RESUMO
Objetivo: Mostrar imagens com diferentes características encontradas nos diagnósticos de lipoblastoma. Relato do Caso: Foram realizadas análises de imagens e quadros clínicos de três crianças menores de 3 anos que apresentaram lesão expansiva de crescimento rápido, cujo diagnóstico histopatológico da lesão foi lipoblastoma, sendo dois do sexo feminino e um masculino, entre os anos 2014-2022. Discussão: O exame por radiografia dasextremidades demonstrou aumento de partes moles sem comprometimento ósseo associado. As tomografias computadorizadas apresentaram formação expansiva heterogênea com densidade predominantemente de gordura, com areas sólidas e císticas. A ressonância permitiu caracterizar com mais precisão a doença, fornecendo o aspecto da mesma, a intensidade de sinal, realce pelo meio de contraste, permitindoavaliação mais precisa dos tecidos de partes moles e áreas císticas. Conclusão: Uma avaliação imagiológica pré-operatória deve ser realizada para determinar a extensão da doença e ajudar no planejamento cirúrgico. A excisão cirúrgica não mutilante permanece como terapêutica de escolha. Ressaltamos através desse relato de casos que, na identificação de um tumor composto principalmente por gordura, deverá ser aventada a hipótese diagnóstica de lipoblastoma na faixa pediátrica
Objective: We aimed to show images with different characteristics found in lipoblastoma diagnoses. Case Report: We performed Images and clinical analysis on three children under 3 years of age, who suffered a rapidly growing, expansive lesion whose histopathological diagnosis of the lesion was lipoblastoma, two of which were female and one male, among the years 2014-2022. Discussion: Radiographic examination of the extremities demonstrated an increase in soft tissues without associated bone involvement. The CT scans showed heterogeneous expansive formation with predominantly fat density, with solid and cystic areas. MRI allowed us to more accurately characterize the disease, providing its appearance and signal intensity, real through the contrast medium, allowing more precise assessment of soft tissue tissues and cystic areas. Conclusion: A preoperative imaging evaluation should be performed to determine the extent of the disease and aid in surgical planning. Non-mutilating surgical excision remains the therapy of choice. We emphasize through this case report that the identification of a tumor composed mainly of fat should point to the diagnostic hypothesis of lipoblastoma in the pediatric population
Assuntos
Humanos , Masculino , Feminino , Radiografia Torácica , Tomografia , Pré-Escolar , Lipoblastoma , NeoplasiasRESUMO
Se informa de un lipoblastoma perineal en una niña de 5 años localizado en el labio mayor derecho. La lesión aumentó gradualmente en 6 meses. En la ecografía y la resonancia magnética (RM), se observó un tumor heterogéneo sólido limitado con componente graso. Tras su extirpación quirúrgica, el estudio anatomopatológico confirmó un lipoblastoma. El lipoblastoma es un tumor mesenquimatoso benigno poco frecuente de la lactancia y la primera infancia. Los síntomas varían en función de la localización; pueden observarse signos de compresión de los órganos adyacentes. Este tipo de tumores inusuales de los tejidos blandos son más frecuentes en menores de 3 años. Los lipoblastomas se localizan predominantemente en las extremidades, pero también pueden encontrarse en la cabeza y el cuello, el tronco, el mediastino, el riñón, el mesenterio, el retroperitoneo y el perineo. Se debe sospechar su presencia en función de los hallazgos de la ecografía y la RM.
We reported a perineal lipoblastoma in a 5-year-old girl located in the right labia mayor. The lesion gradually increased within 6 months. Ultrasound and magnetic resonance imaging (MRI) showed a limited solid heterogenous tumor with fatty component. After it had been surgically removed, the anatomopathological examination confirmed that it was a lipoblastoma. Lipoblastoma is a rare benign mesenchymal tumor of infancy and early childhood. Symptoms vary depending on localization; signs of compression of adjacent organs may be seen. This type of unusual soft tissue tumors occurred most often in under 3 years old. The localization of lipoblastomas is predominantly in the extremities but may be also found in other sites including the head and neck, trunk, mediastinum, kidney, mesentery, retropritoneum and perineum. The suspicion should be considered according to ultrasound and MRI findings.
