unusual non epithelial tumors of head and neck

UNUSUAL NON-EPITHELIAL TUMOURS OF THE HEAD

AND NECK

Dr. AYUSH GARGPG JR I

RADIOTHERAPY

Classification

Non Epithelial Tumors of Head & Neck

• GLOMUS TUMORS

• HEMANGIPERICYTOMA

• CHORDOMAS

• LETHAL MIDLINE GRANULOMA

• CHLOROMA

• ESTHESIONEUROBLASTOMA

• EXTRAMEDULLARY PLASMACYTOMAS

• NASOPHARYNGEAL ANGIOFIBROMA

• EXTRACRANIAL MENINGIOMAS

• NON LENTIGINOUS MELANOMA

• CUTANEOUS MELANOMAS

• MUCOSAL MELANOMAS

• LENTIGO MALIGNA MELANOMA

• SARCOMAS OF THE HEAD & NECK

GLOMUS TUMORS

• Glomus bodies are found in the jugular bulb and along the tympanic (Jacobson) and auricular (Arnold) branch of the tenth nerve in the middle ear or in other anatomic sites .

• Depending on the location, glomus tumors (chemodectoma or paraganglioma) can be classified as tympanic (middle ear), jugulare, or carotid vagal, or designated as originating from other locations, such as the larynx, adventitia of thoracic aorta, abdominal aorta, or surface of the lungs .

• Glomus tumors consist of large epithelioid (smooth muscle) cells with fine granular cytoplasm embedded in a rich capillary network and fibrous stroma with reticulin fibers, which derive from embryonic neural crest cells.

Anatomy

Epidemiology

• The mean age at diagnosis has been reported to be 44.7 years for carotid body tumors and 52 years for glomus tympanicum.

• These tumors occur three or four times more frequently in women than in men, suggesting a possible estrogen influence .

• Glomus tumors may be familial.In the head and neck region, the most common association is bilateral carotid body tumors or carotid body tumor associated with tympanic-jugular glomus.

Clinical Presentation

•Glomus tumors may arise along the nerve roots. Glomus tumors of the middle ear may initially cause earache or discomfort.• As they expand, eventually they produce pulsatile tinnitus, hearing loss, and, in later stages, cranial nerve paralysis resulting from invasion of the base of the skull in 10% to 15% of patients.•If the tumor invades the middle cranial fossa, symptoms may include temporoparietal headache, retro-orbital pain, proptosis, and paresis of cranial nerves V and VI.

• If the posterior fossa is involved, symptoms may include occipital headache, ataxia, and paresis of cranial nerves V to VII, IX, and XII; invasion of the jugular foramen causes paralysis of nerves IX to XI.

• Chemodectoma of the carotid body usually presents as a painless, slowly growing mass in the upper neck.

• Occasionally the mass may be pulsatile and may have an associated thrill or bruit. As it enlarges, the mass may extend into the parapharyngeal space and be visible on examination of the oropharynx. Very rarely these tumors may be malignant .

• Metastases occur in 2% to 5% of cases.

Diagnostic Work-up

General 1.History 2.Physical examination-demonstrates a red, vascular middle ear mass, although occasionally it may be bluish or white 3.Examination of the neck may occasionally demonstrate a mass in the neck that may be pulsatile or have a bruit or regional lymph node metastases. 4.ENT examination

Radiographic studies

1.Computed tomography scan (with contrast) to define tumor extent and possible central nervous system involvement(High-resolution computed tomography (CT) with contrast has the highest degree of sensitivity and specificity to diagnose this tumor when located in the middle ear or jugular bulb) 2.Magnetic resonance imaging with gadolinium 3.Arteriography to determine bilateral involvement and collateral cerebral blood flow (optional) 4.Jugular phlebography (optional)

Laboratory studies 1.Complete blood cell count

2.Blood chemistry profile

3.Urinalysis

4.Cytochemical techniques demonstrate increased levels of serotonin, epinephrine, and norepinephrine in normal glomus tissue of the carotid body

Special tests

•1. Audiograms to establish baseline hearing loss-may demonstrate conductive hearing loss in the ear involved by tumor

2.Histologic staining to determine presence of catecholamines

3.Biopsy of glomus tumors may result in severe haemorrhage

•StagingThe prognosis of these tumors is closely related to the anatomic location and the volume of the lesion, which is reflected in the Glasscock-Jackson classification.

Glasscock-Jackson classification

Glomus tympanicum

I Small mass limited to promontory

II Tumor completely filling middle ear space

III Tumor filling middle ear and extending into the mastoid

IV Tumor filling middle ear, extending into the mastoid or through tympanic membrane to

fill the external auditory canal; may extend anterior to carotid

Glomus jugular

I Small tumor involving jugular bulb, middle ear, and mastoid

II Tumor extending under internal auditory canal; may have intracranial canal extension

III Tumor extending into petrous apex; may have intracranial canal extension

IV Tumor extending beyond petrous apex into clivus or infratemporal fossa; may have

intracranial canal extension

• General Management

Surgery

Surgery is generally selected for treatment of small tumors that can be

completely excised. Glomus tympanicum tumors are particularly well

managed with excision, via tympanotomy or mastoidectomy. Percutaneous

embolization of a low-viscosity silicone polymer has been used, frequently

as preoperative preparation of the tumor embolization of feeding vessels

allows meticulous microsurgery with virtually complete hemostasis

The local tumor control rate with surgery alone is only about 60%, and there is

significant morbidity, particularly cranial nerve injury and bleeding.

