Unusual Nonepithelial Tumors of the Head and Neck (under construction) Flashcards
Glomus Tumors
In what branches of the tenth nerve are glomus bodies usually found?
tympanic (Jacobson)
auricular (Arnold)
Which of the following is/are FALSE regarding glomus tumors?
I. GT or chemodectomas consist of large epithelioid (striated 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.
II. They are histologically benign.
III. They may extend along the lumen of the vein to regional
lymph nodes, but rarely to distant sites (2 to 5%)
IV. These tissues are responsive to changes
in oxygen and carbon dioxide tensions and pH.
I. smooth muscles. not striated.
Glomus Tumors
What is the mean age of diagnsosis in carotid body tumors?
What is the mean age of diagnsosisin glomus tympanicum?
45
52
Glomus Tumors
They are more common in men.
TRUE or FALSE?
False.
3-4 times more common in women, suggesting a possible estrogen influence.
Glomus Tumors
Glomus tumors may be familial and may be influenced by what hereditary syndromes?
PGL1-5
MEN2
NF1
***
Multiple paragangliomas of the head and neck are rare, with an incidence of 10% of all
patients, but in familial cases, it increases up to 35% to 50%
Glomus Tumors
If the tumor invades the middle cranial fossa, symptoms may include temporoparietal headache, retro-orbital pain, proptosis, and paresis of what cranial nerves?
nerves V and VI.
Glomus Tumors
If the posterior fossa is involved, symptoms may include occipital headache, ataxia, and paresis of what cranial nerves?
nerves V to VII, IX, and XII;
Glomus Tumors
Invasion of the jugular foramen causes paralysis of what cranial nerves?
nerves IX to XI.
Glomus Tumors
Which of the following statements is/are TRUE in the physical examination of glomus tumors?
I. In the majority of glomus tympanicum, physical examination demonstrates a red, vascular middle ear mass, although occasionally it may be bluish or white (the latter resembling a cholesteatoma).
II. Audiography may demonstrate sensorineural hearing loss in the ear involved by tumor as noted in 33 of 49 patients evaluated by Larson et al.; 4 of 33 patients also exhibited tympanic pulsations.
III. 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.
I & III.
***
II is false. it’s conductive hearing loss not SNHL
Glomus Tumors
High-resolution computed tomography (CT) with contrast has a degree of sensitivity and specificity to diagnose this tumor when located in the
middle ear or jugular bulb.
Tumor enhancement is similar to which structure?
temporalis muscle
Glomus Tumors
Bony erosions are typical findings in CT with contrast in patients with glomus tympanicum.
TRUE or FALSE?
False.
***
there were no instances of local bony erosion;
instead, the tumors engulfed the ossicular chain, bulged or protruded through the
tympanic membrane, filled the middle ear, or extended into the eustachian tube
orifice or aditus ad antrum.
This pattern is in contrast to cholesteatomas, which
typically destroy adjacent bony landmarks, including the ossicles, and
progressively erode the petrous bones as they enlarge.
Glomus Tumors
What are the usual findings in a magnification angiography in glomus tympanicum?
hypervascular middle ear mass
- first appears in the middle to late arterial phase
- persists through the capillary phase
- quickly disappears in the venous phase without demonstrably early draining veins
Glomus Tumors
Drape et al. described magnetic resonance imaging (MRI) findings in 31
patients with a clinical suspicion of glomus tumor; gadoterate meglumine was
injected into 19 patients. Twenty-seven of twenty-eight pathologically confirmed
glomus tumors were detected with MRI.
What finding was present in most tumors?
peripheral capsule.
Glomus Tumors
What modality was found to be
more accurate in the diagnosis of glomus tumors, with enhancement in the
arterial phase, when compared with MRI.
Multidetector CT angiography
Glomus Tumors
As GTs show high levels of somatostatin receptor (SSTR) subtypes 2 and 5, fluorine-(18F)-octreotate positron emission tomography (PET) may be useful for diagnostic purposes in a semiquantitative manner and for improving target
volume delineation in radiation therapy planning.
What two tracers were shown to detect SSTR-expressing tumors?
Gallium-68 DOTATOC
Glus-Lys(18)F-TOCA
Glomus Tumors
What are the three subtypes of tumors based on enhancement characteristics on MRI?
vascular
solid
myxoid
Glomus Tumors
What classification system reflects the relationship of prognosis to the anatomic location of the lesion?
