RESUMO
Background: Primary malignant bone tumors are rare however, have a high global mortality rate. Osteosarcoma and chondrosarcoma are the most common bone sarcomas in the pelvis. The surgical management of primary bone tumors in the pelvis is challenging and depends on several factors. Internal hemipelvectomy with extremity preservation has become more popular compared to external hemipelectomy. Objective: Identify and describe characteristics of the surgical management of bone tumors and soft tissues of the pelvis by means of hemipelvectomy. Material and methods: Observational study of consecutive cases, in which patients with bone and soft tissue tumors undergoing hemipelvectomy were identified from January 2010 to December 2020 at the Oncology Surgery Department, using the Musculoskeletal Tumor Society (MSTS) scale. Results: 17 patients with pelvic tumors were included, 10 (58.8%) primary bone sarcomas, 3 (17.6%) soft tissue sarcomas, 3 (17.6%) benign bone tumors, 1 (5.8%) neoplasia of hematological origin. Average follow-up of 30 ± 14. Most frequent surgical complication, surgical wound seroma, 6 patients (37.5%). Conclusions: A multidisciplinary team is required to individualize treatment with the best sequence of options, giving improvement in oncological and functional results.
Introducción: os tumores óseos malignos primarios son raros, pero tienen una alta mortalidad global. El osteosarcoma y el condrosarcoma son los sarcomas óseos más comunes en la pelvis. El manejo quirúrgico de los tumores óseos primarios en la pelvis es desafiante y depende de varios factores. La hemipelvectomía interna con preservación de la extremidad se ha vuelto más popular en comparación con la hemipelvectomía externa. Objetivo: identificar y describir características del manejo quirúrgico de tumores óseos y tejidos blandos de pelvis mediante hemipelvectomía. Material y métodos: estudio observacional de casos consecutivos, en el que se identificaron pacientes con tumores óseos y tejidos blandos sometidos a hemipelvectomía en período de enero del 2010 a diciembre del 2020 en el Servicio de Cirugía Oncología, utilizando la escala MSTS (Musculoskeletal Tumor Society). Resultados: se incluyeron 17 pacientes con tumores pélvicos, 10 (58.8%) sarcomas óseos primarios, 3 (17.6%) sarcomas de tejido blando, 3 (17.6%) tumores óseos benignos, 1 (5.8%) neoplasia de origen hematológico. Seguimiento promedio de 30 ± 14. La complicación quirúrgica más frecuente fue el seroma de herida quirúrgica, presente en 6 pacientes (37.5%). Conclusiones: se requiere equipo multidisciplinario para individualizar tratamiento con la mejor secuencia de opciones, dando mejoría de resultado oncológico y funcional.
Assuntos
Neoplasias Ósseas , Hemipelvectomia , Humanos , Hemipelvectomia/métodos , Feminino , Masculino , Adulto , Neoplasias Ósseas/cirurgia , Pessoa de Meia-Idade , Adulto Jovem , Adolescente , Neoplasias Pélvicas/cirurgia , Idoso , Neoplasias de Tecidos Moles/cirurgia , Estudos Retrospectivos , Sarcoma/cirurgia , Osteossarcoma/cirurgia , Osteossarcoma/mortalidade , Seguimentos , Complicações Pós-Operatórias/etiologia , Condrossarcoma/cirurgiaRESUMO
Background: Osteosarcoma is the most common primary malignant bone neoplasm in young people. Presentation in non-bone tissues comprises 2 to 5% of all osteosarcomas and less than 1% of all soft tissue sarcomas. On rare occasions it presents as a superficial tumor, making it necessary to make a differential diagnosis with benign entities, such as pyogenic granuloma ossificans, and malignant neoplasms, with sarcomatoid differentiation such as carcinosarcoma and dedifferentiated melanoma. Accurate diagnosis requires correlation of the histological, macroscopic and imaging characteristics of the neoplasm, which is of great relevance since the biological behavior and treatment differ from that of bone osteosarcoma and dedifferentiated neoplasms. Clinical case: We present the case of a 52-year-old man with superficial extraskeletal osteosarcoma, with radiological and pathological characteristics characteristic of this neoplasm. Conclusions: Superficial extraskeletal osteosarcoma is extremely rare, representing less than 10% of extraskeletal osteosarcomas, histological evaluation and imaging studies are mandatory for diagnosis, and clinical history is useful both to rule out differential diagnoses and to contribute to the diagnosis. definitive. Superficial extraskeletal osteosarcoma was diagnosed in this patient based on histological criteria. However, the few findings reported prevent us from knowing the prognostic factors.