Radiation Therapy

Irradiation is frequently used in the treatment of glomus tumors,

particularly for those in the tympanicum and jugulare bulb locations.

Tumors with destruction of the petrous bone, jugular fossa, or occipital

bone or patients with jugular foramen syndrome are more reliably

managed with irradiation

• Radiation Therapy TechniquesRadiation therapy techniques are determined by the location and extent of the

tumor, which must be defined before treatment. Usually bilateral, portals should be used for relatively localized glomus tumors, whether or not the treatment is combined with surgery.

A:Portal used for relatively localized glomus tumor. B: Simulation film of patient with glomus tumor. C: Isodose distribution of a mixed-beam unilateral portal for a glomus tympanicum lesion (80% 16-

MeV electrons, 20% 4-MV photons).

• Superior-inferior 60-degree and 45-degree wedged filtered fields.

• Isodose distributions using superior-inferior pairs of 60-degree (A) and 45-degree (B) converging wedge filtered 60Co fields, demonstrating limited volume of irradiation.

HEMANGIOPERICYTOMA

• Hemangiopericytomas are rare soft-tissue neoplasms that account for 3% to 5% of all

soft-tissue sarcomas and 1% of all vascular tumors. Soccur in the head and neck; of

these, approximately 5% occur in the sinonasal area.ome 15% to 30% of all

hemangiopericytomas

• Epidemiology

• Hemangiopericytomas is an unusual tumor; it represents approximately 1% of all vascular

neoplasms; it occurs in both genders with equal frequency and is found primarily in adults.

• In the head and neck, the most common sites are the nasal cavity and the paranasal

sinuses, and usually, the orbital region, the parotid gland, and the neck.

Pathology

• Hemangiopericytomas are composed of a proliferation of tightly packed

pericytes around thin-walled endothelial-lined vascular channels ranging from

capillary-sized vessels to large, gaping sinusoidal spaces , benign tumors may

be difficult to differentiate from malignant ones.

• However, prominent mitoses, foci of necrosis, and increased cellularity are

suggestive of malignancy; the definitive sign is local recurrence or development

of metastases.

• The final diagnosis of hemangiopericytomas is based on the histopathology and

immunochemistry, and whether the tumor is benign or malignant is defined on

the basis of the clinical history. Hemangiopericytomas located in the sinonasal

area is generally benign.

• Clinical Presentation• Soft-tissue hemangiopericytoma is a firm, painless, slowly expanding mass that is

often nodular and well localized.

• In the head and neck, the tumor may constitute a polypoid, soft gray or red mass that grows slowly and may cause nasal obstruction. Epistaxis and nasal obstruction are common symptoms.

• On plain radiographs, hemangiopericytoma appears as a soft-tissue mass in the nasal cavity or other portions of the head and neck.

• On arteriography, according to Yaghmai (293), hemangiopericytoma is the only vascular tumor that has some characteristic angiographic features that include radially arranged or spiderlike branching vessels around and inside the tumor and a long-standing, well-demarcated tumor stain.

• The most distinctive and constant feature of this tumor is its hypervascularity; this tissue characteristic also may be demonstrated with contrast-enhanced CT.


• Complete surgical resection, if possible, combined with preoperative

embolization of the tumor, is the treatment of choice.

• For incompletely resected tumors, postoperative radiation therapy is used.

• The role of chemotherapy in this tumor is not well determined; Doxorubicin, alone or

in combination-drug regimens, is the most effective agent for metastatic

hemangiopericytoma, producing complete and partial remissions in 50% of cases.

Other drugs prescribed when metastasis occurs are cyclophosphamide, dacarbazine,

vincristine, and actinomycin-D.

Radiation Therapy Techniques

• The role of radiation therapy alone in the management of hemangiopericytoma is controversial.

• The main role of irradiation is as an adjuvant after complete excision of the lesion or postoperatively for minimal residual disease.

• The tumor has been considered relatively radioresistant.

• Tumor doses of 60 to 65 Gy in 6 to 7 weeks are required to produce local tumor control in postoperative cases.

• The fields of irradiation should be wide, to encompass the tumor bed with a margin of at least 5 cm to safely avoid marginal recurrence. Portal arrangement and beam selection are similar to those used in treatment of malignant brain tumors or soft-tissue sarcomas.

