TUMORS OF THE HEAD AND NECK
SOURCE: Dept. of Otolaryngology, UTMB, Galveston, TX
RESIDENT PHYSICIAN: Carl "Rusty" Stevens, MD
FACULTY PHYSICIAN: Christopher Rassekh, MD
SERIES EDITOR: Francis B. Quinn, Jr., M.D.
DATE: November 12, 1997
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Vascular tumors of the head and neck region comprises a heterogeneous group of lesions that have very different histology, presentations, clinical courses and treatment options. The difficulties inherent to the study of such a varied group are compounded by the fact that present literature contains a multitude of classification schemes and obscure terms. This grand rounds will attempt to present the material in an orderly fashion with emphasis on the clinical presentation, usual natural history, and basic management techniques. The lesions are grouped into three broad categories including benign lesions, malignant lesions, and those of congenital or acquired origin. A section briefly describing associated syndromes is also included.
Hemangiomas are the most common congenital lesion in man and occur in approximately two to three percent of infants. Their usual natural history dictates that many will not be visible at birth but will declare themselves in the first few months of life. By age one, approximately twelve percent of the population has a hemangioma.1 Fortunately, they are almost always benign and usually involute by age five to seven years. Hemangiomas are actually hamartomas of blood vessels, not true neoplasms, and are often divided into three subgroups based on the size of the vessels.
Capillary (strawberry) hemangiomas are by far the most common and are more frequent in females. They are usually red or pink in color and are often papular with a lobular surface. An often alarming rate of enlargement occurs until the child is ten to twelve months of age at which time the lesions usually stabilize and eventually regress with few remaining by seven years of age. Capillary hemangiomas involving the larynx usually occur in the subglottis and are found mainly in the pediatric population.2,3 These lesions typically present in a previously healthy infant who develops biphasic stridor during the first few months of life. As with other pediatric airway problems, the child may be initially referred to the otolaryngologist for a further work up of recurrent episodes of croup. At endoscopy, the lesions are usually located in the posterolateral subglottis and are submucosal, soft, compressible masses. Because the overlying mucosa is normal, many of these hemangiomas will not display the usual red or pink color of a cutaneous lesion. Pediatric subglottic hemangiomas generally behave like their cutaneous counterparts with eventual regression occurring in most cases. For this reason, a conservative approach to management is usually appropriate as long as the airway is acceptable or is secured by tracheotomy. Medical management, discussed below, as well as excision with the CO2 laser may be appropriate in some cases.3 One-half of these children will have an associated cutaneous hemangioma.
Cavernous hemangiomas also are more frequently found in females and are soft, compressible, often poorly defined lesions. They tend to involve deeper structures and many do not regress. Even with regression, they often leave disfigurement. These hemangiomas may present in skeletal muscle such as the sternocleidomastoid, masseter, or scalene muscles. Mandibular or maxillary bone involvement may be seen with a characteristic "soap bubble" appearance on X-ray. Extraction of a nearby tooth may lead to dangerous bleeding in these cases. The majority of hemangiomas arising in the salivary glands of adults are of the cavernous type.4 When laryngeal hemangiomas present in an adult, they are more commonly cavernous in nature and are usually located in the supraglottis. Hoarseness is the main symptom with bleeding or respiratory compromise rare.2,5
The final subgroup is mixed hemangiomas which are comprised of blood vessels of various sizes. These hemangiomas often develop in the parotid gland and as such, represent the most common pediatric parotid tumor. Their presentation is typical of other hemangiomas with a slowly enlarging mass that is not present at birth but becomes apparent during the first few months of life.
The diagnosis of hemangioma is established by clinical findings and history in most cases although ultrasound, computer tomography, and particularly magnetic resonance imaging may be helpful in certain situations. MRI can accurately determine the extent of the lesion and the finding of serpentine high-volume flow voids surrounded by nonvascular soft tissue is characteristic of hemangiomas.6 Biopsy is rarely indicated and may be dangerous.