Introducción: el osteosarcoma es la neoplasia maligna primaria de hueso más común en jóvenes. La presentación en tejidos no óseos comprende del 2 al 5% de todos los osteosarcomas y menos del 1% de todos los sarcomas de partes blandas. En raras ocasiones se presenta como un tumor superficial, siendo necesario hacer diagnóstico diferencial con entidades benignas, como el granuloma piógeno osificante, y neoplasias malignas, con diferenciación sarcomatoide como el carcinosarcoma y el melanoma desdiferenciado. El diagnóstico preciso requiere correlación de las características histológicas, macroscópicas y de imagen de la neoplasia, siendo de gran relevancia ya que el comportamiento biológico y el tratamiento difiere al del osteosarcoma óseo y al de neoplasias desdiferenciadas. Caso clínico: presentamos el caso de un hombre de 52 años con osteosarcoma extraesquelético superficial, con características radiológicas y patológicas características de esta neoplasia. Conclusiones: el osteosarcoma extraesquelético superficial es extremadamente raro, representa menos del 10% de los osteosarcomas extraesqueléticos, la evaluación histológica y los estudios de imagen son obligatorios para el diagnóstico, y la historia clínica es útil tanto para descartar diagnósticos diferenciales como para contribuir al diagnóstico definitivo. En este paciente se diagnosticó osteosarcoma extraesquelético superficial con base a criterios histológicos. Sin embargo, los pocos hallazgos reportados impiden conocer los factores pronósticos.
Assuntos
Osteossarcoma , Humanos , Osteossarcoma/diagnóstico , Osteossarcoma/patologia , Masculino , Pessoa de Meia-Idade , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/patologiaRESUMO
Liposarcoma is a malignant mesenchymal tumor defined as a rare cancer due to its low incidence rate. The most common location of liposarcoma is in the extremities, followed by retroperitoneum, with the bone and trunk being the less frequent presentations. The most common histological subtype is well-differentiated liposarcoma, which has the highest local recurrence, is slow-growing, and is insensitive to chemo and radiotherapy. We present the case of a 62-year-old male patient with a 10-year-growth mass in the dorsal region. A computed tomography scan showed a huge mass in the right dorsal space with a malignant lipomatous appearance, which required surgical removal of a mass of 2,800 g.
Assuntos
Lipossarcoma , Humanos , Masculino , Lipossarcoma/cirurgia , Lipossarcoma/patologia , Lipossarcoma/diagnóstico por imagem , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia , Neoplasias de Tecidos Moles/diagnóstico por imagemRESUMO
Pediatric sarcomas present heterogeneous morphology, genetics and clinical behavior posing a challenge for an accurate diagnosis. DNA methylation is an epigenetic modification that coordinates chromatin structure and regulates gene expression, determining cell type and function. DNA methylation-based tumor profiling classifier for sarcomas (known as sarcoma classifier) from the German Cancer Research Center (Deutsches Krebsforschungszentrum) was applied to 122 pediatric sarcomas referred to a reference pediatric oncology hospital. The classifiers reported 88.5% of agreement between histopathological and molecular classification confirming the initial diagnosis of all osteosarcomas and Ewing sarcomas. The Ewing-like sarcomas were reclassified into sarcomas with BCOR or CIC alterations, later confirmed by orthogonal diagnostic techniques. Regarding the CNAs profile, osteosarcomas had several chromosomal gains and losses as well as chromothripsis, whereas Ewing sarcomas had few large events, such as amplifications of chromosomes 8 and 12. The molecular classification together with clinical and histopathological assessment could improve the diagnosis of pediatric sarcomas although there are limitations to deal with more rare classes. This study provides an increase in the number of sarcomas evaluated for DNA methylation profiling in the pediatric population.
Assuntos
Neoplasias Ósseas , Metilação de DNA , Osteossarcoma , Sarcoma de Ewing , Humanos , Criança , Neoplasias Ósseas/genética , Neoplasias Ósseas/classificação , Neoplasias Ósseas/patologia , Adolescente , Feminino , Masculino , Pré-Escolar , Sarcoma de Ewing/genética , Sarcoma de Ewing/classificação , Sarcoma de Ewing/patologia , Sarcoma de Ewing/diagnóstico , Osteossarcoma/genética , Osteossarcoma/classificação , Osteossarcoma/patologia , Sarcoma/genética , Sarcoma/classificação , Sarcoma/diagnóstico , Sarcoma/patologia , Perfilação da Expressão Gênica/métodos , Lactente , Neoplasias de Tecidos Moles/genética , Neoplasias de Tecidos Moles/classificação , Neoplasias de Tecidos Moles/patologia , Variações do Número de Cópias de DNARESUMO
Atypical cellular neurothekeoma is a rare benign soft-tissue tumour that usually arises in the head and neck region, shoulder girdles, and proximal extremities, predominantly in young women. This dermal neoplasm is under-reported in the literature and is not uncommonly misdiagnosed as a malignant tumour due to its worrisome histologic characteristics. Currently, the diagnosis of cellular neurothekeoma relies on a panel of non-specific immunohistochemical markers and its etiopathogenesis is unknown.Herein, we present the case of an atypical cellular neurothekeoma in the arm of a 49-year-old woman, describing its microscopic features and immunohistochemical profile. Additionally, we present a novel heterozygous predicted inactivating NF1 mutation, not previously reported, which was identified using high-throughput molecular techniques. Such finding might provide insights into the pathogenesis of neurothekeoma, potentially contributing to future refinements in diagnosis, which would enable more precise identification of this neoplasm.
