Diseases, disorders and conditions - NEOPLASTIC Flashcards

1
Q

What is the most common site of laryngeal cancer?

A

Glottic

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2
Q

What are the 7 different types of squamous cell aberrations occurring in the larynx?

A
Benign hyperplasia
Benign keratosis (no atypia)
Atypical hyperplasia
Keratosis with atypia or dysplasia
Intraepithelial carcinoma
Microinvasive squamous cell carcinoma(SCCA)
Invasive SCCA
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3
Q

What percent of patients with carcinoma in situ of the weal cord will develop inv.uive SCCA after a single
exclsional biopsy?

A

one in six - 16.7%

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4
Q

What is “microinvasive” SCCA of the vocal cord?

A

Invades through the basment membrane but not into the vocalis muscle

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5
Q

What is Ackerman’s tumor?

A

Verrucous carcinoma, thought to be less radiosensitive and less likely to metastasize than SCCA

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6
Q

What are the two most important factors predicting lymph node metastasis in laryngeal cancer?

A

Tumor size and location

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7
Q

True/False: Once invasion of the laryngeal framework occurs, the ossified portions of cartilage have the least
resistance to tumor spread.

A

true

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8
Q

What percent of glottic tumors display perineural and vascular invasion?

A

25%

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9
Q

What percent of patients with a primary laryngeal cancer will eventually develop a 2nd primary?

A

10-20%

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10
Q

What is the stage of a transglottic tumor without vocal cord fixation, cartilage invasion, or extension beyond
the larynx?

A

T2

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11
Q

Which parts of the glottis are most difficult to treat with radiation?

A

Anterior commissure, posterior 1/3 of the vocal cord.

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12
Q

What percent of glottic tumors will metastasize to the cervical lymph nodes?

A

25%

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13
Q

What is the incidence of positive cervical nodes in patients with T3 glottic tumorsl

A

30-40 %

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14
Q

Which type of laryngeal cancer is most likely to metastasize distally?

A

supraglottic

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15
Q

What is the most common site of distant metastasis from laryngeal carcinoma?

A

lungs

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16
Q

How does metastatic disease to the lungs normally present?

A

Multiple small lesions less than 3 mm that are difficult to detect on X-ray.

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17
Q

Where does supraglottic carcinoma most often begin?

A

Junction of the epiglottis and false cords

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18
Q

What anatomic structure serves as a natural barrier to the inferior extension of supraglottic cancers?

A

Ventricle (embryologic developmnent is completely separate from the false cord).

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19
Q

Which kinds of supraglottic cancers are more likely to extend inferiorly to the anterior commissure or
-ventricule
ulcerative or exophytic

A

ulcerative lesions

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20
Q

True/False: Stage I lesions of the supraglottis can be controlled equally well with radiotherapy or surgery.

A

True

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21
Q

What is the risk of cervical metastases in patients with Tl, T2, T3, and T4 tumors of the supraglottis?

A

T1 20%
T2 40%
T3 60%
T4 80%

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22
Q

What percent of patients undergoing supraglottic laryngectomy and unilateral neck dissection will fail in the
contralateral neck?

A

16%, despite receiving radiation therapy to the area.

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23
Q

What percent of laryngeal tumors are primarily subglottic ?

A

5%

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24
Q

What are the differences between primary and secondary subglottic tumors?

A

Primary tumors are less common, usually present with stridor or dyspnea and at a more advanced stage, and have a
worse survival time than secondary tumors.

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25
Q

What is the primary site of lymphatic drainage fom subglottic tumors?

A

Paratracheal nodes.

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26
Q

Compared with supraglottic and glottic tumors, subglottic tumors are at a much higher risk for developing
what?

A

stomal recurrence

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27
Q

What is the treatment of choice for primary subglottic cancer?

A

Total laryngectomy, bilateral neck dissection, near total thyroidectomy, paratracheal node dissection, and
postoperative radiation to the superior mediastinum and stoma; if the anterior cervical esophageal wall is involved,
then laryngopharyngectomy with cervical esophagectomy instead of total laryngectomy.

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28
Q

What is the bat organ-sparing treatment for a patient with stage III SCCA of the supraglottis?

A

Induction chemotherapy followed by radiation therapy.

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29
Q

What are the indications for postoperative radiation after neck dissection?

A

Multiple nodes or extracapsular spread

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30
Q

What is the significance of the number of pathologically positive nodes on prognosis~

A

Greater than 3 pathologically positive nodes is a negative prognostic indicator.

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31
Q

True/False: Chemosensitiw: tumors are usually radiosensitive.

A

True

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32
Q

Which types of radiation beams are used for superficial tumors and why?

A

Electron beams; their finite range spares deeper tissues.

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33
Q

True/False: The dose of radiation necessary to kill hypoxic cells is 2.5-3.0 times greater than that required to
kill well-oxygenated cells.

A

True, as free radical formation requires oxygen.

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34
Q

True/False: Cells undergoing DNA synthesis in the S phase are much more radiosensitiw: than cells in other
phases of the cell cycle.

A

False: They are much more radioresistant in the S phase.

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35
Q

True/False: The cells responsible for acute radiation injuries are rapidly cycling.

A

true

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36
Q

Which type of cancer is most sensitive to radiation therapy: exophytic, infiltrative, or ulcerated?

A

Exophytic

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37
Q

When, after XRI or radiation therapy, is a positive biopsy a reliable indicator of persistent disease?

A

3 months after treatment

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38
Q

How do XRT or radiation therapy failure differ from surgical failures in site of recurrence?

A

XRT or radiation therapy failures often occur in the center of areas that were grossly involved with cancer initially,
whereas surgical failures often occur at the periphery of the original tumor.

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39
Q

What is conventional fractionated radiotherapy?

A

1.8-2.5 Gy every day, live fractions every week, for 4-8 weeks (total dose 60-65 Gy for small tumors, 65-70 Gy for larger tumors).

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40
Q

What can be said of the presence of level V cervical metastases from SCCA of the upper aerodigestive tract?