Assuntos
Humanos , Feminino , Pré-Escolar , Neoplasias de Tecidos Moles , Lipoblastoma/cirurgia , Lipoblastoma/diagnóstico , Lipoblastoma/patologia , Imageamento por Ressonância Magnética , Rim/patologia , Pescoço/patologiaRESUMO
We reported a perineal lipoblastoma in a 5-year-old girl located in the right labia mayor. The lesion gradually increased within 6 months. Ultrasound and magnetic resonance imaging (MRI) showed a limited solid heterogenous tumor with fatty component. After it had been surgically removed, the anatomopathological examination confirmed that it was a lipoblastoma. Lipoblastoma is a rare benign mesenchymal tumor of infancy and early childhood. Symptoms vary depending on localization; signs of compression of adjacent organs may be seen. This type of unusual soft tissue tumors occurred most often in under 3 years old. The localization of lipoblastomas is predominantly in the extremities but may be also found in other sites including the head and neck, trunk, mediastinum, kidney, mesentery, retropritoneum and perineum. The suspicion should be considered according to ultrasound and MRI findings.
Se informa de un lipoblastoma perineal en una niña de 5 años localizado en el labio mayor derecho. La lesión aumentó gradualmente en 6 meses. En la ecografía y la resonancia magnética (RM), se observó un tumor heterogéneo sólido limitado con componente graso. Tras su extirpación quirúrgica, el estudio anatomopatológico confirmó un lipoblastoma. El lipoblastoma es un tumor mesenquimatoso benigno poco frecuente de la lactancia y la primera infancia. Los síntomas varían en función de la localización; pueden observarse signos de compresión de los órganos adyacentes. Este tipo de tumores inusuales de los tejidos blandos son más frecuentes en menores de 3 años. Los lipoblastomas se localizan predominantemente en las extremidades, pero también pueden encontrarse en la cabeza y el cuello, el tronco, el mediastino, el riñón, el mesenterio, el retroperitoneo y el perineo. Se debe sospechar su presencia en función de los hallazgos de la ecografía y la RM.
Assuntos
Lipoblastoma , Neoplasias de Tecidos Moles , Feminino , Humanos , Pré-Escolar , Lactente , Lipoblastoma/diagnóstico , Lipoblastoma/cirurgia , Lipoblastoma/patologia , Imageamento por Ressonância Magnética , Rim/patologia , Pescoço/patologiaRESUMO
PURPOSE: To describe demographic, clinical, diagnostic and therapeutic aspects of pediatric patients with benign adipocytic tumors admitted to a high complexity teaching hospital from 2007 to 2021. METHODS: Retrospective observational descriptive study. Patient information was retrieved from clinical records. A descriptive analysis was carried out for qualitative data and frequencies were calculated for quantitative data. RESULTS: 76 patients were included with a mean age of 7.5 years old where 60.5% were boys. The main symptom was a mass (73.7%) mostly found in the lower limbs (23.6%). Congenital birth defects were identified in 48.6% of the cases. Preoperative imaging was available in 78.9% of the patients allowing characterization of lesions or differential diagnosis. The therapeutic goal was resection with negative margins, which was feasible in all cases except for one case. The histopathological diagnosis was lipoma in 68.4% of the cases followed by lipoblastoma in 13.1%. The mean follow-up period was 17.9 months. 79.7% of the patients were asymptomatic at their last out-patient visit. CONCLUSION: Benign adipocytic tumors constitute a wide spectrum of lesions, which involve diverse anatomic segments from the neural axis to the inguinoscrotal region. The present work contributes to the general understanding of the clinical presentation and differential diagnosis for these infrequent neoplasms.
