Fortis Escorts Jaipur Successfully Treats 41-Year-Old Male with Rare Genetic Disorder and Multiple Cancers

Fortis Escorts Jaipur Successfully Treats 41-Year-Old Male with Rare Genetic Disorder and Multiple Cancers 

Von Hippel Lindau Syndrome: A Rare Genetic Disorder Affecting 1 in 36,000 People 

 Jaipur, May, 2024: A 41-year-old male patient from Madhya Pradesh arrived at Fortis Escorts Jaipur in critical condition, suffering from polycystic disease of the pancreas and cancer in his left kidney. His condition was further complicated by Von Hippel Lindau (VHL) syndrome, a rare inherited disorder that causes tumors and cysts to grow in various parts of the body, including the brain, spinal cord, eyes, adrenal glands, pancreas, and kidneys. Owing to the lack of awareness surrounding Von Hippel Lindau syndrome, the patient faced the unfortunate reality of being refused treatment at another hospital in Jaipur. This lack of understanding about VHL often results in patients being misdiagnosed or denied care, making the journey to proper treatment a significant challenge for those afflicted with this rare genetic disorder. However, after a thorough diagnosis by Dr. Sundeep Jain, Director & Head, Department of GI and Gastrointestinal Oncology, and his team, surgery was identified as the best course of action. Following the successful procedure, the patient was discharged within 10 days without complications. 

  Due to the widespread nature of the cancer and the patient's rare genetic condition, Dr. Sundeep Jain and his oncology team, including Dr. Aditya, Dr. Akhil, and Dr. Anjali, in collaboration with the urology team led by Dr. Madhusudan Patodiya, encountered the daunting challenge of removing the patient's cancer-affected pancreas, spleen, left adrenal gland, and part of the left kidney. This intricate, high-risk surgery required meticulous planning and coordination between the teams to ensure the best possible outcome. After three hours in the operating room, the surgical team successfully completed the procedure. 

Explaining about the critical condition and the surgery, Dr. Sundeep Jain, Director & Head, Department of GI, Gastrointestinal Oncology, Fortis Escorts Hospital Jaipur said, “VHL is a rare condition, and there’s a general lack of awareness about it. Because of this, the patient was denied treatment at other places. In this patient’s case, the pancreas had polycystic disease with multiple NET - Neuroendocrine Tumor (rare tumors that develop in the cells of the neuroendocrine system, which is made up of nerve and gland cells that produce hormones) in pancreas, pheochromocytoma adrenal gland and cancer in left Kidney. In order to treat the patient, our team worked meticulously for the best possible outcome; which, in this case, was an operation to take out his cancer-ridden pancreas along with spleen, left adrenal gland and part of the left kidney. The operation was successful, and the patient is doing well. However, as is with all complex diseases, the road to full recovery will take time. Since the pancreas were completely removed, he will have diabetes all his life. Additionally, there are some enzymes produced by the pancreas for digestion, hence the patient would need enzyme supplements throughout his life; apart from which, the patient can lead a normal life.” 

  Mr. Neerav Bansal, Zonal Director, Fortis Escorts Hospital Jaipur, said, “This is a very rare condition, and we usually don’t often come across such cases. Congratulations to Dr. Sundeep Jain and the entire team for successfully identifying this condition and treating him. With access to advanced technology and the expertise of our medical team, we are proud to lead the way in even the most complex cancers, providing hope and healing to those facing complex and rare conditions.”