As mentioned above, most of these lesions will eventually regress and therefore the most important initial management includes close observation with careful measurements and photodocumentation, in addition to parental reassurance. This includes informing the parents of the expected natural history of hemangiomas and that if definitive surgery is required, it will likely provide a better cosmetic result and will be safer if performed after maximal involution has occurred. However, additional measures including steroids, interferon or excision may be required in rare cases (5-10%).6 Indications for more aggressive management include massive, ulcerative, disfiguring lesions, or those that produce hematologic, cardiovascular or upper aerodigestive tract compromise. Also, large periorbital hemangiomas that involve obstruction of vision are an indication for prompt definitive management. In these cases, medical management is usually attempted initially, and if successful in slowing or halting the proliferative phase, the final cosmetic result will be improved. Steroid treatment, whether systemic or intralesional will provide better results if used early in the course of the disease (before age one). Edgerton describes a course of alternate day, high dose steroids using 40 milligrams of prednisone (for a fifteen pound child) every other day for sixteen days and then tapering the dose by one-half every two weeks.7 He reports that ninety percent will stop growing and begin to regress with this treatment although as many as thirty percent will show rebound growth when the dose drops to 5-10 milligrams per day. In these cases, the dose is increased again to 15 milligrams for another ten days. The child must be closely monitored during this course for any evidence of systemic steroid toxicity. Although these results are encouraging, others have experienced less dramatic response with steroids and recent literature has focused on the use of interferons in the treatment of this disease. The ability of interferon to cause regression of vascular lesions was first noted incidentally through its affect on Kaposiís sarcoma during trials assessing its antiviral benefits in HIV positive patients. Bauman et al recently published a report of ten patients treated with alpha 2a interferon.6 They achieved excellent results utilizing an outpatient regimen of nightly, subcutaneous doses beginning at 1 mU/m2 increasing weekly to a target dose of 3 mU/m2. They note that because of some questions surrounding the affect of interferon on the developing neurologic system, they now increase the dosage monthly instead of weekly and periodic neurologic evaluations are performed. Additionally, CBC and LFTís are monitored closely. The treatments are continued until a complete or near complete resolution has occurred. While the responses were variable, in those patients with complete or nearly complete hemangioma regression, subsequent discontinuation of therapy has not resulted in disease recurrence at a mean follow-up of 17 months. The final major consideration in the treatment of large hemangiomas is surgical excision. This is reserved for very select cases and is usually performed with the assistance of laser technology. Pre-excision superselective angiography with or without embolization may also be helpful with large lesions. An additional conservative measure that may be helpful in limiting the growth of a hemangioma is constant pressure applied via a Jobst garment.8
Lymphangiomas, in contrast to hemangiomas, are true malformations of the lymphatic system. They are often located in the head and neck region and can be divided into four classes clinically and histologically: capillary, cavernous, cystic (hygroma) and lymphangiohemangioma.1 Capillary lymphangiomas clinically present as slightly raised papules on the skin or mucous membranes. The tongue and floor of mouth are common locations for these lesions. Cavernous lymphangiomas are found primarily in the head and neck region and present as a painless swelling that resembles a lipoma in consistency. They occur equally between the sexes. Cystic hygromas also show no gender preferences and are usually located in the neck, particularly the posterior triangle. Lymphangiohemangiomas are mixed lesions that generally behave like other lymphangiomas but, as expected, tend to have episodic bleeding as a prominent feature. Lymphangiomas have variable natural courses but most tend to progress with time. They may also exhibit rapid enlargement during upper respiratory illnesses although they usually return to baseline after the URI resolves. Use of antibiotics during these episodes has been reported to be beneficial.9 The differential diagnosis includes hemangioma, branchial cleft cyst, lipoma, thyroglossal duct cyst and teratomas. Use of MRI is particularly helpful in establishing the diagnosis and estimating the extent of the lesion. It is also helpful in following patients after treatment to detect recurrence.
