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Doctor speaking with patient
Doctor speaking with patient

Rare and Familial Endocrine Tumors

We have uncommon expertise in rare and familial endocrine tumors. These conditions that are often passed down in families raise your risk of cancer and require specialized care. Our team is here for you with comprehensive evaluations and sophisticated treatments that are not widely available.

Mays Cancer Center, home to UT Health San Antonio MD Anderson Cancer Center, is among the few programs in South Texas capable of treating rare and familial endocrine tumors.

We offer the services you need close to home, including genetic testing, cancer screening and leading therapies. Our offerings help you and your family learn about your risks and give you access to trusted care.

Through research, we are also advancing care for rare and familial endocrine tumors. Our renowned researchers are uncovering new methods of identifying and evaluating people at risk of these problems. Our scientists are also evaluating the properties of certain tumors to make care more precise.

What you need to know about rare and familial endocrine tumors

  • Endocrine tumors affect chemical messengers (hormones) that regulate many of your organs. Tumors may or may not be cancerous, but they can grow large enough to affect organ functioning, making you sick.
  • There are many types of rare endocrine tumors. Receiving care at an experienced program such as ours gives you access to a range of therapies, including clinical trials, if you are eligible.
  • Some endocrine tumors are more likely to occur in people with a family history or changes (mutations) in specific genes. Having a gene mutation does not always mean you’ll get cancer.
  • We can take steps to manage your cancer risk. We may recommend regular screening tests to catch early signs or treatments, like surgery, to prevent cancer from forming.

How to talk to your doctor about rare and familial endocrine tumors

Learning that you have or are at risk for a rare or familial endocrine tumor can be overwhelming. Our team is here to help. We encourage you to ask a lot of questions. No topic is too small to explore.

Feel free to ask us about:

  • Tests that may be necessary to check for tumors and how often you need them
  • Genetic testing and counseling is available 
  • Treatments, if necessary, and how they work
  • Support to help you cope with the challenges that come with being at risk for or having cancer

You are welcome to bring loved ones with you to appointments. They can help you remember details about your care plan and help you make important decisions.

Rare and familial endocrine tumors we treat

Our expertise spans a broad range of disorders, including:

Endocrine tumors affecting the kidneys

In rare cases, an adrenal tumor can grow into nearby kidney tissue. Our team includes a fellowship-trained surgical oncologist specializing in urologic disease, including kidney cancer. We perform delicate surgery to remove the tumor while protecting healthy kidney tissue.

We also care for other uncommon conditions, such as kidney tumors made up of cells that resemble thyroid cancer. Few cases of this problem exist, and we have experience treating it. Learn more about kidney cancer.

Hereditary paraganglioma-pheochromocytoma syndrome

This inherited condition causes:

  • Paraganglioma, a tumor that forms in certain nerve cells
  • Pheochromocytoma, a type of adrenal tumor

There are many types of paragangliomas and pheochromocytomas. Each occurs due to different mutations, many of which are passed down in families.

We determine your risk based on the mutations that are present in your genes. Our expertise means we provide reliable information, so you receive additional tests and treatments that are right for your situation.

Medullary thyroid cancer

Medullary thyroid cancer often advances to nearby tissue (lymph nodes), enabling it to spread to other areas of the body. You face a higher risk if you have an inherited condition such as multiple endocrine neoplasia.

This type of cancer typically does not cause symptoms in early stages, making it difficult to detect. If you are at risk, we may recommend removing your thyroid before cancer develops. Find out more about thyroid cancer.

Multiple endocrine neoplasia

This inherited condition causes tumors in multiple areas, including the adrenal, thyroid, parathyroid and pituitary glands. Tumors may start developing as early as childhood.

Our team of experts includes fellowship-trained pediatric and adult cancer doctors, endocrinologists and cancer surgeons. Our experience caring for this condition helps us anticipate your needs so you receive timely care. Read more about multiple endocrine neoplasia.

Pancreatic neuroendocrine tumors (NETs)

Pancreatic NETs typically do not produce hormones or hormone-related symptoms, making them difficult to detect in early stages.

Our team includes endocrinologists, surgeons and cancer doctors (medical oncologists) with a special interest in pancreatic neuroendocrine tumors. You receive a thorough evaluation, including advanced imaging scans, enabling us to tailor care to your needs.

Neuroendocrine tumors may occur anywhere in the body. When they form in the endocrine system, they typically occur in the pancreas. Get more information about neuroendocrine tumors.

Parathyroid jaw tumor syndrome

A small number of people who experience hyperparathyroidism have this hereditary endocrine neoplasia syndrome. You may have parathyroid jaw tumors if there’s a mutation in a gene that makes a specific type of protein (parafibromin).

Jaw tumors may or may not be cancerous. But even noncancerous tumors can grow quickly and affect your appearance and jaw function. Some people also develop other types of tumors.

Our team includes jaw (maxillofacial) surgeons with experience in treating these tumors. We offer advanced surgical care to minimize changes to your teeth and jaw. Find out more about reconstructive maxillofacial surgery.

von Hippel-Lindau (VHL) syndrome

This condition causes multiple tumors to form in the endocrine system and other parts of the body. As a comprehensive cancer center, Mays Cancer Center provides coordinated care.

Patients with VHL can develop tumors including:

  • Brain or spinal cord hemangioblastoma
  • Retinal hemangioblastoma
  • Endolymphatic sac tumor
  • Pancreatic cysts and neuroendocrine tumors
  • Pheochromocytoma
  • Kidney cysts and cancer

Our endocrine tumor experts work with other specialists, including ophthalmologists, urologists, medical oncologists and neurosurgeons, whenever necessary. We perform thorough evaluations and testing that increase the likelihood of detecting tumors early. Mays Cancer Center is part of the VHL alliance. Find out more about von Hippel-Lindau syndrome.