Cargando…

Giant bilateral angiomyolipomas with spontaneous hemorrhage and inferior vena cava thrombosis in a patient with tuberous sclerosis

Renal angiomyolipomas are rare type of benign renal neoplasm. They are composed of vascular, smooth and fat elements and can be associated to phacomatosis as Tuberous Sclerosis disease. Symptomatic presentation is most frequently spontaneous retroperitoneal hemorrhage, which can be fatal. The risk o...

Descripción completa

Detalles Bibliográficos
Autores principales: Menany, Meriem, Khnaba, Safae, Radouane, Bouchaib, Jidal, Mohamed, Amil, Touriya, Saouab, Rachida
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The African Field Epidemiology Network 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5622810/
https://www.ncbi.nlm.nih.gov/pubmed/28979625
http://dx.doi.org/10.11604/pamj.2017.27.223.8379
_version_ 1783267992525602816
author Menany, Meriem
Khnaba, Safae
Radouane, Bouchaib
Jidal, Mohamed
Amil, Touriya
Saouab, Rachida
author_facet Menany, Meriem
Khnaba, Safae
Radouane, Bouchaib
Jidal, Mohamed
Amil, Touriya
Saouab, Rachida
author_sort Menany, Meriem
collection PubMed
description Renal angiomyolipomas are rare type of benign renal neoplasm. They are composed of vascular, smooth and fat elements and can be associated to phacomatosis as Tuberous Sclerosis disease. Symptomatic presentation is most frequently spontaneous retroperitoneal hemorrhage, which can be fatal. The risk of bleeding is proportional to the size of the lesion (>4 cm of diameter). Typical angiomyolipomas are benign but may have alarming properties: nuclear pleomorphism and mitotic activity, extension into the vena cava, and spread to regional lymph nodes without malignant progression. We report a Computed Tomography finding of a rare giant bilateral angiomyolipomas with spontaneous hemorrhage and inferior vena cava thrombus in a patient with tuberous sclerosis, emphasizing the importance of imagery in the positive and etiologic diagnosis.
format Online
Article
Text
id pubmed-5622810
institution National Center for Biotechnology Information
language English
publishDate 2017
publisher The African Field Epidemiology Network
record_format MEDLINE/PubMed
spelling pubmed-56228102017-10-04 Giant bilateral angiomyolipomas with spontaneous hemorrhage and inferior vena cava thrombosis in a patient with tuberous sclerosis Menany, Meriem Khnaba, Safae Radouane, Bouchaib Jidal, Mohamed Amil, Touriya Saouab, Rachida Pan Afr Med J Case Report Renal angiomyolipomas are rare type of benign renal neoplasm. They are composed of vascular, smooth and fat elements and can be associated to phacomatosis as Tuberous Sclerosis disease. Symptomatic presentation is most frequently spontaneous retroperitoneal hemorrhage, which can be fatal. The risk of bleeding is proportional to the size of the lesion (>4 cm of diameter). Typical angiomyolipomas are benign but may have alarming properties: nuclear pleomorphism and mitotic activity, extension into the vena cava, and spread to regional lymph nodes without malignant progression. We report a Computed Tomography finding of a rare giant bilateral angiomyolipomas with spontaneous hemorrhage and inferior vena cava thrombus in a patient with tuberous sclerosis, emphasizing the importance of imagery in the positive and etiologic diagnosis. The African Field Epidemiology Network 2017-07-25 /pmc/articles/PMC5622810/ /pubmed/28979625 http://dx.doi.org/10.11604/pamj.2017.27.223.8379 Text en © Meriem Menany et al. http://creativecommons.org/licenses/by/2.0/ The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Menany, Meriem
Khnaba, Safae
Radouane, Bouchaib
Jidal, Mohamed
Amil, Touriya
Saouab, Rachida
Giant bilateral angiomyolipomas with spontaneous hemorrhage and inferior vena cava thrombosis in a patient with tuberous sclerosis
title Giant bilateral angiomyolipomas with spontaneous hemorrhage and inferior vena cava thrombosis in a patient with tuberous sclerosis
title_full Giant bilateral angiomyolipomas with spontaneous hemorrhage and inferior vena cava thrombosis in a patient with tuberous sclerosis
title_fullStr Giant bilateral angiomyolipomas with spontaneous hemorrhage and inferior vena cava thrombosis in a patient with tuberous sclerosis
title_full_unstemmed Giant bilateral angiomyolipomas with spontaneous hemorrhage and inferior vena cava thrombosis in a patient with tuberous sclerosis
title_short Giant bilateral angiomyolipomas with spontaneous hemorrhage and inferior vena cava thrombosis in a patient with tuberous sclerosis
title_sort giant bilateral angiomyolipomas with spontaneous hemorrhage and inferior vena cava thrombosis in a patient with tuberous sclerosis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5622810/
https://www.ncbi.nlm.nih.gov/pubmed/28979625
http://dx.doi.org/10.11604/pamj.2017.27.223.8379
work_keys_str_mv AT menanymeriem giantbilateralangiomyolipomaswithspontaneoushemorrhageandinferiorvenacavathrombosisinapatientwithtuberoussclerosis
AT khnabasafae giantbilateralangiomyolipomaswithspontaneoushemorrhageandinferiorvenacavathrombosisinapatientwithtuberoussclerosis
AT radouanebouchaib giantbilateralangiomyolipomaswithspontaneoushemorrhageandinferiorvenacavathrombosisinapatientwithtuberoussclerosis
AT jidalmohamed giantbilateralangiomyolipomaswithspontaneoushemorrhageandinferiorvenacavathrombosisinapatientwithtuberoussclerosis
AT amiltouriya giantbilateralangiomyolipomaswithspontaneoushemorrhageandinferiorvenacavathrombosisinapatientwithtuberoussclerosis
AT saouabrachida giantbilateralangiomyolipomaswithspontaneoushemorrhageandinferiorvenacavathrombosisinapatientwithtuberoussclerosis