Craniofacial Tumors

What is a Craniofacial Tumor?

The best way to think about tumors is that they are abnormal collections of abnormal cells that are either detectable visually, by feel (palpation), or radiographically (CT, MRI, or X-ray).  Not all tumors are “cancer”; in fact, most are not cancer at all.  The Craniofacial Team of Texas specializes in the diagnosis and treatment of craniofacial tumors. Most often they are either:

1. An abnormal collection of malformed tissues which occurs during the early development of the child (e.g. vascular malformation, teratoma, fibrous dysplasia).

2. A normal structure that is enlarged (e.g. inflamed lymph node) most often from an inflammatory process.

3. A collection of abnormally proliferating (multiplying) cells which occurs because of an error in cell cycle where the replication process just doesn’t turn off appropriately.  This collection of cells is called a neoplasm. Neoplasms can be benign or malignant (cancerous), distinguished by the fact that the cells of malignant tumors are so dysfunctional that they can now break away from the main collection of cells (primary tumor) and spread via the blood or lymph to other parts of the body.

Terminology for Craniofacial Tumors

In general, doctors are very casual and sloppy about their use of terms when it comes to communicating with patients about a detectable, abnormal volume of tissue. Below is a brief description of terms often used to describe and communicate findings:

Mass

You may often hear the term “mass” used before a diagnostic workup is initiated to refer to an abnormal volume of tissue.  The volume of tissue may be felt, visualized, or seen radiographically.  “Mass” could be a tumor, or it could be something like an infection with inflammation which causes the volume change.  It is the most general and nonspecific term used to describe an abnormal volume of tissue.  After a mass is determined to be a neoplasm (abnormal proliferation of a single type of cell), most doctors will often refer to it as a “tumor”.

Tumor

The word “tumor” is a Latin term that simply means swelling.  So when you twist your ankle and it swells up, the ankle is “tumorous”.   Over time, the word has almost become synonymous with “neoplasm” which is an abnormal proliferation (multiplying at an abnormally high rate) of cells forming a “mass” (detectable volume).  This is where the confusion takes place, because a tumor can also be a collection of malformed cells or structures that occurs as an error of tissue development (embryogenesis).  The malformed cells have normal cell cycle processes, so they are not growing like a neoplasm (see below), but likely at some point when the error occurred, they did multiply too much which is the reason that there is an abnormally large volume of this malformed tissue present. Nonetheless, these malformations do not maintain their proliferative capacity after birth. They are treated very differently than neoplasms. Examples would be vascular malformations and teratomas.

Neoplasm

The term “neoplasm” specifically refers to a collection of cells derived from a single cell that lost its ability to regulate its capacity to multiply normally and consequently, multiplies at an abnormally high rate, ultimately forming a “tumor” or “mass”, a detectable volume of this collection of cells.  Here is where it can get even trickier. Neoplasms can be benign or malignant.  A benign tumor is one where the cells that are proliferating at an abnormally high rate do not have the ability to break away from the original focus and spread to other parts of the body via the blood or lymph system.  The cells of a malignant tumor (cancer) do have the ability to break away from the main tumor and spread through the body.

Why are craniofacial tumors unique?

Craniofacial tumors are unique for a couple of reasons.  First and foremost, the types of tumors that occur in the head and face region are different from other regions of the body.   The craniofacial region is a very busy place, with a number of unique and specialized structures.  Each of these can form tumors that are unique and do not occur in other places of the body.  These unique tumors require a unique skill set and approach from the doctors that treat them. The diagnosis and treatment often requires a very close and collaborative effort among a number of specialists including craniofacial surgeons, neurosurgeons, ophthalmologists, otorhinolaryngologists, oncologists, and radiation oncologists, among others.

Because many of the congenital malformations and syndromes that craniofacial surgeons treat transgress all the regions of the skull and face,and it takes collaborative programs to successfully treat these patients, craniofacial surgeons and programs are uniquely poised to treat craniofacial tumors, as well.  Craniofacial surgeons have unique knowledge about access to every aspect of the craniofacial region. They are also experts at surgically reconstructing every aspect of the craniofacial region from the top of the head to the based of the neck.  Our craniofacial surgeons are not only focused on achieving a permanent cure for the patient, but also restoring function once the tumor is removed.  We are focused on saving the life and “making it the best it can be” for years to come.

What is a skull base tumor?

A subset of craniofacial tumors occur at the base of the cranial cavity at the interface of the orbits, brain, and nose; the brain and spine; the brain and ear cavities; or the brain and the major vessels and nerves that move from the face to the cranial cavity and vice versa.  Because of their unique anatomic position and the difficulty associated with gaining safe access, the tumors are referred to as “skull base tumors”. Because of the strong partnership of the specialists within the Craniofacial Team of Texas, we successfully treat a number of skull base tumors. We have assembled a very unique team encompassing all the skill sets in a culture of collaboration and teamwork to achieve the best results possible in removing the tumor and then reconstructing to restore function and form.

