Elastofibroma Dorsi: experience of a single center.

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.

Fibroma of the Patellar Tendon Sheath: A New Paradigm / Fibroma da bainha do tendão patelar: Um novo paradigma

Abstract The authors present a case of fibroma of the tendon sheath with intra-articular location in the knee, more specifically in the infrapatellar fat; with this specific location, this is the fourth case described of an entity that rarely affects large joints. Clinical and epidemiologi-cal aspects, but especially the imaging findings on magnetic resonance imaging scans, are essential for the differential and definitive diagnosis, which was nevertheless established only after a histological study of the excised mass by miniarthrotomy.

Resumo Os autores apresentam um caso de um fibroma da bainha de tendão com localização intra-articular no joelho e origem na gordura infrapatelar. Esta localização específica é extremamente rara, sendo este o quarto caso descrito de uma entidade que raramente afeta grandes articulações. Para o seu diagnóstico aspetos clínicos, epidemiológicos e sobretudo achados imagiológicos da ressonância magnética são fundamentais. Neste caso o diagnóstico definitvo foi apenas estabelecido após estudo histológico da massa excisada por mini-artrotomia.

Giant lipofibromatous hamartoma of median nerve in a child

Lipofibromatous hamartoma (LFH) is a rare fibrofatty tumor of adipocytes within peripheral nerves, affecting mainly children. It typically presents as a palpable mass surrounding the nerves of the upper limbs, causing pain and neurological deficits in the affected nerve distribution. We report the case of a child with a 2-years presentation of a mass in the right wrist associated with pain and paresthesia, who underwent investigation with magnetic resonance imaging (MRI). It showed thickening of the median nerve with spaghetti-like appearance associated with lipomatous tissue in a coaxial cable-like pattern, both features characteristic of LFH. This case illustrates the importance of MRI in the differential diagnosis of limb masses in the pediatric population.

Giant Right Ventricular Fibroma: Prenatal Diagnosis and Partial Resection in Early Infancy.

Congenital cardiac fibromas are very rare and prenatal diagnosis has been reported in just a few cases. We describe a four-month-old infant presenting a symptomatic giant right ventricular fibroma discovered during prenatal scanning at 33 weeks of gestation, which was confirmed after delivery on echocardiogram and cardiac magnetic resonance imaging. Due to progressive hemodynamic deterioration, partial surgical resection was performed and the patient recovered uneventfully. We report the successful management during early infancy of a giant cardiac fibroma prenatally diagnosed.

Tumores cardíacos pediátricos: características de imagens ecocardiográficas / Pediatric cardiac tumors: echocardiographic imaging features

Tumores cardíacos (TC) em crianças são lesões muito raras, mas seu diagnóstico é fundamental para a conduta adotada para o paciente. A ecocardiografia é a modalidade de imagem cardiovascular mais utilizada na prática clínica para o diagnóstico inicial de TC em pacientes pediátricos. Conhecer as características ecocardiográficas das TCs pode possibilitar um diagnóstico cada vez mais precoce e a identificação de sua etiologia mais provável. Os TCs primários e benignos são os mais frequentes na população pediátrica. Entre os TCs benignos, os mais frequentes em fetos e neonatos são rabdomiomas e teratomas. Em crianças e adolescentes, rabdomiomas e fibromas são os mais comuns. Neste artigo, descrevemos as características ecocardiográficas dos principais TCs em idades pediátricas.(AU)

Cardiac tumors (CTs) in children are very rare, but their diagnosis is crucial for patient management. Echocardiography is the most commonly used cardiovascular imaging modality in clinical practice for the initial diagnosis of CTs in pediatric patients. Knowing the echocardiographic characteristics of CTs can enable an increasingly early diagnosis and the identification of its most likely etiology. Primary and benign CTs are the most frequent types in the pediatric population. Among benign CTs, the most frequent in fetuses and neonates are rhabdomyomas and teratomas. In children and adolescents, rhabdomyomas and fibromas are more common. Here we describe the echocardiographic characteristics of the most common CTs in pediatric patients.(AU)

Acompanhamento a longo prazo de paciente do sexo masculino com fibroma cardíaco assintomático / Long-Term follow-up of an asymptomatic man with a cardiac fibroma

Descreve-se o caso de um homem de 19 anos assintomático com fibroma de ventrículo esquerdo em acompanhamento por 15 anos, sem tratamento.(AU)

Here we describe a case of a 19-year-old asymptomatic man with a left ventricular fibroma on follow-up for 15 years with no treatment required.(AU)

