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Distinct lesions are derived from notochordal cells (NCDL), ranging from benign to malignant ones. This study presents fifty NCDL cases diagnosed in a tertiary hospital of reference from the past 55 years: forty-two conventional chordomas, including one chondroid chordoma subtype, four benign notochordal cell tumors (BNCT), two conventional chordomas with BNCT foci, and two dedifferentiated chordomas. All patients were adults. Three BNCT were incidentally diagnosed, and one case presented local pain. Chordomas began with local pain and/or neurological symptoms. BNCT were well-defined intraosseous lesions, hypointense on T1-weighted images (WI) and hyperintense on T2-WI, without enhancement in the contrast. Conventional chordomas, including its chondroid subtype, were lobulated masses with cortical disruption and soft tissue extension, hypointense on T1-WI and hyperintense on T2-WI, with variable contrast enhancement. BNCT were histologically composed of solid sheets of vacuolated cells with clear cytoplasm and round and central nuclei. No atypia, lobular growth pattern, myxoid matrix, or bone infiltration were seen. Conventional chordomas were histologically composed of physaliphorous cells in a myxoid stroma with lobulated and infiltrating growth patterns. Observational follow-up using radiological controls was decided on for the BNCT cases. None of these cases presented local recurrence or metastasis. En-bloc resection and adjuvant radiotherapy were selected for sacral and vertebral chordoma cases. Sixteen patients died due to tumor-related factors; twenty-eight presented local recurrence, and four developed distant metastases. New therapeutic options are being studied for chordoma cases. Clinical, radiological, and histopathological data are necessary to properly diagnose and follow up of NCDL.
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Giant cell tumor of the bone is a locally aggressive and rarely metastasizing tumor that typically affects the ends of long bones. Less than 1% of giant cell tumor of bone occur in the ribs. Our patients were a 32-year-old woman and a 45-year-old man and were detected following chest traumas. No bone alterations were detected in radiological studies performed immediately after local trauma. Six- and one- months, respectively, following chest trauma, both giant lytic heterogeneous masses arising on an anterior rib arc were radiologically observed. In the computerized tomography-guided needle biopsy, giant cell tumor of bone were diagnosed. Both tumors were completely removed by bloc resection including the adjacent ribs and posterior reconstruction were performed. One patient was preoperatively treated with denosumab. Neither local recurrences nor metastasis have been detected in follow-up. Despite its low frequency and its low degree of suspicion, giant cell tumor of bone should be included in the differential diagnosis of a rapid growing chest mass.
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Hemosiderotic/aneurysmal variant of dermatofibroma (DF) is infrequent and may be misdiagnosed with malignant lesions. We report the case of a giant (7.6cm) subcutaneous hemosiderotic/aneurysmal DF (H/ADF) of the thigh in a 53-year-old female patient. Internal arterial and venous hypervascularity was seen by spectral Doppler ultrasound. Magnetic resonance image showed a discrete homogeneous hypointense in T1-weighted images (WI) and T2-WI mass, with hyperintense areas in fat-suppressed T2-WI. The histology revealed a monotonous fusocelular proliferation without atypia, positive for CD163, factor XIIIa and CD10. Widely distributed hemosiderin pigment and two blood-filled pseudovascular spaces lacking endothelial lining were present. H/ADF was diagnosed. The mass was removed but surgical margins were affected. The patient did not present local relapse or distant metastasis. H/ADF are unusual cutaneous soft tissue tumours that can be clinically, radiologically and histopathologically confused with malignant lesions such as melanomas, vascular lesions or sarcomas, especially in giant cases.
