Malignant Skull Base Tumors
The UCI Health skull base surgery team is experienced at diagnosing and treating the broad range of malignancies that can affect the skull base region. These tumors fall into the following groups:
Squamous cell carcinoma is a type of skin cancer that can occur anywhere on or in the body, but most often it arises on sun-exposed areas such as the face, ears, neck, hands and arms. This cancer may appear as a growing bump or flat, red-colored patch. It may have a scaly or crusted appearance. Other possible signs of squamous cell cancer are a sore that doesn't heal or a mole or wart that has recently changed in appearance.
If left untreated, squamous cell cancer may spread to the sinuses or other areas of the skull base and brain.
Squamous cell cancers are surgically removed (or excised). Deeper lesions may require cutting. Other techniques to remove lesions include the use of specialized lasers. Skull base surgeons may take a minimally invasive approach, using a microscope and an endoscope to enter the ear. For sinus cancers, surgeons may perform endonasal surgery, using an endoscope with a tiny camera and specialized instruments through the nose to reach the tumor.
Radiation may be required if the squamous cell skin cancer has spread to organs or lymph nodes, or if the cancer cannot be treated with surgery.
Melanoma is the most serious form of skin cancer, and it is typically caused by exposure to ultraviolet light from the sun or tanning beds. The tumors arise in pigment-producing skin cells called melanocytes. Melanomas sometimes look like moles or develop from pre-existing moles. Symptoms of cancer include moles that have changed in appearance, such as moles that bleed or change shape. Melanomas are often black or brown, but they can also be flesh-colored, pink, red, blue or white.
Melanomas that are detected early are often confined to one spot in the skin and are not very deep (superficial). Lentigo maligna is a type of superficial melanoma that is characterized by a mottled brown or tan color. Invasive melanomas are cancers that have penetrated deeper into the skin and spread to other parts of the body. Advanced melanoma can spread to the skull base and brain.
Treatment for skull base melanoma involves surgical removal of the lesion followed by other therapies, such as interventional radiation or chemotherapy, depending on the stage of the cancer (localized or invasive). A neurosurgeon may remove the tumor depending on its location.
Non-squamous cell carcinomas are group of malignancies that can arise in different tissues within the body. They include:
- Neuroendocrine tumors, also called NETs, can arise in any part of the body that contains neuroendocrine cells. NETs, which are usually cancerous, vary in size and how fast they grow, thus affecting patients in different ways. Some neuroendocrine tumors are called functional NETs because they produce symptoms caused by the secretion of hormones and other substances.
Treatment options include surgery, chemotherapy, chemoembolization, radiofrequency ablation, radiotherapy and targeted radionuclide therapy.
- Adenocarcinoma is a type of cancer that forms in mucus-secreting glands in the body. Treatment for adenocarcinoma varies depending on where it grows in the body, but may include surgery to remove the affected glandular tissue as well as some surrounding tissue.
Minimally invasive surgery can often be used, which helps reduce side effects and the risk of infection and speeds recovery. Some patients also require radiation therapy or chemotherapy. Using state-of-the-art technologies and imaging devices, radiation can be precisely target the adenocarcinoma tumors while sparing healthy tissues and surrounding organs.
- Sinonasal undifferentiated carcinoma (SNUC) is a rare cancer of the nasal cavity and/or paranasal sinuses. Early symptoms of this condition include bloody nose, runny nose, double vision, bulging eye, chronic infections and nasal obstruction.
Treatment consists of the combined modalities of surgery, radiation and chemotherapy.
- Salivary gland cancer is a rare malignancy that begins in the salivary glands in the mouth, neck or throat. The cancer most commonly occurs in the parotid gland, which is just in front of the ear. The first signs of the disease include the appearance of a painless lump in the ear, cheek, jaw, lip or inside the mouth, fluid draining from the ear, trouble swallowing or opening the mouth widely, numbness or weakness in the face and facial pain.
Treatment can include surgery, radiation therapy, chemotherapy and radiation with radiosensitizers, which are drugs that make a tumor more sensitive to radiation.
- Adenoid cystic carcinoma (ACC) is an uncommon type of cancer that forms in the secretory glands — or salivary — glands of the head and neck. The tumors are typically painless and slow-growing although advanced tumors may cause pain or nerve paralysis as well as changes in vision, speech and breathing.