The treatment of choice for these lesions is conservative resection although recurrence rates are often high. Definitive management is indicated when vital structures are endangered or when episodic hemorrhage occurs. Another indication for surgical management is associated macroglossia which may lead to articulation, deglutition or dental occlusion problems, in addition to airway compromise.9 In a child, excision should be deferred until age three to five if possible to facilitate removal with preservation of all important structures. If the lesion remains stable or regresses during this time, continued observation may be warranted. An adjunct technique is needle aspiration to periodically decompress the lymphangioma. Other techniques such as sclerosing agents, radiation therapy, or steroids have not been shown to be very effective.9
Juvenile Nasopharyngeal Angiofibroma
Juvenile nasopharyngeal angiofibroma (JNA) is a rare, benign neoplasm that almost exclusively affects adolescent males. The classic symptoms at presentation are nasal obstruction and epistaxis. Additionally, persistent, unilateral middle ear effusion in this patient population should alert the physician to the possibility of this diagnosis and the need for appropriate nasopharyngeal examination. As the tumor enlarges, more obvious signs may develop related to the direction of tumor spread. These include orbital findings such as proptosis or limitation of ocular movements, cranial nerve deficits, sinusitis, noticeable cheek swelling or meningitis. On physical exam the lesions are usually gray or red in color and typically begin in the posterolateral nasopharynx. From this origin, angiofibromas may invade adjacent structures through direct extension and bone destruction or, more commonly, by following paths of least resistance. Extension anteriorly leads into the nasal cavity and posteriorly into the sphenoid sinus. Laterally, the tumor may enter the pterygomaxillary fossa via the sphenopalatine foramen. Bowing or erosion of the posterior maxillary wall at this point produces the characteristic findings on plain films (submental vertex) or CT scan. Exit from the pterygomaxillary fossa is usually directed laterally into the cheek and infratemporal fossa. A second route of spread from the pterygomaxillary fossa is into the orbit through the inferior orbital fissure and subsequently into the superior orbital fissure. Further growth leads to intracranial tumor lateral to the cavernous sinus.10 A less common route intracranially involves direct extension through the sphenoid bone resulting in tumor medial to the cavernous sinus. The diagnosis of juvenile nasopharyngeal angiofibroma is usually made clinically and radiographically. Characteristic findings with contrast computer tomography will often establish the diagnosis in addition to accurately determining bony involvement. Soft tissue extension and particularly intracranial involvement are better evaluated using MRI. Angiography is helpful diagnostically in determining the important feeding vessels and therapeutically through preoperative embolization. When possible, diagnostic angiography and embolization should be carried out simultaneously 48-72 hours prior to surgery. While the most common feeding vessels are from the external carotid system (internal maxillary and ascending pharyngeal arteries), internal carotid contribution may occur with large tumors, or those that are intraorbital or intracranial. Also, large lesions, or those that approach the midline, may receive contralateral blood supply and therefore angiography should include bilateral internal and external carotid systems. The only indications for biopsy of a suspected juvenile nasopharyngeal angiofibroma are those cases in which the diagnosis remains unclear after the above evaluation, or those in which primary radiation therapy will be utilized. Biopsy in the later case is needed to provide a tissue diagnosis to rule out malignant lesions such as rhabdomyosarcoma or olfactory neuroblastoma.11 Biopsy should only be undertaken in the operating room where the airway can be secured and appropriate equipment for hemostasis is available.
The treatment of choice for nasopharyngeal angiofibroma is surgical excision. As discussed above, preoperative embolization is important in reducing intraoperative blood loss. Additional measures of managing blood loss that should be considered include autologous blood banking preoperatively and the use of a Cell Saver device during the procedure. Appropriate exposure and visualization is particularly important when resecting these vascular tumors and several approaches have been described. Briefly, these include transpalatal and transpharyngeal routes to achieve access inferiorly and preauricular subtemporal access to the infratemporal fossa laterally. Anteriorly, a lateral rhinotomy or midfacial degloving procedure combined with medial maxillectomy or LeFort I osteotomy, provides exposure of the nasal cavity, ethmoid sinuses, maxillary sinuses, and pterygomaxillary fossa. A maxillary swing or facial translocation procedure can be extended to provide further access to the infratemporal fossa and skull base. Intracranial extension may also require a formal craniotomy. Most of these lesions require a combination of the above techniques based on location and the experience of the surgeon. Primary radiation therapy may be indicated for rare cases involving inoperable intracranial disease, a debilitated patient or one who simply refuses surgical management. While radiation therapy can be expected to offer a chance of cure similar to resection, long-term sequelae including secondary malignancy may also occur. Other modalities including chemotherapy, sclerotherapy and cryosurgery are of limited benefit.