A

Uncommon - 7%

if present, most likely to occur in the presence of IV metastases.

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41
Q

For SCCA of the tongue, invasion beyond ___ is associated with a significantly higher incidence of lymph
node metastasis.

A

4 mm

30% vs 7% if 4mm or less invasion

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42
Q

True/False: Disease-free, but not overall, survival is improved in patients with early oral tongue cancer who
undergo elective neck dissection.

A

True

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43
Q

When SCCA grossly invades the adventitia of the carotid artery, how will resection of the artery affect
survival?

A

it will not improve long term survival

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44
Q

True/False: Hypopharyngeal cancer has the worst prognosis of all head and neck cancers.

A

True
70% of patients present with advanced disease (stage III and IV) and the 5-year disease specific survival rate is only 33%

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45
Q

What are the most common and least common sites of tumor involvement in the hypopharynx?

A

Pyriform sinus is the most common site (75%)

postcricoid space is the least common site 3-4%

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46
Q

How does the behavior of pyriform sinus tumors differ from postcricoid and posterior pharyngeal wall
tumors?

A

tumors of the pyriform sinus tend to infiltrate deeply at early stages, whereas those of postcricoid area and posterior pharyngeal wall tend to remain superficial until achieving ad advanced stage.

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47
Q

What futures of hypopharyngeal tumors distinguish them from other head and neck tumors?

A

tendency for submucosal spread and skip lesions

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48
Q

What significance do these features of submucosa spread of hypopharyngeal tumors have on treatment?

A

Wide surgical margins (4-6 cm inferior to gross, 2-3 cm superior to gross) and wide radiation therapy ports are necessary

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49
Q

What is the incidence of cervical metastases at the time of presentation of pyriform sinus tumors? What
percent are bilateral or fixed?

A

60 % , 25%

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50
Q

True/False: The size of the primary lesion is related to the incidence of lymph node metastases in tumors of
the hypopharynx.

A

false

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51
Q

Where do posterior pharyngeal wall tumors metastasize?

A

bilaterally to level II cervical nodes, mediastinum, and superiorly to the nodes of Rouviere at hte skull base

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52
Q

Where do postcricoid space drain to ?

A

bilaterally into levels IV and VI

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53
Q

True/False: The involvment of the medial (as opposed to lateral) wall of the pyriform sinus significantly
increases the likelihood of bilateral cervical metastasis.

A

True

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54
Q

True/False: Due to the high. incidence of cervical metastases, treatment of the neck is necessary in all patients with hypopharyngeal cancer.

A

True

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55
Q

What is the incidence of a 2nd primary at the time of diagnosis in patients with hypopharyngeal cancer?

A

5-8%

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56
Q

What are the most common presenting symptoms in patients with tumor of the retromolar trigone?

A

referred otalgia and trismus

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57
Q

How many year does it take for a former smoker to have the same probability of developing an oral cavity
cancer as a nonsmoker?

A

16 years

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58
Q

What is the chance that a patient cured of an oral cavity cancer will develop a 2nd primary if they continue
to smoke

A

40 %

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59
Q

What is the incidence of cervical metastases from base of tongue, tonsil, and soft palate SCCA?

A

70 %
60 %
40%

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60
Q

What is the incidence of malignancy in adults with asymmetric tonsils with normal-appearing mucosa and
no cervical Iymphad.enopathyl

A

5%

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61
Q

What percent of T3/T4 tumors of the tonsil can be salvaged after failing primary XRT?

A

50%

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62
Q

What are the risk factors for developing osteosarcoma in the mandible or maxilla?

A

History of ionizing radiation, fibrous dysplasia, retinoblastoma, and prior exposure to thorium oxide (radioactive
scanning agent).

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63
Q

What chromosomal abnormality do osteosarcoma and retinoblastoma have in common?

A

Deletion of the long arm of chromosome 13.

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64
Q

True/False: There is a much lower risk of distant metastases with osteosarcoma of the head and neck than
that of the long bones.

A

true

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65
Q

What is the most common cause of death in osteosarcoma of the head and neck?

A

intracranial extension

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66
Q

True/False: A patient with T3N2aMO SCCA of the base of tongue has a complete response to extemal-beam
radiation therapy both at the primary site and the neck. A planned neck dissection should be done to
increase the rate of regional control.

A

true

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67
Q

What are the three most common odontogenic tumors

A

Ameloblastoma,
cementoma,
odontoma.

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68
Q

What are the three most common odontogenic cystsl

A

Radicular cyst (65%), odontogenic keratocyst, and dentigerous cyst.

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69
Q

What are odontomas composed of

A

Enamel, dentin, cementum, and pulp.

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70
Q

Where does a radicular or periapical cyst occur?

A

Along the root of a nonviable tooth, as the liquefied stage of a dental granuloma.

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71
Q

Where do dentigerous cysts develop?

A

Around the crown of an unerupted, impacted tooth.

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72
Q

Multiple odontogenic keratocyst:a are a manifestation of what syndrome?

A

Basal cell nevus syndrome.

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73
Q

What is a Pindborg tumor

A

Calcified epithelial odontogenic tumor that is less aggressive than ameloblastoma and is associated with an
impacted tooth.

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74
Q

Which mandibular tumor or cyst produces white, keratin-containing fluid

A

Odontogenic keratocyst.

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75
Q

What is the incidence of recurrence after excision of odontogenic keratocystl

A

62% in the first 5 years.

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76
Q

What percent of parotid gland tumors are benign?

A

75-80 %

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77
Q

What is the most common site of a salivary gland neoplasma

A

Parotid gland 73%

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78
Q

What is the least common site of a salivary gland neoplasm

A

submandibular gland 11%

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79
Q

What is the most common site of a malignant salivary gland neoplasm?

A

minor salivary glands (60 % of those 40% occur on the palate)

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80
Q

What is the least common site of a malignant salivary gland neoplasm?

A

Parotid gland 32%

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81
Q

Which salivary gland has the best prognosis for malignant tumors?

