Diseases, disorders and conditions - NEOPLASTIC Flashcards
What is the most common site of laryngeal cancer?
Glottic
What are the 7 different types of squamous cell aberrations occurring in the larynx?
Benign hyperplasia Benign keratosis (no atypia) Atypical hyperplasia Keratosis with atypia or dysplasia Intraepithelial carcinoma Microinvasive squamous cell carcinoma(SCCA) Invasive SCCA
What percent of patients with carcinoma in situ of the weal cord will develop inv.uive SCCA after a single
exclsional biopsy?
one in six - 16.7%
What is “microinvasive” SCCA of the vocal cord?
Invades through the basment membrane but not into the vocalis muscle
What is Ackerman’s tumor?
Verrucous carcinoma, thought to be less radiosensitive and less likely to metastasize than SCCA
What are the two most important factors predicting lymph node metastasis in laryngeal cancer?
Tumor size and location
True/False: Once invasion of the laryngeal framework occurs, the ossified portions of cartilage have the least
resistance to tumor spread.
true
What percent of glottic tumors display perineural and vascular invasion?
25%
What percent of patients with a primary laryngeal cancer will eventually develop a 2nd primary?
10-20%
What is the stage of a transglottic tumor without vocal cord fixation, cartilage invasion, or extension beyond
the larynx?
T2
Which parts of the glottis are most difficult to treat with radiation?
Anterior commissure, posterior 1/3 of the vocal cord.
What percent of glottic tumors will metastasize to the cervical lymph nodes?
25%
What is the incidence of positive cervical nodes in patients with T3 glottic tumorsl
30-40 %
Which type of laryngeal cancer is most likely to metastasize distally?
supraglottic
What is the most common site of distant metastasis from laryngeal carcinoma?
lungs
How does metastatic disease to the lungs normally present?
Multiple small lesions less than 3 mm that are difficult to detect on X-ray.
Where does supraglottic carcinoma most often begin?
Junction of the epiglottis and false cords
What anatomic structure serves as a natural barrier to the inferior extension of supraglottic cancers?
Ventricle (embryologic developmnent is completely separate from the false cord).
Which kinds of supraglottic cancers are more likely to extend inferiorly to the anterior commissure or
-ventricule
ulcerative or exophytic
ulcerative lesions
True/False: Stage I lesions of the supraglottis can be controlled equally well with radiotherapy or surgery.
True
What is the risk of cervical metastases in patients with Tl, T2, T3, and T4 tumors of the supraglottis?
T1 20%
T2 40%
T3 60%
T4 80%
What percent of patients undergoing supraglottic laryngectomy and unilateral neck dissection will fail in the
contralateral neck?
16%, despite receiving radiation therapy to the area.
What percent of laryngeal tumors are primarily subglottic ?
5%
What are the differences between primary and secondary subglottic tumors?
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.
What is the primary site of lymphatic drainage fom subglottic tumors?
Paratracheal nodes.
Compared with supraglottic and glottic tumors, subglottic tumors are at a much higher risk for developing
what?
stomal recurrence
What is the treatment of choice for primary subglottic cancer?
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.
What is the bat organ-sparing treatment for a patient with stage III SCCA of the supraglottis?
Induction chemotherapy followed by radiation therapy.
What are the indications for postoperative radiation after neck dissection?
Multiple nodes or extracapsular spread
What is the significance of the number of pathologically positive nodes on prognosis~
Greater than 3 pathologically positive nodes is a negative prognostic indicator.
True/False: Chemosensitiw: tumors are usually radiosensitive.
True
Which types of radiation beams are used for superficial tumors and why?
Electron beams; their finite range spares deeper tissues.
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.
True, as free radical formation requires oxygen.
True/False: Cells undergoing DNA synthesis in the S phase are much more radiosensitiw: than cells in other
phases of the cell cycle.
False: They are much more radioresistant in the S phase.
True/False: The cells responsible for acute radiation injuries are rapidly cycling.
true
Which type of cancer is most sensitive to radiation therapy: exophytic, infiltrative, or ulcerated?
Exophytic
When, after XRI or radiation therapy, is a positive biopsy a reliable indicator of persistent disease?
3 months after treatment
How do XRT or radiation therapy failure differ from surgical failures in site of recurrence?
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.
What is conventional fractionated radiotherapy?
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).
What can be said of the presence of level V cervical metastases from SCCA of the upper aerodigestive tract?
Uncommon - 7%
if present, most likely to occur in the presence of IV metastases.
For SCCA of the tongue, invasion beyond ___ is associated with a significantly higher incidence of lymph
node metastasis.
4 mm
30% vs 7% if 4mm or less invasion
True/False: Disease-free, but not overall, survival is improved in patients with early oral tongue cancer who
undergo elective neck dissection.