Assuntos
Lipoblastoma , Masculino , Criança , Humanos , Feminino , Estudos Retrospectivos , Diagnóstico Diferencial , Hospitalização , Hospitais de EnsinoRESUMO
Objetivo: El lipoblastoma es una neoplasia benigna poco común que puede presentarse como un tumor localizado o difuso (lipoblastomatosis). Debido a su rareza, se han publicado, en su mayoría, solo reportes de casos. El objetivo de este estudio fue determinar la tasa de recurrencia local y las complicaciones después de la resección marginal de lipoblastomas ubicados en extremidades y tronco. Materiales y métodos: Se realizó una revisión retrospectiva multicéntrica de los registros de pacientes pediátricos sometidos a extirpación quirúrgica de lipoblastomas en cuatro instituciones, entre 2008 y 2018. Se registraron las siguientes variables: datos demográficos, método diagnóstico, volumen de la lesión, tipo de biopsia, complicaciones, recurrencia y necesidad de procedimientos adicionales. Resultados: Durante el período de estudio, 17 pacientes cumplieron los criterios de inclusión para la evaluación. La media de la edad era de 3.9 años y el 65% eran varones. Las ubicaciones más frecuentes fueron: muslos (n = 9), columna lumbar (n = 2) y glúteos (n = 2). El volumen de masa preoperatorio medio fue de 305,5 cm3 (rango: 10,2-1745,8). La duración media del seguimiento fue de 2.8 años (rango: de 8 meses a 5.6 años). Hubo una recurrencia (5,9%) y una cicatriz retraída en el área glútea como complicación. Ninguno requirió una nueva intervención. Conclusión: La resección quirúrgica marginal de lipoblastomas localizados en el dorso o las extremidades genera una baja tasa de recurrencia a los 2.8 años de seguimiento y mínimas complicaciones. Nivel de Evidencia: IV
Objectives: Lipoblastoma is a rare benign neoplasm that resembles white fat and can occur as a localized (lipoblastoma) or diffuse (lipoblastomatosis) tumor. Due to its rarity, the literature is mostly limited to case reports. The purpose of this study was to determine the local recurrence rate and complications after marginal resection of lipoblastomas located in the extremities or the back. Materials and methods: We performed a multicenter retrospective review of the records of pediatric patients who had undergone surgical excision of lipoblastomas at 4 tertiary care institutions from 2008 to 2018. We recorded the demographic data, diagnostic method, the volume of the lesion, type of biopsy, complications, recurrence, and the need for additional procedures. Results: Throughout the study, 17 patients met the inclusion criteria for evaluation. The average patient age was 3.9 years, and 65% were male. The most common locations included thighs (N 9), low back region (N 2), and buttocks (N 2). The mean preoperative mass volume was 305.5 cm3(range: 10.2 cm3 - 1745.8 cm3). The mean duration of follow-up was 2.8 years (range: 8 months to 5.6 years). One patient experienced recurrence (5.9%). One patient had a retracted skin scarring in the gluteal area. Conclusion: Marginal surgical resection of lipoblastomas located in the back or extremities showed a low recurrence rate at 2.8 years of follow-up and minimal complications. Level of Evidence: IV
Assuntos
Pré-Escolar , Criança , Complicações Pós-Operatórias , Resultado do Tratamento , Extremidades , Tronco , Lipoblastoma/cirurgia , Recidiva Local de NeoplasiaRESUMO
OBJECTIVE: To describe two cases of mediastinal lipoblastoma, an infrequent and little-known patho logy, which is extremely rare in the mediastinum, with no cases reported in our country. CLINICAL CASES: Two case reports. Both patients were boys younger than three years, in which a mediastinal mass was found incidentally on a chest x-ray. The study was complemented with a CT scan and with a thoracoscopic biopsy in one of the cases. Complete resection of the tumor was achieved in both patients through thoracotomy. One of the patients presented Claude Bernard Horner syndrome as a complication from surgery, which resolved spontaneously after two years and the second case had no complications. CONCLUSION: Mediastinal lipoblastoma is a very rare pathology. Descriptions found in existing literature are similar to the cases presented in this article. We can conclude that mediastinal lipoblastomas in pediatrics present a very similar pattern and presentation, having a good prognosis if complete resection is achieved. Also, it is essential to distinguish it from its differential diagnoses in order to rule out malignancy.