CHORDOMAS

• Anatomy

• Chordomas are rare neoplasms of the axial skeleton that arise from the remnant of

the primitive notochord (chorda dorsalis). About 50% arise in the sacrococcygeal

area; 35% arise intracranially, where they typically involve the clivus, and the

remaining 15% occur in the midline along the path of the notochord, primarily

involving the cervical vertebrae

Epidemiology

• Chordomas are more common in patients in their 50s and 60s but can occur

in all age groups. In children and young adults the prognosis and long-term

survival appear to be better than in older patients (289). No risk factors have

been identified. Male predominance is reported at a 2:1 to 3:1 ratio.

Pathology

• Chordoma is a soft, lobulated tumor that may have areas of hemorrhage,

cystic changes, or calcification. It is frequently encapsulated but may be

nonencapsulated or pseudoencapsulated. Histologically, it is composed of

cords or masses of large cells (physaliferous cells) with typical vacuoles and

granules of glycogen in the cytoplasm and abundant intercellular mucoid

material. Usually there are few mitotic cells.

• Clinical Presentation

• Chordomas tend to originate from the clivus and chondrosarcomas from the temporal

bone. In the head, extension may be intracranial or extracranial, into the sphenoid

sinus, nasopharynx, clivus, and sellar and parasellar areas, with a resultant mass

effect. In chordomas of the spheno-occipital region, the most common presenting

symptom is headache. Other presentations include symptoms of pituitary

insufficiency, nasal stuffiness, bitemporal hemianopsia, diplopia, and other cranial

nerve deficits.

Diagnostic Work-up for Chordoma

• General History Physical examinationRadiologic studies Plain radiographs Computed tomography scan/magnetic resonance imagingLaboratory studies Complete blood cell count Chemistry UrinalysisSpecial studies Endocrinologic profile (clivus) Visual evaluations (clivus)


• A surgical approach is recommended (when feasible), but complete surgical

extirpation alone is unusual. Regression of preoperative symptoms without additional

postoperative morbidity could be achieved by radical transoral tumor extirpation

documented by MRI. Intracranial spread usually requires steroid coverage and

therapy directed to correction of neurologic deficits that may be present. Because of

the high incidence of local recurrence, combined surgical excision and irradiation is

frequently used. No effective chemotherapeutic agent or combination of drugs has

been identified.


• Basisphenoidal tumors usually are treated by a combination of parallel

opposed lateral fields, anterior wedges, and photon and electron beam

combinations, depending on the extent of the neoplasm. Precision radiation

therapy planning, using CT and MRI, is required because high doses of

external-beam radiation therapy are needed. Three-dimensional CRT or

IMRT provide optimal dose distributions

Lethal Midline Granuloma• Natural History and Pathology

• Lethal midline granuloma (LMG) or midline malignant polymorphic reticulosis is a

clinical entity characterized by progressive, unrelenting ulceration and necrosis of the

midline facial tissues. LMG is associated with Epstein-Barr virus.

• Three clinicopathologic entities: Wegener's granulomatosis, LMG, and polymorphic

reticulosis (PMR).

Clinical Features and Diagnostic Work-Up

• It includes progressive nasal discharge, obstruction, foul odor emanating from the nose,

and, in later stages, pain in the nasal cavity, paranasal areas, and even in the orbits.

• Examination discloses ulceration and necrosis in the nasal cavity, perforation or

destruction of nasal septum and turbinates, and even ulceration of the nose. Edema of

the face and eyelids may be noted, and the bridge of the nose may be sunken.

Radiographic studies initially show soft-tissue swelling, mucosal thickening, and findings

consistent with chronic sinusitis.

• CT is invaluable in demonstrating the full extent of the tumor, including bone or cartilage

destruction.

• General Management and Radiation Therapy Techniques

• When treatment of these patients is planned, it is extremely important to exclude the

diagnosis of Wegener's granulomatosis, a benign process that is commonly treated with

antimicrobial agents, steroids, and systemic chemotherapy .LMG does not respond to

steroids; the treatment of choice is radiation therapy.

• Target volume should encompass all areas of involvement, including adjacent areas at

risk (i.e., for a lesion of the maxillary antrum it will include the antrum as well as all of the

paranasal sinuses) with a 2- to 3-cm margin. Because marginal failures are a significant

problem, wide margins are necessary for treatment of these patients.

• Irradiation techniques are similar to those described for tumors of the paranasal

sinuses, nasal cavity, or nasopharynx. Several investigators have described complete

responses with doses of 30 to 50 Gy; most patients are treated with 35 to 45 Gy in 3 to

4.5 weeks

Chloroma• Natural History

• Chloroma (granulocytic sarcoma, myeloblastoma) is a solid extramedullary tumor

composed of early myeloid precursors usually associated with acute myelocytic

leukemia (41); the most common sites of presentation are in the orbit and other

craniofacial bones. The name chloroma (from the Greek chloros, meaning green)

derives from the green color of affected tissues resulting from the presence of

myeloperoxidase.

• Children are affected more often than adults.Chloromas are found more frequently in

children with the M4 and M5 acute myeloid leukemia subtypes of the French-

American-British Cooperative Group Classification and are also associated with the

8:21 translocation.

Clinical Presentation and Diagnostic Work-Up

• Intraorbital (retrobulbar) chloroma causes progressive exophthalmos or

temporal swelling. Central nervous system involvement causes both local

pressure phenomena and generalized elevation of intracranial pressure with

headaches, nausea, and vomiting.