Subject(s)
Histiocytoma, Benign Fibrous , Thigh , Humans , Female , Middle Aged , Thigh/pathology , Histiocytoma, Benign Fibrous/pathology , Histiocytoma, Benign Fibrous/diagnostic imaging , Magnetic Resonance Imaging , Hemosiderosis/pathology , Hemosiderosis/diagnostic imaging , Skin Neoplasms/pathology , Skin Neoplasms/diagnostic imaging , Hemosiderin/analysis , Aneurysm/pathology , Aneurysm/diagnostic imagingABSTRACT
INTRODUCTION: Osteosarcomas of the jaw (OSJs) are rare tumors with distinct characteristics from osteosarcomas affecting other bones. This study aims to analyze the clinical, pathological, and therapeutic characteristics of OSJs. METHODS: A retrospective, descriptive cross-sectional study including patients diagnosed with OSJ registered at the "La Paz" University Hospital, Madrid, was performed. RESULTS: Data of eight patients with a diagnosis of OSJ were obtained during the study period of 22 years (2002-2024). The mean age of the patients was 41 years. The distribution was 1:1 between the maxilla and mandible. Painful inflammation was the most frequent clinical manifestation. Conventional osteoblastic osteosarcoma was the most predominant histological type. Survival rate at 5 years was 50%, which decreased to 25% at 10 years. CONCLUSIONS: OSJs differ from conventional osteosarcomas of long tubular bones. Surgery continues to be the mainstay of treatment. However, more studies are needed through which more standardized protocols can be proposed for adjuvant therapeutic management.
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Angiosarcoma is a rare soft tissue sarcoma originating from endothelial cells. Given that current treatments for advanced disease have shown limited efficacy, alternative therapies need to be identified. In rare diseases, patient-derived cell models are crucial for screening anti-tumour activity. In this study, cell line models were characterised in 2D and 3D cultures. The cell lines' growth, migration and invasion capabilities were explored, confirming them as useful tools for preclinical angiosarcoma studies. By screening a drug library, we identified potentially effective compounds: 8-amino adenosine impacted cell growth and inhibited migration and invasion at considerably low concentrations as a single agent. No synergistic effect was detected when combining with paclitaxel, gemcitabine or doxorubicin. These results suggest that this compound could be a potentially useful drug in the treatment of AGS.
Subject(s)
Hemangiosarcoma , Sarcoma , Humans , Hemangiosarcoma/drug therapy , Endothelial Cells/pathology , Doxorubicin/pharmacology , Doxorubicin/therapeutic use , Sarcoma/drug therapy , Paclitaxel/pharmacology , Paclitaxel/therapeutic useABSTRACT
Cutaneous fibrous histiocytoma (FH) is considered a benign dermal tumor. The cellular variant is rare and poorly documented. Besides presenting a high risk of local recurrence, it has a low but serious metastatic potential. We present a case of metastatic cellular FH and also review the literature on this tumor, given its unusual metastatic development. A 47-year-old male patient presented with a lesion in the anterior surface of the right thigh, which has been present since adolescence but had grown during last year. Anatomopathological evaluation revealed a cellular FH, and the lesion was completely removed. Six months later, tumor recurrence with multiple compartment muscle involvement and pulmonary metastasis were detected. Both lesions were completely resected and after 3 years of follow-up, the patient is asymptomatic and free of the disease. We conclude that FH should be carefully sampled to detect variants with high local recurrence rates or with some metastatic risk such as the cellular one. We recommend wide surgical resection and a close follow-up including chest x-rays or thorax computed tomography (CT) in all cellular FH cases with local recurrence.