What problems do craniofacial tumors cause?

The form, position, and relationship of all the unique craniofacial structures determine proper function. Any abnormal volume of tissue (tumor) can cause loss of normal function.  Humans use the craniofacial region to think, control the body, breath, eat/drink, smell, see, taste, express emotions, feel, speak, and communicate.  All this is in a relatively tight space that starts pretty small and grows a great deal during childhood.  The volume of a tumor alone may cause a loss of function.  It takes a very small tumor to completely block the nasal airway for example. Tumors that exert pressure on adjacent structures, either because the tumor is growing or just because pressure within it pushes adjacent tissues, often cause remodeling of the adjacent structures.  In the growing child, disturbance of growth occurs as growth around the tumor or in abnormal form.   In the case of cancerous tumors, the multiplying cells can just invade and destroy the adjacent tissue.  As mentioned above, the craniofacial region is very tight and relies on a very specific structure of its components to function properly. Anything that causes destruction or derangement of the tissues will affect function that is almost always impactful to the patient.

Are pediatric craniofacial tumors different than adult craniofacial tumors?

Pediatric tumors are very different than adult tumors.  Whereas most pediatric tumors are not malignant neoplasms (cancers), many adult tumors are.  A significant portion of pediatric craniofacial tumors is benign tumors or malformations.  While some of them have the potential to become cancers over time, most cause problems by growth and compression of adjacent critical structures or disturbing the normal growth of adjacent structures.  Additionally, pathologists have a very difficult time determining the biological behavior of many pediatric tumors because they tend to be rare, so there are few series that clinically trace the behavior back to the pathology. The cellular findings are often somewhat mixed with some features of a cancer and some features of a benign tumor, as well.  Our pathologists often consult national pathological experts to consult on cases to ensure proper diagnosis, so treatment decisions are made with the best information possible.  Experience with this unique set of tumors is paramount to proper diagnosis and treatment.  Having the proper focus and organization is just as important.  Through our multidisciplinary care platforms and case management conferences, the Craniofacial Team of Texas is ready to treat the most complex craniofacial tumors.

What therapies are used to treat craniofacial tumors and how are treatment decisions made?

Depending on the nature of the tumor, there a number of treatment modalities used to manage the broad range of tumors in this region.  Vascular malformations, for example, are treated with various combinations of sclerotherapy, laser, medications, and surgery; whereas, cancerous tumors of the craniofacial region are treated with various combinations of surgery, chemotherapy, and radiation.  Once a diagnosis is made, patients are often managed through specific care conferences and programs that specialize in the specific disease process, and where disease coordinators ensure proper data collection, specialists’ collaboration, and decision-making.

Vascular malformations are specifically referred to the Vascular Malformation Center where dermatologists, craniofacial/plastic surgeons, interventional radiologists, hematologists/oncologists, and radiologists that specialize in these unique tumors come together to evaluate, diagnose, plan treatment, and manage these unique patients. (see Vascular Malformations Section)

Neoplasms are unique in that they represent a higher risk for local or, in the case of cancerous tumors, distant recurrence and spread.  Neoplasms are managed through the Neuro-Oncology Program where oncologists, neurosurgeons, radiation oncologists, radiologists, pathologists, palliative care, and craniofacial surgeons collaboratively work together to decide on proper diagnosis, treatment, management, and long-term surveillance of each unique patient.  The Neuro-Oncology Program manages its patients through case coordination and a collaborative case conference, the Neuro-Onc Conference.

Types of Craniofacial Tumors

Malignant Neoplasms

• Rhabdosarcomas
• Chondrosarcomas
• Ewing’s Sarcoma
• Osteosarcoma
• Malignant ameloblastoma
• Dermatofibrosarcoma protuberans
• Malignant peripheral nerve sheath tumors
• Langerhans Cell Histiocytosis
• Esthesioneuroblastoma
• Plus many others

Benign Neoplasms

• Hemangiomas
• Hemangiopericytomas
• Myofibromas
• Osteoma
• Osteoblastoma
• Giant Cell Granuloma
• Desmoid tumors
• Ameloblastoma
• Odontogenic tumors
• Osteochondroma (Exostosis)
• Fibroma
• Lipomas
• Enchondroma
• Neurofibroma (Neurofibromatosis)
• Fibrous dysplasia
• Plus many others

Malformations

• Harmartomas
• Teratomas
• Dermoid cysts (non-ovarian)
• Vascular Malformations (AVM, Venous, Lymphatic, etc.)
• Plus many others

If you would like more information about craniofacial tumors, please contact the Craniofacial Team of Texas by calling 512-377-1142 or toll free 877-612-7069 to schedule an appointment or complete an online appointment request.