Fibroma Cardíaco: seguimento a longo prazo por ressonância magnética cardíaca / Cardiac Fibroma: long-term follow-up by cardiac magnetic resonance

As neoplasias cardíacas primárias são raras, e o diagnóstico correto é essencial para planejar o tratamento mais adequado. O objetivo deste estudo foi demonstrar o papel da ressonância magnética cardíaca na avaliação, no diagnóstico e no acompanhamento de fibroma cardíaco. Paciente do sexo feminino, 21 anos, com massa miocárdica ao ecocardiograma. Realizou ressonância magnética com diagnóstico de fibroma cardíaco. Foi acompanhada durante 6 anos com estabilidade do quadro. Fibromas cardíacos correspondem à segunda neoplasia mais comum em crianças e jovens. À ressonância magnética, caracterizam-se por realce tardio intenso e homogêneo.(AU)

Central odontogenic fibroma: an international multicentric study of 62 cases.

OBJECTIVE: The aim of this study was to report the clinicopathologic features of 62 cases of central odontogenic fibroma (COdF). STUDY DESIGN: Clinical and radiographic data were collected from the records of 13 oral pathology laboratories. All cases were microscopically reviewed, considering the current World Health Organization classification of tumors and were classified according to histopathologic features. RESULTS: There were 43 females and 19 males (average age 33.9 years; range 8-63 years). Clinically, COdF lesions appeared as asymptomatic swellings, occurring similarly in the maxilla (n = 33) and the mandible (n = 29); 9 cases exhibited palatal depression. Imaging revealed well-defined, interradicular unilocular (n = 27), and multilocular (n = 12) radiolucencies, with displacement of contiguous teeth (55%) and root resorption (46.4%). Microscopically, classic features of epithelial-rich (n = 33), amyloid (n = 10), associated giant cell lesion (n = 7), ossifying (n = 6), epithelial-poor (n = 3), and granular cell (n = 3) variants were seen. Langerhans cells were highlighted by CD1a staining in 17 cases. Most patients underwent conservative surgical treatments, with 1 patient experiencing recurrence. CONCLUSIONS: To the best of our knowledge, this study represents the largest clinicopathologic study of COdF. Most cases appeared as locally aggressive lesions located in tooth-bearing areas in middle-aged women. Inactive-appearing odontogenic epithelium is usually observed within a fibrous/fibromyxoid stroma, occasionally exhibiting amyloid deposits, multinucleated giant cells, or granular cells.

Fibromatosis mamaria. Reporte de dos casos clínicos y revisión bibliogáfica / Mammary fibromatosis. Two case report and review of literatura

RESUMEN Introducción y objetivos: La fibromatosis produce tumores benignos pero localmente agresivos, que afectan a los tejidos blandos. A nivel mamario, representa tan sólo el 0.2% de las neoplasias de la mama. Nuestro objetivo con el presente artículo es profundizar en el conocimiento de la fibromatosis mamaria, a través del estudio de dos casos clínicos, mostrando sus características clínico-radiológicas e histológicas, e intentar establecer un protocolo de actuación adecuado. Métodos: Estudio retrospectivo de dos casos clínicos de fibromatosis mamaria diagnosticados en el Hospital Universitario La Paz entre los años 2018 y 2019. Resultados: Presentaremos dos pacientes con diagnóstico de fibromatosis mamaria, ambas debutaron con la autopalpación de un nódulo mamario. Al realizarles una ecografía, se visualizó un nódulo sólido, mal definido y axila ecográficamente negativa, que precisó de biopsia-aspiración con aguja gruesa. En los dos casos, se decidió resección quirúrgica de la lesión. Seguimiento mediante exploración mamaria y pruebas de imagen periódicas. Conclusiones: Aunque se trata de una entidad benigna, la fibromatosis mamaria puede simular un proceso maligno, tanto clínica como radiológicamente, por lo que precisa de un estudio histológico. A pesar de que la diseminación metastásica es muy poco frecuente, no se debe olvidar el carácter agresivo a nivel local de esta patología, y sus altas tasas de recurrencia. Como tratamiento, se debe realizar una resección quirúrgica, aunque recientemente se ha contemplado la opción de vigilancia estrecha sin tratamiento. No existe evidencia científica que justifique la utilización de otros tratamientos como la radioterapia o el tratamiento hormonal.

