Unusual nonepithelial tumors of the head and neck

1. Unusual Nonepithelial Tumors of
the Head and Neck- An overview
Dr Sasikumar Sambasivam
DNB Resident
Dept. of Radiation Oncology

2. 1. Glomus Tumors
2. Hemangiopericytoma
3. Chordomas
4. Lethal Midline Granuloma
5. Chloroma
6. Esthesioneuroblastoma
7. Extramedullary Plasmacytomas
8. Nasopharyngeal Angiofibroma
9. Extracranial Meningiomas
10.Nonlentiginous Melanoma
11.Mucosal Melanomas
12.Lentigo Maligna Melanoma
13.Sarcomas of the Head and Neck

3. 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

4. • These tissues are responsive to changes in oxygen and
carbon dioxide tensions and pH.
• 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
• Although histologically benign, they may extend along
the lumen of the vein to regional lymph nodes, but
rarely to distant sites.

5. Epidemiology
•Mean age
– 44.7 years for carotid body tumors
– 52 years for glomus tympanicum
•3 or 4times more frequently in women than in men,
suggesting a possible estrogen influence
•Glomus tumors may be familial; they also occur in
multiple sites in 10% to 20% of patients
•Bilateral carotid glomus tumors were reported in six
of 16 patients (38%) with a positive family history for
these lesions but in only 17/206 patients (8%)
without such a history

6. Clinical Presentation
• Glomus tumors of the middle ear may initially
cause earache or discomfort
• Pulsatile tinnitus
• Hearing loss
• Cranial nerve paralysis
• Middle cranial fossa, symptoms may include
temporoparietal headache, retro-orbital pain,
proptosis, and paresis of cranial nerves V and VI
• 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

7. • Chemodectoma of the carotid body usually
presents as a painless, slowly growing mass in
the upper neck.
• Mass may be pulsatile and may have an
associated thrill or bruit
• 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

8. Work up
History
Physical examination
ENT examination
Basic Investigations
Radiographic 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)
Special tests
Audiograms to establish baseline hearing loss
Histologic staining to determine presence of catecholamines

9. Glasscock-Jackson Classification of Glomus Tumors
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 jugulare
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

10. Modification of McCabe and Fletcher Classification of Chemodectomas
Group I: Tympanic tumors
Absence of bone destruction on x-rays of the mastoid bone and jugular fossa
Absence of facial nerve weakness
Intact eighth nerve with conductive deafness only
Intact jugular foramen nerves (cranial nerves IX, X, and XI)
Group II: Tympanomastoid tumors
X-ray evidence of bone destruction confined to the mastoid bone and not involving the petrous
bone
Normal or paretic seventh nerve
Intact jugular foramen nerves
No evidence of involvement of the superior bulb of the jugular vein on retrograde venogram
Group III: Petrosal and extrapetrosal tumors
Destruction of the petrous bone, jugular fossa, and/or occipital bone on x-rays
Positive findings on retrograde jugulography
Evidence of destruction of the petrous or occipital bones on carotid arteriogram
Jugular foramen syndrome (paresis of cranial nerves IX, X, or XI)
Presence of metastasis

11. Modification of McCabe and Fletcher Classification of Chemodectomas
Group I: Tympanic tumors
Absence of bone destruction on x-rays of the mastoid bone and jugular fossa
Absence of facial nerve weakness
Intact eighth nerve with conductive deafness only
Intact jugular foramen nerves (cranial nerves IX, X, and XI)
Group II: Tympanomastoid tumors
X-ray evidence of bone destruction confined to the mastoid bone and not involving the petrous
bone
Normal or paretic seventh nerve
Intact jugular foramen nerves
No evidence of involvement of the superior bulb of the jugular vein on retrograde venogram
Group III: Petrosal and extrapetrosal tumors
Destruction of the petrous bone, jugular fossa, and/or occipital bone on x-rays
Positive findings on retrograde jugulography
Evidence of destruction of the petrous or occipital bones on carotid arteriogram
Jugular foramen syndrome (paresis of cranial nerves IX, X, or XI)
Presence of metastasis

12. Management
• Surgery is generally selected for treatment of small
tumors
• Glomus tympanicum tumors- 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
• Surgical treatment of a glomus tumor arising in the
jugular bulb- piece-by-piece removal
• Preoperative embolization via a transarterial
approach has proved beneficial but is often limited
by vascular anatomy and unfavorable locations

13. • The local tumor control rate with surgery alone is
only about 60%, and there is significant morbidity,
particularly cranial nerve injury and bleeding.
• In a retrospective review of all skull-base surgery
cases treated at Baylor University 175
jugulotympanic glomus tumors and nine
malignant cases (5.1%) were identified. The 5year survival rate was 72%.