Assuntos
Mutação , Neurofibromina 1 , Neurotecoma , Humanos , Feminino , Neurotecoma/patologia , Neurotecoma/genética , Neurotecoma/diagnóstico , Pessoa de Meia-Idade , Neurofibromina 1/genética , Neoplasias de Tecidos Moles/genética , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/diagnóstico , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/análise , Imuno-HistoquímicaRESUMO
Background and Objectives: This study aims to translate and culturally adapt the Toronto Extremity Salvage Score (TESS) for Latin American Spanish-speaking patients, enhancing the tool's accessibility for evaluating postsurgical functional outcomes in sarcoma patients across Latin America. Methods: The TESS questionnaires for lower extremity (LE) and upper extremity (UE) were translated and adapted following international guidelines. The process included forward and backward translation, expert committee review, and pretesting with cognitive interviewing. Patients treated for bone or soft tissue tumors in LE or UE were recruited to complete the adapted questionnaires. Test-retest reliability was evaluated by having participants complete the questionnaire again 2 weeks after the initial assessment. Results: A total of 89 participants completed the questionnaires. The study found high internal consistency, with Cronbach's alpha values reaching 0.9437 for LE and 0.9402 for UE. An agreement rate of 98.4% for the global score of TESS-LE (95% confidence interval [CI]: 0.909-1.059) and 93.9% for TESS-UE (95% CI: 0.882-0.995) was observed, demonstrating strong test-retest reliability. Conclusions: The Latin American Spanish version of TESS for both lower and upper extremities is a reliable and culturally appropriate tool for assessing physical function in limb sarcoma patients. Further validation across diverse Latin American populations is encouraged to strengthen its broad applicability.
Assuntos
Neoplasias Ósseas , Salvamento de Membro , Sarcoma , Traduções , Humanos , Sarcoma/cirurgia , Masculino , Feminino , Inquéritos e Questionários , Pessoa de Meia-Idade , América Latina/etnologia , Adulto , Neoplasias Ósseas/cirurgia , Neoplasias Ósseas/etnologia , Reprodutibilidade dos Testes , Idoso , Comparação Transcultural , Neoplasias de Tecidos Moles/cirurgia , Adulto Jovem , Idioma , Extremidade Inferior/cirurgia , Extremidade Inferior/fisiopatologiaRESUMO
OBJECTIVE: To evaluate the quality of life among patients with Soft Tissue Sarcomas treated at the Evangelic Mackenzie Hospital (HUEM) from 2018 to 2024 and undergoing surgical treatment. MATERIAL AND METHODS: descriptive and cross-sectional analysis of 23 patients with soft tissue neoplasia who underwent surgery and whether they underwent neoadjuvant or adjuvant clinical treatments. Epidemiological, clinical, and pathological data were considered. The EORTC 30 protocol was the instrument used for assessing the patients' quality of life. RESULTS: the main cases were located at extremity in females. Mean age at diagnosis was 47 years. All patients were symptomatic at diagnosis, with pain and a palpable mass being the most prevalent symptom and clinical sign, respectively. The mean time from the onset of symptoms to the date of diagnosis was 9 months. The mean tumor size was 11.68cm. Considering the functional scale, the items physical functioning, role performance and social function were the most affected in the assessment of quality of life. Fatigue and loss of appetite were the most common sign and symptom, respectively. A global measure of quality of life achieved high rates when 50% of patients rated as excellent. CONCLUSION: Continuous and multidisciplinary oncological care provided to the patient allows for better symptom control, resulting in higher quality of life, which positively impacts the patient's adherence to treatment, their progression, and possibly their survival.