ABSTRACT Introduction and objectives: Fibromatosis produces benign but locally aggressive tumours that affect soft tissues. At breast level, it represents only 0.2% of breast neoplasms. Our goal with this article is to increase knowledge on breast fibromatosis, through the study of two clinical cases; explaining their clinical-radiologic and histological characteristics. Additionally, try to establish an adequate protocol, for the management of the disease and for its subsequent monitoring. Methods: A retrospective study about two clinical cases of breast fibromatosis diagnosed in La Paz Hospital between 2018-2019. Results: both patients presented with clinical manifestations, autopalpation of a breast nodule. A breast ultrasound was performed and a solid nodule was visualized, with poorly defined edges and ecographically negative armpit. A core needle biopsy was performed to confirm the histological diagnosis. In both clinical cases, the treatment was surgical resection of the lesion. Periodic revisions are being performed in order to exclude recurrence. Conclusions: Although it is a benign disease, breast fibromatosis can simulate a malignancy, both in a clinical and radiological way, so histological study is mandatory in order to achieve an accurate diagnosis. Even metastatic dissemination is extremely rare, the local aggressive nature and high rates of recurrence for fibromatosis makes surgical excision, with wide free margins, the most important tool in treatment, although the possibility of close surveillance without treatment is recently being contemplated. There is no scientific evidence to justify the use of other treatments such as radiotherapy or hormonal treatment.

Tumor desmoides cervical: reporte de caso / Cervical desmoid tumor: case report

El tumor desmoides es un tumor benigno raro, de origen fibroblastico no inflamatorio, algunas veces referido como fibromatosis no agresiva. Su etiología aún no está completamente clara, sin embargo, se asocia habitualmente a trauma previo y/o procedimientos quirúrgicos. La ubicación más frecuente es abdominal, el cual posee características imagenologicas claras, al igual que su presentación musculo esquelética plantar. La presentación fuera de estos lugares es infrecuente y difícil de diagnosticar. Presentamos un caso de un tumor desmoides cervical que genero un gran desafío diagnóstico, identificando sus claves diagnósticas y realizando una revisión de la bibliografía al respecto para esta ubicación.

Desmoid tumours are a rare benign tumour of fibroblastic non inflammatory origin, sometimes referred as non aggressive fibromatosis.The etiology is not yet completely clear, however, it is usually associated with previous trauma and / or surgical procedures. The most frequent location is in the abdomen, which has typical images characteristics, as well as its skeletal muscle presentation at the plantar level. The presentation outside these places is infrequent and difficult to diagnose. We present a case of a cervical desmoid tumour that generated a great diagnostic challenge, identifying its key imaging characteristics and performing a literature review of the bibliography regarding this location.

[Embolic stroke secondary to a mitral valve fibroelastoma. Case report]. / Accidente cerebrovascular embólico secundario a fibroelastoma papilar de válvula mitral.

Embolic stroke secondary to cardiac tumors is uncommon. However, 25-30% of cardiac tumors may cause systemic emboli. We report a 29-year-old male consulting for a sudden episode of aphasia and right hemiparesis, compatible with infarct of the left middle cerebral artery territory. Transthoracic echocardiography reported an ovoid tumor of 8 × 7 × 7 mm in relation to the sub valvular apparatus of the mitral valve. After neurologic stabilization, surgical treatment was performed. Approached by median sternotomy and in cardiopulmonary bypass, the mitral valve was explored. A macroscopic tumor consistent with a papillary fibroelastoma curled in sub valvular chordae was found. It was deployed and resected from its base, while the anterior mitral leaflet was preserved intact. Histopathological examination confirmed the intraoperative macroscopic diagnosis. The patient recovered uneventfully postoperatively and was discharged on the fifth day after surgery. He currently is in functional capacity I without cardiovascular symptoms at five years follow-up.

Tumores cardiacos primarios fetales: revisión de la literatura / Primary fetal cardiac tumors: review of the literature

Los tumores cardiacos primarios fetales representan una entidad de baja incidencia en la práctica clínica. La gran mayoría corresponde a lesiones benignas, siendo el rabdomioma el tipo más común. Si bien, es frecuente la regresión espontánea de este tipo de lesiones durante el embarazo, los tumores cardiacos fetales pueden asociarse a complicaciones como obstrucción del flujo cardíaco, insuficiencia valvular, arritmias, insuficiencia cardíaca e hidrops fetal, pudiendo conducir incluso a la muerte fetal. El mayor desarrollo de técnicas imagenológicas ha permitido un aumento en el número y precisión de los diagnósticos prenatales de tumores cardiacos, generando al mismo tiempo, nuevos desafíos y alternativas en relación al abordaje terapéutico. El objetivo del presente artículo de revisión es exponer la evidencia actual en relación al diagnóstico prenatal, manejo, complicaciones y condiciones asociadas de los tumores cardiacos fetales más frecuentes.