True
When SCCA grossly invades the adventitia of the carotid artery, how will resection of the artery affect
survival?
it will not improve long term survival
True/False: Hypopharyngeal cancer has the worst prognosis of all head and neck cancers.
True
70% of patients present with advanced disease (stage III and IV) and the 5-year disease specific survival rate is only 33%
What are the most common and least common sites of tumor involvement in the hypopharynx?
Pyriform sinus is the most common site (75%)
postcricoid space is the least common site 3-4%
How does the behavior of pyriform sinus tumors differ from postcricoid and posterior pharyngeal wall
tumors?
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.
What futures of hypopharyngeal tumors distinguish them from other head and neck tumors?
tendency for submucosal spread and skip lesions
What significance do these features of submucosa spread of hypopharyngeal tumors have on treatment?
Wide surgical margins (4-6 cm inferior to gross, 2-3 cm superior to gross) and wide radiation therapy ports are necessary
What is the incidence of cervical metastases at the time of presentation of pyriform sinus tumors? What
percent are bilateral or fixed?
60 % , 25%
True/False: The size of the primary lesion is related to the incidence of lymph node metastases in tumors of
the hypopharynx.
false
Where do posterior pharyngeal wall tumors metastasize?
bilaterally to level II cervical nodes, mediastinum, and superiorly to the nodes of Rouviere at hte skull base
Where do postcricoid space drain to ?
bilaterally into levels IV and VI
True/False: The involvment of the medial (as opposed to lateral) wall of the pyriform sinus significantly
increases the likelihood of bilateral cervical metastasis.
True
True/False: Due to the high. incidence of cervical metastases, treatment of the neck is necessary in all patients with hypopharyngeal cancer.
True
What is the incidence of a 2nd primary at the time of diagnosis in patients with hypopharyngeal cancer?
5-8%
What are the most common presenting symptoms in patients with tumor of the retromolar trigone?
referred otalgia and trismus
How many year does it take for a former smoker to have the same probability of developing an oral cavity
cancer as a nonsmoker?
16 years
What is the chance that a patient cured of an oral cavity cancer will develop a 2nd primary if they continue
to smoke
40 %
What is the incidence of cervical metastases from base of tongue, tonsil, and soft palate SCCA?
70 %
60 %
40%
What is the incidence of malignancy in adults with asymmetric tonsils with normal-appearing mucosa and
no cervical Iymphad.enopathyl
5%
What percent of T3/T4 tumors of the tonsil can be salvaged after failing primary XRT?
50%
What are the risk factors for developing osteosarcoma in the mandible or maxilla?
History of ionizing radiation, fibrous dysplasia, retinoblastoma, and prior exposure to thorium oxide (radioactive
scanning agent).
What chromosomal abnormality do osteosarcoma and retinoblastoma have in common?
Deletion of the long arm of chromosome 13.
True/False: There is a much lower risk of distant metastases with osteosarcoma of the head and neck than
that of the long bones.
true
What is the most common cause of death in osteosarcoma of the head and neck?
intracranial extension
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.
true
What are the three most common odontogenic tumors
Ameloblastoma,
cementoma,
odontoma.
What are the three most common odontogenic cystsl
Radicular cyst (65%), odontogenic keratocyst, and dentigerous cyst.
What are odontomas composed of
Enamel, dentin, cementum, and pulp.
Where does a radicular or periapical cyst occur?
Along the root of a nonviable tooth, as the liquefied stage of a dental granuloma.
Where do dentigerous cysts develop?
Around the crown of an unerupted, impacted tooth.
Multiple odontogenic keratocyst:a are a manifestation of what syndrome?
Basal cell nevus syndrome.
What is a Pindborg tumor
Calcified epithelial odontogenic tumor that is less aggressive than ameloblastoma and is associated with an
impacted tooth.
Which mandibular tumor or cyst produces white, keratin-containing fluid
Odontogenic keratocyst.
What is the incidence of recurrence after excision of odontogenic keratocystl
62% in the first 5 years.
What percent of parotid gland tumors are benign?
75-80 %
What is the most common site of a salivary gland neoplasma
Parotid gland 73%
What is the least common site of a salivary gland neoplasm
submandibular gland 11%
What is the most common site of a malignant salivary gland neoplasm?
minor salivary glands (60 % of those 40% occur on the palate)
What is the least common site of a malignant salivary gland neoplasm?
Parotid gland 32%
Which salivary gland has the best prognosis for malignant tumors?
Parotid gland
Which salivary gland has the worst prognosis for malignant tumors?
submandibular gland
What is the most common tumor of the parotid gland?