Assuntos
Lipoblastoma/diagnóstico por imagem , Neoplasias do Mediastino/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Pré-Escolar , Humanos , Lactente , Lipoblastoma/cirurgia , Masculino , Neoplasias do Mediastino/cirurgia , ToracotomiaRESUMO
Introducción: El lipoblastoma es una neoplasia benigna de presentación infrecuente originada en el tejido adiposo, de presentación casi exclusiva en pacientes pediátricos antes de los tres años de edad, con predominio en el sexo masculino. Se localiza principalmente en las extremidades y tronco, como una tumoración indolora de crecimiento progresivo. El tratamiento de elección es quirúrgico y tiene pronóstico favorable. Las recidivas se presentan en los casos en los que la resección no pudo ser completa. Caso clínico: Lactante mujer de 7 meses de edad. Desde los tres meses de edadpresentó aumento progresivo del volumen de la extremidad inferior izquierda.Al examen físico se evidenciógran tumoraciónde 12 x 7 centímetrosque compromete la cara posterior del muslo izquierdo: Masa indolora y bien delimitada. Sin compromiso de la movilidad, sin edema de miembro. Taller diagnóstico: La Resonancia Magnética reportó unatumoración sólida de muslo izquierdo hiper-intensa en T1 y T2, hipo-intensa en STIR con septos finos en su interior, se extiende desde el musculo abductor magno del bíceps femoral y mide 11.2 x 7.9 x 8.4 cm en sus ejes longitudinal, anteroposterior y transversal respectivamente, desplazando y comprimiendo a los músculos semitendinoso, semimembranoso y gracilis. La paciente fue sometida a escisión completa y amplia de la masa, mediante abordaje posterolateral. Desenlace:Patología reportóun tumor constituido por tejido adiposo con tejido mixoide,sin atipia nuclear; se evidencia infiltración parcial del musculo esquelético adyacente, sin afectación de piel, sin necrosisy bordes quirúrgicos negativos para neoplasia. Inmunohistoquímica con marcador de proliferación celular KI-67 resultado positivo de 1%, y estudio de MDM2 (inhibidor de la activación transcripcional de p53) negativo; hallazgos compatibles con lipoblastoma. El estudio citogenético no fue realizado. Evolución: La paciente fue dada de alta al cuarto día post-operatorio sin complicaciones. Con una recuperación completa, en el quinto mes de seguimiento se realizónuevo estudio de resonancia magnética en la cual no se visualizan imágenes que sugieran tumor residual ni recidiva tumoral. Conclusión: El lipoblastoma debe ser tomada en cuenta como diagnóstico diferencial en niños con tumores de partes blandas, su tratamiento es eminentemente quirúrgico con un buen pronóstico si la extirpación es completa
Introduction: Lipoblastoma is an infrequent benign neoplasm originating in adipose tissue, presenting almost exclusively in pediatric patients before the age of three years, predominantly in males. It is located mainly on the limbs and trunk, as a painless, progressively growing tumor. The treatment of choice is surgical and has a favorable prognosis. Recurrences occur in cases in which the resection could not be complete. Clinical case: 7-month-old female infant. From the age of three months, he presented a progressive increase in the volume of the left lower limb. Physical examination revealed a large mass measuring 12 x 7 centimeters that involves the posterior aspect of the left thigh: a painless and well-defined mass. No compromise of mobility, no limb edema. Diagnostic workshop: Magnetic resonance imaging reported a hyper-intense solid tumor of the left thigh in T1 and T2, hypo-intense in STIR with fine septa inside it, extending from the abductor magnus muscle of the biceps femoris and measuring 11.2 x 7.9 x 