Assuntos
Qualidade de Vida , Sarcoma , Humanos , Feminino , Estudos Transversais , Sarcoma/cirurgia , Sarcoma/psicologia , Pessoa de Meia-Idade , Masculino , Adulto , Idoso , Neoplasias de Tecidos Moles/cirurgia , Adulto JovemRESUMO
INTRODUCTION: surface sarcomas are a rare entity that need correct diagnosis to differentiate parosteal (cPOS), periosteal and the high grade surface osteosarcomas (HGSO). HGSO has malignant behavior similarities with osteosarcomas and wide resection is the key to a successful treatment.1 The Capanna and Hemi-Capanna reconstruction techniques have being developed in order to avoid amputation after an oncological resection, allowing structural support from an allograft and biological advantages from a vascularised autograft. CASE PRESENTATION: 46 years old male presenting with knee pain and 4 × 3 cm soft tissue tumor on the right tibial surface diagnosed of High Grade Surface Osteosarcoma (HGSO). Was treated by oncological resection followed by reconstruction with allograft and ipsilateral fibula autograft following the "Hemi-Capanna" technique and pedicled medial gastrocnemius flap. CONCLUSION: sufficient evidence supports the use of the Capanna technique in major musculoskeletal reconstructions. The new "Hemi-Capanna" technique has less evidence but proves to be an easier surgical technique with good functional results and little complications.
INTRODUCCIÓN: los sarcomas de superficie son una entidad rara que necesita un diagnóstico correcto para diferenciar el parostio (cPOS), el perióstico y el osteosarcoma de superficie de alto grado (HGSO). HGSO tiene similitudes de comportamiento maligno con los osteosarcomas y la resección amplia es la clave para un tratamiento exitoso. Las técnicas de reconsrucción Capanna y Hemi-Capanna han sido desarrolladas para evitar la amputación después de una resección por cáncer, permitiendo el soporte estructural de un aloinjerto y las ventajas biológicas de un autoinjerto vascularizado. PRESENTACIÓN DEL CASO: varón de 46 años que presenta dolor en rodilla y tumor de tejido blando de 4 × 3 cm en superficie tibial derecha diagnosticado con osteosarcoma de superficie de alto grado (OSAG). Se trató mediante resección oncológica seguida de reconstrucción con aloinjerto y autoinjerto de peroné ipsilateral siguiendo la técnica de "Hemi-Capanna" y colgajo pediculado de gastrocnemio medial. CONCLUSIÓN: existe evidencia suficiente que respalda el uso de la técnica de Capanna en reconstrucciones musculoesqueléticas mayores. La nueva técnica "Hemi-Capanna" tiene menos evidencia, pero demuestra ser una técnica quirúrgica más sencilla, con buenos resultados funcionales y pocas complicaciones.
Assuntos
Neoplasias Ósseas , Osteossarcoma , Humanos , Osteossarcoma/cirurgia , Masculino , Pessoa de Meia-Idade , Neoplasias Ósseas/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Tíbia/cirurgia , Retalhos Cirúrgicos , Fíbula/transplante , Fíbula/cirurgia , Transplante Ósseo/métodos , Neoplasias de Tecidos Moles/cirurgiaRESUMO
BACKGROUND: Different giant cell-rich tumors may occur in the jaws. Recently, a new condition known as keratin-positive giant-cell rich tumor harboring recurrent HMGA2::NCOR2 fusions has been described. Interestingly, the mononuclear cells of this tumor are immunoreactive with the AE1/AE3 keratin. Considering the similarities of central and peripheral giant cell granuloma of the jaws with the keratin-positive giant cell-rich tumor of the soft tissue and bone, we hypothesized whether the keratin-positive tumors could also occur in the maxillary bones. METHODS AND RESULTS: An immunohistochemical investigation of AE1/AE3 in a cohort of 16 cases of peripheral and central giant cell granuloma of the jaws was carried out. None of the cases was keratin-positive. CONCLUSIONS: Although no immunopositivity for keratin was observed in the present giant cell granulomas cohort, we cannot completely exclude the possibility of keratin-positive giant cell-rich tumors occurring in the jaws. Therefore, oral pathologists should be aware about this condition and further studies using cohorts from different laboratories are necessary.