• All patients require complete hematologic and neurologic testing as is true

for any patient with suspected leukemia. Open biopsy remains the best

diagnostic tool. Plain radiographic findings consist mainly of localized bone

destruction with predominantly lytic lesions and associated soft-tissue

masses in orbital and periorbital chloromas.

• Radiation Therapy Techniques

• Chloromas are extremely radiosensitive.The maximum dose needed for treatment of

chloromas, it appears that 30 Gy is the maximum required for local control. There

appears to be a relationship between the size of the chloroma and the total dose of

irradiation required for control. The target volume is the tumor mass and an adequate

margin (2 to 3 cm). Irradiation techniques depend on the location of the infiltrate. For

superficial lesions, electron beam is recommended.

Esthesioneuroblastoma(EBN)• Rare tumors thought to arise in the olfactory receptors in the nasal mucosa or the

cribriform plate of the ethmoid bone. The olfactory nerves perforate grooves in the

ethmoid bone in the cribriform plate and continue into the subarachnoid spaces,

accounting for the high incidence of intracranial extension.

• Epidemiology

• ENB constitutes 3% of all endonasal neoplasms.

• There appears to be a slight male predominance. The age incidence has a bimodal

distribution, with peaks at 11 to 20 years and 40 to 60 years, the highest incidence at

51 to 60 years.

• Clinical Presentation

• These tumors tend to be friable and bleed easily. The most common clinical symptoms are

epistaxis and nasal blockage. Patients also may have local pain or headache, visual

disturbances, rhinorrhea, tearing, proptosis, or swelling in the cheek. The symptoms may be

associated with a mass in the neck.

• Diagnostic Work-Up

• Physical examination may show the inferior aspect of a polypoid friable mass in the nasal

cavity. Ocular findings or a mass in the nasopharynx may be present. With early lesions,

radiographs or CT or MRI may show only nonspecific opacification, soft-tissue swelling, and

occasionally bone destruction. Octreotide is a somatostatin analog that, when coupled to a

radioisotope, produces a scintigraphic image of neuroendocrine tumors (NET) expressing

somatostatin type 2 (SSR 2) receptors. Octreotide scintigraphy (OS) may be useful in

confirming the preoperative diagnosis of certain head and neck NET, such as paragangliomas,

Merkel cell carcinomas, medullary thyroid carcinomas, and esthesioneuroblastomas.

Diagnostic Work-up

General History Physical examination ENT examinationRadiographic studies Computed tomography scan (with contrast) to define tumor extent and possible central nervous system involvement Magnetic resonance imaging with gadolinium Arteriography to determine bilateral involvement and collateral cerebral blood flow (optional) Jugular phlebography (optional)Laboratory studies Complete blood cell count Blood chemistry profile UrinalysisSpecial tests Audiograms to establish baseline hearing loss Histologic staining to determine presence of catecholamines

Staging

• Pathologic Features and Prognostic Factors

• ENBs are polypoid, frequently reddish, soft, and vascular tumors with neuroblasts

and neurocytes.ENB contains epithelial components serving as a supporting stroma

and have a nerve component that corresponds to the olfactory cells. Rosettes are the

main feature, consisting of several rows of cells arranged around the central area.

General Management

• Surgery alone appears to be adequate treatment for small, low-grade tumors

confined to the ethmoids in which negative surgical margins can be obtained .

An ethmoidomaxillary resection with or without orbital sparing is usually

necessary. This procedure is combined with preoperative or postoperative

irradiation.

• For advanced lesions, in which disseminated disease is likely, chemotherapy

may improve tumor control and decrease the incidence of distant metastases.

A combination of thiotepa, cyclophosphamide, doxorubicin, vincristine,

nitrogen mustard, and actinomycin-D has been used.

Elective Neck Treatment

• Esthesioneuroblastoma has been shown to metastasize to the neck and

remote sites.Cervical lymph nodes are the most common site.In general,

because of the low incidence of cervical lymph node metastasis in early-

stage disease, elective irradiation of the neck or a dissection is not indicated

. However, in patients with Kadish stage C disease, the cervical metastatic

rate climbed to 44% . Thus, with advanced-stage disease, cervical nodes

should be initially managed by irradiation, radical neck dissection, or a

combination of both.

• Radiation Therapy Techniques

• A combination of photons and electrons with anterior fields provides good coverage for

limited ethmoidal disease when the tumor is confined anteriorly.

• When intracranial or posterior extension is present or tumor has spread into the

maxillary sinus, a pair of perpendicular (anteroposterior and lateral) portals with

wedges or two lateral wedge fields in conjunction with an open anterior photon field

will give good coverage of the treatment volume with the dose inhomogeneity around

10% to 20%.

• Occasionally, an anterior electron beam field may be needed to supplement low-dose

areas. When the electron beam is used over air cavities, some dosimetry problems

result. Eye blocks must be positioned precisely to avoid undesirable side effects.