8.4 cm in its longitudinal, anteroposterior and transverse axes respectively, displacing and compressing the semitendinosus, semimembranosus and gracilis muscles. The patient underwent a complete and wide excision of the mass using a posterolateral approach. Outcome: Pathology reported a tumor made up of adipose tissue with myxoid tissue, without nuclear atypia; partial infiltration of the adjacent skeletal muscle is evidenced, without skin involvement, without necrosis and negative surgical margins for neoplasia. Immunohistochemistry with a cell proliferation marker KI-67, a positive result of 1%, and a negative MDM2 study (inhibitor of transcriptional activation of p53); findings consistent with lipoblastoma. The cytogenetic study was not performed. Evolution:The patient was discharged on the fourth postoperative day without complications. With a complete recovery, in the fifth month of follow-up a new magnetic resonance study was performed in which no images that suggest residual tumor or tumor recurrence are visualized Conclusion: Lipoblastoma should be taken into account as a differential diagnosis in children with soft tissue tumors, its treatment is eminently surgicalwith a good prognosis if the removal is complete
Assuntos
Humanos , Aberrações Cromossômicas , Lipoblastoma , Lactente , Relatos de CasosRESUMO
Resumen: Objetivo: Describir dos casos de lipoblastoma mediastínico, patología de baja incidencia y poco conocida, siendo aún más infrecuente en mediastino, sin haber reportes descritos en nuestro país. Casos Clínicos: 2 pacientes, ambos de sexo masculino, menores de 3 años, en los que se encontró como ha llazgo incidental una masa mediastínica en una radiografía de tórax. El estudio se complementó con una tomografía computada y en uno de los pacientes con una biopsia toracoscópica. En ambos se logró ex tirpación completa de la lesión vía toracotomía, siendo apoyado vía toracoscópica previa a la extracción de la lesión en uno de ellos. Uno de los pacientes evolucionó con Síndrome de Claude Bernard Horner el cual resolvió de manera espontánea luego de dos años y el segundo caso no presentó complicaciones. Conclusiones: el lipoblastoma mediastínico es una patología muy poco frecuente, las descripciones de la literatura se asemejan a los casos expuestos en este artículo, concluyendo que presentan un patrón muy similar entre ellos. Teniendo un pronóstico favorable si se logra la resección completa y siendo fundamental diferenciarlo de sus diagnósticos diferenciales para descartar malignidad.
Abstract: Objective: To describe two cases of mediastinal lipoblastoma, an infrequent and little-known patho logy, which is extremely rare in the mediastinum, with no cases reported in our country. Clinical Cases: Two case reports. Both patients were boys younger than three years, in which a mediastinal mass was found incidentally on a chest x-ray. The study was complemented with a CT scan and with a thoracoscopic biopsy in one of the cases. Complete resection of the tumor was achieved in both patients through thoracotomy. One of the patients presented Claude Bernard Horner syndrome as a complication from surgery, which resolved spontaneously after two years and the second case had no complications. Conclusion: Mediastinal lipoblastoma is a very rare pathology. Descriptions found in existing literature are similar to the cases presented in this article. We can conclude that mediastinal lipoblastomas in pediatrics present a very similar pattern and presentation, having a good prognosis if complete resection is achieved. Also, it is essential to distinguish it from its differential diagnoses in order to rule out malignancy.