A

Parotid gland

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82
Q

Which salivary gland has the worst prognosis for malignant tumors?

A

submandibular gland

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83
Q

What is the most common tumor of the parotid gland?

A

Pleomorphic adenoma in adults

Hemangioma in children

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84
Q

How does metastasizing pleomorphic adenoma differ from carcinoma ex-pleomorphic adenoma?

A

It is histologically benign, lacking malignant epithelial components.

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85
Q

What is the most common malignant tumor of the parotid gland in adults?

A

Mucoepidermoid carcinoma

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86
Q

What is the most important prognostic factor for malignant salivary gland neoplasms?

A

Stage

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87
Q

Which five salivary gland tumors have the worst prognosis?

A

High-grade mucoepidermoid, adenocarcinoma,
squamous cell carcinoma, undifferentiated carcinoma,
carcinoma ex-pleomorphic adenoma.

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88
Q

What are the indications for postoperative radiation after parotidectomy?

A

High probability of residual microscopic disease; positive margins; advanced stage; high grade; deep lobe tumors;
recurrent tumors; the presence of regional metastases; and angiolymphatic invasion.

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89
Q

What factors are predictors of occult regional disease in parotid cancer?

A

Extracapsular extension, preoperative facial paralysis, age >54 years, and perilymphatic invasion

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90
Q

What are the indications for neck dissection in the treatment of salivary gland malignancies?

A

Clinical metastasis, submandibular tumor, SCCA, undifferentiated carcinoma, size >4 cm, and high-grade
mucoepidermoid carcinoma.

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91
Q

What is the most common site of distant metastasis for adenoid cystic carcinoma?

A

lungs

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92
Q

What is the most common salivary gland malignancy following radiation?

A

Mucoepidermoid.

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93
Q

What is the most common malignancy of the submandibular and minor salivary glands?

A

Adenoid cystic.

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94
Q

What type of tumor comprises 50% of all lacrimal gland neoplasms?

A

Adenoid cystic.

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95
Q

What are the four types of growth patterns of adenoid cystic carcinoma and which is most common?

A

Cribriform (most common-looks like Swiss cheese), tubular/ductular, trabecular, and solid.

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96
Q

Which type of radiation therapy does adenoid cystic carcinoma respond best to

A

Neutron beam

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97
Q

What is the most common salivary gland malignancy to occur bilaterally?

A

Acinic cell

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98
Q

What are the two most common malignant tumors of the parotid gland in children younger than 12?

A

Mucoepidermoid is the most common, followed by acinic cell.

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99
Q

What is the incidence of cervical metastasis of mucoepidermoid carcinomas?

A

30-40%

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100
Q

Your patient has a mucoepidermoid carcinoma of the parotid gland. Histologic evaluation of the biopsy
specimen reveals a scant amount of mucin. There is no clinical evidence of regional metastasis. Do you treat
the neck?

A

yes

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101
Q

What is the second most common malignant tumor of the minor salivary glands?

A

Adenocarcinoma

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102
Q

What are the four types of monomorphic adenomas?

A

Basal cell
trabecular
canalicular
tubular

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103
Q

Which salivary gland tumor is more common in women with a history of breast cancer?

A

Mucoepidermoid carcinoma

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104
Q

What is the treatment of choice for metastatic cutaneous SCCA to the parotid?

A

Total parotidectomy with preservation of VII (unless invaded by tumor) and postoperative radiation therapy to the
parotid area and ipsilateral neck.

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105
Q

What percent of malignant tumors of the parotid gland present with facial nerve weakness or paralysis?

A

20%

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106
Q

Which salivary gland tumor has a high propensity for perineural invasion?

A

Adenoid cystic carcinoma

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107
Q

What are the clinical features of salivary duct carcinomas?

A

Most commonly involve the parotid gland and present as an asymptomatic mass; higher incidence in males; distant
metastases are the most common cause of death.

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108
Q

Parapharyngeal tumors arising &om the deep lobe of the parotid will involve which compartment?

A

prestyloid compartment

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109
Q

What happens to the carotid sheath with deep lobe parotid tumors extending into the parapharyngeal space?

A

It is displaced posteriorly.

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110
Q

Which compartment are neurogenic tumors most likely to arise in?

A

Poststyloid compartment

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111
Q

What is the most common tumor of the parapharyngeal space?

A

Pleomorphic adenoma.

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112
Q

What percent of head and neck paragangliomas are familial?

A

7-10 %

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113
Q

What is the most common paraganglioma of the head and neck?

A

Carotid body tumor

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114
Q

What percent of carotid body tumors are multicentric?

A

10%

30-40% in the hereditary form

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115
Q

What is the inheritance pattern of familial carotid body tumors?

A

Autosomal dominant but only the genes passed from the paternal side are expressed (maternal genomic imprinting).

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116
Q

What is a “nonchromaffin’’ paraganglioma?

A

one that does not secret significant amounts of catecholamines

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117
Q

What percent of cervical paragangliomas secrete catecholamines ?

A

5%

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118
Q

What is Shamblin’s dassification system for carotid body tumors

A

Group I: Small and easily excised.
Group II: Adherent to the vessels; resectable with careful subadventitial dissection.
Group III: Encase the carotid; require partial or complete vessel resection

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119
Q

What are the two types of temporal bone paragangliomas?

A

Glomus jugulare involving the adventitia of the jugular bulb and glomus tympanicum involving Jacobson’s nerve
(jugulotympanic glomus if unable to discern site of origin).

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120
Q

How do glomus tumors differ clinically from carotid body tumors

A

More common in females, less likely to secrete catecholamines or metastasize, and are more radiosensitive.

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121
Q

What is the primary advantage of stereotactic radiosurgery for treatment of recurrent glomus jugulare
tumors compared with surgery and conventional radiation

A

lower incidence of cranial nerve injury

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122
Q

When is stereotactic radiosurgery contraindicated in the treaunent of recurrent glomus jugulare tumors?

A

For larger tumors (> 3.0-4.0 cm).

