Tumors – Head & Neck
Overview
Many lesions can occur in the head, neck, or face which require treatment by craniofacial techniques. The initial appearance of a lesion may be a swelling or a lump or bump. It could appear on the skin of a newborn infant or on an older patient which in either case needs to be investigated and differentiated from any number of lesions. Most of these occur in children but may continue into adulthood. They are primarily related to embryologic and/or abnormalities of growth cells which are present in the child at the time of birth, or appear during early childhood development.
Cystic Hygroma
Case Studies
Preoperative frontal view of a 9-year-old with a facial mass. Postoperative frontal view 6 months after resection of the tumor. Pre and postoperative submental vertex views.
Fibrous Dysplasia
Description
Fibrous dysplasia represents a benign bony tumor which can occur in any portion of the craniofacial skeleton. Rarely, if ever, does this turn into a malignant tumor or a sarcomatous lesion. They usually develop in early childhood or teenage years and are very slow growing and progressive. They may appear as a bump on the forehead or head which gradually increases in size. It may also occur around the orbit with displacement of the eye and orbital area which may cause double vision. It may also occur anywhere in the bones of the face with gradual occlusion of the surrounding structures and end up presenting with a mass or lump in that bone or region.
The cause of fibrous dysplasia is not known. It is due to the overgrowth of fibrous cellular components which give rise to this slow growing tumor. Most frequently they are seen in children. These are of the mono-ostotic type which occur in the craniofacial region.
Expectations & Treatment
Evaluation is both clinical and radiographic. A C.T. scan examination is generally diagnostic and serves to define the entire scope of the lesion. One note of importance is that this lesion may involve the maxilla or the bones of the orbit. A complete opthalmological exam may be required pre-operatively.
Treatment consists of surgical extirpation of the involved skeletal bone with reconstruction using techniques such as split cranial bone grafting. In some special cases, the optic nerve may need to be released so as to minimize any compressive risk to this stucture. The prognosis is good with complete resection, or near total removal, usually being accomplished and an excellent aesthetic result expected with minimal disability or deformity.
Case Study
Hemangioma
Description of Hemangioma
Hemangioma and vascular malformations are frequently discussed in the same setting with the thinking being that these two conditions are due to their vascular nature. In fact, these lesions are completely different with different presentations, treatments, and long term outcomes.
A hemangioma is a non-malignant tumor which is made up of rapidly growing endothelial or vascular cells. They may be capillary, cavernous or mixed in origin. Usually these lesions appear at the time of birth, or shortly thereafter, as a small spot that appears pink or red. They may present on the face or elsewhere on the body. In may cases, this is the only manifestation of the process.
However, there are times when these lesions will undergo rapid growth during the newborn period. This rapid growth phase will last from 6 to10 months and during this time may grow very large. Problems that may occur during this stage include bleeding, infection, mass effect and the possible obstruction of critical structures such as the mouth, nose, eyes or ears, which would require early treatment.
Expectations & Treatment
Active therapies available today include laser treatment, injection and or oral corticosteroids, the utilization of interferon, pressure dressings, or direct surgical excision.Frequently, hemangiomas go on natural involution and begin to disappear after 10 months of age. In fact, 50% of patients will note complete involution of the lesion by 5 years of age. It may leave only a residual lump or bump of tissue which may need surgical excision.
Case Studies
Neurofibromatosis
Description of Neurofibromatosis
Neurofibromatosis is well known and was first felt to be occurring in James Merrick, or the “elephant man”. Today we know that his diagnosis was Proteus syndrome. The cause of neurofibromatosis, or von Recklinghausen disease, is unknown but there is a genetic predisposition.
The characteristics present in an infant with simple cafe au lait spots and minimal deformity. In time, this may progress into more massive deformities. Three basic types exists. Plexiform neurofibroma are flat lesions of moderate size which tend to follow the the nerve or nerve axis with marked enlargement. It infiltrates through the skin, subcutaneous tissue, deep planes, and even cause distortion of growth of the bone.
Mollusca fibrosa are small round nodules which may be present in large numbers. Elephantiasis neurofibromatosa is the most impressive manifestation due to its size. In this setting the lesion will lead to massive overgrowth of both the skin and the associated soft tissue.
Expectations & Treatment
Frequent, repeated surgical excision is performed and can give improvement. But since infiltration is throughout all of the planes of the tissues involved, it can be a long term progressive problem.
Case Studies
Dermoid Cysts
Description of Dermoid Cysts
Dermoid cysts are thought to be embryological remnants which form during the development of a child in utero. They may arise from the foramen cecum as small outpouchings of the dura, or they may only be superficial. In either case, a small firm cystic lesion is noted at birth. The lesion may occur anywhere along the mid-axial line of the face but are most commonly noted at the bridge of the nose, or lateral portion of the eyebrow.
Expectations & Treatment
Lateral dermoids tend to be of superficial origin and diagnosis is generally limited to a thorough clinical exam. Treatment is surgical and consists of cyst removal with mobilization and advancement of the surrounding tissues in order to close the cavity created by the cyst. Frequently, a dermoid will cause a small bony defect and this too will be masked by a well designed plastic surgical closure.
Medial or midline dermoids require a slightly more involved diagnostic work-up. In this setting, there is a high rate of communication between the dermoid and the underlying dura through the foramen cecum. Due to this possibility, a C.T. scan is required prior to any surgical intervention. If there is communication with the underlying dura, the aid of our pediatric neurosurgeon is required to remove the lesion. If the lesion does not communicate with the dura, a limited surgical approach is all that is needed.
Facial dermoids are benign lesions and as such, treatment plans tend to be highly successful. Surgical intervention usually provides a complete resolution of all symptoms and recurrence is rare.
Juvenile Ossifying Fibroma
Case Study