Assuntos
Humanos , Masculino , Lactente , Pré-Escolar , Tomografia Computadorizada por Raios X , Lipoblastoma/diagnóstico por imagem , Neoplasias do Mediastino/diagnóstico por imagem , Toracotomia , Lipoblastoma/cirurgia , Neoplasias do Mediastino/cirurgiaRESUMO
Los tumores óseos y de partes blandas localizados en el pie son muy poco frecuentes. El lipoblastoma es una neoplasia benigna de partes blandas rara que se presenta exclusivamente en la población pediátrica, con predilección por el sexo masculino. Su asiento preferente son las extremidades, pero raramente afecta el pie. Presentamos un caso de lipoblastoma de pie en un niño de 13 meses de edad y una revisión de la bibliografía. Nivel de Evidencia: IV
Bone and soft tissue tumors of the feet are uncommon. Lipoblastoma is a rare benign soft tissue tumor, exclusive to the pediatric population, with predilection for boys. Lipoblastomas most commonly occur in the extremities, but rarely affect the foot. We present a case of lipoblastoma occurring in the foot of a 13-month-old boy, as well as our literature review. Level of Evidence: IV
Assuntos
Lactente , Neoplasias de Tecidos Moles , Lipoblastoma/cirurgia , Pé/patologia , Doenças do PéAssuntos
Lipoblastoma/diagnóstico por imagem , Neoplasias Hepáticas/diagnóstico por imagem , Apetite , Doenças Assintomáticas , Pré-Escolar , Proteínas de Ligação a DNA/genética , Rearranjo Gênico , Humanos , Achados Incidentais , Lipoblastoma/genética , Neoplasias Hepáticas/genética , Masculino , Tomografia Computadorizada por Raios XRESUMO
El lipoblastoma forma parte de los tumores derivados del tejido adiposo. Es una neoplasia benigna que se puede presentar de forma localizada o difusa, que afecta a niños en la primera infancia y que, generalmente, se localiza en el tronco y las extremidades. Estos tumores son poco frecuentes en la edad pediátrica, pero cuando uno de ellos se presenta, constituye un desafío que se debe resolver rápidamente por el riesgo de malignidad que implican otras lesiones neoplásicas, de similares características. El diagnóstico preoperatorio es dificultoso, ya que los métodos por imágenes no aportan información específica que permita diferenciarlos de otros tumores, como los liposarcomas. El diagnóstico definitivo es anatomopalógico y, en casos dudosos, es necesaria la confirmación citogenética. Se presenta el caso de un niño de 14 meses de edad con diagnóstico de lipoblastoma de localización retroperitoneal de gran tamaño.
Lipoblastoma is part of tumors derived from adipose tissue. It is a benign neoplasm that can be localized or diffuse, affecting children in early childhood and usually located in the trunk and extremities. These tumors are uncommon in the pediatric age, but when one of them is present, it is a challenge that we must resolve quickly due to the risk of malignancy that involves other similar neoplastic lesions. The preoperative diagnosis is difficult, because the imaging methods do not provide specific information that allows us to differentiate them from other tumors, such as liposarcomas. The definitive diagnosis is anatomopathological and, in doubtful cases, cytogenetic confirmation is necessary. We present the case of a 14-month-old boy with diagnosis of large sized lipoblastoma of retroperitoneal localization.
Assuntos
Humanos , Masculino , Lactente , Neoplasias Retroperitoneais/patologia , Lipoblastoma/patologia , Carga TumoralRESUMO
Lipoblastoma is part of tumors derived from adipose tissue. It is a benign neoplasm that can be localized or diffuse, affecting children in early childhood and usually located in the trunk and extremities. These tumors are uncommon in the pediatric age, but when one of them is present, it is a challenge that we must resolve quickly due to the risk of malignancy that involves other similar neoplastic lesions. The preoperative diagnosis is difficult, because the imaging methods do not provide specific information that allows us to differentiate them from other tumors, such as liposarcomas. The definitive diagnosis is anatomopathological and, in doubtful cases, cytogenetic confirmation is necessary. We present the case of a 14-month-old boy with diagnosis of large sized lipoblastoma of retroperitoneal localization.
El lipoblastoma forma parte de los tumores derivados del tejido adiposo. Es una neoplasia benigna que se puede presentar de forma localizada o difusa, que afecta a niños en la primera infancia y que, generalmente, se localiza en el tronco y las extremidades. Estos tumores son poco frecuentes en la edad pediátrica, pero cuando uno de ellos se presenta, constituye un desafío que se debe resolver rápidamente por el riesgo de malignidad que implican otras lesiones neoplásicas, de similares características. El diagnóstico preoperatorio es dificultoso, ya que los métodos por imágenes no aportan información específica que permita diferenciarlos de otros tumores, como los liposarcomas. El diagnóstico definitivo es anatomopalógico y, en casos dudosos, es necesaria la confirmación citogenética. Se presenta el caso de un niño de 14 meses de edad con diagnóstico de lipoblastoma de localización retroperitoneal de gran tamaño.