Paraganglia are nests of cells originating from neural crest cells that have migrated in close association with the autonomic nervous system ganglion cells. The term paraganglioma applies simply to a neoplastic process, usually benign, that involves one of the paraganglia. Other terms such as glomus tumors (general term used to indicate a cluster of specialized cells and more appropriately applied to tumors of the skin and superficial tissues of the extremities), chemodectomas (describing a tumor from chemoreceptor origin), and nonchromaffin tumors (related to staining characteristics) have been used in the past but are less accurate in describing the pathology. Unfortunately, many continue to be used in current literature, particularly the term glomus tumor referring to temporal bone paragangliomas. However, it should be remembered that all paragangliomas are closely related to one another, as well as to pheochromocytomas of the adrenal medulla. Their histologic appearance is similar to the normal histology of paraganglia and includes two types of cells. Type I (chief) cells are APUD type cells with copious cytoplasm and large round or oval nuclei. Their cytoplasm contains dense core granules that store and release catecholamines. Type II (sustentacular) cells are elongated cells that closely resemble Schwann cells although their function is not entirely clear. The two cell types are arranged into clusters with a core of chief cells surrounded by sustentacular cells embedded in a fibrous stroma. These clusters make up the fundamental histologic structure ( termed "Zellballen") and may be somewhat enlarged in paragangliomas. Also, there are often areas of spindle-shaped cells ("sarcomatoid foci") and highly vascular areas that may resemble an angioma. Nuclear pleomorphism and cellular hyperchromatism are common in benign paragangliomas and should not be considered evidence of malignancy. In fact, there are no clear histologic characteristics of malignancy with these lesions. This diagnosis should be reserved for the presence of local, regional or distant metastasis.
These lesions can be divided into cervical and temporal bone classifications which, although similar in most respects, do have some differences in presentation, epidemiology, clinical behavior and treatment options.
The most common cervical paraganglioma, the carotid body paraganglioma, arises from the similarly named chemoreceptor located on the posteromedial wall of the common carotid artery at its bifurcation.
Grossly this paraganglioma is dark, tan to purple in color and is usually fairly well circumscribed although there may be only a very thin fibrous capsule. They tend to splay the carotid bifurcation as they enlarge and can extend along the internal carotid artery to the skull base. As presented above, carotid body paragangliomas also resemble pheochromocytomas but are chemically active less often than this adrenal counterpart. Carotid body paragangliomas occur in both familial and sporadic forms with clearly defined differences between the two types. The sporadic form is more common than the inherited variety and
is multicentric in approximately 10% of cases with bilateral carotid body lesions being the most common combination. Malignancy occurs in 6-12.5% of cases which ranks carotid body paragangliomas as the most frequently malignant head and neck paraganglioma. The hereditary form occurs in 7-9% of cases and is more frequently multicentric (30-40%). The inheritance pattern in these cases is autosomal dominant modified by genomic imprinting. Although the allele can be passed from either parent, only those from the father will lead to the paraganglioma phenotype in the children. It is thought that this occurs because the allele is only activated during spermatogenesis and not during oogenesis. Since treatment of smaller tumors carries a much lower risk of morbidity and mortality, and because of the autosomal dominant pattern of inheritance, routine examination and screening with MRI every two years for at risk individuals older than 16 to 18 years of age is recommended.12 This costly approach may be eliminated in the future if a reliable genetic screening test can be developed.