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123
Q

What is basal cell-nevoid syndrome

A

Autosomal dominant disorder characterized by multiple basal cell carcinomas, odontogenic keratocysts, rib
abnormalities, palmar and plantar pits, and calcification of the falx cerebri.

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124
Q

What are some other genetic disorders that are associated with a high risk of cutaneous malignancies?

A

Xeroderma pigmentosum, albinism, epidermodysplastic verruciformis, epidermolysis bullosa dystrophica, and
dyskeratosis congenital.

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125
Q

What is Marjolin’s ulcer

A

Burn or ulcer associated with the development of malignancy.

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126
Q

What is the most common premalignant skin lesion of the head and neck?

A

Actinic keratosis.

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127
Q

What is the name of the skin lesion, most commonly located on the nose, characterized by rapid growth with
a central area of ulceration followed by spontaneous involution

A

Keratoacanthoma.

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128
Q

What is Bowen’s disease?

A

Squamous cell carcinoma in situ of the skin.

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129
Q

True/False: Adnexal carcinomas of the skin are very aggressive and have a poor prognosis

A

true

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130
Q

Which adnexal skin carcinoma ames from a pluripotential basal cell within or around the hair cells?

A

Merkel cell carcinoma.

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131
Q

What virus is strongly associated with Merkel cell carcinoma that is known to cause cancer in animals?

A

Merkel cell polyomavirus.

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132
Q

What is the 5-year survival of patients with Merkel cell carcinoma?

A

30%

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133
Q

What test should be ordered in the workup of Merkel cell carcinoma?

A

Positron emission tomography scan.

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134
Q

Should the N0 neck be treated in patients with Merkel cell carcinoma?

A

yes

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135
Q

What is the most common type of skin sarcoma

A

Malignant fibrous histiocytoma.

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136
Q

What are the five main types of basal cell carcinomas?

A
nodular
cystic
superficial multicentric
morpheaform
keratotic
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137
Q

most common basal cell ca

A

nodular

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138
Q

basal cell carcinoma commonly found on extremities and trunk

A

superficial multicentric

139
Q

Which of these is a variant of nodular basal cell carcinomas and produces pigment?

A

cystic

140
Q

basal cell carcinoma commonly resemble scar

A

morpheaform

141
Q

most agressive basal cell carcinoma

A

keratotic

142
Q

which areas of the face are most susceptible to basal cell carcinomas?

A

Inner canthus,
philtrum,
mid-lower chin,
nasolabial groove, preauricular area, retroauricular sulcus.

143
Q

What proportion of incompletely excised basal cell cancers will recur

A

one third

144
Q

True/False: squamous cell carcinomas arising in sun-exposed areas tend to behave less aggressively than those
arising de novo

A

true

145
Q

What percent of squamous cell carcinoma arising in areas of actinic change metastasize?

A

3-5 %

146
Q

What percent of squamous cell carcinoma arising de novo metastasize?

A

8%

147
Q

What percent of squamous cell carcinoma arising in areas of scar or chronic inflammation metastasize?

A

10-30%

148
Q

What are the five histopathologic types of squamous cell carcinoma?

A
Generic, 
adenoid, 
bowenoid, 
verrucous,
spindle-pleomorphic.
149
Q

Which of these SSC typically arises in areas of actinic change?

A

generic

150
Q

Which of these SSC is more common in the oral mucosa?

A

Verrucous

151
Q

Which of SSC is the least common

A

Spindle-pleomorphic.

152
Q

What factors increase the likelihood of recurrence for squamous cell carcinoma?

A

Tumors on the midface, diameter >2 cm or thickness >4 mm, perineural invasion, or regional metastases.

153
Q

What factors increase the likelihood of regional metastasis of squamous cell carcinoma?

A

Tumors arising on the ear, diameter >2 cm or >4 mm thickness, poorly differentiated histology, and recurrent tumors.

154
Q

What are the four types of melanoma

A

Superficial spreading, lentigo maligna, acrallentiginous, and nodular sclerosing.

155
Q

most common type of melanoma

A

superficial spreading

156
Q

best prognosis type of melanoma

A

superficial spreading

157
Q

What is the most common form of hereditary cutaneous melanoma?

A

Dysplastic nevus syndrome.

158
Q

Which type of melanoma occurs on palms, soles, nail beds, and mucous membranes

A

Acral lentiginous melanoma.

159
Q

What percent of melanomas occur in the head and neck?

A

20%

160
Q

What percent of tumors (melanomas) are not pigmented (amelanotic)?

A

5%

161
Q

What mutation has been found in more than half of malignant melanomaa?

A

BRAF somatic missense mutations; a single substitution (V599E) accounts for 80% of these.

162
Q

What cells are melanomas composed of

A

Melanocytes, which are derived from neural crest cells.

163
Q

What are the risk factors for developing melanoma?

A
Family history, 
multiple atypical or dysplastic nevi, 
Hutchinson's freckle,
presence of large congenital nevi, 
blond or red hair, 
marked freckling on upper back, 
history of three or more blistering sunburns prior to age 20, 
presence of actinic keratoses.
164
Q

What is the risk of melanomatous transformation of giant congenital nevi?

A

14%

165
Q

What percent of patients with xeroderma pigmentosa develop melanoma?

A

3%

166
Q

How should a lesion suspicious for melanoma be biopsied?

A

A sample should be taken of the tumor and the underlying tissue so that depth can be ascertained; a shave biopsy
should never be performed.

167
Q

What is the most important prognostic factor of melanomas?

A

depth of invasion

168
Q

What is the incidence of nodal metastases if the depth of the tumor is >4.0 mm

A

> 70%

169
Q

What is the incidence of nodal metastases if the depth of the tumor is <1.5 mm

A

8%

170
Q

True/False: Women with melanoma have a better prognosis than men regardless of tumor depth.

A

True.

171
Q

What tumor factor, other than depth, influences regional metastasis in melanoma?

A

ulceration

172
Q

Involvement of which areas of the body also increases the risk of metastases (of melanoma)?