Assuntos
Lipoblastoma/patologia , Neoplasias Retroperitoneais/patologia , Humanos , Lactente , Masculino , Carga TumoralRESUMO
Introducción El lipoblastoma es una neoplasia benigna del tejido adiposo, de presentación infrecuente y casi exclusiva en niños menores de 3 años. Usualmente se presenta en las extremidades como una masa indolora de crecimiento progresivo, estableciéndose su diagnóstico definitivo mediante análisis histológico y citogenético. Objetivo Presentar un caso clínico de lipoblastoma de ubicación inhabitual en una lactante y revisar la literatura al respecto. Caso clínico Lactante mayor de 16 meses, con aumento de volumen abdominal de 6 meses de evolución, asociado a una ingesta alimentaria disminuida, sin otros síntomas. El estudio de imagen reveló una imagen de aspecto lipoideo que comprometía casi la totalidad de la cavidad abdominal, muy sugerente de lipoblastoma, realizándose la resección de un tumor retroperitoneal de 18 cm de diámetro que rechazaba los órganos vecinos. El análisis histológico fue suficiente para confirmar el diagnóstico. En el seguimiento no hubo recidiva. Conclusión Tomando en cuenta la baja frecuencia de esta afección y su inusual presentación, se reporta el caso de esta paciente, para considerarlo dentro del diagnóstico diferencial de masa abdominal en un lactante.
Introduction Lipoblastoma is a benign neoplasia of the adipose tissue. It is a rare conditionand almost exclusively presents in children under 3 years old. It usually occurs in extremities as a painless volume increase of progressive growth, with the definitive diagnosis being established by pathological and cytogenetic analysis. The treatment of choice is complete resection, and follow-up period of up to five years is recommended due to a recurrence of up to 25%. Objective To present an unusual location of this uncommon condition in an infant, and review the related literature. Case report A sixteen-month child with an increase in abdominal growth of six-months progression, associated with a decreased food intake, and with no other symptoms. The imaging study revealed a lipoid-like image compromising almost the entire abdominal cavity, very suggestive of lipoblastoma. A resection was performed on an 18 cm diameter retroperitoneal tumour that rejected the adjacent organs. Histological analysis was enough to confirm diagnosis without the need for cytogenetic analysis. The follow-up showed no recurrence of the disease. Conclusion Given the rarity of this disease and its unusual presentation, we communicate this clinical case, in order to be considered in the differential diagnosis of abdominal mass in chilhood.
Assuntos
Humanos , Feminino , Lactente , Neoplasias Retroperitoneais/diagnóstico , Lipoblastoma/diagnóstico , Neoplasias Retroperitoneais/cirurgia , Neoplasias Retroperitoneais/patologia , Seguimentos , Progressão da Doença , Diagnóstico Diferencial , Lipoblastoma/cirurgia , Lipoblastoma/patologiaRESUMO
INTRODUCTION: Lipoblastoma is a benign neoplasia of the adipose tissue. It is a rare conditionand almost exclusively presents in children under 3 years old. It usually occurs in extremities as a painless volume increase of progressive growth, with the definitive diagnosis being established by pathological and cytogenetic analysis. The treatment of choice is complete resection, and follow-up period of up to five years is recommended due to a recurrence of up to 25%. OBJECTIVE: To present an unusual location of this uncommon condition in an infant, and review the related literature. CASE REPORT: A sixteen-month child with an increase in abdominal growth of six-months progression, associated with a decreased food intake, and with no other symptoms. The imaging study revealed a lipoid-like image compromising almost the entire abdominal cavity, very suggestive of lipoblastoma. A resection was performed on an 18cm diameter retroperitoneal tumour that rejected the adjacent organs. Histological analysis was enough to confirm diagnosis without the need for cytogenetic analysis. The follow-up showed no recurrence of the disease. CONCLUSION: Given the rarity of this disease and its unusual presentation, we communicate this clinical case, in order to be considered in the differential diagnosis of abdominal mass in chilhood.