Subject(s)
Histiocytoma, Benign Fibrous , Lung Neoplasms , Skin Neoplasms , Male , Adolescent , Humans , Middle Aged , Histiocytoma, Benign Fibrous/pathology , Skin Neoplasms/pathology , Lung Neoplasms/secondaryABSTRACT
INTRODUCTION: A soft tissue aneurysmal bone cyst is an extremely rare tumor. The objective of the article is to present the clinical, radiological, and histopathological features of a very unusual neoplasm of soft tissues. CASE REPORT: A 13-year-old male patient presented a painful, mobile, and rapidly growing mass on the posteromedial aspect of his left knee. Imaging studies revealed a mass that arose from the medial surface of the distal sartorius muscle, with extension to the subcutaneous fat tissue. It was a well-circumscribed solid tumor with a peripheral rim calcification on plain film, computerized tomography, and ultrasound (zonal phenomenon). On magnetic resonance imaging, a heterogenous mass on T1-weighted images (WI) and T2-WI was seen, with a peripheral hypointense rim in both sequences. An outstanding edema on T2-WI extending to the soft tissue and muscles of the medial compartment of the knee was detected. The mass was resected, and the "tumoral mimickers" histopathological and molecular (next-generation sequencing) diagnoses confirmed a soft tissue aneurysmal bone cyst. A follow-up showed that the patient was free of disease 12 months after surgery. CONCLUSION: Soft tissue aneurysmal bone cyst is a rare tumor. Appropriate clinical and radiological correlation should be performed to differentiate it from other tumor mimickers.
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Background Infantile fibrosarcoma is the most frequent soft tissue sarcoma in newborns or children under one year of age. This tumour often implies high local aggressiveness and surgical morbidity. The large majority of these patients carry the ETV6NTRK3 oncogenic fusion. Hence, the TRK inhibitor larotrectinib emerged as an efficacious and safe alternative to chemotherapy for NTRK fusion-positive and metastatic or unresectable tumours. However, real-world evidence is still required for updating soft-tissue sarcoma practice guidelines. Objective To report our experience with the use of larotrectinib in pediatric patients. Methods Our case series shows the clinical evolution of 8 patients with infantile fibrosarcoma under different treatments. All patients enrolled in this study received informed consent for any treatment. Results Three patients received larotrectinib in first line. No surgery was needed with larotrectinib, which led to the rapid and safe remission of tumours, even in unusual anatomical locations. No significant adverse effects were observed with larotrectinib. Conclusion Our case series supports that larotrectinib may be a therapeutic option for newborn and infant patients with infantile fibrosarcoma, especially in uncommon locations (AU)
Subject(s)
Humans , Infant, Newborn , Infant , Child, Preschool , Soft Tissue Neoplasms/drug therapy , Soft Tissue Neoplasms/genetics , Fibrosarcoma/drug therapy , Fibrosarcoma/genetics , Antineoplastic Agents/therapeutic use , Soft Tissue Neoplasms/pathology , Fibrosarcoma/pathology , Treatment OutcomeABSTRACT
Background: Oral cancer is the 11th most common type of cancer in the world, with established major risk factors as tobacco and alcohol, and recently included high-risk human papillomavirus types 16 and 18. HPV types 16 and 18 are the etiologic agents of cervical cancers and a proportion of oropharyngeal cancers. However, the picture of HPV and the clinical implications of oral cancers are not clear with most reports combining oral cancer data with head and neck cancers. It has been confirmed as a favorable prognostic factor in oropharyngeal cancer. However, the prognostic value of HPV in oral squamous cell carcinoma is still unclear. Material and Methods: The main objective of this article is to present the evidence encountered following a bibliographical review of recent publications specifically related to oral cancer and its differences from oropharyngeal cancer. The secondary goals are to present the findings of a five-year retrospective observational study of the prevalence of HPV infection in oral cancer patients treated by the Oral and Maxillofacial Surgery Department at La Paz University Hospital (Madrid, Spain), and finally, we to evaluate and compare our country's HPV prevention program in comparison to other European countries. Results: According to the review of the literature, HPV positive oral squamous cell carcinoma is associated with significantly decreased overall survival and distant control. Bibliographic review suggest HPV infection can be used as a negative prognostic factor in oral squamous cell carcinoma. Conclusions: As regards diagnostic testing for HPV, it should be extended to as many cases of oral cavity squamous cell carcinoma as possible, especially in those with risk factors. The current vaccination program in Spain does not have adequate coverage and is significantly under the level of other European Union countries; it should be expanded and catch-up strategies should be included. Key words:HPV, OSSC, Papillomavirus, oral carcinoma, prevention.