A

BANS: back, arms, neck, and scalp.

173
Q

What is the chance that a patient with melanoma will develop a second melanoma?

A

5%

174
Q

Is melanoma radiosensitive?

A

It may be sensitive to large dose fractions (600 cGy) but not to standard fractionation radiotherapy (180-200 cGy).

175
Q

What is the role of large-dose fraction radiotherapy in the management of melanoma?

A

Decreases incidence of locoregional recurrence among NO patients.

176
Q

What is the risk of developing esophageal cancer in patients who smoke and drink compared with those who do not

A

100 times higher

177
Q

In which areas of the world is the incidence of esophageal cancer highest

A

Middle East, southern and eastern Africa, and northern China.

178
Q

What are the risk factors for developing esophageal cancer?

A

Tobacco, alcohol, achalasia, Plummer-Vinson syndrome, prior head and neck cancer, tylosis, and Barrett’s disease.

179
Q

What are the clinical features of Plummer-Vinson syndrome

A

Iron-deficiency anemia, upper esophageal web, hypothyroidism, glossitis/cheilitis, gastritis, and dysphagia.

180
Q

In patients with Plummer-Vinson syndrome, where is squamous cell carcinoma of the esophagus most likely
to occur?

A

Postcricoid area.

181
Q

Metaplasia of the distal esophagus is otherwise known as what

A

Barrets eosphagus

182
Q

What percent of people with gastroesophageal reflux disease have Barrett’s esophagus and what percent of these people will develop adenocarcinoma?

A

5%

5-10%

183
Q

Cancer of the cervical esophagus is usually what typ

A

SCC

184
Q

What is the usual cause of death from esophageal cancer?

A

aspiration pneumonia

185
Q

When do patients with synovial sarcoma usually present?

A

between age of 25-36%

186
Q

Where are most synovial sarcomas of the head and neck located?

A

hypopharynx and parapharyngeal space

187
Q

What is the incidence of regional metastasis in synovial sarcomas of the head and neck

A

12.5%

188
Q

What is the most common cause of death from synovial sarcoma of the head and neck

A

lung metastises

189
Q

What is the primary mode of treatment for synovial sarcoma of the head and neck?

A

wide surgical excision and postoperative radiation therapy

190
Q

5 years survival rate of synovial sarcoma

A

40-50%

191
Q

What prognostic significance does the presence of microcalcifications have in synovia sarcoma?

A

better prognosis

192
Q

Nasopharyngeal cancer accounts for what percent of all cancers diagnosed in the Kwangtung province of
southern China?

A

20%

193
Q

What is the incidence of nasopharyngeal cancer among native-born Chinese compared with that among
Caucasians?

A

118 times higher

194
Q

What EBV product is likely to play a role in malignant transformation of nasopharyngeal epithelium

A

Latent membrane protein (LMP-1).

195
Q

What environmental factor is most strongly linked to nasopharyngeal carcinoma?

A

Frequent consumption of dried salted fish.

196
Q

What is the most common site of origin of nasopharyngeal cancer

A

Fossa of rosenmuller

197
Q

Which nodal groups does naaopharyngeal cancer spread to

A

retropharyngeal nodes of rouviere
juggulodigastic nodes
spinal accessory chain

198
Q

In the staging system desaibed by Ho, poorer prognosis is associated with cervical metastases to which area of the neck

A

Inferior to a plane spanning from the contralateral sternal head of the clavicle to the ipsilateral superir margin of the trapezius muscle

199
Q

What is the incidence of skull base erosion in patients with nasopharyngeal carcinoma

A

25%

200
Q

What is the most common site of distant metastases (nasopharyngeal ca)

A

Bones

201
Q

Smooth, submucosal nasopharyngeal masses located in the midline are most often what

A

Embryologic remnants

Thornwald’s cysts, pharyngeal bursa remnants

202
Q

What factors, described by Ho and Neel, are regarded as important adverse prognostic indicators in patients
with nasopharyngeal carcinoma?

A

Length and symptomatology of disease, extension of tumor outside of the nasopharynx, presence of inferior cervical
adenopathy, keratinizing histologic architecture, cranial nerve and skull base extension, presence of distant
metastases, and low ADCC titers.

203
Q

Extension into which space is associated with the worst prognosis in patients with nasopharyngeal
carcinoma

A

anterior masticator space

204
Q

What is the primary treatment modality for nasopharyngeal cancer?

A

Radiation therapy to the nasopharynx (66-70 Gy) and neck (60 Gy).

205
Q

Why is the clinically negative neck treated (nasopharynx)?

A

Studies have shown improved local control and disease-free survival for prophylactic irradiation of the clinically
negative neck in patients with nasopharyngeal carcinoma.

206
Q

What are the complications from radiation overdosage in the treatment of nasopharyngeal carcinoma?

A
Osteoradionecrosis 
brain necrosis 
transverse myelitis
hearing loss
hypopituitraism
hypothyroidism
optic neuritis
207
Q

What is the role of induction chemotherapy for treatment of nasopharyngeal carcinoma?

A

No survival advantage has been proven.

208
Q

What is the standard treatment protocol for stage III and IV nasopharyngeal carcinoma?

A

Concomitant cisplatin and XRT followed by adjuvant chemotherapy with cisplatin and 5-FU.

209
Q

How does treatment failure usually manifest in nasopharyngeal carcinoma?

A

disease at both primary site and cervical lymph nodes

210
Q

What is the most common site of recurrent/persistent nasopharyngeal carcinoma?

A

Lateral wall of the nasopharynx.

211
Q

What are the treatment options for recurrent/persistent nasopharyngeal carcinoma at the primary site?

A

Reirradiation with larger therapeutic dose than initial treatment; stereotactic radiotherapy; brachytherapy with split
palate implantation of radioactive gold grains; surgical resection.

212
Q

What is the most common benign sinonasal neoplasm?

A

Inverting papiloma

213
Q

What is the most common malignant sinonasal neoplasm?