The vast majority of carotid body paragangliomas present as slowly enlarging (~5mm per year), non-tender neck masses located just anterior to the sternocleidomastoid muscle at the level of the hyoid. The classic finding is a mass in this location that is mobile in the lateral plane but limited in the cephalocaudal direction. Occasionally the mass may transmit the carotid pulse or demonstrate a bruit or thrill.13 As these tumors enlarge, progressive symptoms of dysphagia, odynophagia, hoarseness and other cranial nerve (IX-XII) deficits appear. The history should include questioning as to the presence of similar lesions in family members and symptoms associated with catecholamine production such as fluctuating hypertension, blushing and palpitations. Initial imaging includes MRI with gadolinium (tumors as small as 5 mm) and contrast CT which are effective modalities in this area and are non-invasive. Although it is an invasive test, carotid angiography has traditionally been the most useful diagnostic test for paragangliomas. This modality can establish the diagnosis, demonstrate multiple lesions, determine the size and vascularity of the tumor, as well as evaluate its blood supply. Additionally, it can be modified to include selective, controlled balloon occlusion of the internal carotid artery to evaluate the cerebral cross-flow. This information is important in preoperative planning and counseling of the patient as to the relative risk of surgery. The classic, pathognomonic finding on arteriogram is widening of the carotid bifurcation by a well-defined tumor blush ("lyre sign"). It should be emphasized that angiography of both carotid systems is required to rule out bilateral tumors. When available, magnetic resonance angiography can non-invasively provide detailed mapping of the carotid vessels and identification of additional neoplasms. Since embolization is not often utilized with carotid body paragangliomas, MRA may be an appropriate alternative to angiography in many cases.14 Biopsy, including fine needle aspiration is unnecessary, dangerous, and contra-indicated in the evaluation of suspected paragangliomas. Routine screening for urinary metanephrines and VMA, and serum catecholamines is probably only indicated for multiple or familial paragangliomas or in the presence of catecholamine related symptoms.15 However, considering the hazards associated with operating on a previously unsuspected, metabolically active tumor, an argument can be made for obtaining these studies in all cases.
The treatment of choice for most carotid body paragangliomas is surgical excision. However, because of their location in close approximation to important vessels and nerves, there is a very real risk of morbidity (mainly cranial nerve X-XII deficits and vascular injuries) and mortality which is estimated as 3-9%.16,17 Tumor size is important because those greater than 5 cm in diameter have a markedly higher incidence of complications ( 67% vs 15% for <5 cm).12 A classification system based on size and difficulty of resection has been developed by Shamblin that describes Group I tumors as small and easily dissected away from the vessels. Group II includes paragangliomas of medium size that are intimately associated with the vessels but are separable with careful subadventitial dissection. Group III tumors are large and typically encase the carotid requiring partial or complete vessel resection and replacement.18 An extensive and complete preoperative work up is essential for safe resection of carotid body paragangliomas. In cases where the internal carotid may require resection, a vascular surgeon should be available to assist if necessary. Perioperative alpha and beta adrenergic blockers should be given for all catecholamine producing carotid paragangliomas. Although some older articles recommend angiographic embolization preoperatively, other authors discourage this because it seems to be associated with an inflammatory phase that makes the subadventitial dissection more difficult. The approach is usually transcervical but occasionally a superiorly based carotid paraganglioma or one that reaches the skull base may require a skull base approach. Wide exposure is a must as is meticulous hemostasis. Early control of the proximal and distal vessels is then accomplished. Important neurovascular structures are identified and appropriately retracted. The tumor is then carefully dissected from the common carotid in a subadventitial plane. This dissection is carried superiorly, making every effort to protect the internal carotid. If necessary, the external carotid artery can be sacrificed in most cases. If the common or internal carotid is encased in tumor or damaged during resection, immediate repair/replacement should be performed. Again, the preoperative work up should indicate the likelihood of vascular involvement and give some indication as to how well the patient will tolerate temporary internal carotid compromise. Radiotherapy, either alone or in conjunction with surgery, is a second consideration and an area of some controversy. Historically, cervical paragangliomas have been considered radioresistant. Although several articles report good responses, others have demonstrated persistent disease in lesions treated with primary radiation therapy. Most authors still recommend XRT only for very large tumors, recurrent tumors or for those patients who are poor surgical candidates.19