Assuntos
Granuloma de Células Gigantes , Doenças Maxilomandibulares , Queratinas , Humanos , Granuloma de Células Gigantes/patologia , Granuloma de Células Gigantes/metabolismo , Feminino , Masculino , Adulto , Pessoa de Meia-Idade , Doenças Maxilomandibulares/patologia , Doenças Maxilomandibulares/metabolismo , Adulto Jovem , Imuno-Histoquímica , Idoso , Neoplasias Maxilomandibulares/patologia , Neoplasias Maxilomandibulares/metabolismo , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/metabolismo , Adolescente , Tumores de Células Gigantes/patologia , Tumores de Células Gigantes/metabolismoRESUMO
OBJECTIVE: Elastofibroma dorsi (ED) is a rare benign tumor located in the subscapular region. The aim of this study was to evaluate our clinical findings, surgical approach, and management of ED patients based on single-center data with the relevant literature. METHODOLOGY: A retrospective evaluation was conducted on 20 patients who were operated on for ED. RESULTS: Of the 16 (80%) female patients and 4 (20%) male patients, the main complaint was swelling (80%), and 10 cases (50%) had unilateral involvement. All patients were operated on using standard surgical procedures. Despite a long follow-up period (6-53 months, mean of 26.6 months), no recurrences were observed. Two patients (10%) required simple needle aspiration due to post-operative seroma, and one patient, due to infection, required evacuation (5%). CONCLUSION: Although rare, ED should not be overlooked in patients with swelling in the back region. Our data suggests that surgery can be safely performed in such patients after a clinical and radiological diagnosis of ED has been established.
OBJETIVO: Evaluar los hallazgos clínicos, el enfoque quirúrgico y el manejo de los pacientes con urgencias a partir de los datos de un solo centro y la literatura relevante. MÉTODO: Se realizó una evaluación retrospectiva de 20 pacientes que fueron operados de ED. RESULTADOS: En los 16 (80%), pacientes del sexo femenino y cuatro (20%) del sexo masculino, la queja principal fue la tumefacción (80%) y 10 casos (50%) tuvieron afectación unilateral. Todos los pacientes fueron operados utilizando procedimientos quirúrgicos estándar. Con un largo periodo de seguimiento (6-53 meses, media de 26.6 meses), no se observaron recurrencias. Dos pacientes (10%) requirieron aspiración con aguja simple por seroma posoperatorio y un paciente (5%) requirió evacuación por infección. CONCLUSIONES: Aunque es raro, el ED no debe pasarse por alto en pacientes con hinchazón en la región de la espalda. Nuestros datos sugieren que la cirugía se puede realizar de manera segura en estos pacientes después de haber establecido el diagnóstico clínico y radiológico de ED.
Assuntos
Fibroma , Neoplasias de Tecidos Moles , Humanos , Masculino , Feminino , Estudos Retrospectivos , Fibroma/cirurgia , Fibroma/diagnóstico por imagem , Fibroma/patologia , Pessoa de Meia-Idade , Adulto , Idoso , Neoplasias de Tecidos Moles/cirurgia , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/diagnóstico por imagem , Complicações Pós-Operatórias/etiologia , Seroma/etiologiaRESUMO
BACKGROUND: Radiation-associated soft tissue sarcomas (RA-STS) are rare complications of patients receiving radiation therapy (RT) and are generally associated with a poor prognosis. Most of the literature surrounding RA-STS of the chest is centered on angiosarcoma. Therefore, we aim to document the management and outcome of patients with non-angiosarcoma RA-STS of the chest. METHODS: We reviewed 17 patients (all female, median age 65 years) diagnosed with RA-STS. The most common primary malignancy was breast carcinoma (n = 15), with a median RT dose of 57.9 Gy. All patients underwent surgical resection; five patients (29%) received radiotherapy; and five patients (29%) received peri-operative chemotherapy. RESULTS: The 5-year local recurrence and metastatic-free survival were 61% and 60%, while the 5-year disease-specific survival was 53%. Local recurrence was associated with death due to disease (HR 9.06, p = 0.01). Complications occurred in nine of patients, most commonly due to a wound complication (n = 7). At the most recent follow-up, the median Musculoskeletal Tumor Society Score was 63%. CONCLUSION: RA-STS involving the chest wall are aggressive tumors with a high risk of local relapse and death due to disease. Local recurrence was associated with death due to disease; as such, we recommend aggressive surgical management with evaluation for adjuvant therapies.
Assuntos
Recidiva Local de Neoplasia , Sarcoma , Humanos , Feminino , Idoso , Pessoa de Meia-Idade , Sarcoma/radioterapia , Sarcoma/patologia , Sarcoma/mortalidade , Sarcoma/terapia , Sarcoma/cirurgia , Recidiva Local de Neoplasia/patologia , Neoplasias Induzidas por Radiação/patologia , Neoplasias Induzidas por Radiação/mortalidade , Neoplasias Induzidas por Radiação/etiologia , Neoplasias Induzidas por Radiação/cirurgia , Idoso de 80 Anos ou mais , Estudos Retrospectivos , Adulto , Neoplasias Torácicas/radioterapia , Neoplasias Torácicas/patologia , Neoplasias Torácicas/mortalidade , Parede Torácica/patologia , Parede Torácica/efeitos da radiação , Seguimentos , Neoplasias de Tecidos Moles/radioterapia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/terapia , Neoplasias de Tecidos Moles/cirurgia , Neoplasias da Mama/patologia , Neoplasias da Mama/radioterapia , Neoplasias da Mama/mortalidade , Neoplasias da Mama/terapiaRESUMO
Spinocellular carcinoma is a tumor lesion that frequently occurs in photo-exposed areas, presenting characteristics such as keratinization, scaly areas and even ulcerations. Its potential for metastasis makes early identification and diagnosis essential in order to carry out correct treatment of said lesion. In the spectrum of spinocelullar carcinomas is Keratoacanthoma, which has been in debate about its origin and its benignity. We present the clinical evolution, treatment, results, and bibliographic review of a keratoacanthoma.
