Von Hippel–Lindau Syndrome: A Case Report

BACKGROUND AND OBJECTIVE: Von Hippel–lindau syndrome is a rare syndrome. Von Hippel–Lindau is an autosomal dominant condition manifested by cerebellar and spinal hemangioblastomas, retinal angiomas, clear cell RCC, cysts of the pancreas, kidney, and epididymis, epididymal cystadenimas, pheochromocyt...

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Autores principales:	H Shafi, M Rafati, B Ataee, A Ali Ramaji, AR Firoozjahi, B Jahed
Formato:	article
Lenguaje:	EN FA
Publicado:	Babol University of Medical Sciences 2009
Materias:	von hippel-lindau syndrome autosomal dominant multisystems different clinical manifestations Medicine R Medicine (General) R5-920
Acceso en línea:	https://doaj.org/article/091439b5dc674bfba9b855d27c7bdbce
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spelling	oai:doaj.org-article:091439b5dc674bfba9b855d27c7bdbce2021-11-10T09:03:01ZVon Hippel–Lindau Syndrome: A Case Report1561-41072251-7170https://doaj.org/article/091439b5dc674bfba9b855d27c7bdbce2009-10-01T00:00:00Zhttp://jbums.org/article-1-3262-en.htmlhttps://doaj.org/toc/1561-4107https://doaj.org/toc/2251-7170BACKGROUND AND OBJECTIVE: Von Hippel–lindau syndrome is a rare syndrome. Von Hippel–Lindau is an autosomal dominant condition manifested by cerebellar and spinal hemangioblastomas, retinal angiomas, clear cell RCC, cysts of the pancreas, kidney, and epididymis, epididymal cystadenimas, pheochromocytoma, and endolymphatic sac tumors. The aim of this report was to introduce one case of Von Hippel–Lindau syndrome that patients multiple organs were involved. CASE: The patient was a 28 year old male that nine years ago because of right big cyst epididymis underwent surgery and about seven years ago he was hospitalized because of headache, ataxia and vertigo. Cerebellum hemangioblastomas was diagnosed by MRI, and three years ago abnormal gait caused by spinal hemangioblastoma in L2 and L3. The patient underwent surgery and then radiation treatment. Also, he suffered from blindness in the right eye caused by retinal angioma. Neurosurgeon referred the patient to urology center that a tumor in the incidentaloma of right kidney was discovered and underwent right radical nephrectomy. Pathologists report showed renal cell carcinoma. Now he referred due to infertility and undergoes diagnostic biopsy. Unfortunately he can not be a candidate for microinjection because histology revealed immature cells.CONCLUSION: With regard to the reported case of Von Hippel–Lindau syndrome that his multiple organs were involved coordination of different specialties in medical cares and the earlier identification of tumor and more careful surveillance after surgery due to multiple characteristics of tumor seem to be necessary.H Shafi,M RafatiB AtaeeA Ali RamajiAR Firoozjahi,B JahedBabol University of Medical Sciencesarticlevon hippel-lindau syndromeautosomal dominantmultisystemsdifferent clinical manifestationsMedicineRMedicine (General)R5-920ENFAMajallah-i Dānishgāh-i ̒Ulūm-i Pizishkī-i Bābul, Vol 11, Iss 4, Pp 75-80 (2009)
institution	DOAJ
collection	DOAJ
language	EN FA
topic	von hippel-lindau syndrome autosomal dominant multisystems different clinical manifestations Medicine R Medicine (General) R5-920
spellingShingle	von hippel-lindau syndrome autosomal dominant multisystems different clinical manifestations Medicine R Medicine (General) R5-920 H Shafi, M Rafati B Ataee A Ali Ramaji AR Firoozjahi, B Jahed Von Hippel–Lindau Syndrome: A Case Report
description	BACKGROUND AND OBJECTIVE: Von Hippel–lindau syndrome is a rare syndrome. Von Hippel–Lindau is an autosomal dominant condition manifested by cerebellar and spinal hemangioblastomas, retinal angiomas, clear cell RCC, cysts of the pancreas, kidney, and epididymis, epididymal cystadenimas, pheochromocytoma, and endolymphatic sac tumors. The aim of this report was to introduce one case of Von Hippel–Lindau syndrome that patients multiple organs were involved. CASE: The patient was a 28 year old male that nine years ago because of right big cyst epididymis underwent surgery and about seven years ago he was hospitalized because of headache, ataxia and vertigo. Cerebellum hemangioblastomas was diagnosed by MRI, and three years ago abnormal gait caused by spinal hemangioblastoma in L2 and L3. The patient underwent surgery and then radiation treatment. Also, he suffered from blindness in the right eye caused by retinal angioma. Neurosurgeon referred the patient to urology center that a tumor in the incidentaloma of right kidney was discovered and underwent right radical nephrectomy. Pathologists report showed renal cell carcinoma. Now he referred due to infertility and undergoes diagnostic biopsy. Unfortunately he can not be a candidate for microinjection because histology revealed immature cells.CONCLUSION: With regard to the reported case of Von Hippel–Lindau syndrome that his multiple organs were involved coordination of different specialties in medical cares and the earlier identification of tumor and more careful surveillance after surgery due to multiple characteristics of tumor seem to be necessary.
format	article
author	H Shafi, M Rafati B Ataee A Ali Ramaji AR Firoozjahi, B Jahed
author_facet	H Shafi, M Rafati B Ataee A Ali Ramaji AR Firoozjahi, B Jahed
author_sort	H Shafi,
title	Von Hippel–Lindau Syndrome: A Case Report
title_short	Von Hippel–Lindau Syndrome: A Case Report
title_full	Von Hippel–Lindau Syndrome: A Case Report
title_fullStr	Von Hippel–Lindau Syndrome: A Case Report
title_full_unstemmed	Von Hippel–Lindau Syndrome: A Case Report
title_sort	von hippel–lindau syndrome: a case report
publisher	Babol University of Medical Sciences
publishDate	2009
url	https://doaj.org/article/091439b5dc674bfba9b855d27c7bdbce
work_keys_str_mv	AT hshafi vonhippellindausyndromeacasereport AT mrafati vonhippellindausyndromeacasereport AT bataee vonhippellindausyndromeacasereport AT aaliramaji vonhippellindausyndromeacasereport AT arfiroozjahi vonhippellindausyndromeacasereport AT bjahed vonhippellindausyndromeacasereport
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Von Hippel–Lindau Syndrome: A Case Report

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