Other related paragangliomas include those involving the vagus nerve, larynx, orbit and nasal cavity. All tend to have similar histology and sex distribution. Vagal neoplasms are similar to carotid body paragangliomas in clinical presentation and malignant potential. One diagnostic difference between the two is that vagal paragangliomas displace the internal carotid artery anteriorly and medially, whereas carotid body tumors usually splay the vessels as mentioned above. Paragangliomas of the larynx and orbit may occur and are often locally aggressive. Treatment of these paragangliomas is again primarily surgical excision with radiotherapy being particularly ineffective for laryngeal, orbital and nasal tumors.19
Temporal bone paragangliomas
Paraganglia in the adventitia of the jugular bulb or along the course of Jacobsonís nerve (usually over the promontory) may give rise to paragangliomas. Although these tumors are similar to the paragangliomas discussed above, the terms glomus jugulare and glomus tympanicum have persisted and will therefore be used in this text. Often the lesion is large at initial diagnosis and the paraganglia of origin not easily determined. In these cases the term jugulotympanic glomus is employed. As with cervical paragangliomas, these lesions are slow growing and locally invasive. Because of their location in the temporal bone, intracranial extension and cranial nerve involvement are more common with these tumors. They also tend to have a higher female predominance compared to carotid body and vagal paragangliomas. However, the malignant potential ( 2-3 %) and tendency for catecholamine secretion ( 1-3 %) is less with temporal bone tumors.19,20 Symptoms related to glomus jugulare or tympanicum include pulsatile tinnitus, hearing loss or aural fullness in addition to those related to cranial nerve involvement. A red, pulsatile mass behind the tympanic membrane is the classic finding on exam when the lesion involves the middle ear. A full head and neck exam including cranial nerve testing and audiometry, as well as a detailed medical and family history, should be carried out initially. As with other paragangliomas, further work up includes CT and/or MRI with contrast to evaluate bony and soft tissue extension respectively. Four-vessel cerebral angiography is indicated with large lesions or those possibly involving the carotid artery. In addition to ruling out a second paraganglioma, this study helps accurately determine the extent and vascularity of the tumor and may be combined with preoperative embolization of large lesions.20 As with cervical tumors, magnetic resonance angiography (MRA) may non-invasively provide similar diagnostic information. Clinical suspicion of catecholamine production should prompt 24 hour urine analysis for metanephrines, vanillylmandelic acid (VMA), epinephrine, and norepinephrine. If these vasoactive amines are identified, pheochromocytoma should be ruled out prior to crediting the glomus tumor with their production.20
Temporal bone glomus tumors differ from other paragangliomas in that they are considered radiosensitve.19 Both surgical resection and radiation therapy have associated advantages and disadvantages, and therefore the treatment of these lesions must be individualized to the patient and tumor. While a detailed discussion is beyond the scope of this text, general considerations include the age and overall health of the patient. Younger patients should strongly consider definitive surgical resection while radiation therapy may be more appropriate for an older individual or someone who is considered a poor surgical candidate. Also, close observation with repeated imaging may be a viable option for an elderly person with a very small, asymptomatic temporal bone paraganglioma.
As discussed above, there are no significant histologic differences between benign and malignant paragangliomas. The later diagnosis is reserved for those lesions that exhibit regional or distant spread. Although rare, carotid body and vagal body paragangliomas display malignant characteristics more frequently than those from other locations. Presentation, diagnosis and evaluation are similar to those for benign lesions, however treatment may include adjuvant radiation therapy. It should also be noted that malignant paragangliomas often behave in a manor similar to the benign lesions in that they are slow growing and patients may survive many years with extensive disease.19
Angiosarcomas are malignant lesions arising from endothelial cells and most commonly occur in the skin of the head and neck region. They are usually found in older individuals with men being affected approximately four times as often as women.21 The lesions typically present as a red discoloration of the skin and may have significant dermal spread. They are usually divided into high and low grade lesions based on microscopic findings but treatment is surgical excision with adjuvant radiation therapy in either case. The five year prognosis is poor at less than 50%.21,22
These rare malignant lesions develop from pericytes which are cells that surround endothelial cells. Approximately 16 % are located in the head and neck region and these tend to be less aggressive than those located in other areas.22,23 They usually appear as slowly growing, painless, rubbery, gray lesions. Wide surgical excision is the treatment of choice but recurrence occurs in approximately fifty-seven percent of cases.22