Pleomorphic adenoma in adults
Hemangioma in children
How does metastasizing pleomorphic adenoma differ from carcinoma ex-pleomorphic adenoma?
It is histologically benign, lacking malignant epithelial components.
What is the most common malignant tumor of the parotid gland in adults?
Mucoepidermoid carcinoma
What is the most important prognostic factor for malignant salivary gland neoplasms?
Stage
Which five salivary gland tumors have the worst prognosis?
High-grade mucoepidermoid, adenocarcinoma,
squamous cell carcinoma, undifferentiated carcinoma,
carcinoma ex-pleomorphic adenoma.
What are the indications for postoperative radiation after parotidectomy?
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.
What factors are predictors of occult regional disease in parotid cancer?
Extracapsular extension, preoperative facial paralysis, age >54 years, and perilymphatic invasion
What are the indications for neck dissection in the treatment of salivary gland malignancies?
Clinical metastasis, submandibular tumor, SCCA, undifferentiated carcinoma, size >4 cm, and high-grade
mucoepidermoid carcinoma.
What is the most common site of distant metastasis for adenoid cystic carcinoma?
lungs
What is the most common salivary gland malignancy following radiation?
Mucoepidermoid.
What is the most common malignancy of the submandibular and minor salivary glands?
Adenoid cystic.
What type of tumor comprises 50% of all lacrimal gland neoplasms?
Adenoid cystic.
What are the four types of growth patterns of adenoid cystic carcinoma and which is most common?
Cribriform (most common-looks like Swiss cheese), tubular/ductular, trabecular, and solid.
Which type of radiation therapy does adenoid cystic carcinoma respond best to
Neutron beam
What is the most common salivary gland malignancy to occur bilaterally?
Acinic cell
What are the two most common malignant tumors of the parotid gland in children younger than 12?
Mucoepidermoid is the most common, followed by acinic cell.
What is the incidence of cervical metastasis of mucoepidermoid carcinomas?
30-40%
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?
yes
What is the second most common malignant tumor of the minor salivary glands?
Adenocarcinoma
What are the four types of monomorphic adenomas?
Basal cell
trabecular
canalicular
tubular
Which salivary gland tumor is more common in women with a history of breast cancer?
Mucoepidermoid carcinoma
What is the treatment of choice for metastatic cutaneous SCCA to the parotid?
Total parotidectomy with preservation of VII (unless invaded by tumor) and postoperative radiation therapy to the
parotid area and ipsilateral neck.
What percent of malignant tumors of the parotid gland present with facial nerve weakness or paralysis?
20%
Which salivary gland tumor has a high propensity for perineural invasion?
Adenoid cystic carcinoma
What are the clinical features of salivary duct carcinomas?
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.
Parapharyngeal tumors arising &om the deep lobe of the parotid will involve which compartment?
prestyloid compartment
What happens to the carotid sheath with deep lobe parotid tumors extending into the parapharyngeal space?
It is displaced posteriorly.
Which compartment are neurogenic tumors most likely to arise in?
Poststyloid compartment
What is the most common tumor of the parapharyngeal space?
Pleomorphic adenoma.
What percent of head and neck paragangliomas are familial?
7-10 %
What is the most common paraganglioma of the head and neck?
Carotid body tumor
What percent of carotid body tumors are multicentric?
10%
30-40% in the hereditary form
What is the inheritance pattern of familial carotid body tumors?
Autosomal dominant but only the genes passed from the paternal side are expressed (maternal genomic imprinting).
What is a “nonchromaffin’’ paraganglioma?
one that does not secret significant amounts of catecholamines
What percent of cervical paragangliomas secrete catecholamines ?
5%
What is Shamblin’s dassification system for carotid body tumors
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
What are the two types of temporal bone paragangliomas?
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).
How do glomus tumors differ clinically from carotid body tumors
More common in females, less likely to secrete catecholamines or metastasize, and are more radiosensitive.
What is the primary advantage of stereotactic radiosurgery for treatment of recurrent glomus jugulare
tumors compared with surgery and conventional radiation
lower incidence of cranial nerve injury
When is stereotactic radiosurgery contraindicated in the treaunent of recurrent glomus jugulare tumors?
For larger tumors (> 3.0-4.0 cm).
What is basal cell-nevoid syndrome
Autosomal dominant disorder characterized by multiple basal cell carcinomas, odontogenic keratocysts, rib
abnormalities, palmar and plantar pits, and calcification of the falx cerebri.
What are some other genetic disorders that are associated with a high risk of cutaneous malignancies?
Xeroderma pigmentosum, albinism, epidermodysplastic verruciformis, epidermolysis bullosa dystrophica, and
dyskeratosis congenital.
