scholarly journals Bilateral Pheochromocytoma in von Hippel-Lindau Syndrome Simultaneously Removed by Lateral Retroperitoneal Endoscopic Approach

2012 ◽  
Vol 4 (3) ◽  
pp. 102-104
Author(s):  
Georgi Todorov ◽  
Konstantin Grozdev ◽  
Tsonka Lukanova ◽  
Biljana Mioljevikj-Miserliovska ◽  
Risto Miserliovski
Keyword(s):  
Suppressor Gene ◽  
Endoscopic Approach ◽  
Von Hippel Lindau ◽  
Endoscopic Adrenalectomy ◽  
Bilateral Pheochromocytoma ◽  
Increased Risk ◽  
Liver And Pancreas ◽  
Vascular Proliferation ◽  
The Central Nervous System ◽  
Von Hippel Lindau Syndrome

ABSTRACT Von Hippel-Lindau (VHL) syndrome is an autosomal dominant familial neoplastic syndrome caused by mutation in VHL tumor suppressor gene localized on chromosome 3p25. The disease is characterized by abnormal vascular proliferation and increased risk of developing renal cell carcinoma, pheochromocytoma, hemangioblastoma of the central nervous system, tumors of the endolymphatic bag, cysts of the kidney, liver and pancreas, epididymal cystadenomas, neuroendocrine tumors of the pancreas, angiomas in the retina. We report a case of a bilateral pheochromocytoma, simultaneously removed by unilateral total and contralateral subtotal retroperitoneal endoscopic adrenalectomy. How to cite this article Todorov G, Grozdev K, Lukanova T, Mioljevikj-Miserliovska B, Miserliovski R. Bilateral Pheochromocytoma in von Hippel-Lindau Syndrome Simultaneously Removed by Lateral Retroperitoneal Endoscopic Approach. World J Endoc Surg 2012;4(3):102-104.

Pregnancy complicated by neurological and neurosurgical conditions – The evidence regarding mode of delivery

2021 ◽  
pp. 1753495X2110001
Author(s):  
Adam Morton
Keyword(s):  
Caesarean Section ◽  
Health Professionals ◽  
Mode Of Delivery ◽  
Maternal Outcomes ◽  
Von Hippel Lindau ◽  
Healthy Pregnancy ◽  
Increased Risk ◽  
The Central Nervous System ◽  
Von Hippel Lindau Syndrome ◽  
Considerable Concern

Neurological and neurosurgical conditions complicating pregnancy may precipitate considerable concern regarding the risk of complications, and uncertainty regarding the preferred mode of delivery and anaesthesia. Caesarean section is known to be associated with significantly increased risk of adverse maternal outcomes compared with vaginal delivery in healthy pregnancy. Nevertheless, a common perception exists among the general population and some health professionals that caesarean section is safer for the mother and baby in high-risk pregnancies. This manuscript examines the literature regarding the risks and outcomes related to mode of delivery in pregnancy complicated by disorders of the central nervous system. With the exception of women with raised intracranial pressure, and some women with Von Hippel-Lindau syndrome, the available evidence suggests that the mode of delivery should be based upon obstetric indications.

Hemangioblastomas of the central nervous system

1989 ◽  
Vol 70 (1) ◽  
pp. 24-30 ◽  
Author(s):  
Hartmut P. H. Neumann ◽  
Hans R. Eggert ◽  
Klaus Weigel ◽  
Hartmut Friedburg ◽  
Otmar D. Wiestler ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Screening Program ◽  
Family Background ◽  
Renal Cysts ◽  
Pancreatic Cysts ◽  
Content Type ◽  
Von Hippel Lindau ◽  
The Central Nervous System ◽  
Von Hippel Lindau Syndrome

✓ The findings of a 10-year study (1976 to 1986) conducted in southwest Germany on hemangioblastomas (HBL's) of the central nervous system (CNS) are presented. During that period, 47 HBL's were diagnosed and surgically removed in 44 patients, with a good postoperative survival rate and prognosis. The majority (83%) of these tumors were located in the cerebellum. By thorough clinical examination of the patients and careful evaluation of their family background, it was found that 23% of the HBL patients were afflicted with von Hippel-Lindau syndrome. In addition to the CNS tumors, 14 neoplastic or similar lesions were detected in other tissues. These included angiomatosis of the retinae, pheochromocytomas, pancreatic cysts, renal cysts, and renal carcinoma. The diagnosis of von Hippel-Lindau syndrome was thus established in seven families. The authors suggest the need for a screening program for patients with HBL of the CNS which is designed to confirm or exclude ocular or visceral lesions associated with von Hippel-Lindau syndrome.