Glasscock-Jackson
Glomus Tumors
What is the Glasscock-Jackson classification for:
glomus tympanicum involving the mastoid?
at least III
(I - limited to promontory)
(II - middle ear space)
(III - mastod)
(IV - TM, external canal, may extend anterior to carotid)
Glomus Tumors
What is the Glasscock-Jackson classification for:
glomus jugulare involving the mastoid?
I
bulb, middle ear, and mastoid are all I.
***
II. Tumor extending under internal auditory canal; may have intracranial canal extension
III. Tumor extending into the petrous apex; may have intracranial canal extension
IV. Tumor extending beyond the petrous apex into the clivus or infratemporal fossa; may have
intracranial canal extension
Glomus Tumors
What is the McCabe and Fletcher classification for tumors with jugular foramen syndrome?
Group III.
(Intact jugular foramen nerves are groups I & II)
***
Other group III descriptions:
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
Glomus Tumors
usual RT dose prescription using conventional RT?
45 to 55/1.8-2
H&N - Hemangiopericytoma
What are the definitive signs of a malignant HPC?
local recurrence
development of metastases
H&N - Hemangiopericytoma
Findings in pathologic examination that are highly suggestive of malignancy that differentiates benign HPC from malignant HPCs.
prominent mitoses >4/hpf
foci of necrosis
increased cellularity
H&N - Hemangiopericytoma
In general, tumors of the CNS, lower extremity, and
mediastinum tend to be more benign than H&N tumors.
TRUE or FALSE?
False.
They tend to be more malignant, with local recurrence occuring in up to 50% of cases.
H&N - Hemangiopericytoma
Which of the following is/are TRUE re HPC epidemiology?
I. HPC is an unusual tumor; representing approximately 1% of all vascular neoplasms;
II. It occurs in both genders with equal frequency and is found primarily in adults.
III. In the head and neck, the most common sites are the nasal cavity and the paranasal sinuses and, less frequently, the orbital region, the parotid gland, and the neck.
IV. HPCs represent 3% to 4% of all meningeal and <1% of CNS tumors.
All
H&N - Hemangiopericytoma
Which of the following is/are TRUE re HPC?
I. Hemangiopericytomas (HPCs) are rare soft tissue neoplasms that account for 3%
to 5% of all soft tissue sarcomas and 1% of all vascular tumors.
II. They may resemble pituitary adenomas in the central nervous system (CNS), clinically, and on imaging studies.
III. Vagal paragangliomas originate within the first 2 cm of the extracranial stretch of the
vagus nerve and are associated with the inferior ganglion.
IV. These tumors are
believed to originate from the pericytes of Zimmerman extravascular cells, morphologically resembling smooth muscle, found around the capillaries or from primitive mesenchymal cells.
I, III, and IV.
***
II - should be meningiomas
H&N - Hemangiopericytoma
The skin overlying the mass does not have any discoloration or redness to indicate its vascular origin.
TRUE or FALSE?
True
H&N - Hemangiopericytoma
On T2-weighted imaging, the
punctate black flow voids in cross-section within the relatively bright tumor,
creating a characteristic “______” appearance in tumors >2 cm in diameter.
salt-and-pepper
H&N - Hemangiopericytoma
HPC is the only vascular tumor that has radially arranged or spiderlike branching vessels around and inside the tumor and a long-standing, well demarcated tumor stain.
TRUE or FALSE?
True.
Well, at least according to Yaghmai
H&N - Hemangiopericytoma
In general, what is the treatment of choice?
complete surgical resection after pre-operative embolization of the feeding arteries.
H&N - Hemangiopericytoma
HPCs are generally radiosensitive tumors.
TRUE or FALSE?
False.
H&N - Hemangiopericytoma
In general, what doses are prescribed to produce local control in post-operative cases?
60 to 65 Gy
H&N - Hemangiopericytoma
The fields of irradiation should be wide to encompass the tumor bed with a margin of at least __cm to safely avoid marginal recurrence.
5
H&N - Chordoma
Where do 50% of chordomas arise?
sacrococcygeal area
***
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.
(this one from benign chapter)
Hence, they occur along the axial skeleton in the skull base (35%), vertebral column (15%), or sacral regions (50%).
(this one from osteosarc chapter)
This corresponds well with the anatomic distribution of chordoma, with half arising in the sacrococcygeal region and one-third at the base of skull, typically the clivus.