• When combined therapy is used, preoperative doses of 45 Gy and postoperative

doses of 50 to 60 Gy are indicated, depending on the status of the surgical margins.

Doses of 65 to 70 Gy are delivered with irradiation alone in patients with inoperable

tumors.High doses per fraction (exceeding 2 Gy) increase the possibility of late

sequelae such as blindness and bone and brain necrosis.

Extramedullary Plasmacytomas• Solitary plasmacytomas are rare tumors of plasma cell origin making up 4%

of all plasma cell tumors.

• Multiple myeloma occurs about 40 times more frequently than solitary

plasmacytoma.

• Monoclonal extramedullary plasmacytoma (EMP) is a rare, low-grade

lymphoma found predominantly in the head and neck region.

• Diagnostic procedures exclude a benign polyclonal plasmacytoma, multiple

myeloma, and solitary bone plasmacytoma.

• The slow natural progression of the disease and the rarity of secondary

multiple myeloma favor nonmutilating local surgery whenever possible to

avoid the long-term sequelae of radiation.

Epidemiology

• The annual incidence of EMP is 0.04 cases per 100,000 population.

They constitute only 0.5% of all upper respiratory tract

malignancies. Male patients exceed female patients by a ratio of

4:1, and 75% of patients are 40 to 60 years of age.

• The most common sites in the head and neck are the nasopharynx,

nasal cavity, paranasal sinuses, and tonsils.

Clinical Presentation and Diagnostic Work-Up

• The most common symptoms are nasal obstruction, local pain and swelling, and epistaxis.

• Grossly, plasmacytomas tend to be sessile in the nasal cavity and paranasal sinuses and pedunculated in the nasopharynx and larynx. The masses are soft, pliable, and pale gray. The lesion may remain localized or may infiltrate and destroy the surrounding soft tissue and bone.

• The usual criteria for solitary plasmacytomas, either medullary or extramedullary, include a biopsy-proven plasma cell tumor with one or, at the most, two solitary foci, absence of Bence-Jones protein in the urine, bone marrow taken some distance from the primary site not involved by tumor (<10% of plasma cells), hemoglobin of 13 g/mL or more, and a normal serum protein level or serum electrophoresis at the time of the diagnosis.

• Basically, the diagnosis of solitary plasmacytoma is made by exclusion, that

is, by eliminating the possibility of multiple myeloma. Diagnosis is based on

histology along with special immunoperoxidase staining for immunoglobulin

lambda and kappa light chains

• Bone destruction is not a particularly bad prognostic sign, although some

investigators report that it adversely affects prognosis. Bony invasion is

common in the more malignant types

• The exact relationship between EMP and multiple myeloma is unclear;

however, approximately 20% to 30% of EMP cases will convert to multiple

myeloma


• Irradiation techniques vary with the location of the primary tumor. The

techniques are similar to those used for primary tumors in comparable

locations (i.e., nasopharynx, tonsil, paranasal sinuses). Solitary

plasmacytomas respond well to doses of 50 to 60 Gy in 2-Gy fractions. The

local tumor control rate with radiation therapy alone is about 85%.

•

Nasopharyngeal Angiofibroma

Epidemiology• Juvenile nasopharyngeal angiofibroma (JNPA) is found more

frequently in young pubertal boys; it has been shown to contain androgen receptors and occasionally to regress with estrogen therapy. androgen receptors in 75% cases, whereas only 8.3% were positive to progesterone.

• JNPA comprises <0.05% of head and neck tumors . Patient age at presentation ranges from 9 to 30 years, with a median of 15 years. Females comprise <4% of the total cases.

• The tumor is believed to originate from the posterolateral wall of the nasal cavity where the sphenoidal process of the palatine bone meets the horizontal ala of the vomer and the roof of the pterygoid process because it is always involved. Other investigators agree, because involution of tumor after irradiation usually occurs in this direction.

Clinical Presentation and Pathology

• Symptoms usually occur 2 to 48 months before diagnosis. The most

common complaints are nasal obstruction or epistaxis, followed by nasal

voice or discharge, cheek swelling, proptosis, diplopia, hearing loss, and

headaches.

• Nasopharyngeal angiofibroma may initially extend into the nasal fossae

and maxillary antrum and push the soft palate downward, then through

the pterygopalatine fossa and superoanteriorly through the inferior orbital

fissure or laterally through the pterygomaxillary fissure to the cheek and

temporal regions

Diagnostic Work-Up• After the history and physical examination, CT scans with and without

contrast should be obtained. The pattern of enhancement in this highly

vascular tumor is diagnostic.

• CT scans are especially helpful in regions involving thin bony structures

(paranasal sinuses, orbits), where CT performs better than MRI. In the

nasopharynx and parapharyngeal space MRI is superior to CT.

Staging and Prognostic Factors

• Two staging schemes have been proposed: (a) the system of Chandler et al. and (b) a radiographic staging system by Sessions et al. . • Stage Ia is limited to the nasopharynx and posterior nares; • stage Ib extends to the paranasal sinuses; • stages IIa, IIb, and IIc extend to other extracranial locations; and • stage III is intracranial.• In a retrospective review of 44 cases of juvenile nasopharyngeal

angiofibroma, invasion of the skull affected two thirds of the patients, and the rate of recurrence was 27.5%.