Histologically, Kaposiís sarcoma is a vascular malignancy composed of large, malignant appearing endothelial cells surrounded by spindle-shaped cells. Today, it is mainly found in patients with AIDS although it may occur in other groups. However, the head and neck region is disproportionately represented in patients who are HIV positive. Clinically, it presents as a nonpruritic, painless, flat or nodular, pigmented lesion that may be located on any skin or mucosa of the head and neck region. It may also occur primarily in lymph nodes. The lesions are usually purple in caucasians and nearly black in darker pigmented populations. While skin lesions are mainly a cosmetic concern, oral or aerodigestive tract tumors often cause functional problems related to pain, bleeding and obstruction. Excluding potentially lethal pulmonary Kaposiís sarcoma, treatment is usually geared toward reduction in symptoms and improved cosmesis. This should be accomplished when possible without further damage to the patients immune system. Radiation and chemotherapy are very effective but may have serious side effects in these patients. Other treatments such as interferon may also be effective but recurrence is common. Intralesional injection of vinblastine, cryotherapy and laser excision may be helpful with small tumors. Angiosarcoma is occasionally associated with Kaposiís sarcoma and is often rapidly lethal in this patient population.24
Congenital Malformations and Acquired Lesions
Port-Wine Stains (nevus flammeus)
Although often classified as an intradermal hemangioma, the histology and clinical presentation of these lesions more appropriately places them into the vascular malformation group. As such, they demonstrate vessels with mature endothelial lining and no hypercellularity on microscopic exam. They are present at birth, grow proportionately with other structures and rarely proliferate.1 Association with sensory nerves, particularly the trigeminal nerve in head and neck cases, is common. Initially, port-wine stains are pink, flat and sharply demarcated lesions. In older individuals, they often become more nodular and verrucous in appearance and a darker, scarlet or purple color develops. Treatment with a variety of techniques including tattooing, surgical excision, and camouflage with cosmetics has been reported. Additionally, the argon laser has been described as being an effective treatment, particularly with older, darker colored lesions.1 As with hemangiomas, there occurrence in various syndromes must be kept in mind to facilitate appropriate detection and management of other associated conditions.
AV Fistulas and Aneurysms
AV fistulas and aneurysms may be congenital or develop after trauma. Congenital AV fistulas, as with other vascular malformations, are usually present at birth. Proportionate growth with overall body growth is again the rule with these lesions although some may exhibit increased growth during puberty or pregnancy. They typically present as a pulsatile mass that is surrounded by hypertrophic, discolored tissue that may contain varicose veins. Also hyperhidrosis, hypertrichosis, and hyperthermia may be present in the area.1 Symptoms of a systolic bruit and throbbing pain may also be present. Some AV fistulae may demonstrate a palpable thrill. Cardiovascular overload and decompensation related to the lesion may be present although this is rare. Ultrasound and angiography are useful in confirming the clinical diagnosis and determining the extent of the lesion as well as delineating the vascular supply. Treatment is complete surgical excision after superselective embolization when possible. Ligation of a main feeding vessel may lead to rapid recurrence that is larger than the initial lesion. This is thought to be related to increased arterial pressure in smaller adjacent fistulae that then become dilated and clinically apparent. Aneurysms in the head and neck may present with similar symptoms and findings and treatment is again excision although this may be difficult in certain locations.
Telangiectasias are another benign vascular abnormality that affects the head and neck region. These are primarily associated with hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu disease. This autosomal dominant syndrome is characterized by telangiectasias of the nasal and oral mucosa as well as scalp and facial skin. The most common symptom is epistaxis related to nasal septal lesions but bleeding from oral telangiectasias may also be problematic. Treatment is directed toward eradication of the telangiectasias, classically by septal dermoplasty. Hormonal and laser treatments may also be helpful.
Many of the above vascular lesions are associated with well defined clinical syndromes. Consideration of these associations is important to the patientís overall care. When detected early, many of these other abnormalities can be effectively managed. A summary of several important syndromes with vascular components is provided below.1,25
Osler-Weber-Rendu syndrome (hereditary hemorrhagic telangiectasia):
Von Hippel-Lindau disease:
Blue Rubber Bleb Nevus syndrome:
Vascular tumors of the head and neck comprises a diverse group that includes benign, malignant and congenital/acquired lesions. They differ significantly in their presentation, course and treatment options. Biopsy is often not needed for diagnosis and may be contraindicated therefore a general understanding of the characteristics of each lesion is important to ensure that it is considered early in the work up. Many of these lesions are also associated with important systemic syndromes. Identifying other conditions involved in the syndrome may be important to the patients overall care.
