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.

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.

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)

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.

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.

Excisão de um fibroma de células gigantes com laser cirúrgico / Excisión de un fibroma de células gigantes con láser quirúrgico / Giant-cell fibroma removal with surgical laser

Introdução: O fibroma de células gigantes é uma neoplasia fibrosa benigna, considerada rara, com fatores etiológicos incertos e características clinico-patológicas peculiares. Objetivo: Descrever a exérese do fibroma de células gigantes, em mucosa jugal direita, utilizando laser cirúrgico. Relato de caso: Paciente do sexo feminino, 33 anos, parda, atendida na clínica de Estomatologia da Universidade Estadual da Paraíba, motivada por uma lesão neoplásica, de crescimento lento em região de mucosa jugal direita. Clinicamente, observou-se massa tumoral única, assintomática, com aproximadamente dois centímetros, de base séssil, normocorada, de consistência firme e superfície lisa. Após exame clínico, foi realizada uma biópsia excisional com fins diagnósticos, utilizando o laser cirúrgico. O diagnóstico, após o resultado do exame histopatológico, revelou um fibroma de células gigantes. A abordagem da biópsia excisional, além de ter fins de diagnóstico bucal, foi responsável pelo tratamento da lesão, visto que proporcionou a remoção completa da patologia. Optou-se por cicatrização por segunda intenção, e para acelerar esse processo, foi realizada aplicação local com laser de baixa potência de espectro de luz vermelha. No acompanhamento de sete dias, observou-se cicatrização adequada, com mínima alteração tecidual. Após oito meses, notou-se regeneração tecidual adequada sem recidiva da lesão. Conclusão: A remoção de um fibroma de células gigantes, utilizando laser de diodo de alta potência, se mostrou como uma abordagem terapêutica viável para o tratamento dessa patologia(AU)

Fibroma testicular / Testicular fibroma

El fibroma paratesticular es un proceso proliferativo benigno de origen incierto, probablemente inflamatorio reactivo no tumoral. El objetivo del trabajo es presentar un caso de fibroma paratesticular, así como la secuencia diagnóstica y terapéutica adoptada. Se presenta un paciente de 55 años de edad con antecedentes de hipertensión arterial. Acude a la consulta externa de Urología por presentar aumento de volumen del testículo derecho, de un año de evolución, que en los últimos tres meses le había ocasionado molestias leves. Al interrogatorio no se recogieron antecedentes de traumatismos o inflamaciones en ese testículo. En el examen físico presentó, hacia el polo inferior del testículo y cola del epidídimo derechos, una masa de consistencia pétrea, irregular, de 3 a 4 cm de diámetro, fija e indolora. El ultrasonido testicular reportó la presencia de una imagen compleja, hacia polo inferior, heterogénea hipo e hiperecoica calcificada en su interior, que medía 33 x 18 x 34 mm para 11cm³ de volumen, y con ligero hidrocele asociado. El paciente fue sometido a orquiectomia radical derecha, proceder que transcurrió sin complicaciones. El resultado histopatológico fue proliferación fibrosa nodular e hialinizante que afecta la túnica albugínea, fibroma calcificado del testículo derecho. La evolución posoperatoria fue satisfactoria. Aunque el diagnóstico definitivo de fibroma paratesticular es histopatológico, sus características clínicas y ecográficas lo hacen ser uno de los diagnósticos diferenciales a tener en cuenta, con los tumores malignos del testículo(AU)

Paratesticular fibroma is a benign proliferative process of uncertain origin, probably non-tumor reactive inflammatory. The aim of the study is to present a case of paratesticular fibroma, as well as the diagnostic and therapeutic sequence implemented. We present a 55 year old patient with a history of hypertension. This patient visits the Urology outpatient clinic because of an increase in volume of the right testicle, a year of evolution, which in the last three months had caused slight discomfort. No history of trauma or inflammation was recorded in the testicle. On the physical examination he presented, to the lower pole of the testis and tail of the right epididymis, a mass of irregular stony consistency, 3 to 4 cm in diameter, fixed and painless. The testicular ultrasound reported the presence of a complex image, towards the lower pole, heterogeneous hypo and hyperechoic calcified inside, which measured 33 x 18 x 34 mm for 11 cm3 volume, and with associated light hydrocele. The patient underwent right radical orchiectomy with no complications. The histopathological result was nodular and hyalinizing fibrous proliferation affecting the tunica albuginea, calcified fibroma of the right testicle. Postoperative evolution was satisfactory. Although the definitive diagnosis of paratesticular fibroma is histopathological, its clinical and ultrasound characteristics make it one of the differential diagnoses to be considered with malignant tumors of the testis(AU)

Fibroma osificante juvenil, presentación de un caso clínico y revisión de la literatura / Juvenil ossifying fibroma, case report and review of the literature

Se presenta el caso de una paciente de seis años, de sexo femenino sin antecedentes mórbidos ni familiares de importancia, quien consulta por proptosis izquierda en abril de 2011. Se realiza resonancia magnética de cerebro evidenciándose un tumor de seno etmoidal con compromiso de órbita izquierda que ocasiona exoftalmo. La biopsia endoscópica nasal fue compatible con fibroma osificante juvenil. Posteriormente se realiza antrostomía con etmoidectomía en dos tiempos, con resultados satisfactorios para la paciente. Aunque el fibroma osificante juvenil es un tipo de lesión benigna poco frecuente, puede llegar a ser muy agresiva y con una alta tasa de recidiva en ciertas ocasiones, por lo que se debe tener en cuenta para realizar un diagnóstico y tratamiento precoz, con un seguimiento programado a largo plazo.