• Extensions to the intratemporal fossa, sphenoid sinus, base of pterygoids and clivus, the cavernous sinus (medial), foramen lacerum, and anterior fossa were correlated with more frequent recurrence.

• Long-term radiographic follow-up showed residual disease in nine asymptomatic patients: these remnants gradually involuted.

Staging of Nasopharyngeal Angiofibromas

Stage I Confined to the nasopharynx

Stage II Extension to nasal cavity and/or sphenoid sinus

Stage III Extension to one or more: antrum, ethmoid, pterygomaxillary and infratemporal fossae, orbit, and/or cheek

Stage IV Intracranial extension

General Management

• The decision of whether surgery or radiation therapy, depends in part on the

initial extent of the disease. In patients with extracranial tumors, surgery is

the treatment of choice and yields near-zero mortality or any long-term

morbidity.

• Tumor remnants in symptom free patients should be kept under surveillance

by repeated CT scanning, since involution may occur. Recurrent symptoms

may be treated by radiation therapy rather than by extended surgery or

combined procedures.

• When there is intracranial tumor extension, the risk of surgically related

death increases. Most of these patients are best treated with irradiation.

• Preoperative intraarterial tumor vessel embolization at the time of diagnostic

bilateral carotid angiography can be used, claiming a decrease in operative

bleeding.

• Salvage with embolization of polyvinyl alcohol has been described.

• Others have reported anecdotal evidence of partial regression with the use of

estrogens, believed to be the result of feedback inhibition of the pituitary's

production of gonadotropin-releasing hormone

• Although radiation therapy is equally effective in extracranial tumors, the low but

existing risk of secondary malignancies should limit its use to the most advanced

tumors only.

Radiation Therapy Techniques• Photon irradiation should be used for these patients, and fields must be

individualized to cover the tumor completely with a margin (1 to 2 cm).

• • Treatment portals are similar to those used in carcinoma of the nasopharynx

(without irradiating the cervical lymph nodes) or carcinoma of the paranasal sinuses when these structures or the nasal cavity is involved. Opposing lateral portals are suitable in most patients, with larger fields and compensators used for tumors extending into the nose.

• More extensive disease requires three-field or wedge-pair arrangements of 3DCRT or IMRT that can yield excellent dose distributions, particularly when there is nasopharyngeal or intracranial tumor extension.

• In all cases the eyes are protected as much as possible.

• The recommended tumor dose ranges from 30 Gy in 15 fractions in 3 weeks to 50 Gy in 24 to 28 fractions in 5 weeks. A conventional setup uses 6- to 18-MV photons to treat the lesion with parallel-opposed fields to 50 Gy (2-Gy fractions)

• Diagrams of the most commonly used conventional field arrangements for treatment of nasopharynx angiofibroma: lateral opposed field pair and three-field technique.

Diagrams of the most commonly used conventional field arrangements for treatment of nasopharynx angiofibroma: lateral opposed field pair and three-field technique.

Extracranial Meningiomas• Extracranial meningiomas of the sinonasal tract are rare tumors. The overall

prognosis is good, without a difference in outcome between benign and atypical meningiomas.

• Mean 47.5 years.

• Patients presented clinically with a mass, epistaxis, sinusitis, pain, visual changes, or nasal obstruction, dependent on the anatomic site of involvement.

• Tumors size ranged from 1 to 8 cm (mean, 3.5 cm).

• Histologically, the tumors demonstrated features similar to intracranial meningiomas; the majority of the meningothelial type, although there were three atypical meningiomas.

• Immunohistochemical studies confirmed the diagnosis with reactions for epithelial membrane antigen and vimentin. The differential diagnosis included paraganglioma, carcinoma, melanoma, psammomatoid ossifying fibroma, and angiofibroma.

Nonlentiginous Melanoma

• Malignant melanoma accounts for 11% of primary head and neck malignancies. Of all malignant melanomas, 20% to 35% are located in the head and neck area.

Cutaneous Melanomas• Of all head and neck malignant melanomas, 64% to 78% were cutaneous, 6%

to 8% were mucosal, and 14% to 30% were ocular.• • The superficial spreading and nodular types of malignant melanoma have a

metastatic potential of 10% to 30% and 50%, respectively.

• Neurotropic melanoma is an uncommon variant of cutaneous melanoma, with a higher propensity to invade peripheral nerves. A thorough evaluation with CT scans should determine if there is intracranial or base of the skull involvement.

• Treatment of cutaneous melanomas has typically been wide excision of the lesion with a minimum 3-cm margin. More recently, margins of at least 2 cm have been used in the head and neck area compared with wider margins for stage I melanomas, with equivalent success as noted by the local failure rate of 3% to 6%.