El carcinoma espinocelular es una lesión tumoral que se da frecuentemente en zonas foto-expuestas, presentando características tales como queratinización, zonas descamativas e incluso ulceraciones. Su potencial de metástasis hace imprescindible la identificación y diagnóstico precoz para poder realizar un correcto tratamiento de dicha lesión. Dentro de su espectro se encuentra el Queratoacantoma, el cual ha estado en debate sobre su origen y su benignidad. Nosotros presentamos la evolución clínica, tratamiento, resultados y revisión bibliográfica de un queratoacantoma.
Assuntos
Humanos , Feminino , Idoso de 80 Anos ou mais , Neoplasias de Tecidos Moles/cirurgia , Neoplasias de Tecidos Moles/diagnóstico por imagem , Ceratoacantoma , Tumor de Células Granulares/cirurgia , Tumor de Células Granulares/diagnóstico por imagemRESUMO
Introduction: Soft tissue sarcomas (STS) are low-incidence tumors whose clinical and histopathological factors are associated with adverse oncological outcomes. This study evaluated prognostic factors (PF) associated with tumor recurrence and overall survival (OS) in patients diagnosed with STS of the extremities, treated at the Instituto Nacional de Cancerología (INC), Bogotá, Colombia. Materials and Methods: An analytical observational study of a historical cohort was carried out, including patients diagnosed with STS and managed surgically in the Functional Unit for Breast and Soft Tissue Tumors of the INC from January 2008 to December 2018. Results: A total of 227 patients were included; 74.5% had tumors greater than 5 cm. Most patients (29.1%) were in stage IIIB at diagnosis. Age was associated with higher mortality (HR = 1.01; CI95%: 1-1.02; p = 0.048). Tumor persistence at admission to the INC (HR = 2.34; CI95%: 1.25-4.35; p = 0.007) and histologic grade III (HR = 5.36; CI95%: 2.29-12.56; p = <0.001) showed statistical significance in the multivariate analysis for recurrence of any type, as did the PFs associated with a higher risk of local recurrence (HR = 2.85; CI95%: 1.23-6.57; p = 0.014 and HR = 6.09; CI95%: 2.03-18.2; p = 0.001), respectively. Tumor size (HR = 1.03; CI95%: 1-1.06; p = 0.015) and histologic grade III (HR = 4.53; CI95%: 1.42-14.49; p = 0.011) were associated with a higher risk of distant recurrence. Conclusions: This cohort showed that in addition to histologic grade and tumor size, tumor persistence at the time of admission has an impact on disease recurrence, so STS should be managed by a multidisciplinary team with experience in this pathology in high-volume reference centers.
Assuntos
Extremidades , Recidiva Local de Neoplasia , Sarcoma , Humanos , Feminino , Masculino , Sarcoma/mortalidade , Colômbia/epidemiologia , Pessoa de Meia-Idade , Extremidades/patologia , Prognóstico , Adulto , Idoso , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Idoso de 80 Anos ou maisRESUMO
BACKGROUND: Soft tissue sarcomas are rare malignant tumors with significant heterogeneity. The importance of classifying histological grades is fundamental to defining the treatment approach. OBJECTIVE: To evaluate magnetic resonance imaging (MRI) in predicting the histological grade of soft tissue sarcomas. METHODS: A retrospective observational study included patients over 18 years undergoing MRI and primary tumor surgery at AC Camargo Cancer Center from January 2015 to June 2022. Two radiologists evaluated MRI criteria (size, margin definition, heterogeneity of the T2 signal, high-intensity peritumoral signal on T2, and postperitumoral contrast), and a grading prediction score was calculated. χ2 and logistic regression analyses were conducted. RESULTS: Sixty-eight patients were included (38 men; median: 48 years). Moreover, 52 high-grade and 16 low-grade tumors were observed. The MRI criteria associated with histological grade were peritumoral high-intensity T2-weighted signals (p < 0.001) and peritumoral postcontrast enhancement (p = 0.006). Logistic regression confirmed their significance (odds ratio [OR]: 11.8 and 8.8, respectively). Each score point increment doubled the chance of high-grade tumors (OR: 2.0; p = 0.014). CONCLUSION: MRI effectively predicts histological grades of soft tissue sarcomas. Peritumoral high-intensity T2-weighted signals and peritumoral postcontrast enhancement are valuable indicators of high-grade tumors. This highlights MRI's importance in treatment decision-making for sarcoma patients.