A

SSC 80%

214
Q

What is the 2nd most common malignant sinonasal neoplasm?

A

Adenocarcinoma

215
Q

What are the most common locations of sinonasal squamous cell carcinoma?

A

Maxillary sinus followed by nasal cavity then ethmoid sinuses

216
Q

Are elective neck dissections warranted in patients with sinonasal squamous cell carcinoma?

A

No

incidence of occult cervical lymph nodes metastases is 10%

217
Q

What percentage of sinonasal tumors can be attributed to occupational exposures?

Where are they usually located ?

A

up to 44%

Lateral nasal wall, adjacent to the middle turbinate

218
Q

Which substances are thought to predispose to sinonasal neoplasms?

A

Nickel, chromium, isopropyl oils, volatile hydrocarbons, organic fibers from wood, shoe, and textile refineries

219
Q

Which substance exposure classically associated with SCCA?

A

Nickel

220
Q

Which substance exposure classically associated with adenocarcinoma?

A

Hardwood dust and leather tanning substances

221
Q

Which virus is thought to play a role in the etiology of sinonasal tumors?

A

Human papilloma virus (HPV), particularly typres 6 and 12

222
Q

True/False: Smoking by itself is not a significant etiologic factor for sinonasal tumors.

A

True

223
Q

Which nasal masses should not be biopsied in the clinic?

A

Masses in children or adolescents and masses suspicious for angiofibroma-some also recommend delaying biopsy
of any nasal mass until after imaging has been obtained.

224
Q

What are the three subtypes of Schneiderian papillomas?

A

Fungiform
Inverting
Cylindrical

225
Q

Where do inverting papillomas most commonly arise?

A

Lateral nasal wall

226
Q

What factor is most related to the chance of recurrence for inverting papilloma?

A

Method of removal

227
Q

What is the incidence of recurrence after resection of inverting papilloma via lateral rhinotomy/medial
maxillectomyl

A

13-15%

228
Q

In patients who undergo resection of inftrting papilloma via lateral rhinotomy/medial maxillectomy, what is
the most important factor related to risk for recurrence?

A

Mitotic index

229
Q

What is the differential diagnosis of a small cell sinonasal tumor?

A

Esthesioneuroblastoma, plasmacytoma,
melanoma,
lymphoma,
sarcoma,
poorly differentiated squamous cell carcinoma,
Ewing’s sarcoma,
peripheral neuroectodermal tumor (PNET),
SNUC (sinonasal undifferentiated carcinoma).

230
Q

What is a SNUC?

A

Sinonasal undifferentiated carcinoma

very aggrissive small cell tumor

231
Q

In what age group is olfactory neuroblastoma typically seenl

A

Bimodal distribution-people in their 20s and 50s.

232
Q

Kadish system of classification of neuroblastoma

A

Kadish A: confined to nasal cavity (18%)
Kadish B: extends to paranasal sinuses (32%)
Kadish C: extends beyond nasal cavity and paranasal sinuses (49%)
Kadish D: lymph node or distant metastases

233
Q

What are the three most common malignant bone tumors of the paranasal sinuses?

A

Multiple myeloma, osteogenic sarcoma, chondrosarcoma.

234
Q

What is the pathophysiology of fibrous dysplasia?

A

Normal medullary bone is replaced by collagen, fibroblasts, and osteoid.

235
Q

Where is fibrous dysplasia most commonly found in the head and neck

A

maxilla

236
Q

Where is adenoid cystic carcinoma of the head and neck most commonly found

A

Palate, followed by major salivary glands, then paranasal sinuses.

237
Q

Where is melanoma most commonly found in the nose and paranasal sinuses

A

Nasal septum

238
Q

How does nasal melanoma differ from cutaneous melanoma

A

More aggressive with a worse prognosis and an unpredictable course-local recurrence is the most common cause
of failure.

239
Q

What is the most common type of lymphoma of the nose and paranasal sinuses~

A

Non-Hodgkin’s lumphoma

240
Q

What is Ohngren’s line and how is it significant?

A

Imaginary line from the medial canthus to the angle of the mandible; tumors below the line have a better prognosis
than tumors above the line (with the palate as an exception).

241
Q

What tumor Comprises only 3% of Schneiderian papillomas

A

Cylindrical papiloma

242
Q

What tumor is Most common type of Schneiderian papilloma, typically seen on the nasal septum

A

Fungiform papiloma

243
Q

2-13% of these benign sinonasal tumors have malignant potential

A

inverting papillomas

244
Q

Has a predilection for the mandible and a sunray appearance on X-ray

A

Osteogenic sarcoma

245
Q

More than 90% will have invaded through at least one wall of the
involved sinus at presentation

A

SCC

246
Q

Benign tumor, most commonly seen in patients less than 20 years old
and has a ground glass appearance on X-ray

A

Fibrous dysplasia

247
Q

Benign tumor most commonly found in the frontal sinus

A

Osteoma

248
Q

Encapsulated, benign tumor that arises from the surface of nerve fibers

A

Neurilemoma

249
Q

Unencapsulated tumor that arises from within a nerve; 15% become malignant (when associated with von Recklinghausen’s disease}

A

Neurofibroma

250
Q

Second most common malignant sinonasal tumor; tend to be located superior to Ohngren’s line

A

Adenocarcinoma

251
Q

Arise from pericytes of Zimmerman and considered neither benign nor malignant

A

Hemangiopericytoma

252
Q

Arise from stem cells of neural crest origin that differentiate into
olfactory sensory cells; Homer Wright rosettes are characteristic

A

Olfactory neuroblastoma or

esthesioneuroblastoma

253
Q

Most common tumor to metastasize to the sinonasal area

A

Renal cell

254
Q

Well-circumscribed, mobile, painless benign lesion most commonly found on the tongue that has malignant potential and histopathology shows polygonal cells with abundant eosinophils

A

Granular cell tumor

255
Q

Metastasizes to the brain more frequently than any other soft-tissue
sarcoma

A

Alveolar soft part sarcoma

256
Q

Which sinonasal neoplasnm remodel rather than erode bone?