What is Marjolin’s ulcer
Burn or ulcer associated with the development of malignancy.
What is the most common premalignant skin lesion of the head and neck?
Actinic keratosis.
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
Keratoacanthoma.
What is Bowen’s disease?
Squamous cell carcinoma in situ of the skin.
True/False: Adnexal carcinomas of the skin are very aggressive and have a poor prognosis
true
Which adnexal skin carcinoma ames from a pluripotential basal cell within or around the hair cells?
Merkel cell carcinoma.
What virus is strongly associated with Merkel cell carcinoma that is known to cause cancer in animals?
Merkel cell polyomavirus.
What is the 5-year survival of patients with Merkel cell carcinoma?
30%
What test should be ordered in the workup of Merkel cell carcinoma?
Positron emission tomography scan.
Should the N0 neck be treated in patients with Merkel cell carcinoma?
yes
What is the most common type of skin sarcoma
Malignant fibrous histiocytoma.
What are the five main types of basal cell carcinomas?
nodular cystic superficial multicentric morpheaform keratotic
most common basal cell ca
nodular
basal cell carcinoma commonly found on extremities and trunk
superficial multicentric
Which of these is a variant of nodular basal cell carcinomas and produces pigment?
cystic
basal cell carcinoma commonly resemble scar
morpheaform
most agressive basal cell carcinoma
keratotic
which areas of the face are most susceptible to basal cell carcinomas?
Inner canthus,
philtrum,
mid-lower chin,
nasolabial groove, preauricular area, retroauricular sulcus.
What proportion of incompletely excised basal cell cancers will recur
one third
True/False: squamous cell carcinomas arising in sun-exposed areas tend to behave less aggressively than those
arising de novo
true
What percent of squamous cell carcinoma arising in areas of actinic change metastasize?
3-5 %
What percent of squamous cell carcinoma arising de novo metastasize?
8%
What percent of squamous cell carcinoma arising in areas of scar or chronic inflammation metastasize?
10-30%
What are the five histopathologic types of squamous cell carcinoma?
Generic, adenoid, bowenoid, verrucous, spindle-pleomorphic.
Which of these SSC typically arises in areas of actinic change?
generic
Which of these SSC is more common in the oral mucosa?
Verrucous
Which of SSC is the least common
Spindle-pleomorphic.
What factors increase the likelihood of recurrence for squamous cell carcinoma?
Tumors on the midface, diameter >2 cm or thickness >4 mm, perineural invasion, or regional metastases.
What factors increase the likelihood of regional metastasis of squamous cell carcinoma?
Tumors arising on the ear, diameter >2 cm or >4 mm thickness, poorly differentiated histology, and recurrent tumors.
What are the four types of melanoma
Superficial spreading, lentigo maligna, acrallentiginous, and nodular sclerosing.
most common type of melanoma
superficial spreading
best prognosis type of melanoma
superficial spreading
What is the most common form of hereditary cutaneous melanoma?
Dysplastic nevus syndrome.
Which type of melanoma occurs on palms, soles, nail beds, and mucous membranes
Acral lentiginous melanoma.
What percent of melanomas occur in the head and neck?
20%
What percent of tumors (melanomas) are not pigmented (amelanotic)?
5%
What mutation has been found in more than half of malignant melanomaa?
BRAF somatic missense mutations; a single substitution (V599E) accounts for 80% of these.
What cells are melanomas composed of
Melanocytes, which are derived from neural crest cells.
What are the risk factors for developing melanoma?
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.
What is the risk of melanomatous transformation of giant congenital nevi?
14%
What percent of patients with xeroderma pigmentosa develop melanoma?
3%
How should a lesion suspicious for melanoma be biopsied?
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.
What is the most important prognostic factor of melanomas?
depth of invasion
What is the incidence of nodal metastases if the depth of the tumor is >4.0 mm
> 70%
What is the incidence of nodal metastases if the depth of the tumor is <1.5 mm
8%
True/False: Women with melanoma have a better prognosis than men regardless of tumor depth.
True.
What tumor factor, other than depth, influences regional metastasis in melanoma?
ulceration
Involvement of which areas of the body also increases the risk of metastases (of melanoma)?
BANS: back, arms, neck, and scalp.
What is the chance that a patient with melanoma will develop a second melanoma?
5%
Is melanoma radiosensitive?
It may be sensitive to large dose fractions (600 cGy) but not to standard fractionation radiotherapy (180-200 cGy).
What is the role of large-dose fraction radiotherapy in the management of melanoma?
Decreases incidence of locoregional recurrence among NO patients.
What is the risk of developing esophageal cancer in patients who smoke and drink compared with those who do not
100 times higher
In which areas of the world is the incidence of esophageal cancer highest
Middle East, southern and eastern Africa, and northern China.