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INTRODUCTION: Intranodal palisaded myofibroblastoma (IPM) is an exceedingly rare benign mesenchymal tumor of the lymph nodes. Magnetic resonance imaging (MRI) findings are unspecific, which may present diagnostic challenges to fine-needle aspiration cytology (FNAC). The histological and immunohistochemical features of IPM are unique. CASE REPORT: A previously healthy 40-year-old male patient presented a slow-growing solitary left inguinal mass. FNAC revealed clustered cells within a metachromatic stroma, single spindle cells without atypia, hemosiderin pigment, and siderophages. An MRI showed a central hyperintense septum in fat-suppressed, T2-weighted sequences. The excised lymph node contained central haphazard fascicles of spindle cells with focal nuclear palisading, hemosiderin pigment, extravasated erythrocytes, and hemorrhagic areas. Vimentin and smooth muscle actin were diffusely positive. Amianthoid collagen fibers were not clearly observed. CONCLUSION: IPM is an extremely rare mesenchymal benign intranodal tumor that should be included in the differential diagnosis of spindle cell lesions in the inguinal region.
Subject(s)
Hemosiderin , Neoplasms, Muscle Tissue , Male , Humans , Adult , Lymph Nodes/pathology , Biopsy, Fine-Needle , Neoplasms, Muscle Tissue/pathology , CytodiagnosisABSTRACT
BACKGROUND: Infantile fibrosarcoma is the most frequent soft tissue sarcoma in newborns or children under one year of age. This tumour often implies high local aggressiveness and surgical morbidity. The large majority of these patients carry the ETV6-NTRK3 oncogenic fusion. Hence, the TRK inhibitor larotrectinib emerged as an efficacious and safe alternative to chemotherapy for NTRK fusion-positive and metastatic or unresectable tumours. However, real-world evidence is still required for updating soft-tissue sarcoma practice guidelines. OBJECTIVE: To report our experience with the use of larotrectinib in pediatric patients. METHODS: Our case series shows the clinical evolution of 8 patients with infantile fibrosarcoma under different treatments. All patients enrolled in this study received informed consent for any treatment. RESULTS: Three patients received larotrectinib in first line. No surgery was needed with larotrectinib, which led to the rapid and safe remission of tumours, even in unusual anatomical locations. No significant adverse effects were observed with larotrectinib. CONCLUSION: Our case series supports that larotrectinib may be a therapeutic option for newborn and infant patients with infantile fibrosarcoma, especially in uncommon locations.
Subject(s)
Fibrosarcoma , Sarcoma , Soft Tissue Neoplasms , Infant , Humans , Child , Infant, Newborn , Fibrosarcoma/drug therapy , Fibrosarcoma/genetics , Fibrosarcoma/pathology , Sarcoma/drug therapy , Soft Tissue Neoplasms/drug therapy , Soft Tissue Neoplasms/genetics , Soft Tissue Neoplasms/pathology , Protein Kinase Inhibitors/therapeutic useABSTRACT
BACKGROUND: Survival in patients who have Ewing sarcoma is correlated with postchemotherapy response (tumor necrosis). This treatment response has been categorized as the response rate, similar to what has been used in osteosarcoma. There is controversy regarding whether this is appropriate or whether it should be a dichotomy of complete versus incomplete response, given how important a complete response is for in overall survival of patients with Ewing sarcoma. The purpose of this study was to evaluate the impact that the amount of chemotherapy-induced necrosis has on (1) overall survival, (2) local recurrence-free survival, (3) metastasis-free survival, and (4) event-free survival in patients with Ewing sarcoma. METHODS: In total, 427 patients who had Ewing sarcoma or tumors in the Ewing sarcoma family and received treatment with preoperative chemotherapy and surgery at 10 international institutions were included. Multivariate Cox proportional-hazards analyses were used to assess the associations between tumor necrosis and all four outcomes while controlling for clinical factors identified in bivariate analysis, including age, tumor volume, location, surgical margins, metastatic disease at presentation, and preoperative radiotherapy. RESULTS: Patients who had a complete (100%) tumor response to chemotherapy had increased overall survival (hazard ratio [HR], 0.26; 95% CI, 0.14-0.48; p < .01), recurrence-free survival (HR, 0.40; 95% CI, 0.20-0.82; p = .01), metastasis-free survival (HR, 0.27; 95% CI, 0.15-0.46; p ≤ .01), and event-free survival (HR, 0.26; 95% CI, 0.16-0.41; p ≤ .01) compared with patients who had a partial (0%-99%) response. CONCLUSIONS: Complete tumor necrosis should be the index parameter to grade response to treatment as satisfactory in patients with Ewing sarcoma. Any viable tumor in these patients after neoadjuvant treatment should be of oncologic concern. These findings can affect the design of new clinical trials and the risk-stratified application of conventional or novel treatments.