A
Sarcomas, 
minor salivary gland carcinomas,
hernangiopericytomas,
extramedullary plasmacytomas, 
large cell lymphomas, 
and olfactory neuroblastomas.
257
Q

What is the primary modality of treatment for extramedullary plasmacytomas?

A

Radiation

258
Q

After benign lymphoid hyperplasia, what is the most common benign nasopharyngeal tumor?

A

Juvenile nasopharyngeal angiofibroma (JNA)

259
Q

From which site in the nasopharynx does this tumor develop?

A

Trifurcation of the palatine bone,

horizontal ala of the vomer, and the root of the pterygoid process.

260
Q

Where does the main blood supply to these tumors (Juvenile nasopharyngeal angiofibroma) most often come from?

A

Internal maxillary artery or the ascending pharyngeal artery.

261
Q

What is the second leading cause of death among children ages 1-14?

A

Cancer

262
Q

What is the most common solid malignant tumor in infants < 1 year?

A

Neuroblastoma

263
Q

What are the precursor cells of neuroblastoma?

A

Neural crest cells

264
Q

What is the survival rate after complete excision of lesions in children < 1

A

90%

265
Q

What is the most common head and neck tumor of children?

A

Lymphoma

266
Q

In what age groups is Hodgkin’s lymphoma most common

A

Bimodal peak incidence, with one peak in the 15- to 34-year-old age group and another in later adulthood.

267
Q

What percent of Hodgkin’s lymphoma cases are associated with EBV?

A

40%

268
Q

True/False: Axillary, inguinal, and Waldeyer’s ring involvement is uncommon in patients with Hodgkin’s
lymphoma.

A

true

269
Q

Which lymphoma accounts for 50% of childhood malignancies in equatorial Africa

A

Burkitt’s lymphoma

270
Q

What is the most common soft tissue sarcoma of the head and neck in childrern

A

Rhabdomyosarcoma

271
Q

In what age groups is rhabdomyosarcoma most common?

A

Ages 2-5 and 15-19.

272
Q

What is the treatment for rhabdomyosarcoma?

A

Multirnodality; primary chemoradiation followed by surgery for recurrent or residual disease.

273
Q

Involvement in which area of the head and neck by rhabdomyosarcoma has the best prognosis

A

orbit

274
Q

What is the most common type of well-differentiated thyroid carcinoma in children

A

Papillary

275
Q

What is the most common benign neoplasm of the larynx in children?

A

papillomas

276
Q

What percent of patients with recurrent respiratory papillomatosis (RRP) devdop distal tracheal and
pulmonary spread of papillomas

A

5%

277
Q

What percent of patients with RRP ( recurrent respiratory papillomatosis) require tracheostomy

A

15%

278
Q

What percent of patients with distal spread have had a previous tracheostomy

A

95%

279
Q

What is the incidence of stomal papilloma recurrence rate after tracheostomy for RRP( recurrent respiratory papilomatosis) ?

A

> 50%

280
Q

What are the most common subtypes of HPV isolated from RRP

A

6 and 11

found in >95%

281
Q

What are the most common respiratory complications of distal RRP

A

Pneumatocele, abscess, and tracheal stenosis

282
Q

Why is aspergillus infection a risk factor for ear and temporal bone tumors?

A

It produces aflatoxin B, a known carcinogen.

283
Q

What are other risk factors for development of ear and temporal bone tumors?

A

History of radiation to the head and neck, chronic chromate burns secondary to using matchsticks to clean
the ear canal.

284
Q

What is the most common site of ear and temporal bone tumors?

A

External auditory canal (EAC).

285
Q

What is the most common route of spread of tumors in the cartilaginous portion of the EAC

A

Through the fissures of Santorini.

286
Q

What is the most common histologic type of tumor involving the EAC or middle ear

A

SCC

287
Q

Where do most basal cell carcinomas of the EAC arise?

A

Concha

288
Q

What is the most common tumor of glandular origin to involve the EAC or middle ear?

A

Adenoid cystic carcinoma

289
Q

What are the most common types of sarcoma of the temporal bone?

A

Rhabdomyosarcoma, chondrosarcoma, and osteosarcoma.

290
Q

What are the most common sites of origin of metastatic tumors of the temporal bone?

A

Breast, lung, and kidney

291
Q

Tumors that metastasize to the temporal bone hematogenously most often involve which area of the
temporal bone?

A

Petrous apex

292
Q

Tumors that metastasize to the temporal bone via the meninges most often traverse what structure?

A

IAC internal auditory canal

293
Q

What is the most common presentation of tumors of the EAC

A

Unremitting pain and serosanguinous otorrhea

294
Q

What percent of patients with a tumor in the EAC will present with cervical metastases?

A

10%

295
Q

What percent of patients with a tumor in the middle ear will present with facial nerve palsy?

A

20-40%

296
Q

What is the most common tumor of the cerebellopontine angle (CPA)?

A

Vestibular schwannoma

297
Q

What is the differential diagnosis of a CPA tumor?

A

Schwannoma, meningioma, epidermoid, lipoma, arachnoid cyst, cholesterol granuloma.

298
Q

What is the incidence of patients with vestibular schwannomas who have normal hearing at presentation?

A

5%

299
Q

What is the nature of vertigo in the majority of patients with a vestibular schwannoma

A

Chronic disequilibrium with self-limiting episodes of vertigo

300
Q

What is the most wmmon type of hearing loss in patients with a vestibular schwannoma

A

High-frequency unilateral SNHL.

301
Q

What is a typical word discrimination score in a patient with a vestibular sc:hwannoma

A

0-30% in >50% of patients with acustic neuroma

302
Q

Adenocarcinoma of the endolymphatic sac is more common in patients with what disease

A

Von Hippel-Lindau disease.

303
Q

What percent of thyroid cancers are well differentiated

A

95%

304
Q

What percent of thyroid nodules are malignant?

A

< 5%

305
Q

What is the most common thyroid nodule?