What are the risk factors for developing esophageal cancer?
Tobacco, alcohol, achalasia, Plummer-Vinson syndrome, prior head and neck cancer, tylosis, and Barrett’s disease.
What are the clinical features of Plummer-Vinson syndrome
Iron-deficiency anemia, upper esophageal web, hypothyroidism, glossitis/cheilitis, gastritis, and dysphagia.
In patients with Plummer-Vinson syndrome, where is squamous cell carcinoma of the esophagus most likely
to occur?
Postcricoid area.
Metaplasia of the distal esophagus is otherwise known as what
Barrets eosphagus
What percent of people with gastroesophageal reflux disease have Barrett’s esophagus and what percent of these people will develop adenocarcinoma?
5%
5-10%
Cancer of the cervical esophagus is usually what typ
SCC
What is the usual cause of death from esophageal cancer?
aspiration pneumonia
When do patients with synovial sarcoma usually present?
between age of 25-36%
Where are most synovial sarcomas of the head and neck located?
hypopharynx and parapharyngeal space
What is the incidence of regional metastasis in synovial sarcomas of the head and neck
12.5%
What is the most common cause of death from synovial sarcoma of the head and neck
lung metastises
What is the primary mode of treatment for synovial sarcoma of the head and neck?
wide surgical excision and postoperative radiation therapy
5 years survival rate of synovial sarcoma
40-50%
What prognostic significance does the presence of microcalcifications have in synovia sarcoma?
better prognosis
Nasopharyngeal cancer accounts for what percent of all cancers diagnosed in the Kwangtung province of
southern China?
20%
What is the incidence of nasopharyngeal cancer among native-born Chinese compared with that among
Caucasians?
118 times higher
What EBV product is likely to play a role in malignant transformation of nasopharyngeal epithelium
Latent membrane protein (LMP-1).
What environmental factor is most strongly linked to nasopharyngeal carcinoma?
Frequent consumption of dried salted fish.
What is the most common site of origin of nasopharyngeal cancer
Fossa of rosenmuller
Which nodal groups does naaopharyngeal cancer spread to
retropharyngeal nodes of rouviere
juggulodigastic nodes
spinal accessory chain
In the staging system desaibed by Ho, poorer prognosis is associated with cervical metastases to which area of the neck
Inferior to a plane spanning from the contralateral sternal head of the clavicle to the ipsilateral superir margin of the trapezius muscle
What is the incidence of skull base erosion in patients with nasopharyngeal carcinoma
25%
What is the most common site of distant metastases (nasopharyngeal ca)
Bones
Smooth, submucosal nasopharyngeal masses located in the midline are most often what
Embryologic remnants
Thornwald’s cysts, pharyngeal bursa remnants
What factors, described by Ho and Neel, are regarded as important adverse prognostic indicators in patients
with nasopharyngeal carcinoma?
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.
Extension into which space is associated with the worst prognosis in patients with nasopharyngeal
carcinoma
anterior masticator space
What is the primary treatment modality for nasopharyngeal cancer?
Radiation therapy to the nasopharynx (66-70 Gy) and neck (60 Gy).
Why is the clinically negative neck treated (nasopharynx)?
Studies have shown improved local control and disease-free survival for prophylactic irradiation of the clinically
negative neck in patients with nasopharyngeal carcinoma.
What are the complications from radiation overdosage in the treatment of nasopharyngeal carcinoma?
Osteoradionecrosis brain necrosis transverse myelitis hearing loss hypopituitraism hypothyroidism optic neuritis
What is the role of induction chemotherapy for treatment of nasopharyngeal carcinoma?
No survival advantage has been proven.
What is the standard treatment protocol for stage III and IV nasopharyngeal carcinoma?
Concomitant cisplatin and XRT followed by adjuvant chemotherapy with cisplatin and 5-FU.
How does treatment failure usually manifest in nasopharyngeal carcinoma?
disease at both primary site and cervical lymph nodes
What is the most common site of recurrent/persistent nasopharyngeal carcinoma?
Lateral wall of the nasopharynx.
What are the treatment options for recurrent/persistent nasopharyngeal carcinoma at the primary site?
Reirradiation with larger therapeutic dose than initial treatment; stereotactic radiotherapy; brachytherapy with split
palate implantation of radioactive gold grains; surgical resection.
What is the most common benign sinonasal neoplasm?
Inverting papiloma
What is the most common malignant sinonasal neoplasm?
SSC 80%
What is the 2nd most common malignant sinonasal neoplasm?
Adenocarcinoma
What are the most common locations of sinonasal squamous cell carcinoma?
