Epithelioid Angiomyolipoma in a Child—A Diagnostic Dilemma

Gupta, Rakesh Kumar; Sharma, Mehar Chand; Chitragar, Sanjeev; Sinha, K.; Agarwala, Sandeep; Sharma, Raju

doi:10.1016/j.urology.2013.12.044

Cited by 5 publications

(3 citation statements)

References 12 publications

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“…Malignant tumours such as renal‐cell carcinoma or renal medullary carcinoma can be detected in this age group 7 . This can explain the general attitude to perform an open radical nephrectomy, like we observed in all cases previously described 8–10 and in which histological diagnosis was only later refined as PEComas. This is the reason why we suggest to perform a percutaneous cutting needle biopsy (PCNB) in selected cases with the purpose to avoid the risk of an over‐treatment (benign tumours) or an under‐treatment (malignant tumours) either in terms of chemotherapy or to the extent of surgery.…”

Section: Discussionsupporting

confidence: 69%

Individualized robotic organ‐sparing surgery in a paediatric case of renal perivascular epithelioid cell tumour

Silveri

Natali

Cobellis³

et al. 2020

Robotics Computer Surgery

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Background: Perivascular epithelioid cell tumours (PEComas) represent a rare group of mesenchymal tumours with an unpredictable outcome. They are usually observed in middle-aged women whilst they are very rare in children, with limited cases described. Due to the rare anatomical location in kidneys, a correct diagnosis and treatment is really challenging. Methods: We observed and treated a case of kidney's PEComa in a 14-year-old boy. Results: An individualized approach based on anatomical (3D CT-reconstructions) and histopathological (US-guided preliminary biopsy) features lead us to perform a successful robotic-sparing surgery enabling the preservation of two-thirds of the kidney involved. Conclusions: A meticulous preoperative planning in selected patients can lead to a minimally invasive approach even in some paediatric kidney's neoplasms.

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Section: Discussionsupporting

confidence: 69%

Individualized robotic organ‐sparing surgery in a paediatric case of renal perivascular epithelioid cell tumour

Silveri

Natali

Cobellis³

et al. 2020

Robotics Computer Surgery

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“…Epithelioid angiomyolipoma (EAML) is a rare variant of AML tumours in the kidney with a mean age of diagnosis being 38 years 1 . Only a few cases of EAML have been diagnosed in children and the youngest reported in the literature was a four-year-old girl with a tuberous sclerosis (TS) 2 . In this paper, we present a case of a 30 month old male with EAML and idiopathic chylothorax, the youngest case reported to our knowledge date in the literature.…”

Section: Introductionmentioning

confidence: 99%

A large epithelioid angiomyolipoma of the kidney in a very young child with idiopathic chylothorax: A diagnostic dilemma

Kakaje¹,

Mujahed²,

Hamdan³

et al. 2020

Urology Case Reports

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Epithelioid angiomyolipoma (EAML) is a subtype of angiomyolipoma, the most common benign renal tumour and usually asymptomatic until adulthood.This paper reports a patient with EAML identified at 30 months, the youngest age of presentation with this tumour reported in the literature. In this case the tumour was extremely large andwas successfully surgically removed. Furthermore, the patient had no other TS features, presented with acute symptoms, and had bilateral idiopathic chylothorax which was managed by octreotide and diet.

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“…La otra forma de presentación está asociada a la esclerosis tuberosa y corresponde al 20%, siendo más frecuente en hombres jóvenes con compromiso renal bilateral y multifocal [2][3][4][5] . En la literatura científica se encuentran escasos reportes de esta entidad en la población pediátrica, en las cuales no se observa predilección por sexo y edad 6,7 .…”

Section: Introductionunclassified

Angiomiolipoma renal epitelioide: reporte de un caso y revisión de la literatura

Lomonaco

Barreto

Daniels

2016

Urología Colombiana

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ResumenIntroducción: El angiomiolipoma renal epitelioide es una entidad infrecuente. Se presenta en el 0,3% de la población general y corresponde al 3% de las masas renales sólidas. Su comportamiento clínico es agresivo, de aparición esporádica; predomina en el sexo femenino en la tercera década de la vida. Puede asociarse a esclerosis tuberosa (20%), y en tal caso se observa una tendencia a la bilateral y en el sexo masculino. Materiales y métodos: Mujer de 49 años que consulta por síntomas urinarios irritativos y polaquiuria. Recibe tratamiento, sin mejoría. La tomografía de abdomen y pelvis simple y contrastada evidencia lesión sólida bien definida en el contorno lateral del riñón derecho de 31 × 21 mm. Se realiza nefrectomía parcial, con conservación del parénquima residual, el cual se encontraba indemne. Resultados: El estudio histopatológico reporta angiomiolipoma renal epitelioide, confirmado por inmunohistoquímica, con positividad para actina de músculo liso, HMB-45 y CD 117. Discusión: Los angiomiolipomas renales epitelioides son una variante poco común del angiomiolipoma clásico. Son considerados hamartomas que, debido a sus características histológicas, pertenecen a la familia de los denominados PEComas (células epitelioides perivasculares). Macroscópicamente son masas únicas de tamaño mediano, color amarillo con áreas hemorrágicas, localizadas hacia la corteza renal. Se constituyen por tejido adiposo maduro de configuración epitelioide, músculo liso y vasos sanguíneos irregulares, infiltrando el parénquima renal, lo cual fue evidenciado en el caso actual. Conclusión: El angiomiolipoma epitelioide es un tumor de curso clínico insidioso, único y bien delimitado, con pronóstico desfavorable. El tratamiento está supeditado a la evolución clínica.

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Epithelioid Angiomyolipoma in a Child—A Diagnostic Dilemma

Cited by 5 publications

References 12 publications

Individualized robotic organ‐sparing surgery in a paediatric case of renal perivascular epithelioid cell tumour

Individualized robotic organ‐sparing surgery in a paediatric case of renal perivascular epithelioid cell tumour

A large epithelioid angiomyolipoma of the kidney in a very young child with idiopathic chylothorax: A diagnostic dilemma

Angiomiolipoma renal epitelioide: reporte de un caso y revisión de la literatura

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