A

follicular adenoma

306
Q

What is the significance of age with thyroid nodules?

A

More likely to be malignant in women over 50 and men over 40 and in both men and women under 20.

307
Q

What percent of solitary thyroid nodules in children are malignant?

A

50%

308
Q

What is the significance of size with thyroid nodules?

A

More likely to be malignant if >4 cm in diameter.

309
Q

What are the three types of well-differentiated thyroid malignancies?

A

Follicular, papillary, and Hurthle cell.

310
Q

Which of these well differentiated thyroid malignancies is associated with iodine deficiency?

A

papillary

311
Q

well differentiated thyroid malignancies relatively unresponsive to ablation with radioactive iodine

A

Hurthle cell

312
Q

A 65-year-old woman presents with a cervical lymph node that is found to have well-differentiated thyroid
tissue but the thyroid has no palpable abnormality. What is the next step in management?

A

Total thyroidectomy and modified radical neck dissection

313
Q

What factor best correlates with the presence of lymph node metastases in papillary carcinoma?

A

Age

314
Q

True/False: Microscopic lymph node involvement does not change the long-term survival in patients with
papillary thyroid cancer.

A

true

315
Q

What is the incidence of multicentric disease on pathological examination of the entire thyroid in patients
with papillary carcinoma (> 1 cm)

A

70-80%

316
Q

What histological subtypes of thyroid tumors are associated with an increased risk of local recurrence and
metastasis

A

Tall cell, columnar, insular, solid variant, and poorly differentiated.

317
Q

A 36-year-old woman presents with a 3 cm papillary carcinoma and no clinical evidence of lymph node
inwlvement, no intrathyroidal vascular invasion, and no gross or microscopic multifocal disease. She has no
history of neck radiation and no family history of thyroid cancer. She was treated with a total thyroidectomy.
Is radioiodine ablation therapy indicated

A

no

318
Q

What is the most common site of metastasis from follicular thyroid cancer?

A

bone

319
Q

How is the definitive diagnosis of follicular thyroid cancer made?

A

By demonstration of capsular invasion at the interface of the tumor and the thyroid gland

320
Q

What is the most important prognostic indicator of follicular thyroid cancer?

A

Degree of angioinvasion

321
Q

True/False: Follicular cell carcinoma is more aggressive than Hurthle cell.

A

false

322
Q

What are the indications for adjuvant thyroid hormone in patients with well-differentiated thyroid
carcinoma?

A

All patients with well-differentiated carcinoma should be treated with thyroid hormone to suppress TSH for life,
regardless of the extent of their surgery.

323
Q

In what four settings does medullary thyroid carcinoma (MTC) arise

A

Sporadic, familial, and in association with multiple endocrine neoplasia Ila or lIb.

324
Q

Medullary thyroid ca - best prognosis

A

familial

325
Q

Medullary thyroid ca - tends to occur unilaterally

A

sporadic

326
Q

Medullary thyroid ca - worst prognosis

A

sporadic

327
Q

What percentage of MTC occurs sporadically?

A

70-80 %

328
Q

What are the characteristics of familial MTC?

A

Autosomal dominant inheritance pattern; not associated with any other endocrinopathies

329
Q

What other disorders are present in patients with MEN Ila?

A

Pheochromocytoma, parathyroid hyperplasia

330
Q

True/False: All patients with MEN IIa will have MTC.

A

true

331
Q

What other disorders are present in patients with MEN lIb

A

Pheochromocytoma, multiple mucosal neuromas, marfanoid body habitus.

332
Q

What is the surgical treatment for MTC?

A

Total thyroidectomy with central node dissection, lateral cervical lymph node sampling of palpable nodes, and a
modified radical neck dissection, if positive.

333
Q

What are the two types of anaplastic thyroid cancer?

Which is more common?

Which of these is usually responsive to radiation therapy?

A

Large cell and small cell.

Large cell - more common

small cell responsive to radiation

334
Q

What is the appropriate management for a patient with an anaplastic thyroid carcinoma?

A

Debulking and tracheostomy may be performed for palliation of airway obstruction.

335
Q

What is the beat treatment for primary non-Hodgkin’s lymphoma of the thyroid gland

A

Chemoradiation.

336
Q

A 44-yea.r-old man present with a 5 cm thyroid nodule. FNA returns fluid, the nodule disappears, and the
cytology is benign. What is the next step in management?

A

Total thyroid lobectomy with isthmusectomy should be considered because there is an increased chance of
malignancy in large cysts.

337
Q

A 56-year-old man with no risk factors presents with a thyroid nodule. The FNA is nondiagnostic. What is
the treatment of choice?

A

Total thyroid lobectomy with isthmusectomy.

338
Q

What are the indications for postoperative radioiodine ablation therapy in thyroid

A

Known distant metastases, gross extrathyroidal extension of tumor, tumors larger than 4 cm, tumors 1-4 cm when
T and N status, age, and histological features predict an intermediate to high rate of recurrence.

339
Q

Known distant metastases, gross extrathyroidal extension of tumor, tumors larger than 4 em, tumors 1-4 em when
T and N status, age, and histological features predict an intermediate to high rate of recurrence.

A

true

340
Q

Which medication improves quality of life when preparing patients for radioiodine scanning and ablation
therapyl

A

Recombinant TSH stimulation (rTSH).

341
Q

How are patients with MTC managed postoperatively?

A

Receive L-thyroxine and 2 weeks of calcium and vitamin D supplementation; serial measurements of calcitonin and
CEA.

342
Q

Severe cerebellar symptoms with a normal MRI suggests what condition?

A

Paraneoplastic cerebellar degeneration

343
Q

What are the two primary subtypes of paraneoplastic cerebellar degeneration?

A

Vestibulocerebellar syndrome and opsoclonus-myoclonus syndrome.

344
Q

Which malignancies most commonly cause paraneoplastic cerebellar degeneration?

A

In adults, ovarian, uterine, breast, and small cell lung cancer.
In children, neuroblastoma.