Maxillary sinus followed by nasal cavity then ethmoid sinuses
Are elective neck dissections warranted in patients with sinonasal squamous cell carcinoma?
No
incidence of occult cervical lymph nodes metastases is 10%
What percentage of sinonasal tumors can be attributed to occupational exposures?
Where are they usually located ?
up to 44%
Lateral nasal wall, adjacent to the middle turbinate
Which substances are thought to predispose to sinonasal neoplasms?
Nickel, chromium, isopropyl oils, volatile hydrocarbons, organic fibers from wood, shoe, and textile refineries
Which substance exposure classically associated with SCCA?
Nickel
Which substance exposure classically associated with adenocarcinoma?
Hardwood dust and leather tanning substances
Which virus is thought to play a role in the etiology of sinonasal tumors?
Human papilloma virus (HPV), particularly typres 6 and 12
True/False: Smoking by itself is not a significant etiologic factor for sinonasal tumors.
True
Which nasal masses should not be biopsied in the clinic?
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.
What are the three subtypes of Schneiderian papillomas?
Fungiform
Inverting
Cylindrical
Where do inverting papillomas most commonly arise?
Lateral nasal wall
What factor is most related to the chance of recurrence for inverting papilloma?
Method of removal
What is the incidence of recurrence after resection of inverting papilloma via lateral rhinotomy/medial
maxillectomyl
13-15%
In patients who undergo resection of inftrting papilloma via lateral rhinotomy/medial maxillectomy, what is
the most important factor related to risk for recurrence?
Mitotic index
What is the differential diagnosis of a small cell sinonasal tumor?
Esthesioneuroblastoma, plasmacytoma,
melanoma,
lymphoma,
sarcoma,
poorly differentiated squamous cell carcinoma,
Ewing’s sarcoma,
peripheral neuroectodermal tumor (PNET),
SNUC (sinonasal undifferentiated carcinoma).
What is a SNUC?
Sinonasal undifferentiated carcinoma
very aggrissive small cell tumor
In what age group is olfactory neuroblastoma typically seenl
Bimodal distribution-people in their 20s and 50s.
Kadish system of classification of neuroblastoma
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
What are the three most common malignant bone tumors of the paranasal sinuses?
Multiple myeloma, osteogenic sarcoma, chondrosarcoma.
What is the pathophysiology of fibrous dysplasia?
Normal medullary bone is replaced by collagen, fibroblasts, and osteoid.
Where is fibrous dysplasia most commonly found in the head and neck
maxilla
Where is adenoid cystic carcinoma of the head and neck most commonly found
Palate, followed by major salivary glands, then paranasal sinuses.
Where is melanoma most commonly found in the nose and paranasal sinuses
Nasal septum
How does nasal melanoma differ from cutaneous melanoma
More aggressive with a worse prognosis and an unpredictable course-local recurrence is the most common cause
of failure.
What is the most common type of lymphoma of the nose and paranasal sinuses~
Non-Hodgkin’s lumphoma
What is Ohngren’s line and how is it significant?
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).
What tumor Comprises only 3% of Schneiderian papillomas
Cylindrical papiloma
What tumor is Most common type of Schneiderian papilloma, typically seen on the nasal septum
Fungiform papiloma
2-13% of these benign sinonasal tumors have malignant potential
inverting papillomas
Has a predilection for the mandible and a sunray appearance on X-ray
Osteogenic sarcoma
More than 90% will have invaded through at least one wall of the
involved sinus at presentation
SCC
Benign tumor, most commonly seen in patients less than 20 years old
and has a ground glass appearance on X-ray
Fibrous dysplasia
Benign tumor most commonly found in the frontal sinus
Osteoma
Encapsulated, benign tumor that arises from the surface of nerve fibers
Neurilemoma
Unencapsulated tumor that arises from within a nerve; 15% become malignant (when associated with von Recklinghausen’s disease}
Neurofibroma
Second most common malignant sinonasal tumor; tend to be located superior to Ohngren’s line
Adenocarcinoma
Arise from pericytes of Zimmerman and considered neither benign nor malignant
Hemangiopericytoma
Arise from stem cells of neural crest origin that differentiate into
olfactory sensory cells; Homer Wright rosettes are characteristic
Olfactory neuroblastoma or
esthesioneuroblastoma
Most common tumor to metastasize to the sinonasal area
Renal cell
Well-circumscribed, mobile, painless benign lesion most commonly found on the tongue that has malignant potential and histopathology shows polygonal cells with abundant eosinophils
Granular cell tumor
Metastasizes to the brain more frequently than any other soft-tissue
sarcoma
Alveolar soft part sarcoma
Which sinonasal neoplasnm remodel rather than erode bone?
