Paraplegia in a patient with Von Hippel Lindau syndrome: surgical and reconstructive treatment of Marjolin’s ulcer. A case report

Being able to live an active and meaningful life is important for mental health of every individual. In this case report we examine the life of an oncology patient who developed depression six years ago. The patient is a fifty seven year old woman who has been suffering from Von Hippel-Lindau syndrome for the last forty years. Her father and two uncles died from the same disease. She had her first operation when she was seventeen years old and has had numerous operations since then. During this time she has undergone four neurosurgical operations, nephrectomy, spine and pancreas operation and eye enucleation. Despite the fact that by being a chronic oncology patient she was prone to depression, she did not develop depressive symptoms. It did not happen even as her husband went to war and left her to take care of their child. It did not come afterwards as they struggled financially. Only after they moved to a new apartment and as she finished decorating it, did depression finally occur. During entire life she was an active, outgoing person, who took pleasure in socialising and various hobbies. She only developed depression after she was pensioned, left with the responsibility to care for her old mother. Following the psychiatric treatment she regained interest in people and become active in different cancer support groups. This example accentuates the importance of every-day pleasurable activities as a defence mechanism against depression.Disclosure of interestThe authors have not supplied their declaration of competing interest.

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Adrenal adenoma in von Hippel–Lindau syndrome: A case report with review of literature

Journal of Cancer Research and Therapeutics ◽

10.4103/jcrt.jcrt_127_18 ◽

2019 ◽

Vol 15 (8) ◽

pp. 163

Author(s):

Jayaprakash Sahoo ◽

Rajan Palui ◽

Sadishkumar Kamalanathan ◽

LalgudiNarayanan Dorairajan ◽

Bhawana Badhe ◽

...

Keyword(s):

Case Report ◽

Adrenal Adenoma ◽

Review Of Literature ◽

Von Hippel Lindau ◽

Von Hippel Lindau Syndrome

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Ovarian steroid cell tumour in an adolescent with von Hippel-Lindau syndrome: A case report and review of the literature

10.26226/morressier.596dfd56d462b8029238797a ◽

2017 ◽

Author(s):

Ana Marques

Keyword(s):

Case Report ◽

Cell Tumour ◽

Review Of The Literature ◽

Ovarian Steroid ◽

Von Hippel Lindau ◽

Von Hippel Lindau Syndrome

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Bilateral Adrenal Incidentalomas: A Case Report and Review of Diagnostic Challenges

Case Reports in Endocrinology ◽

10.1155/2013/953052 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4

Author(s):

Anders L. Carlson ◽

Annis M. Marney ◽

Scott R. Anderson ◽

Matthew P. Gilbert

Keyword(s):

Case Report ◽

Genetic Testing ◽

Adrenal Mass ◽

Diagnostic Approach ◽

Diagnostic Strategy ◽

Adrenal Incidentalomas ◽

Von Hippel Lindau ◽

Additional Testing ◽

Adrenal Masses ◽

Von Hippel Lindau Syndrome

Incidentally discovered adrenal masses (incidentalomas) are common and present challenges both in diagnosis and management. When incidentally discovered adrenal masses are bilateral, a refined diagnostic approach is warranted since bilateral disease is more likely to be pathologic. We review a case of a 34-year-old man with incidentally discovered bilateral adrenal nodules. A comprehensive diagnostic strategy led to the diagnosis of bilateral pheochromocytoma caused by von Hippel-Lindau syndrome. He was successfully treated with bilateral laparoscopic adrenalectomy and has recovered well. While the initial diagnostic approach is similar to the unilateral incidentaloma, additional testing and/or genetic testing should be considered in the case of the bilateral adrenal mass.

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Von Hippel-Lindau syndrome and renal tumours: radiological diagnostic and treatment options. A case report and literature review

Acta medica Lituanica ◽

10.6001/actamedica.v27i1.4263 ◽

2020 ◽

Vol 27 (1) ◽

pp. 25-32

Author(s):

Audrius Untanas ◽

Mantas Trakymas ◽

Indrė Lekienė ◽

Rūta Briedienė

Keyword(s):

Case Report ◽

Renal Dialysis ◽

Distant Metastases ◽

Correct Treatment ◽

Von Hippel Lindau ◽

Treatment Techniques ◽

Contrast Enhanced ◽

Increased Risk ◽

Renal Tumours ◽

Von Hippel Lindau Syndrome

Background. Von Hippel-Lindau disease (VHL) is a rare autosomal dominant syndrome diagnosed for 1 out of 36000–45000 newborns and 90% of the patients have a clinical manifestation before 65 years of age. Affected individuals have an increased risk of developing tumours in several organs or their systems. The most common tumours are retinal or central nervous system hemangioblastomas (60–80%) and VHL-associated renal lesions. Contrast-enhanced computer tomography (CECT) is the gold standard for the diagnosis and characterization of renal tumours. The best treatment option for VHL syndrome-caused renal tumours are nephron-sparing treatment techniques (cryotherapy, radiofrequency, or microwave ablation), which require imaging control. All these innovative treatment techniques are extremely important for VHL patients, because they increase the quality of life by staving off renal dialysis and preventing distant metastases. Case report. Our case report presents a 16-year-old female with multiple renal cysts observed on ultrasound examination and clinically and molecularly diagnosed with Von Hippel-Lindau syndrome (deletion of the entire VHL gene). After that, for past 11 years multiple renal tumours were removed by cryoablation and patient monitoring on contrast-enhanced magnetic resonance (MRI) and CECT control scans was conducted. Conclusions. Active multidisciplinary patient follow-up, routine radiological examinations, and correct treatment tactics allow controlling the progression of renal cell carcinoma and other tumours associated with VHL syndrome, maintaining a normal organ function for a long time, and preventing distant metastases and fatal disease outcomes.

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