Hemangioblastomas of the Central Nervous System in von Hippel-Lindau Syndrome and Sporadic Disease

Neurosurgery ◽  
2001 ◽  
Vol 48 (1) ◽  
pp. 55-63 ◽  
Author(s):  
James E. Conway ◽  
Dean Chou ◽  
Richard E. Clatterbuck ◽  
Henry Brem ◽  
Donlin M. Long ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Von Hippel Lindau ◽  
The Central Nervous System ◽  
Sporadic Disease ◽  
Von Hippel Lindau Syndrome

scholarly journals Identification of a VHL gene mutation in atypical Von Hippel-Lindau syndrome: genotype–phenotype correlation and gene therapy perspective

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Dali Tong ◽  
Yao Zhang ◽  
Jun Jiang ◽  
Gang Bi
Keyword(s):  
Gene Therapy ◽  
Gene Mutation ◽  
Suppressor Gene ◽  
Pancreatic Cysts ◽  
Vhl Gene ◽  
Von Hippel Lindau ◽  
Genetic Examination ◽  
Examination Methods ◽  
Vhl Disease ◽  
Von Hippel Lindau Syndrome

Abstract Background Classical von Hippel Lindau (VHL) disease/syndrome includes CNS hemangioblastoma, renal or pancreatic cysts, pheochromocytoma, renal carcinoma and exodermic cystadenoma. The syndrome is caused by mutation of VHL tumor suppressor gene. The most prevalent mutations are present in VHL syndrome. To date, > 500 mutations of gene related to the progression of VHL syndrome have been reported. VHL gene mutation presented in single lung or pancreatic tumor has been reported occasionally, but there is no report of both. Methods In this paper, we used CT scan, pathological and genetic examination methods to diagnose a rare atypical VHL syndrome. Results We reported a rare case of atypical VHL syndrome with authenticated VHL mutation at p.Arg167Gln, that was associated with not only bilateral pheochromocytoma but also lung carcinoid and neuroendocrine tumor of pancreas. Based on literature reviews, the patient was recommended to be further subjected to octreotide-based radionuclide therapy. Conclusions Combined with gene detection and clinical diagnosis, we found the inherent relationship between VHL genotype and phenotype, and constructed the standard diagnosis and treatment process of disease with rare VHL mutation from the perspective of gene therapy.

Bilateral pheochromocytoma in von hippel-lindau syndrome revealed by a hemangioblastoma

2021 ◽  
Author(s):  
Elyes Kamoun ◽  
Imen Rojbi ◽  
Rihab Laamouri ◽  
Marwa Majdoub ◽  
Chayma Besrour ◽  
...  
Keyword(s):  
Von Hippel Lindau ◽  
Bilateral Pheochromocytoma ◽  
Von Hippel Lindau Syndrome

Haemangioblastoma

2019 ◽  
pp. 367-374
Author(s):  
Donald McArthur ◽  
Ammar Natalwala
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Optimal Timing ◽  
Significant Morbidity ◽  
Rapid Disease Progression ◽  
Von Hippel Lindau ◽  
Complete Surgical Resection ◽  
The Central Nervous System ◽  
Benign Tumours ◽  
Von Hippel Lindau Syndrome

Haemangioblastomas are rare and benign tumours which favour the infratentorial structures in the central nervous system. Around 30% of these tumours are associated with Von Hippel-Lindau syndrome, which if the diagnosis is missed can lead to rapid disease progression and significant morbidity and mortality. Complete surgical resection is the main treatment but other techniques including stereotactic radiosurgery are described in this chapter. Screening for Von Hippel-Lindau (VHL), the optimal timing, and mode of treatment remain controversial due to the diversity of clinical presentation in these patients. This chapter also outlines the general principles of managing patients with haemangioblastomas and the factors which impact on prognosis.

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