphyseal trabecular bones of the capitulum and trochlea was replaced by a tan to pink, expansile mass that was surrounded by a thin rim of cortical bone. Microscopically, the tumour was composed of a bland, osteoid producing spindle cell population within a well-vascularized fibrous stroma. Radiographical and histological features were consistent with osteoblastoma. Osteoblastoma and the related osteoid osteoma are uncommon, benign osteoblastic tumours that are reported rarely in animals. These tumours should be considered as differential diagnoses for slow growing, cystic bony lesions in cats.

Occult phosphaturic mesenchymal tumour of femur cortex causing oncogenic osteomalacia - diagnostic challenges and clinical outcomes.

BACKGROUND: Tumor induced osteomalacia (TIO) are extremely rare paraneoplastic syndrome with less than 300 reported cases. This report highlights the pitfalls and challenges in diagnosing and localizing TIO in patients with refractory and resistant osteomalacia. PATIENT AND METHODS: 41- year gentleman with 4-year history of musculoskeletal weakness and pathologic fractures presented in wheelchair bound incapacitated state of 1-year duration. Investigations were significant for severe hypophosphatemia, severe phosphaturia, normal serum calcium, reduced 1,25-dihydroxy vitamin-D, elevated ALP, elevated intact parathyroid hormone (iPTH), and pseudo-fractures involving pelvis and bilateral femur. Whole body MRI and 99mTc methylene diphosphonate bone-scan were also normal. Whole body FDG-PET scan involving all 4 limbs revealed a small FDG avid lesion at lateral border of lower end of left femur (SUV max 3.9), which was well characterized on 3-dimensional CT reconstruction. Plasma C-terminal fibroblast growth factor (FGF)-23 was 698 RU/ mL (normal < 150 RU/ml). Wide surgical excision of the tumor was done. Histopathology confirmed mesenchymal tumor of mixed connective tissue variant. Serum phosphorous normalized post-surgery day-1. High dose oral calcium and vitamin-D was continued. FGF-23 normalized post surgery (73RU/ml). Physical strength improved significantly and now he is able to walk independently. CONCLUSION: TIO is frequently confused with normocalcemic hyperparathyroidism and vitamin-D resistant rickets/osteomalacia, which increases patient morbidity. Imaging for tumor localization should involve whole body from head to tip of digits, cause these tumors are notoriously small and frequently involve digits of hands and legs. Complete surgical removal of the localized tumor is key to good clinical outcomes.

Osteopoikilosis found incidentally in a 17-year-old adolescent with femoral shaft fracture: A case report.

RATIONALE: Osteopoikilosis is a rare and asymptomatic disease of the bone, which is often discovered occasionally on radiography for irrelevant complaints. Characterized by multiple, small, circular, or oval-shaped radiodense lesions, it may be confused with bony metastatic tumors. PATIENT CONCERNS: The present study describes a case of a 17-year-old adolescent who suffered from pain and movement limitation of his left thigh following a fall from standing height. DIAGNOSES: Plain radiographs showed spiral fracture in left femoral shaft; besides, multiple scattered sclerotic lesions of variable size were also observed over the bilateral proximal femurs, left distal femur, proximal tibia, and distal tibia and fibula through X-rays, computed tomography, and magnetic resonance imaging. The patient was finally diagnosed with left femoral shaft fracture and osteopoikilosis. INTERVENTIONS: The patient underwent reduction and internal fixation with intramedullary nail a week after injury. OUTCOMES: The patient was discharged without any complications 12 days after the surgery. At the 3-month follow-up, the patient recovered well and remained symptom-free with no changes to his sclerotic lesions. LESSONS: Although this case is not so complicated, we have to be cautious when differentiating osteopoikilosis and bony metastases in clinical practice in future, which should avoid causing undue distress to both the patients and doctors.

Condrosarcoma pulmonar metastásico: descripción clínica y revisión de la literatura / Metastatic Pulmonary Chondrosarcoma: Clinical Description and Literature Review

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Benign bone tumors.

Benign bone lesions are a broad category that demonstrates a spectrum of activities from latent to aggressive. Differentiating the various tumors is important in order to properly determine necessary intervention. This chapter focuses on the presentation, imaging, diagnostic features, and treatment of the most common benign bone tumors in order to help guide diagnosis and management.

Primary osseous tumors of the spine.

Primary osseous spinal tumors are relatively rare in comparison with metastatic disease, myeloma, and lymphoma. Despite their rarity, the interpreting radiologist must be aware of the typical imaging features to provide appropriate diagnosis for guidance of clinical management. The age of occurrence, distribution longitudinally in the spine, and distribution axially within the vertebra combined with typical imaging appearances can help indicate the correct diagnosis. This article reviews the diagnostic features of benign and malignant primary bone spinal tumors.