Subject(s)
Bone Neoplasms , Sarcoma, Ewing , Humans , Sarcoma, Ewing/drug therapy , Sarcoma, Ewing/surgery , Sarcoma, Ewing/pathology , Neoadjuvant Therapy/adverse effects , Bone Neoplasms/pathology , Neoplasm Recurrence, Local/drug therapy , Neoplasm Recurrence, Local/etiology , Necrosis/etiology , Retrospective StudiesABSTRACT
OBJECTIVE: To analyze the incidence and survival of patients with oligometastases (solitary and normal) when they are treated in centers 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 Center (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 centers 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 histiotype of the neoplasm.
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Objetivo: Analizar la incidencia, factores pronósticos, indicación de tratamiento quirúrgico y supervivencia de los pacientes con criterios de oligometástasis óseas con los criterios de solitaria y normal ósea tratados en centros expertos en la resección tumoral de lesiones óseas. Material y método: Análisis retrospectivo de 414 pacientes afectos de metástasis óseas secundarias a carcinomas del Hospital Universitario La Paz y Hospital MD Anderson Cancer Center (Madrid), entre mayo de 2006 y mayo de 2019. Fueron excluidos las metástasis localizadas en pelvis y esqueleto axial, analizándose un total de 28 pacientes que cumplían con el criterio de metástasis solitaria u oligometástasis con criterio normal. La estimación de la supervivencia del estudio se llevó a cabo siguiendo el método estadístico de Kaplan-Meier. Resultados: La supervivencia de los pacientes siguiendo los criterios de oligometástasis (solitaria y normal) fue del 53%. El cáncer de mama fue el más prevalente y presentó una supervivencia superior al 70%. La edad promedio de los pacientes fue de 58 años. Discusión: Los tratamientos sistémicos en el tratamiento del cáncer han conseguido mejorar las curvas de supervivencia libre de enfermedad y nos lleva a reflexionar sobre el paradigma del tratamiento de las oligometástasis, planteando que el tratamiento debería realizarse en centros expertos en la resección tumoral de lesiones óseas. Conclusiones: La elección del tratamiento quirúrgico de los pacientes afectos de oligometástasis en sentido estricto (solitaria) y normal deben ser evaluada por equipos multidisciplinarios, según el pronóstico del paciente, localización anatómica e histiotipo de la neoplasia. Nivel de evidencia 3.(AU)
Objective: To analyze the incidence and survival of patients with oligometastases (solitary and normal) when they are treated in centers 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 Center (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 KaplanMeier 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 centers 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 histiotype of the neoplasm. Level of evidence 3.(AU)
Subject(s)
Humans , Male , Female , Medical Oncology , Neoplasm Metastasis , Neoplasms , Bone and Bones/injuries , Incidence , Prognosis , Survivorship , Orthopedics , Spain , Retrospective Studies , Traumatology , Wounds and Injuries , General SurgeryABSTRACT
Objetivo: Analizar la incidencia, factores pronósticos, indicación de tratamiento quirúrgico y supervivencia de los pacientes con criterios de oligometástasis óseas con los criterios de solitaria y normal ósea tratados en centros expertos en la resección tumoral de lesiones óseas. Material y método: Análisis retrospectivo de 414 pacientes afectos de metástasis óseas secundarias a carcinomas del Hospital Universitario La Paz y Hospital MD Anderson Cancer Center (Madrid), entre mayo de 2006 y mayo de 2019. Fueron excluidos las metástasis localizadas en pelvis y esqueleto axial, analizándose un total de 28 pacientes que cumplían con el criterio de metástasis solitaria u oligometástasis con criterio normal. La estimación de la supervivencia del estudio se llevó a cabo siguiendo el método estadístico de