Discussion by Christopher H. Rassekh, M.D.
Vascular tumors of the head and neck is a topic that includes many different types of tumors with a variable spectrum of behavior. Included in the classification of such tumors are benign neoplasms, malignant neoplasms and nonneoplastic growths. Although many of these lesions are rarely encountered in clinical practice, a solid understanding of the diagnosis and management of these lesions is very important. As described, the management of patients with such lesions varies from observation or medication to major high risk surgical procedures. I personally had a "strawberry" hemangioma of the conchal bowl as an infant that was treated with a radioactive gold seed (brachytherapy). This was effective and interestingly, the seed fell out just a few years ago. When I was a little older child, an x-ray revealed the lesion and my pediatrician, unaware of the implant done at a different hospital panicked and was concerned that I had aspirated a pin. Fortunately, it was clarified before an unnecessary esophagoscopy. We recently removed a hemangioma of the nasal cavity via a lateral rhinotomy because of severe recurrent epistaxis.
The advent of modern radiographic techniques particularly angiographic procedures has made the diagnostic workup of major vascular lesions much easier. Even with the use of MR and CT, however, some vascular tumors go unrecognized. Embolization and refinement of surgical approaches has allowed resection of lesions that were once unmanageable. I have had the experience of doing fine needle aspiration of a "glomus tumor" of the neck that was uneventful. I was misled by a nasopharyngeal ulcer and a neck mass that was more firm and mobile equally in the vertical and horizontal plane. The needle biopsy was interpreted as a nasopharyngeal carcinoma. A subsequent CT scan revealed that the tumor was almost certainly a chemodectoma (carotid body paraganglioma). When I sat down to review the cytology with the CT information, the cytopathologist said that the two entities could indeed be confused by cytology and that he could not exclude either one. The nasopharynx was biopsied and was negative (it was probably a Thornwaldt's cyst). We removed the 4.5 cm carotid body tumor with preservation of all the cranial nerves and the internal carotid and the patient did very well. We prefer surgical management of these lesions even if they are minimally symptomatic because the risk of resection increases with size (according to Shamblin and the experience in the literature, 5 cm is perhaps the magic number for likely necessity of internal carotid artery sacrifice). In elderly individuals or patients who do not tolerate test balloon occlusion, observation may be preferable to radiation because of the morbidity of radiation in the elderly. On the other hand, the dose of radiation required (usually 45-50gy is recommended) is less than what we use for malignant tumors. Octreotide scintigraphy is a newer technique that has been proposed to screen patients and families with paragangliomas for multiple tumors.
Juvenile nasopharyngeal angiofibroma is approached with a similar philosophy in that it is a histologically benign but clinically malignant tumor.
We recently completely removed a hemangiopericytoma of the deep lobe of the parotid and a consensus of our multidisciplinary team was that postoperative radiation should be given because the tumor is considered malignant and security of margins in the area is difficult to assess despite a very clean excision and the tumor was removed preserving a functional facial nerve. We have also seen these tumors in the nose and sinuses and neck.
There are other tumors that present in the head and neck or cranial base region that can be hypervascular but are not classified as vascular tumors per se. These include meningioma, esthesioneuroblastoma, plexiform neurofibroma, lymphoma and fibromyxoma. Some lesions are difficult to diagnose even with full imaging and histology and there are vascular tumor "mimics" like granulomatous diseases and angiolymphoid hyperplasia. Arteriovenous malformations can be very difficult to manage if vessel ligation has been performed previously preempting embolization therapy. One must respect the danger of all these different types of lesions and consider the particular anatomy, histology and biologic behavior in choosing management strategies.
1. Stal S, Hamilton S, Spira M. Hemangiomas, lymphangiomas, and vascular malformations of the head and neck. Oto Clinics 1986;19(4):769-96.
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