Adenoid cystic carcinomas are surgically removed. Some patients also undergo radiation therapy or neutron beam therapy, which is a type of radiation therapy that uses a beam of high-energy neutrons instead of x-rays.
- Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare cancer that arises in olfactory bulb, the part of the brain that interprets smell. Symptoms vary depending on the location and extent of the tumor. The most common symptoms are nasal obstruction, nasal bleeding, headaches, facial pain and decreased sense of smell.
Esthesioneuroblastomas are surgically removed by a neurosurgeon and/or otolaryngologist and may be followed with radiation and/or chemotherapy.
Sarcomas are tumors that arise in the body's connective tissues. There are several types, including:
- Chondrosarcomas, which consist mostly of cartilage and can range in severity from cancers that are unlikely to spread to aggressive tumors that have a high risk of spreading (metastasizing). Symptoms may include a deep, dull pain that often occurs at night.
Chondrosarcomas require surgery. Radiotherapy and chemotherapy may also be performed after surgery if the tumor resides in a surgically inaccessible area.
- Osteosarcoma is a cancerous bone tumor that usually develops in teenagers during rapid growth spurts. It typically arises in the bones of the shin, thigh and upper arm. Symptoms can include bone fractures from seemingly routine movements, pain, limitations in range of motion, swelling, tenderness or redness at the site of the tumor.
Surgery is recommended to remove the tumor, followed by chemotherapy to target any remaining tumor cells.
- Rhabdomyosarcoma is a malignant tumor of muscle tissue where it attaches to the bones. It can occur in many places in the body. The most common sites are the structures of the head and neck, the urogenital tract and the arms or legs. Rhabdomyosarcoma is the most common soft tissue tumor in children.
The appearance of a mass is usually the first sign of the disease. However, tumors that develop in the nose or throat may cause bleeding, congestion, swallowing problems or neurological problems if they extend into the brain. Tumors in the brain may cause visual disturbances or hearing loss.
Treatment usually begins with radiation and/or chemotherapy followed by surgery.
- Ewing's sarcoma is a cancerous bone tumor that affects children. The most common symptom is pain and, occasionally, swelling at the site of the tumor. Children may also break a bone at the site of the tumor after a seemingly minor injury (called a pathologic fracture). Fever may also be a symptom.
Treatment includes chemotherapy, radiation therapy and surgery.
- Chordoma is a rare type of sarcoma that occurs most at the base of the skull or the base of the spine (or sacrum). Subtypes include conventional, chondroid and de-differentiated chordomas. The most common signs of a skull base chordoma are headaches, neck pain or visual disturbances.
Patients with chordomas undergo surgery followed by radiation therapy, including stereotactic body radiation (which relies on three-dimensional imaging to precisely focus radiation beams on the tumor), to reduce the risk of recurrence. Clinical trials of targeted cancer medications to prevent chordoma recurrence are ongoing.
Primary lymphoma is a cancer of the white blood cells in the brain. Symptoms include changes in speech or vision, confusion, fever, hallucinations, headaches, leaning to one side when walking, loss of coordination, numbness to hot and cold temperatures, numbness to pain, personality changes, seizures, weakness in the hands and weight loss.
Primary lymphoma of the brain is treated with chemotherapy. Patients usually receive medications called corticosteroids to control swelling and improve symptoms. Radiation therapy of the whole brain is reserved for patients who do not respond to chemotherapy.
Multiple myeloma is a cancer that starts in the plasma cells in bone marrow, the spongy tissue found inside most bones. Bone marrow helps make blood cells.
This disease typically causes a low red blood cell count (anemia), which can lead to fatigue and shortness of breath. It can also cause low white blood cell count, which increases the risk of infections. Multiple myeloma also causes low platelet count, which can lead to abnormal bleeding.
Chemotherapy is usually recommended to treat multiple myeloma. Radiation therapy may be used to relieve bone pain or treat a bone tumor. Some patients with multiple myeloma undergo bone marrow or stem cell transplants. There are two types:
- Autologous bone marrow transplantation — also called stem cell transplantation — is done using the person's own stem cells.
- Allogeneic transplantation involves the use of someone else’s stem cells. This treatment has serious risks, but may offer the chance of long-term cure.
For more information or to schedule a consultation, call 714-456-7017 for head and neck surgery services or 714-456-6966 for neurosurgery services.
