Successful Surgical Management of Multiple Tumours (VHL Syndrome) Followed by High-Risk Pregnancy in a 30-year-old Woman

In a rare and medically complex case, 30-year-old Parul Shukla from Jhansi came to Sarvodaya Hospital, Sec 8, Faridabad with a history of infertility and tumours.

While undergoing fertility treatment in Jhansi, routine investigations revealed an unexpected and alarming finding: a “knot” in her abdomen. Further evaluation through a CT scan confirmed the presence of Pheochromocytoma (a rare tumour) in both the right and left adrenal glands, along with severe hypertension.

First Diagnosis - Pheochromocytoma

Pheochromocytoma is a rare tumour arising from the adrenal glands, which are located above the kidneys and are responsible for producing hormones that regulate blood pressure and stress response. The presence of tumours in both adrenal glands at the age of 30 is extremely uncommon. Her blood pressure was fluctuating dangerously, raising immediate concern.

Second Diagnosis at Sarvodaya - VHL Syndrome and Brain Tumour

At Sarvodaya, she was first evaluated by Dr. Bhawna Atri, Consultant, Endocrinology. Given the unusual presentation of bilateral adrenal tumours at a young age, genetic testing was advised. The results confirmed a rare hereditary condition known as Von Hippel-Lindau (VHL) syndrome. Further imaging also revealed a tumour in her brain, adding another layer of complexity to the case.

VHL Syndrome is a rare genetic disorder that leads to the formation of tumours and cysts in multiple parts of the body, including the brain, spinal cord, adrenal glands, kidneys, pancreas, and eyes. It is estimated to occur in approximately 1 in 36,000 to 1 in 50,000 individuals worldwide, and some severe manifestations may be identified even in childhood.

Multidisciplinary Treatment Plan with Tumour Board

Parul’s case required meticulous planning and a highly coordinated multidisciplinary approach. The first priority was to stabilize her fluctuating blood pressure, which is critical in pheochromocytoma patients.

Once her blood pressure was medically optimized, a team of specialists, including endocrinologists, surgeons, anesthesiologists, genetic experts, and critical care specialists,sat together to chart out a step-by-step treatment plan at tumour board.

First Surgery - Laparoscopic Adrenal Glands Removal

The first major milestone was laparoscopic surgery for the removal of both adrenal glands, performed by Dr. Arjun Goel, Senior Consultant & Head (Unit II) – General & Minimally Invasive Surgery and Dr. Naveen Sanchety, Director, Surgical Oncology.

Adrenal gland surgery is inherently complex because the glands are small, deeply located structures hidden above the kidneys, surrounded by vital organs and major blood vessels. In Parul’s case, the presence of tumours in both glands made it even more challenging.

During the surgery, her blood pressure fluctuated significantly, a known risk in pheochromocytoma operations due to sudden hormone release during tumour handling. However, the experienced anesthesia team, led by Lt. Col (Dr.) Rajiv Gupta (Retd.), HOD & Director - Anaesthesia, managed these rapid changes expertly, ensuring her safety throughout the procedure. The use of minimally invasive laparoscopic techniques allowed precise tumour removal with smaller incisions, reduced blood loss, less post-operative pain, and faster recovery.

After successful surgery and stabilization, Parul was discharged home to recover.

Second Surgery - Brain Tumour Removal

Once she regained strength, attention shifted to the brain tumour detected earlier. Dr. Yawar Shoaib Ali, Director & Head - Unit (I) - Neurosurgery, performed a carefully planned surgery to remove the tumour. Given the underlying VHL syndrome and her recent major surgery, this required meticulous coordination and monitoring. The procedure was successful, and she recovered well.

What followed was perhaps the most remarkable chapter of her journey.

High-Risk Pregnancy Management and Safe Delivery 

After overcoming multiple complex surgeries and a rare genetic diagnosis, Parul conceived. Her pregnancy, however, was categorised as high-risk and required constant monitoring and hormonal management.

Her pregnancy was carefully managed by Dr. Raina Chawla, Associate Director, Gynaecology and Dr. Nidhi Sharma, Consultant, Obstetrics and Gynaecology, who provided specialised high-risk obstetric care. With regular follow-ups, close monitoring of hormone levels, blood pressure surveillance, and coordinated multidisciplinary support, Parul safely carried her pregnancy to term and delivered successfully, fulfilling the dream that had initially led her to seek medical care.

Understanding VHL Syndrome

VHL syndrome is a rare inherited condition caused by a gene change that affects how cells grow. It can be passed from a parent, but sometimes it appears for the first time in a family.

It can cause:

• Hemangioblastomas (tumours of the brain and spinal cord)
• Pheochromocytomas (adrenal gland tumours)
• Kidney Tumours and Cysts
• Pancreatic Cysts and Neuroendocrine Tumours
• Retinal Angiomas

The condition cannot be completely prevented because it is genetic. However, early diagnosis through genetic testing, especially in young patients with unusual tumour presentations, can allow regular screening and timely treatment. Genetic counseling is strongly recommended for affected individuals and their families.

The Power of a Multidisciplinary Approach at Sarvodaya

Parul’s case highlights the importance of a coordinated, multidisciplinary model of care. From endocrinology and genetics to advanced Radiology & Imaging Tests, Laparoscopic Surgery, Neurosurgery, Anesthesia, Critical Care, and High-risk Obstetrics, every department worked together to provide end-to-end care.

Key benefits of the multidisciplinary approach at Sarvodaya include:

• Early and accurate diagnosis through advanced genetic testing
• Preoperative optimization to reduce surgical risk
• Expertise in minimally invasive surgery
• Advanced anesthesia management
• Specialized neurosurgical care
• Comprehensive high-risk pregnancy management
• Modular Operating Theaters and recovery rooms
• Seamless coordination across departments