Assuntos
Imageamento por Ressonância Magnética , Gradação de Tumores , Sarcoma , Humanos , Masculino , Pessoa de Meia-Idade , Imageamento por Ressonância Magnética/métodos , Feminino , Estudos Retrospectivos , Sarcoma/patologia , Sarcoma/diagnóstico por imagem , Sarcoma/cirurgia , Adulto , Idoso , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/diagnóstico por imagem , Adulto Jovem , SeguimentosRESUMO
A 56-year-old woman debuted with a palpable painless mass in the anterior thorax wall at the level of the second and third right parasternal intercostal space, which progressively increased in size over 5 months accompanied by localized skin rash, mild dyspnea and chest pain when changing position. Imaging studies showed a soft tissue mass measuring 75 × 62 mm and a density of 34 Hounsfield Units that had caused the lysis of the costal arches and grew expansively towards the anterior mediastinum, without identifying mediastinal adenopathies only by this imaging method. Core biopsy was performed, which was initially diagnosed as histiocytic sarcoma (HS); however, when the diagnostic panel was expanded to include molecular and NGS studies, the final diagnosis was anaplastic large cell lymphoma with ALK::ATIC fusion. Here, we report a very rare neoplasm with unusual clinical presentation, histopathology and molecular features.
Assuntos
Sarcoma Histiocítico , Linfoma Anaplásico de Células Grandes , Humanos , Feminino , Pessoa de Meia-Idade , Sarcoma Histiocítico/patologia , Sarcoma Histiocítico/genética , Linfoma Anaplásico de Células Grandes/genética , Linfoma Anaplásico de Células Grandes/patologia , Linfoma Anaplásico de Células Grandes/diagnóstico , Quinase do Linfoma Anaplásico/genética , Diagnóstico Diferencial , Neoplasias de Tecidos Moles/genética , Neoplasias de Tecidos Moles/patologia , Neoplasias Torácicas/patologia , Neoplasias Torácicas/genéticaRESUMO
Introducción. El cáncer de riñón es la undécima neoplasia maligna más común en los Estados Unidos Mexicanos. El carcinoma de células claras de riñón (CCR) es considerado la estirpe más frecuente y representa el 2-3 % de todos los cánceres a nivel mundial. En el contexto de la enfermedad metastásica, por lo general se identifica un tumor renal primario y las metástasis se localizan en pulmón, hueso, hígado, cerebro y, raramente, en tejidos blandos. Los pacientes con metástasis a tejidos blandos no tienen síntomas en las etapas iniciales y generalmente se identifican sólo cuando las lesiones aumentan de tamaño o durante el estudio de la pieza de resección quirúrgica. Caso clínico. Se presenta el caso de una paciente en la séptima década de la vida, con una metástasis en tejidos blandos de la región sacra, de 10 años de evolución posterior a una nefrectomía secundario a CCR. Resultados. Hallazgos clínicos e imagenológicos de un tumor bien delimitado. Se realizó resección quirúrgica de la lesión, bajo anestesia regional, con extirpación completa. Conclusión. Se recomienda que los pacientes con un sitio metastásico resecable y solitario sean llevados a resección quirúrgica con márgenes libres, como fue el caso de nuestra paciente, por su fácil acceso y ser una lesión única. En el CCR, además de su tratamiento quirúrgico inicial, es indispensable una estrecha vigilancia con examen físico e imágenes transversales, para detectar la presencia de metástasis y con ello evitar tratamientos tardíos.
Introduction. Kidney cancer is the eleventh most common malignancy in the United States of Mexico. Carcinoma renal cell (CRC) is considered the most frequent type and represents 2-3% of all cancers worldwide. In the setting of metastatic disease, a primary renal tumor is usually identified, and metastases are located in the lung, bone, liver, brain, and rarely in soft tissue. Patients with soft tissue metastases do not have symptoms in the initial stages and are generally found only when the lesions increase in size or during the study of the surgical resection piece. Clinical case. In this case, we report a female patient in the seventh decade of life with a soft tissue metastasis located in the sacral region, 10 years after a nephrectomy secondary to CRC. Results. Clinical and radiological findings of a well-defined tumor. Surgical resection of the lesion is performed under regional anesthesia with complete excision. Conclusions. It is recommended that patients with a resectable and solitary metastatic site be candidates for surgical resection with free margins, as was the case with our patient due to its easy access and single lesion. In CRC, in addition to its initial surgical treatment, close surveillance with physical examination and cross-sectional images is essential to monitor the presence of metastases and thus avoid late treatments.