Fetal primary cardiac tumors represent a low incidence entity in clinical practice. The vast majority corresponds to benign lesions, with rhabdomyoma being the most common type. Although spontaneous regression of this type of lesions during pregnancy is frequent, fetal cardiac tumors can be associated with complications such as obstruction of cardiac flow, valvular insufficiency, arrhythmias, heart failure and fetal hydrops, which can even lead to fetal death. The greater development of imaging techniques has allowed an increase in the number and precision of prenatal diagnoses of cardiac tumors, generating at the same time, new challenges and alternatives in relation to the therapeutic approach. The objective of this review article is to present the current evidence regarding the prenatal diagnosis, management, complications and associated condition s of the most frequent fetal cardiac tumors.

Fibromixoma Odontogénico Agresivo en Maxila: Abordaje Quirúrgico / Aggressive Odontogenic Fibromixoma in Maxilla: Surgical Approach

RESUMEN: El fibromixoma odontogénico (FM) es una infrecuente lesión benigna que muestra un comportamiento invasivo. Considerada una variante del mixoma odontogénico, presenta un origen mesenquimal controvertido y ligera predilección por el sexo femenino. La lesión está asociada a zonas con estructuras dentales adyacentes, siendo su localización más frecuente la región mandibular posterior. El objetivo de este trabajo fue mostrar un caso agresivo de FM en una paciente, localizado en la zona posterior de maxila, destacando su abordaje quirúrgico. Se reporta un caso de una mujer de 52 años, la cual acudió a la consulta por presentar una masa indolora, firme a la palpación en el lado derecho de la maxila. Radiográficamente se observaba como una imagen radiopaca, de márgenes mal definidos que se extendía en el seno maxilar ipsilateral; la tomografía computarizada pudo evidenciar la expansión de las paredes del antro y la fosa nasal, con compromiso del cigomático y el piso de órbita. Se realizó biopsia incisional y el diagnóstico histopatológico fue de FM. El tratamiento de elección consistió en la resección en bloque, mediante abordaje Weber-Fergusson, acompañada de reconstrucción con placa y malla de titanio para garantizar la suspensión del globo ocular; un nuevo estudio histopatológico, confirmó el diagnóstico inicial. Al año del procedimiento quirúrgico no se evidenció recidiva, observando que el material de reconstrucción mantiene su posición, proporcionado estética y funcionabilidad a la paciente.

ABSTRACT: Odontogenic fibromyxoma (FM) is an infrequent benign lesion that shows with invasive characteristics. Considered a variant of the odontogenic myxoma, it presents a controversial mesenchymal origin and is somewhat more frequent in women. The lesion is associated with areas with adjacent dental structures, with its most frequent location in the posterior mandibular region. The objective of this study was to show an aggressive case of FM in a female, located in the posterior area of the maxilla, emphasizing its surgical approach. A case of a 52-year-old woman, who came to the clinic because of a painless, firm mass on the right side of the maxilla. Radiographically, it was seen as a radiopaque image, with poorly defined margins extending in the ipsilateral maxillary sinus; computed tomography showed the expansion of the walls of the antrum and the nasal cavity, with zygomatic compromise and the orbital floor. An incisional biopsy was performed and the histopathological diagnosis was FM. The treatment of choice consisted of block resection, using a WeberFerguson approach, along with reconstruction with a titanium plaque and mesh to guarantee suspension of the eyeball; a new histopathological study, confirmed the initial diagnosis. One year after the surgical procedure, there is no recurrence, and the reconstruction material maintains its position, providing aesthetic and functionality to the patient.

Accidente cerebrovascular embólico secundario a fibroelastoma papilar de válvula mitral / Embolic stroke secondary to a mitral valve fibroelastoma: case report

Embolic stroke secondary to cardiac tumors is uncommon. However, 25-30% of cardiac tumors may cause systemic emboli. We report a 29-year-old male consulting for a sudden episode of aphasia and right hemiparesis, compatible with infarct of the left middle cerebral artery territory. Transthoracic echocardiography reported an ovoid tumor of 8 × 7 × 7 mm in relation to the sub valvular apparatus of the mitral valve. After neurologic stabilization, surgical treatment was performed. Approached by median sternotomy and in cardiopulmonary bypass, the mitral valve was explored. A macroscopic tumor consistent with a papillary fibroelastoma curled in sub valvular chordae was found. It was deployed and resected from its base, while the anterior mitral leaflet was preserved intact. Histopathological examination confirmed the intraoperative macroscopic diagnosis. The patient recovered uneventfully postoperatively and was discharged on the fifth day after surgery. He currently is in functional capacity I without cardiovascular symptoms at five years follow-up.