Assuntos
Lipoblastoma/diagnóstico , Neoplasias Retroperitoneais/diagnóstico , Diagnóstico Diferencial , Progressão da Doença , Feminino , Seguimentos , Humanos , Lactente , Lipoblastoma/patologia , Lipoblastoma/cirurgia , Neoplasias Retroperitoneais/patologia , Neoplasias Retroperitoneais/cirurgiaRESUMO
O lipoblastoma é um tumor mesenquimal raro, composto de lipoblastos que continuam sua proliferação após o período pós-natal e que acometem, predominantemente, a população pediátrica. Apresenta prognóstico excelente, apesar do potencial de invasão local e do crescimento rápido. Os autores relatam o caso de uma paciente pediátrica portadora de volumosa lesão em antebraço direito, ocasionando importante comprometimento funcional do membro acometido. Aspectos relevantes no diagnóstico diferencial e manejo são discutidos neste trabalho, visto tratar-se de lesão com potencial risco incapacitante futuro, caso não manejada corretamente.
Lipoblastoma is a rare mesenchymal tumor occurring primarily in pediatric patients and formed by lipoblasts that proliferate after the postnatal period. Despite its potential for local invasion and rapid growth, its prognosis is excellent. In this study, we report the case of a pediatric patient with a ponderous lesion in the right forearm that caused considerable functional impairment of the affected limb. We also discuss the relevant aspects concerning the differential diagnosis and management of the disease, as it has the potential to cause incapacity without proper treatment.
Assuntos
Humanos , Feminino , Lactente , História do Século XXI , Neoplasias de Tecidos Moles , Cirurgia Plástica , Ferimentos e Lesões , Literatura de Revisão como Assunto , Tecido Adiposo , Adipócitos , Estudo de Avaliação , Lipoma , Neoplasias de Tecidos Moles/cirurgia , Neoplasias de Tecidos Moles/patologia , Cirurgia Plástica/métodos , Ferimentos e Lesões/cirurgia , Tecido Adiposo/embriologia , Tecido Adiposo/metabolismo , Adipócitos/fisiologia , Adipócitos/metabolismo , Lipoblastoma , Lipoblastoma/cirurgia , Lipoblastoma/patologia , Lipoma/cirurgia , Lipoma/patologiaRESUMO
Lipoblastoma and lipoblastomatosis are rare benign soft-tissue tumoral lesions resembling fetal adipose tissue. A total of 16 cases of lipoblastoma of the neck were reported in the literature, and only 3 were described in the posterior side of the neck. Hibernoma is a rare benign adipose tumor composed of brown fat cells and only about ten cases occurring in the cervical area have been reported. We reported two rare cases of adipose tissue tumors. The first case was a male infant aged 12 months who had a cervical mass on the posterior side of the neck. He underwent a complete resection of the lesion and the pathologic study revealed lipoblastomatosis. The second case was a 36-year-old man with an anterior cervical mass, which moved with swallowing. A resection was made and the histological analysis showed hibernoma.
Assuntos
Neoplasias de Cabeça e Pescoço/patologia , Lipoblastoma/patologia , Lipoma/patologia , Adulto , Biópsia por Agulha Fina , Diagnóstico Diferencial , Humanos , Lactente , MasculinoRESUMO
Lipoblastoma is a rare benign tumor arising from embryonic white fat and occurs exclusively in infants and children. This neoplasia has an excellent prognosis but its growth rate can be rapid and reach a large size. In veterinary literature lipoblastoma has been reported only in a new born (2 day old) male calf. This case report describes the clinical, histochemical and immunohistochemical features of a lipoblastoma in a dog. The dog was followed up for 20 months after surgery and found to be healthy with no signs of recurrence.(AU)