• Another approach to the treatment of recurrent or unresectable cutaneous melanomas is combined hyperthermia and high-fraction radiation therapy

Diagnostic Work-Up

• An excisional biopsy should be performed when feasible because some reports have suggested possible local or metastatic spread secondary to a punch or incisional biopsy, although this has not been noted in cutaneous melanomas. one third of these lesions were amelanotic.

• Metastatic melanoma to the mucosa of the head and neck area is less common. It can be differentiated from primary tumors by the presence of normal tissue between subepidermal tumor and the basal layer of melanocytes. In a review of the literature. The larynx, tongue, and tonsils are the most common locations for metastases.

Prognostic Factors

• >0.5 mm invasion, be a poor prognostic factor. Lymph node involvement is not a prognostic factor. Mucosal melanomas fare worse than their cutaneous counterparts, suggesting a lack of immunologic competence.

Management and Results of Therapy

• A local tumor control rate for stage I and II disease of 58% (7/12) in surgically treated patients with oral melanomas and a minimum follow-up of 3 years. Similar rates of failure have been reported, even with radical en bloc excisions (20% to 42%).

• Postoperative radiation therapy was with 60Co or 4-MV photons, 50 to 60 Gy, 1.5 to 2 Gy per fraction, over a period of 5 to 6 weeks.

• Patients with nasal cavity/paranasal mucosal melanoma have a median survival of 24 months.

• Because the results with irradiation are comparable with those of surgical series and because of the poor survival of these patients due to distant metastases and not locoregional failure, irradiation alone, with surgery for salvage, should be seriously considered as the primary treatment for mucosal melanomas of the head and neck

Lentigo Maligna Melanoma

Natural History• Lentigo maligna (Hutchinson's melanotic freckle or

circumscribed precancerous melanosis of Dubreuilh) and its invasive counterpart, lentigo maligna melanoma (LMM), are well-recognized clinicopathologic entities. LMM comprises about 10% of all melanomas in the head and neck, occurs predominantly on the face and ears of elderly persons, and generally has a very long natural history, frequently reaching a large size before diagnosis. Approximately one third of lentigo maligna lesions, if left untreated, will eventually transform into invasive LMM.

Clinical Presentation and Diagnostic Work-Up• These lesions appear as circumscribed and later as more diffuse areas of

hyperpigmentation of the skin. They may develop some superficial

nodularity and eventual ulceration as they become more invasive. In 10% of

the latter patients, regional and distant metastases eventually develop. The

10% metastatic spread in LMM contrasts with the 25% metastatic tendency

in nodular melanomas arising in superficial spreading melanomas and a

50% metastatic spread in nodular melanomas arising de novo.

• The diagnostic work-up of these patients is similar to that of patients

suspected of having malignant melanoma. Biopsies of the lesion are

required to obtain histopathologic confirmation of the diagnosis. Careful

physical examination must rule out any areas of extension or regional or

distant spread.

General Management• The usual treatment of lentigo maligna and LMM has been surgery, with

approximately 1- to 2-cm margin of normal skin.

• Because of the low incidence of regional lymph node metastases, elective

lymph node dissection is not indicated. For larger lesions, wider surgical

excision with skin grafting has been reported to give poor cosmetic results.


• Entire tumor with adequate margin (1 cm for lesions <2 cm and 2 cm for larger tumors).

• Because Miescher's irradiation technique used very superficial x-rays, with 50% depth dose being at approximately 1 mm, there is the possibility of local recurrence if dermal extension is unrecognized. minimum x-ray energies of 100 keVp is used and preferably 140 to 175 keVp to treat these patients.

• Superficial x-rays (100 to 200 keVp) with adequate filtration or electrons (6 to 9 MeV) with appropriate thickness of bolus (about 1.5 cm) are adequate for most patients.

• Doses of 45 to 50 Gy in 15 to 25 fractions delivered over 3 to 5 weeks will control the disease in most patients.

• It is recommended delivering 3 to 3.5 Gy, three times weekly, every other day, to a total of 50 Gy, depending on the size and thickness of the lesion.

• Elective irradiation of the regional lymphatics is not necessary.

• In patients on whom surgical excision is performed, postoperative irradiation is recommended if positive margins are found. Doses are similar to those stated earlier.

Sarcomas of the Head and NeckNatural History

• Sarcomas account for <1% of malignant neoplasms in the head and neck.

The most frequent histological type is malignant fibrohistocytoma, while the

least common is liposarcoma (1%).

• The histology is complex and requires immunochemical analysis including

osteosarcoma, angiosarcoma, chondrosarcoma, hemangiosarcoma,

leiomyosarcoma, liposarcoma, malignant fibrous sarcoma,

rhabdomyosarcoma, malignant schwannoma, neurofibrosarcoma, and

synovial sarcoma. Fibrosarcoma, angiosarcoma, leiomyosarcoma, and

rhabdomyosarcoma are the most common types but this varies in published

reports.

• Distribution of these sarcomas was 33% in the scalp or face, 26% in the orbit

or paranasal sinuses, 14% arising from upper aerodigestive tract including

larynx, and 27% in the neck.