Sarcomas, minor salivary gland carcinomas, hernangiopericytomas, extramedullary plasmacytomas, large cell lymphomas, and olfactory neuroblastomas.
What is the primary modality of treatment for extramedullary plasmacytomas?
Radiation
After benign lymphoid hyperplasia, what is the most common benign nasopharyngeal tumor?
Juvenile nasopharyngeal angiofibroma (JNA)
From which site in the nasopharynx does this tumor develop?
Trifurcation of the palatine bone,
horizontal ala of the vomer, and the root of the pterygoid process.
Where does the main blood supply to these tumors (Juvenile nasopharyngeal angiofibroma) most often come from?
Internal maxillary artery or the ascending pharyngeal artery.
What is the second leading cause of death among children ages 1-14?
Cancer
What is the most common solid malignant tumor in infants < 1 year?
Neuroblastoma
What are the precursor cells of neuroblastoma?
Neural crest cells
What is the survival rate after complete excision of lesions in children < 1
90%
What is the most common head and neck tumor of children?
Lymphoma
In what age groups is Hodgkin’s lymphoma most common
Bimodal peak incidence, with one peak in the 15- to 34-year-old age group and another in later adulthood.
What percent of Hodgkin’s lymphoma cases are associated with EBV?
40%
True/False: Axillary, inguinal, and Waldeyer’s ring involvement is uncommon in patients with Hodgkin’s
lymphoma.
true
Which lymphoma accounts for 50% of childhood malignancies in equatorial Africa
Burkitt’s lymphoma
What is the most common soft tissue sarcoma of the head and neck in childrern
Rhabdomyosarcoma
In what age groups is rhabdomyosarcoma most common?
Ages 2-5 and 15-19.
What is the treatment for rhabdomyosarcoma?
Multirnodality; primary chemoradiation followed by surgery for recurrent or residual disease.
Involvement in which area of the head and neck by rhabdomyosarcoma has the best prognosis
orbit
What is the most common type of well-differentiated thyroid carcinoma in children
Papillary
What is the most common benign neoplasm of the larynx in children?
papillomas
What percent of patients with recurrent respiratory papillomatosis (RRP) devdop distal tracheal and
pulmonary spread of papillomas
5%
What percent of patients with RRP ( recurrent respiratory papillomatosis) require tracheostomy
15%
What percent of patients with distal spread have had a previous tracheostomy
95%
What is the incidence of stomal papilloma recurrence rate after tracheostomy for RRP( recurrent respiratory papilomatosis) ?
> 50%
What are the most common subtypes of HPV isolated from RRP
6 and 11
found in >95%
What are the most common respiratory complications of distal RRP
Pneumatocele, abscess, and tracheal stenosis
Why is aspergillus infection a risk factor for ear and temporal bone tumors?
It produces aflatoxin B, a known carcinogen.
What are other risk factors for development of ear and temporal bone tumors?
History of radiation to the head and neck, chronic chromate burns secondary to using matchsticks to clean
the ear canal.
What is the most common site of ear and temporal bone tumors?
External auditory canal (EAC).
What is the most common route of spread of tumors in the cartilaginous portion of the EAC
Through the fissures of Santorini.
What is the most common histologic type of tumor involving the EAC or middle ear
SCC
Where do most basal cell carcinomas of the EAC arise?
Concha
What is the most common tumor of glandular origin to involve the EAC or middle ear?
Adenoid cystic carcinoma
What are the most common types of sarcoma of the temporal bone?
Rhabdomyosarcoma, chondrosarcoma, and osteosarcoma.
What are the most common sites of origin of metastatic tumors of the temporal bone?
Breast, lung, and kidney
Tumors that metastasize to the temporal bone hematogenously most often involve which area of the
temporal bone?
Petrous apex
Tumors that metastasize to the temporal bone via the meninges most often traverse what structure?
IAC internal auditory canal
What is the most common presentation of tumors of the EAC
Unremitting pain and serosanguinous otorrhea
What percent of patients with a tumor in the EAC will present with cervical metastases?
10%
What percent of patients with a tumor in the middle ear will present with facial nerve palsy?
20-40%
What is the most common tumor of the cerebellopontine angle (CPA)?
Vestibular schwannoma
What is the differential diagnosis of a CPA tumor?
Schwannoma, meningioma, epidermoid, lipoma, arachnoid cyst, cholesterol granuloma.
What is the incidence of patients with vestibular schwannomas who have normal hearing at presentation?
5%
What is the nature of vertigo in the majority of patients with a vestibular schwannoma
Chronic disequilibrium with self-limiting episodes of vertigo
What is the most wmmon type of hearing loss in patients with a vestibular schwannoma
High-frequency unilateral SNHL.