14. Radiation Therapy
• Tumors with destruction of the petrous bone,
jugular fossa, or occipital bone or patients with
jugular foramen syndrome are more reliably
managed with irradiation.
• Some surgeons, such as Glasscock et al. and
Spector et al., have questioned the effectiveness
of radiation therapy in the treatment of
chemodectomas because on histologic sections,
obtained even many years after irradiation, it is
possible to find chromophilic cells remaining in the
tumor. However, there is also evidence of fibrosis
and decreased vascularity

15. • Suit and Gallager demonstrated in a murine mammary
carcinoma model that morphologically intact cells may
have lost their reproductive ability after irradiation,
which is the ultimate end point of cell killing
• Extremely unusual to observe clinical regrowth of a
glomus tumor after irradiation, even if they do not
regress completely.
• Some reports describe successful combinations of
surgery with either preoperative or postoperative
irradiation or preoperatively in an attempt to make an
unresectable tumor operable, postoperatively when
obvious tumor could not be resected.

16. Radiation Therapy Techniques
• Radiation therapy techniques are determined
by the location and extent of the tumor
• Limited, usually bilateral, portals should be
used for relatively localized glomus tumors
• Electrons (15 to 18 MeV) with a lateral portal
or combined with cobalt-60 (60Co) or 4- to 6-MV
photons (20% to 25% of total tumor dose)
render a good dose distribution

17. • In patients in whom tumor has spread into the
posterior fossa, it may be necessary to use
parallel opposed portals with 6- to 18-MV
photon
• Treatment is given at the rate of 1.8 to 2 Gy
tumor dose per day with five treatments per
week for a total tumor dose of 45 to 55 Gy in
5 weeks. Three-dimention (3D) conformal
radiotherapy (RT) or image-guided radiation
therapy (IMRT) are highly desirable
techniques to treat these tumors, with
excellent dose distributions

18. View Figure
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% 4MV photons)

19. View Figure
Isodose
distributions
using
superiorinferior pairs
of 60-degree
(A) and 45degree (B)
converging
wedge filtered
60
Co fields,
demonstrating
limited
volume of
irradiation

20. • Leber et al. reported on 13 patients with glomus
tumors treated with radiosurgery because of
recurrences after surgical removal in six patients.
• Two patients had partial embolization before
Gamma Knife (Elekta, Norcross, GA) treatment
• Mean follow-up was 42 months (range, 14 to 72
months).
• Within the follow-up period there was no tumor
progression and no clinical deterioration in any
patient
• 64% of the patients had an improvement of their
symptoms, and in 36% the volume of the lesion
decreased in size
• There was no radiation-related morbidity

21. Results of Therapy
•
•
•
•
Postirradiation change in tumor size is slow
Increase in proliferative and perivascular fibrosis
Minimal alterations in the chief epithelial cells
Histologic evaluation of tumor cell viability is not
reliable
• Despite the persistence of tumor both clinically
and angiographically, amelioration of symptoms,
absence of disease progression, and occasional
return of cranial nerve function have been
reported

22. • Seventeen patients were treated for glomus tympanicum
tumors at Washington University
• In five patients initial treatment consisted of irradiation
alone, and all were tumorfree at last follow-up (4.5 years in
one patient) or at death
• Seven of eight patients irradiated for surgical recurrence
were free of disease 4.5 to 19 years after irradiation
• The remaining four patients were treated preoperatively or
postoperatively; only one had recurrence and was salvaged
surgically and tumorfree 10 years later
• Of six patients with glomus jugulare lesions treated with
irradiation, two with extensive lesions died of their disease,
whereas the glomus tumor was controlled in four, including
two patients with intracranial extension. Irradiation doses
ranged from 46 to 52 Gy, with 86% to 100% tumor control
with doses over 46 Gy and 50% (two of four) with doses
below 46 Gy.

23. • Of 19 patients treated with irradiation at the M.D.
Anderson Cancer Center, five had only a biopsy
without any surgical excision and 14 had partial
excision, Ten patients had bony destruction; five of
these had petrous pyramid and jugular foramen
destruction, with accompanying multiple cranial nerve
paralysis. Seventeen patients were treated with 60Co
anterior-posterior or superior-inferior wedged filtered
fields, and two patients received electrons and
photons (3:1) via a single lateral field. Of 18 patients
surviving a minimum of 5 years (13 surviving more
than 10 years), all are alive and free of disease or have
died of other causes.