• Synovial sarcomas are rare soft-tissue malignancies in the head and neck

region; they account for 3% to 5% of head and neck tumors.

• Histologic, immunohistochemical, and characteristic chromosomal

translocation findings are necessary for diagnosis.

• The poor prognosis of this sarcoma justifies radical surgery with postoperative

radiation.

Clinical Presentation and Diagnostic Work-Up• Clinical presentation varies with the primary site of disease.

• nasal bleeding, a palpable mass in the neck, or difficulty in swallowing or

breathing, cranial nerve deficit. Diagnostic work-up follows that of soft-tissue

sarcomas of other sites in the body. With early lesions, radiographs or CT

may show only nonspecific opacification, soft-tissue swelling, and

occasionally bone destruction. MRI, especially with gadolinium contrast,

may be used as a supplement or alternative to CT scanning. A CT scan of

the chest is also mandatory for staging work-up.

Prognostic Factors• Prognostic factors for predicting local recurrence or disease-free survival

include anatomic site, treatment modality, tumor histology and grade, tumor

size, extension of disease, and surgical margins

• In addition to tumor grade and size, direct tumor extension to neurovascular

structures, bone, contiguous organs, and skin was associated with a higher

incidence of distant metastasis. The actuarial 5-year freedom-from-distant-

metastasis rates were 70% and 100% for tumors with or without direct

extension, respectively

• Stage IA T1a NO NX M0 G1-2 G1 LowT1b N0 NX M0 G1-2 G1 Low

• Stage IB T2a N0 NX M0 G1-2 G1 LowT2b N0 NX M0 G1-2 G1 Low

• Stage IIA T1a N0 NX M0 G3-4 G2-3 HighT1b N0 NX M0 G3-4 G2-3 High

• Stage IIB T2a N0 NX M0 G3-4 G2-3 High

• Stage III T2b N0 NX M0 G3-4 G2-3 High

• Stage IV Any T N1 M0 Any G Any G High or Low Any TAny N M1 Any G Any G High or

Low

STAGE GROUPING

General Management

• Surgery is the preferred initial treatment modality for sarcomas.

• Unfortunately, it is often difficult to achieve complete resection of the tumor, and a

high recurrence rate has been observed with surgery alone. Extracapsular

enucleation of the tumor results in 90% local recurrence because of the presence

of microscopic pseudopodia, which tend to grow through the pseudocapsule into

the surrounding tissue and the presence of skipped lesions some distance from

the main tumor mass. Pathologic analysis of the surgical bed often discloses

microscopic extension of tumor.

• This resulted in inferior overall survival for sarcomas of the head and neck when

compared with extremity sarcomas. Wide local excision, with a 5-cm margin

around the pseudocapsule in extremity sarcomas, is associated with better

outcome, although approximately 20% will have local recurrence.

•

• The criteria for surgical resection are impractical for head and neck sarcomas because of

anatomic limitations. Some retrospective studies have suggested improved local tumor control

when combined surgery and external irradiation are used. Patients treated with combined-

modality treatment (surgery and irradiation) had less extensive surgery, yet local recurrencefree

survival was longer.

• Radiation therapy, by external beam or brachytherapy, plays an important adjunctive role in the

management, especially for tumors where en-bloc resection with negative margin is not possible.

• Chemotherapy regimens are available for soft-tissue neoplasms primarily designed to improve

local tumor control. Survival is predicted on the incidence of local recurrence and risk of distant

metastasis, both of which are influenced by tumor grade.

Radiation Therapy Techniques• The general principles for radiation therapy of head and neck sarcomas are

similar to those of soft-tissue sarcomas.

• Complete coverage of the surgical bed and scar with adequate margins (3 to

5 cm) is required. However, because of the proximity of critical and

radiosensitive organs (eyes, spinal cord, brainstem), selecting optimal portal

margins without seriously compromising the functioning of these organs is an

art.

• Techniques similar to those used in epithelial tumors of the head and neck

can be applied to sarcomas.

• In general, 55 to 60 Gy is needed for postoperative adjuvant irradiation, and

an additional 10- to 15-Gy boost is recommended if the surgical margins are

close (<3 mm) or involved by tumor.

• Some institutions prefer preoperative irradiation of 45 to 50 Gy. Special

attention should be directed to limiting the dose to critical structures.

Chemotherapy• Head and neck soft-tissue sarcomas frequently metastasize.

• The role of adjuvant chemotherapy to improve diseasefree survival in

sarcoma of the head and neck is controversial.

• Unlike with soft-tissue sarcomas of the extremities, in which distant

metastasis is the most common cause of death, the majority of deaths in

sarcomas of the head and neck are associated with local failure.

Approximately half of the distant metastases were detected after local

recurrence occurred. Chemotherapy did not appear to affect local tumor

control

• For preoperative neoadjuvant chemotherapy, which supplements radiation

therapy to downstage disease before surgery, satisfactory results are

available only for sarcomas of extremities.

• With the exception of rhabdomyosarcoma, postoperative adjuvant

chemotherapy should be given only in a clinical trial setting.

unusual non epithelial tumors of head and neck

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