What is a typical word discrimination score in a patient with a vestibular sc:hwannoma
0-30% in >50% of patients with acustic neuroma
Adenocarcinoma of the endolymphatic sac is more common in patients with what disease
Von Hippel-Lindau disease.
What percent of thyroid cancers are well differentiated
95%
What percent of thyroid nodules are malignant?
< 5%
What is the most common thyroid nodule?
follicular adenoma
What is the significance of age with thyroid nodules?
More likely to be malignant in women over 50 and men over 40 and in both men and women under 20.
What percent of solitary thyroid nodules in children are malignant?
50%
What is the significance of size with thyroid nodules?
More likely to be malignant if >4 cm in diameter.
What are the three types of well-differentiated thyroid malignancies?
Follicular, papillary, and Hurthle cell.
Which of these well differentiated thyroid malignancies is associated with iodine deficiency?
papillary
well differentiated thyroid malignancies relatively unresponsive to ablation with radioactive iodine
Hurthle cell
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?
Total thyroidectomy and modified radical neck dissection
What factor best correlates with the presence of lymph node metastases in papillary carcinoma?
Age
True/False: Microscopic lymph node involvement does not change the long-term survival in patients with
papillary thyroid cancer.
true
What is the incidence of multicentric disease on pathological examination of the entire thyroid in patients
with papillary carcinoma (> 1 cm)
70-80%
What histological subtypes of thyroid tumors are associated with an increased risk of local recurrence and
metastasis
Tall cell, columnar, insular, solid variant, and poorly differentiated.
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
no
What is the most common site of metastasis from follicular thyroid cancer?
bone
How is the definitive diagnosis of follicular thyroid cancer made?
By demonstration of capsular invasion at the interface of the tumor and the thyroid gland
What is the most important prognostic indicator of follicular thyroid cancer?
Degree of angioinvasion
True/False: Follicular cell carcinoma is more aggressive than Hurthle cell.
false
What are the indications for adjuvant thyroid hormone in patients with well-differentiated thyroid
carcinoma?
All patients with well-differentiated carcinoma should be treated with thyroid hormone to suppress TSH for life,
regardless of the extent of their surgery.
In what four settings does medullary thyroid carcinoma (MTC) arise
Sporadic, familial, and in association with multiple endocrine neoplasia Ila or lIb.
Medullary thyroid ca - best prognosis
familial
Medullary thyroid ca - tends to occur unilaterally
sporadic
Medullary thyroid ca - worst prognosis
sporadic
What percentage of MTC occurs sporadically?
70-80 %
What are the characteristics of familial MTC?
Autosomal dominant inheritance pattern; not associated with any other endocrinopathies
What other disorders are present in patients with MEN Ila?
Pheochromocytoma, parathyroid hyperplasia
True/False: All patients with MEN IIa will have MTC.
true
What other disorders are present in patients with MEN lIb
Pheochromocytoma, multiple mucosal neuromas, marfanoid body habitus.
What is the surgical treatment for MTC?
Total thyroidectomy with central node dissection, lateral cervical lymph node sampling of palpable nodes, and a
modified radical neck dissection, if positive.
What are the two types of anaplastic thyroid cancer?
Which is more common?
Which of these is usually responsive to radiation therapy?
Large cell and small cell.
Large cell - more common
small cell responsive to radiation
What is the appropriate management for a patient with an anaplastic thyroid carcinoma?
Debulking and tracheostomy may be performed for palliation of airway obstruction.
What is the beat treatment for primary non-Hodgkin’s lymphoma of the thyroid gland
Chemoradiation.
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?
Total thyroid lobectomy with isthmusectomy should be considered because there is an increased chance of
malignancy in large cysts.
A 56-year-old man with no risk factors presents with a thyroid nodule. The FNA is nondiagnostic. What is
the treatment of choice?
Total thyroid lobectomy with isthmusectomy.
What are the indications for postoperative radioiodine ablation therapy in thyroid
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.
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.
true
Which medication improves quality of life when preparing patients for radioiodine scanning and ablation
therapyl
Recombinant TSH stimulation (rTSH).
How are patients with MTC managed postoperatively?
Receive L-thyroxine and 2 weeks of calcium and vitamin D supplementation; serial measurements of calcitonin and
CEA.
Severe cerebellar symptoms with a normal MRI suggests what condition?
Paraneoplastic cerebellar degeneration
What are the two primary subtypes of paraneoplastic cerebellar degeneration?
Vestibulocerebellar syndrome and opsoclonus-myoclonus syndrome.
Which malignancies most commonly cause paraneoplastic cerebellar degeneration?
In adults, ovarian, uterine, breast, and small cell lung cancer.
In children, neuroblastoma.