Assuntos
Humanos , Carcinoma de Células Renais , Neoplasias Renais , Inoculação de Neoplasia , Neoplasias de Tecidos Moles , Diagnóstico Diferencial , Metástase NeoplásicaRESUMO
PURPOSE: Multidisciplinary molecular tumor boards (MTBs) decode complex genomic data into clinical recommendations. Although MTBs are well-established in the oncology practice in developed countries, this strategy needs to be better explored in developing countries. Herein, we describe the possible benefits and limitations of the first MTB established in Colombia. METHODS: Demographic, clinical, and genomic information was collected between August 2020 and November 2021. By mid-2020, an MTB strategy was created to discuss clinical cases with one or more genomic alterations identified by next-generation sequencing using an open-access virtual platform. We characterized the patient population as benefiting from the recommended treatment option. We assessed the benefits and access to available targeted therapies that have the potential to change clinical management by making recommendations to treating oncologists on the basis of genomic profiling. However, we did not assess the treatment oncologists' compliance with MTB recommendations because they were not intended to replace clinical judgment/standard of care. RESULTS: A total of 146 patients were included in the discussions of the MTB. The median age was 59 years, and 59.6% were women. Genomic results prompting a change in therapeutic decisions were obtained in 53.1% of patients (95% CI, 44.9 to 61.3). The most prevalent malignancy was non-small-cell lung cancer (51%). Other malignancies represented 60%, 50%, and 30% of patients with soft-tissue sarcomas, brain tumors, and breast cancer, respectively. CONCLUSION: Using an open-access virtual platform, MTBs were feasible in low- and middle-income countries on the basis of the capability to provide the benefits and access to available targeted therapies that are not standard of care. Furthermore, MTB recommendations were made available to the treating oncologist in different locations across Colombia, providing the option to modify clinical management in most of these patients.
Assuntos
Hispânico ou Latino , Neoplasias , Avaliação de Resultados em Cuidados de Saúde , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias da Mama , Carcinoma Pulmonar de Células não Pequenas , Neoplasias Pulmonares , Oncologia , Sarcoma , Neoplasias Encefálicas , Neoplasias de Tecidos Moles , Neoplasias/terapia , Resultado do TratamentoRESUMO
BACKGROUND: Preoperative radiation therapy following by limb-sparing or conservative surgery is a standard approach for limb and trunk STS. Data supporting hypofractionated radiotherapy schedules are scarce albeit biological sensitivity of STS to radiation would justify it. We sought to evaluate the impact of moderate hypofractionation on pathologic response and its influence on oncologic outcomes. MATERIAL AND METHODS: From October 2018 to January 2023, 18 patients with limb or trunk STS underwent preoperative radiotherapy at a median dose of 52.5 Gy (range 49.5-60 Gy) in 15 fractions of 3.5 Gy (3.3-4 Gy) with or without neoadjuvant chemotherapy. A favorable pathologic response (fPR) was considered as ≥ 90% tumor necrosis on specimen examination. RESULTS: All patients completed planned preoperative radiotherapy. Eleven patients (61.1%) achieved a fPR, and 7 patients (36.8%) a complete pathologic response with total disappearance of tumor cells. Nine patients (47%) developed grade 1-2 acute skin toxicity, and 7 patients (38.8%) had wound complications on follow-up. With a median follow-up of 14 months (range 1-40), no cases of local relapse were observed, and actuarial 3-year overall survival (OS) and distant metastases-free survival (DMFS) are 87% and 76.4%, respectively. In the univariate analysis, the presence of a favorable pathologic response (fPR) was associated with improved 3-year OS (100% vs. 56.03%, p = 0.058) and 3-year DMFS (86.91% vs. 31.46%, p = 0.002). Moreover, both complete or partial RECIST response and radiological stabilization of the tumor lesion showed a significant association with higher rates of 3-year distant metastasis-free survival (DMFS) (83% vs. 83% vs. 56%, p < 0.001) and 3-year overall survival (OS) (100% vs. 80% vs. 0, p = 0.002). CONCLUSIONS: Preoperative moderate hypofractionated radiation treatment for STS is feasible and well tolerated and associates encouraging rates of pathologic response that could have a favorable impact on final outcomes.