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

Autor: Meriem Menany, Safae Khnaba, Bouchaib Radouane, Mohamed Jidal, Touriya Amil, Rachida Saouab
Jazyk: English<br />French
Rok vydání: 2017
Předmět:
Zdroj: The Pan African Medical Journal, Vol 27, Iss 223 (2017)
Druh dokumentu: article
ISSN: 1937-8688
DOI: 10.11604/pamj.2017.27.223.8379
Popis: 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.
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