Associations of single nucleotide polymorphisms with malignant and borderline bone tumors.

Bone neoplasms - are a rare group of diseases, which ethiology and pathogenesis are not fully understood. We have studied 6 single nucleotide polymorphisms rs792/(GHI), rs7956547(IGFI), rs3761243(GNRH2), rs11737764(FGF2), rs6599400(FGFR3), and rs1690916(MDM2) associations with bone tumors. In our work we've detected significant associations with some single nucleotide polymorphisms: IGFl.rs7956547, GNRH2.rs3761243 and FGFR3.rs6599400 in patients with malignant and borderline bone tumors.

Tratamiento mediante aloinjertos óseos estructurales en resecciones por tumores óseos de huesos largos. Revisión de 37 casos / Treatment using structural bone allografts in long bone tumour resections. A review of 37 cases

Los aloinjertos óseos estructurales han supuesto una alternativa al tratamiento de los tumores óseos de miembros, con posibilidad de cirugía de conservación del mismo. Presentamos un estudio retrospectivo observacional del manejo de los aloinjertos óseos estructurales en tumores óseos de huesos largos en nuestro hospital, durante los años 1993 a 2010, en el que obtenemos una muestra de 37 pacientes subsidiarios de esta técnica quirúrgica. Mediante la obtención de datos clínicos de la muestra aplicamos las escalas de funcionalidad de Mankin y EVACOM HUVA con resultados excelentes, muy buenos o buenos del 84%, y con los datos radiológicos aplicamos la escala de osteointegración ISOLS con un 95,6% de resultados excelentes a los 24 meses. Estos resultados nos muestran que los aloinjertos óseos estructurales constituyen una técnica válida y reproducible en pacientes con tumores óseos destructivos de huesos largos (AU)

Structural bone allografts have become an alternative in the treatment of limb bone tumours with a chance of limb-saving surgery. We present an observational retrospective study on the use of structural bone allografts in bone tumours of the long bones in our hospital between January 1993 and January 2010, with a sample of 37 patients subjected to this surgical technique. After obtaining clinical information from our sample we applied the Mankin and EVACOM HUVA functional scales with excellent, very good and good results in 84%, and with the radiological information we applied the ISOLS osseointegration scale, with 95.6% of excellent results after 24 months. These results demonstrate that structural bone allografts are a valid and reproducible technique in patients with destructive long bone tumours (AU)

Ossifying fibromyxoid tumour: a rare soft tissue tumour of intermediate malignancy.

Ossifying fibromyxoid tumours (OFTs) are uncommon soft tissue tumours. A 73-year-old male presented with a painless exophytic mass located in the left scapular area which was identified 1 year earlier. An incisional biopsy was performed and microscopic features were compatible with OFT. Radiologic evaluation revealed multiple pulmonary metastasis. Although many cases persue an indolent biologic behaviour, local recurrence and metastasis have been reported and long-term follow-up should be considered.

Papel de la radioterapia en tumores óseos benignos de células gigantes / Role of radiotherapy for benign giant cell tumors of bone

El papel de la radioterapia (RT) en los tumores de células gigantes es incierto. El tratamiento estándar es la cirugía, y el uso de adyuvancia es controvertido. Tras la presentación de un caso clínico se analiza la indicación de la RT en estos tumores y se lleva a cabo una revisión retrospectiva, con historias clínicas de pacientes diagnosticados en el Hospital General Universitario Gregorio Marañón con informe de tumor de células gigantes, análisis y discusión de la literatura. En los tumores de células gigantes en los que éstos se presentan en localizaciones difíciles de realizar una resección amplia, la RT debe ser el tratamiento estándar posterior a la biopsia, alcanzando excelentes tasas de respuesta local. La RT de rescate tras recidiva proporciona una alta tasa de control local sin añadir una morbilidad importante, por lo que debería indicarse como tratamiento en los casos mencionados o como tratamiento paliativo(AU)

The rol of the radiotherapy (RT) in the giant-cell tumor is unknown. The standart treatment is surgery and the use of adjuvant therapy is controversial. After a presentation of a clinical case, the indication of the RT is analyzed in these tumors, and a retrospective review is carried out with patients clinical histories diagnosed in the Hospital General Universitario Gregorio Marañón with report of tumor of giant cells, with analysis and discussion of the literature. In giant cells tumors in which the tumor appears in locations difficult to realize a wide resection, the radiotherapy must be the standard treatment after biopsy, reaching excellent rates of local response. Rescue RT, after recurrence, provides a high rate of local control without adding an additional important morbidity, by it should be indicated as treatment in the mentioned cases or as palliative treatment(AU)