H&N - Chordoma
Which is FALSE regarding chordomas.
I. Chordomas are more common in patients in their 50s and 60s but can occur in all age groups.
II. In children and young adults, the prognosis and long-term survival appear to be better than in older patients.
III. No risk factors have been identified.
IV. Male predominance is reported at a 2:1 to 3:1 ratio.
V. Median age at presentation is 60 years, but base-of-skull location typically present at a younger age, usually in the third to fourth decade of life.
None of the above.
All are true.
I-IV from H&N chapter.
V from osteosarc/bone chapter
H&N - Chordoma
Which among these have a better prognosis?
chondroid
conventional
dedifferentiated
chondroid
H&N - Chordoma
Treatment?
Surgery +/- RT (at least 60 Gy)
H&N - Lethal Midline Granuloma
What virus is associated with LMG.
The rare subtype 2 of which may relate to a covert immune defect?
EBV
H&N - Lethal Midline Granuloma
LMG/PMR is believed to be a part of the spectrum of what malignancy?
NK/T-cell lymphoma
H&N - Lethal Midline Granuloma
Treatment of choice?
RT
H&N - Lethal Midline Granuloma
Treatment of choice?
RT
H&N - Lethal Midline Granuloma
Because marginal failures are a significant problem, wide margins are necessary for treatment of these patients.
What is the preferred margin?
2-3 cm
H&N - Lethal Midline Granuloma
Dose range: RT for LMG
30-50 Gy/2Gy
45-50, 35-45
H&N - Nonepithelial Tumors
Also called granulocytic sarcoma, or myeloid blastoma, what is this solid extramedullary tumor composed of early myeloid precursor usually associated with AML and NLL?
Chloroma
H&N - Chloroma
Which of the following statements is/are TRUE of chloromas?
I. GS is usually an ominous sign, suggesting imminent conversion to
acute myelocytic leukemia or blast crisis. Chloromas may appear during bone marrow remission before an
increase in blasts is detected in the bone marrow, so they may herald relapse.
II. Children are affected more often than adults.
III. Chloromas are found more frequently in children with the M6 and M7
acute myeloid leukemia (AML) subtypes of the French-American-British
Cooperative Group Classification and are also associated with the 9:22
translocation
I & II.
***
III is false: M4/M5, 8:21
H&N - Chloroma
Which of the following statements is/are FALSE regarding MRI appearance of chloromas?
I. Isointense relative to gray matter on TI-weighted images
II. Isointense to white matter on T2-weighted images.
III. Demonstrates almost uniform enhancement with gadolinium
None.
All are TRUE
H&N - Chloroma
Usual RT dose prescribed, as described in series of experiences.
24 Gy/12 fx to tumor + 2-3 cm margin
H&N - Esthesioneuroblastoma
Which statement is false about ENB?
I. There appears to be a slight male predominance.
II. The age incidence has a bimodal distribution, with peaks at 11 to 20 years and 40 to 60 years
III. The highest incidence at 51 to 60 years.
IV. Most are Kadish C tumors.
None.
All are TRUE
H&N - Esthesioneuroblastoma
Which statement is false about ENB?
I. The expansile tendency of olfactory neuroblastoma is characterized by bowing of the sinus walls.
II. Dopamine β-hydroxylase and catecholamines are produced by these tumors, and their measurements or vanillylmandelic acid excretion levels are proven clinically useful.
III. These tumors tend to be friable and bleed easily.
II.
Although dopamine β-hydroxylase and catecholamines are produced by these
tumors, their measurements or vanillylmandelic acid excretion levels have not
proven clinically useful.
H&N - Esthesioneuroblastoma
Differentiate Kadish A, B, and C from each other.
A - nasal cavity
B - + paranasal sinus/es
C - outside A and B
H&N - Esthesioneuroblastoma
ENB must be distinguished from other poorly differentiated neoplasms, including sinonasal undifferentiated carcinoma (SNUC), which is derived from the Schneiderian epithelium.
How?
SNUC lacks rosettes and intercellular fibrils.
JNA
JNA is mainly a male disease.
TRUE or FALSE?
True
Juvenile nasopharyngeal angiofibroma (JNPAF) is found more frequently in
young pubertal boys; it has been shown to contain androgen receptors209 and
occasionally to regress with estrogen therapy.
Females
comprise <4% of the total cases.
Some investigators have suggested
chromosomal studies in affected women