Kaplan-Meier. Resultados: La supervivencia de los pacientes siguiendo los criterios de oligometástasis (solitaria y normal) fue del 53%. El cáncer de mama fue el más prevalente y presentó una supervivencia superior al 70%. La edad promedio de los pacientes fue de 58 años. Discusión: Los tratamientos sistémicos en el tratamiento del cáncer han conseguido mejorar las curvas de supervivencia libre de enfermedad y nos lleva a reflexionar sobre el paradigma del tratamiento de las oligometástasis, planteando que el tratamiento debería realizarse en centros expertos en la resección tumoral de lesiones óseas. Conclusiones: La elección del tratamiento quirúrgico de los pacientes afectos de oligometástasis en sentido estricto (solitaria) y normal deben ser evaluada por equipos multidisciplinarios, según el pronóstico del paciente, localización anatómica e histiotipo de la neoplasia. Nivel de evidencia 3.(AU)
Objective: To analyze the incidence and survival of patients with oligometastases (solitary and normal) when they are treated in centers 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 Center (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 KaplanMeier 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 centers 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 histiotype of the neoplasm. Level of evidence 3.(AU)
Subject(s)
Humans , Male , Female , Medical Oncology , Neoplasm Metastasis , Neoplasms , Bone and Bones/injuries , Incidence , Prognosis , Survivorship , Orthopedics , Spain , Retrospective Studies , Traumatology , Wounds and Injuries , General SurgeryABSTRACT
Introduction: Ameloblastic fibroma is a rare benign mixed odontogenic tumor that usually presents as a painless swelling in young patients. In this article we performed a literature review and present our experience in the management of fibroblastic ameloblastoma in 4 cases. Material and methods: We performed a review of cases published in the literature in PubMed between 2015 and 2022. Regarding our experience, research in the Anatomical Pathology department was done to find cases of ameloblastic fibroma that had been histologically confirmed. Results: The search rendered 29 results, of which only 9 were selected. All articles were single case reports, and most of them were located in the mandible (77,7 %). Most patients underwent enucleation and curettage, and no recurrence were reported by any of the authors. We found 4 cases operated in our department in the last 15 years. Two lesions were located in the maxilla and 2 in the mandible. In all cases, enucleation and curettage was performed, including extraction of the teeth involved, with a very favorable evolution and no recurrences. Discussion: Management can be conservative, performing enucleation and curettage with a very favorable evolution. Variable rates of recurrence have been described and a malignant variant has been identified, so long-term follow-up must be done. In the literature we found only single case reports, with most of the cases presenting in the mandible and undergoing conservative treatment with favorable results. In our series we did not observe recurrences, with good bone regeneration and receiving subsequent orthodontic treatment. (AU)
Introducción: El fibroma ameloblástico es un tumor odontogénico mixto benigno y poco frecuente que generalmente se presenta como un abombamiento indoloro en pacientes jóvenes. En este artículo realizamos una revisión de la literatura y presentamos nuestra experiencia en el manejo del ameloblastoma fibroblástico en 4 casos. Material y métodos: Realizamos una revisión de los casos publicados en la literatura en Pub-Med entre 2015 y 2022. En cuanto a nuestra experiencia, se realizó una búsqueda en el departamento de Anatomía Patológica de los casos de fibroma ameloblástico confirmados histológicamente. Resultados: La búsqueda arrojó 29 resultados, de los cuales solo 9 fueron seleccionados. Todos los artículos fueron