This paper presents the case of a patient of six years old female without background or important morbidly in her family, who consults for left-sided proptosis in April of 2011. It performs brain magnetic resonancebeing demonstrated a tumor of theethmoid sinus with commitment to the left orbit that causes exophthalmus. Nasal endoscopic biopsy was compatible with juvenile ossifying fibroma. It was subsequently performed ananthrostomy whitethmoidectomy in two times, with satisfactory results for the patient. Although the Juvenile ossifying fibroma is a rare type of benign lesion, can be very aggressive and have a high recurrence rate in certain occasions, by what should be taken into account to make an early diagnosis and treatment, with a scheduled follow up in the long term.

Multidetector Computed Tomography Findings of a Papillary Fibroelastoma of the Aortic Valve: A Case Report

Papillary fibroelastoma is a rare benign cardiac tumor that represents 10% of all primary cardiac tumors. Diagnosis is accomplished incidentally by echocardiography that is usually performed for another purpose. Most papillary fibroelastomas are asymptomatic, but the lesions are recognized as a cause of embolisms. To the best of our knowledge, there has been no case report of computed tomography findings of a papillary fibroelastoma. We report a case of a papillary fibroelastoma in a 78-yr-old woman who had dyspnea and chest tightness. Echocardiography revealed a small lobulated mobile echogenic mass attached to the aortic valve, and CT demonstrated a lobulated soft tissue density mass with a thin stalk at the sinotubular junction of the aortic valve.

Fascitis nodular en región facial en edades pediàtricas / Nodular fasciitis present in facial area in children

Propósito: Presentar 3 casos de fascitisnodular de región facial. Método: Se realizó un estudio de los pacientes que comprendió examen físico, anàlisis de laboratorio y radiológico. Se describió la conducta quirúrgica o médica realizada en cada caso y su diagnóstico anatomopatológico. Resultados: Se mostró el método científico como única vía de llegar al diagnóstico certero, a pesar de enfrentar el reto de diagnosticar una entidad nosológica inusual en la cirugía maxilofacial. Se discutió la evolución de cada paciente y la literatura consultada. Conclusiones: Resulta muy útil la presentación de estos casos, pues esta tumoración presenta una localización muy poco frecuente en cara y su rápido crecimiento y evolución clínica son habitualmente difíciles de interpretar(AU)

Aim: To present 3 cases of nodular fasciitis in facial area. Method: A study was carried out in patients including physical examination, laboratory and radiologic analysis. Surgical or medical behavior was described present in each case with an anatomical and the pathological diagnosis was made. Results: Scientific method was the only route to achieve an accurate diagnosis despite the challenge of diagnosing an uncommon disease entity in maxillofacial surgery. In each patient the course was discussed as well as a literature review. Conclusions: The presentation of these cases is very essential since this tumor has a not much location in the face and its fast developmental and clinical course are often difficult to interpret(AU)

Elastofibroma dorsi: an under-diagnosed entity -clinical, imaging and pathological features

To report clinical, radiological and histopathological findings of elastofibroma dorsi.Thepurposeis to increase awareness among radiologists and clinicians of this under-diagnosed and interesting benign lesion. Retrospective review of 624 consecutive thoracic Computed Tomography [CT], 92 ultrasounds and 130 Magnetic Resonance Image [MRI] scans for detection of elastofibroma dorsi along with review of the clinical and histopathological findings. Three radiologists evaluated the imaging features using previously definedcriteria. A histopathologist reviewed the pathological findings. A tertiary level hospital and a specialized orthopedic center. Patients who were subjected to CT, Ultrasound and MRI scans of the thorax Interventions: CT, MRI, Ultrasound scans of the thorax and surgical excision. Detection of elastofibromasand their clinical, radiological and histopathologic findings. Only 50% of patients with elastofibrom a dorsicouldbe detected by all modalities though, on review, all lesions showed characteristic location, morphology, imaging as well as typical clinical and pathological features. Significant contrast enhancement of all lesions on MRI was an unusual finding in our study. An awareness of the radiological findingsand enhancement patterns on CT and MRI can help in the proper diagnosis of elastofibroma dorsi an entity often misdiagnosed in a high percentage of patients