24. Local Control with Radiation Therapy for Chemodectoma of the
Temporal Bone (Glomus Tympanicum and Jugulare)
Institution (Reference)
Local Control
Princess Margaret Hospital (53) 42/45a
Nominal Dosage Schedule
35 Gy/3 wk
Queen Elizabeth Hospital,
Birmingham (6)
45–50 Gy/4–5 wk
19/20b
University of Washington (240 10/13
)
8–65 Gy/4–7 wk
Rotterdamsch RadioTherapeutisch Instituut,
Netherlands (166)
40–60 Gy/4–6 wk
19/19
University of Minnesota (172) 13/14
30–60 Gy/3.5–7.5 wk
University of Virginia (104)
14/17
40–50 Gy/4–5 wk
University of Michigan
11/11
Total
128/139 (92%)

25. Chordomas

26. 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

27. 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
• No risk factors have been identified
• Male predominance is reported at a 2:1 to 3:1
ratio

28. Natural History
• Slowly growing, chordomas are locally invasive,
destroying bone and infiltrating soft tissues
• Basisphenoidal chordomas tend to cause
symptoms earlier and may be difficult to
differentiate histologically from chondromas and
chondrosarcomas and radiographically from
craniopharyngiomas, pineal tumors, and
hypophyseal and pontine glioma
• Lethality of these tumors rests on their critical
location, aggressive local behavior, and extremely
high local recurrence rate

29. • Incidence of metastasis, which has been
reported to be as high as 25%, is higher than
previously believed and may be related to the
long clinical history
• Most common site of distant metastasis is the
lungs, followed by liver and bone
• Lymphatic spread is uncommon.

30. Pathology
• Chordoma is a soft, lobulated tumor that may have areas of
hemorrhage, cystic changes, or calcification
• 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
• Heffelfinger et al. postulated that a chondroid variant of chordoma
may exist, being prevalent in the spheno-occipital area
• Patients with this type of histologic variant have improved survival
• Aside from the previously mentioned histologic features, the
prognostic factors that most influence the choice of treatment are
location and local extent of tumor.

31. Clinical Presentation
• Clinical symptoms vary with the location and extent of the
tumor
• 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
• Fuller and Bloom reported on 13 patients with clivus
chordoma, all of whom had multiple cranial nerve palsies.
Facial pain was present in 11/13 patients.

32. Diagnostic Work-up for Chordoma
General
History
Physical examination
Radiologic studies
Plain radiographs
Computed tomography scan/magnetic resonance imaging
Laboratory studies
Complete blood cell count
Chemistry
Urinalysis
Special studies
Endocrinologic profile (clivus)
Visual evaluations (clivus)

33. General Management
• Because of their surgical inaccessibility and relative resistance to
radiation therapy, clivus chordomas represent a formidable
therapeutic challenge.
• The general management of the patient is dictated by the anatomic
location of the tumor and the direction and extent of spread
• 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.

34. Radiation Therapy Techniques
• Depending on the location of the tumor along
the craniospinal axis
• Basisphenoidal tumors usually are treated by
a combination of parallel opposed lateral
fields, anterior wedges, and photon and
electron beam combinations
• 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

35. • Because of the slow proliferative nature of chordomas, high
linear energy transfer may prove useful in their management
• Brachytherapy can be used for recurrent tumors of the base
of skull or adjacent to the spine when a more aggressive
surgical exposure is offered.
• Three of five chordomas were rendered stable when treated
with iodine-94 (94I) implants by Gutin et al., performed with CT
stereotactic technique. Kumar et al. reported use of 94I
intraoperative interstitial implantation in two patients with
recurrent chordomas.
• Disease was effectively controlled in both.

36. • Survival in some patients with chordoma may
be long term, the salient feature of this
unusual neoplasm is local recurrence with
eventual death
• Overall 5-year diseasefree survival rate is
<10% to 20%. At M.D. Anderson Cancer
Center, of 19 patients treated definitively,
three were alive and free of disease with
relatively short follow-up of 3, 6, and 7.5
years, respectively.

37. • Catton et al. analyzed the long-term results of
treatment for patients with chordoma of the sacrum,
base of skull, and mobile spine treated
predominantly with postoperative photon
irradiation. In 20 base of skull chordomas, most of
them irradiated with conventionally fractionated
radiation to a median dose of 50 Gy in 25 fractions
for 5 weeks (range 25 Gy to 50 Gy), median survival
was 62 months (range 4 to 240 months) from
diagnosis with no difference between clival and
nonclival presentations.

38. • There was no survival advantage to patients
receiving radiation doses >50 Gy (median 60
Gy) compared with lower doses <50 Gy
(median 40 Gy).
• Hyperfractionation regimens did not influence
the degree or duration of symptomatic
response or progressionfree survival.
• Median survival after retreatment was 18
months.

39. Protons
• The best results in the treatment of
chordomas have been obtained with radical
surgical procedures followed by high-dose
proton irradiation
• Tatsuzaki and Urie (261) described the use of
proton beam therapy at high doses for
chordomas and chondrosarcomas of the base
of the skull and cervical spine