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

El objetivo del presente trabajo es compartir con el lector nuestra experiencia en el tratamiento percutáneo con radiofrecuencia guiada por tomografía computarizada de los tumores óseos benignos tras haber realizado más de 100 intervenciones en los últimos 8 años. En la actualidad, puede afirmarse que esta técnica debe ser el tratamiento de elección de la inmensa mayoría de los osteomas osteoides y que también tiene aplicaciones, como tratamiento definitivo, en muchos casos de osteoblastomas o de condroblastomas así como en otros tumores óseos benignos más infrecuentes. El procedimiento ha demostrado ser altamente eficaz y ha presentado escasas complicaciones, permitiendo a los pacientes unos tiempos de recuperación muy rápidos (AU)

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 (AU)

Calvarial lesions: a radiological approach to diagnosis.

Calvarial lesions are frequently identified in radiological studies. A wide variety of neoplasms and non-neoplastic lesions can involve the calvarium, and their imaging appearances vary according to their pathologic features. These lesions are usually asymptomatic but may manifest as a lump with or without associated pain. Clinical information, including the age of patient, is an important factor in the diagnostic process. In this paper, we illustrate the value of cross-sectional imaging techniques by computed tomography (CT) and magnetic resonance imaging (MRI) in evaluating these lesions. We also review the literature and discuss the specific imaging characteristics of the most common calvarial lesions in order to provide information that can guide radiological diagnosis or limit differential diagnosis.

Spinal tumors in children.

Pediatric spine tumors encompass a diverse group of pathologic diagnoses that differ markedly based on the location and age of the child. Children can be affected by primary and metastatic tumors, making the differential diagnosis and treatment options extensive. This article discusses the features of spinal tumors in children based primarily on location: extradural, intradural-extramedullary, and intramedullary tumors. Because this article deals with such a broad topic, detailed descriptions and outcomes of surgical and nonsurgical treatments for each particular tumor are limited. Rather, the key clinical, diagnostic, and therapeutic features of each tumor are discussed.

Extraosseous osteosarcoma arising from the small intestinal mesentery.

Extraskeletal osteosarcoma (EOS) is a highly aggressive and exceedingly rare mesenchymal tumor. Due to the rare nature of the disease, the diagnosis can be difficult and is often confirmed only after diagnostic laparotomy and histopathology. We describe the clinical history, radiologic and histomorphologic presentation, and clinical management of a 61-year-old patient who presented with abdominal pain. Abdominal ultrasound and computerized tomography (CT) scan revealed a calcified intra-abdominal mass. Following an explorative laparotomy, histology showed a large extraosseous osteosarcoma of the small bowel mesentery. Therapy according to the Cooperative Sarcoma Study-96 (COSS-96) was commenced. Diagnosis, management, and outcome in the context of the current literature are discussed. To our knowledge, this is the first description of an extraosseous osteosarcomas in the small bowel mesentery in the literature.

Tumors of the spine in children.

In children, tumors of the spine are much rarer than intracranial tumors. They are classified into intramedullary, intradural-extramedullary, and extradural tumors. Magnetic resonance imaging provides crucial information regarding the extent, location, and internal structure of the mass, thus critically narrowing the differential diagnosis and guiding surgery.

Cutaneous malignant ossifying fibromyxoid tumor.

A 76-year-old woman presented with a well-circumscribed 3 cm mass of her right buttock. The tumor, partially surrounded by a shell of woven and lamellar bone, had a lobular arrangement of highly cellular islands of tumor cells embedded in a variably fibrous to myxoid stroma. The lesional cells had well defined cytoplasmic membranes with varying amounts of clear to lightly eosinophilic cytoplasm. The nuclei exhibited moderate to severe nuclear atypia. Areas of tumor necrosis were present. The mitotic rate was 17 MF/50 high-power fields. The tumor was diagnosed as an ossifying fibromyxoid tumor (OFMT). OFMT is a rare tumor first described in 1989. Although OFMT usually occurs in deep soft tissue, up to 11% of reported lesions presented as cutaneous tumors. OFMT usually present in adults on the extremities or trunk. Most are histologically bland and apparently benign tumors, but OFMT with high nuclear grade, high cellularity, and >2 MF/50 high-power fields have shown potential for aggressive behavior including metastasis. OFMT with these features should be considered sarcomas. Given the histologic features, this tumor was considered a malignant OFMT. The patient had a wide excision. The patient died secondary to unrelated comorbidities without evidence of recurrence or metastasis.