reportes de casos únicos, y la mayoría de ellos se localizaron en la mandíbula (77,7 %). La mayoría de los pacientes se sometieron a enucleación y curetaje, y ninguno de los autores informó de recurrencia. Encontramos 4 casos operados en nuestro departamento en los últimos 15 años. Dos lesiones se localizaron en el maxilar y dos en la mandíbula. En todos los casos se realizó enucleación y curetaje, incluyendo exodoncia de los dientes afectados, con evolución muy favorable y sin recidivas. Discusión: El manejo puede ser conservador, realizándose enucleación y curetaje con una evolución muy favorable. Se han descrito tasas variables de recurrencia y se ha identificado una variante maligna, por lo que se debe realizar un seguimiento a largo plazo. En la literatura encontramos solo informes de casos únicos, la mayoría de los casos se presentaron en la mandíbula y se sometieron a un tratamiento conservador con resultados favorables. En nuestra serie no observamos recidivas, con buena regeneración ósea y recibiendo tratamiento de ortodoncia posterior. (AU)
Subject(s)
Humans , Fibroma , Ameloblastoma , Pathology , Mandible , MaxillaABSTRACT
OBJECTIVES: To evaluate the relationship of the immune-checkpoint PD-1/PD-L1 with the clinical evolution of OSCC; to assess survival in OSCC based on the characteristics of TME and histologic risk score; to evaluate the clinical and histopathological relationship of OSCC with immunological TME. MATERIAL AND METHODS: A retrospective study was carried out on 65 samples from patients with OSCC on the floor of the mouth or tongue. Clinicopathological variables and the expression of the biomarkers PD-1, PD-L1, FoxP3, CD4, CD8, CSF1R, and p16 were recorded. The relationship of the clinical and histological variables with the expression of the biomarkers and survival was studied. RESULTS: The univariate and multivariate analysis indicated that positive PD-1 expression was an independent protective factor for survival (overall, disease-free, disease-specific survival) and that high PD-L1 also improved survival. Poorly differentiated histological grades and metastasis were associated with a worse prognosis. CONCLUSIONS: PD-1 is a protective survival factor that is maintained independently of PD-L1 expression. High values of PD-L1 expression also improve survival. Higher expression of PD-1 is observed in smaller tumors, and higher expression of PD-L1 is more likely in women. No relationship between the tumor microenvironment and histologic risk score was found to influence the survival patterns studied in the OSCC. There is no evidence of a relationship between the histopathological features and the studied markers, although the positive PD-1 and PD-L1 cases have a lower risk of a high WPOI score, and positive PD-1 expression was associated with a lower DOI.
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BACKGROUND: Eribulin has shown antitumour activity in some soft tissue sarcomas (STSs), but it has only been approved for advanced liposarcoma (LPS). METHODS: In this study, we evaluated the effect of eribulin on proliferation, migration and invasion capabilities in LPS, leiomyosarcoma (LMS) and fibrosarcoma (FS) models, using both monolayer (2D) and three-dimensional (3D) spheroid cell cultures. Additionally, we explored combinations of eribulin with other drugs commonly used in the treatment of STS with the aim of increasing its antitumour activity. RESULTS: Eribulin showed activity inhibiting proliferation, 2D and 3D migration and invasion in most of the cell line models. Furthermore, we provide data that suggest, for the first time, a synergistic effect with ifosfamide in all models, and with pazopanib in LMS as well as in myxoid and pleomorphic LPS. CONCLUSIONS: Our results support the effect of eribulin on LPS, LMS and FS cell line models. The combination of eribulin with ifosfamide or pazopanib has shown in vitro synergy, which warrants further clinical research.