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– Combination regimens commonly used (no signicant improvement com-
pared with DTIC alone)
Cisplatin, vinblastine, DTIC
Cisplatin, DTIC, carmustine, tamoxifen
• Melanoma of unknown primary prognosis: survival similar to stage III disease
(55 and 44%, 5- and 10-year survival). Treatment should include aggressive surgical approach and consider for adjuvant therapy.
K. Wong et al.
Temporal Bone Malignancies
Overview
• Rare, <0.2% of all H&N tumors
• Most commonly arise from pinna and lateral concha (BCC, SCC), medial
spread to EAC
• SCC=most common primary EAC tumor; “meaty” or polypoid lesions
Presentation
• Symptoms
– Chronic otalgia (80–85%), otorrhea (40–75%)
– Hearing loss (45–80%), tinnitus (8–10%)
CHL from canal obstruction.
SNHL and vertigo→labyrinthine involvement, aggressive lesions
– Cranial nerve paresis (30%)
CN V, IX, XI
– Parotid mass (19%)
– Others: vertigo, auricular lesion, external canal mass, skin lesions
Etiology/Pathogenesis
• Squamous cell carcinoma is the most common TB malignancy
– Commonly well differentiated

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• Chronic otitis media and cholesteatoma common
• Chronic suppurative otitis media→squamous metaplasia
• History of RT
• Other TB carcinomas
– Basal cell carcinoma: second most common, ulceration, absence of pearly
edges (thin, adherent canal epithelium)
– Melanoma: third most common, arising from auricle or EAC
– Rhabdomyosarcoma: most common pediatric TB malignancy; 10% of all
rhabdomyosarcomas occur in ear
– Adenoid cystic: rare in TB; epithelium-covered, “small pimple,” signi-
cant pain
– Endolymphatic sac papillary tumor (aka Heffner’s tumor): hearing loss, cra-
nial nerve decits
Usually isolated, 11–30% associated with von Hippel-Lindau disease
– Others: adenocarcinoma (ceruminous), giant cell tumor, chondrosarcoma,
osteosarcoma, verrucous carcinoma
Diagnosis
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• History: prolonged otalgia, recurrent/persistent ear infections, h/o
cholesteatoma
• Physical exam:
– Complete H&N exam including otoscopy and tuning fork
– Inspection of pinna, EAC, middle ear
– Cranial nerve exam
– Brown sign: blanching middle ear mass with application of pressure suggests
glomus tumor
• Audiogram
• High-resolution CT temporal bone with contrast
– Soft tissue detail, bony erosion, medial extension
• Metastatic work-up
– CT Chest, abdomen, pelvis
– PET/CT (rule out lung metastasis for SCC)
• Carotid angiography
– If carotid involvement suspected
• Balloon occlusion test

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K. Wong et al.
Staging
University ofPittsburgh Staging forSquamous Cell Carcinoma
oftheTemporal Bone
Primary tumor (T)
T1: Tumor limited to EAC without bony erosion or soft tissue involvement
T2: Bony erosion limited to EAC (not full thickness), limited soft tissue involvement (<0.5cm)
T3: Bony erosion through EAC (full thickness), limited soft tissue involvement (<0.5cm), or
involving middle ear, mastoid, or both
T4: Tumor erosion into cochlea, petrous apex, medial wall of middle ear, carotid canal, jugular
foramen of dura, extensive soft tissue involvement (>0.5cm) (e.g., TMJ, stylomastoid
foramen), or facial paresis
Regional lymph nodes (N)
N1: Single ipsilateral lymph node ≤3cm in greatest dimension
N2: Single ipsilateral lymph node >3cm but ≤6cm in greatest dimension
N2b: Multiple ipsilateral lymph nodes, ≤ 6cm in greatest dimension
N2c: Bilateral or contralateral lymph nodes ≤6cm in greatest dimension
N3: Lymph node >6cm in greatest dimension
Distant metastasis (M)
Mx: Distant metastasis cannot be assessed
M0: No distant metastasis
M1: Distant metastasis present
Final staging
Stage 0 Tis, N0, M0
Stage I T1, N0, M0
Stage II T2, N0, M0
Stage III T3, N0, M0
T1, N1, M0
T2, N1, M0
T3, N1, M0
Stage IV T4, N0, M0
T4, N1, M0
Any T, N2, M0
Any T, N3, M0
Any T, any N, M1
T4a, N2, M0
Stage IVB Any T, N3, M0
T4b, any N, M0
Stage IVC Any T, any N, M1

24 Cutaneous andTemporal Bone Malignancies
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Treatment forSquamous Cell Carcinoma
oftheTemporal Bone
• Dependent on location of carcinoma: cartilaginous vs. bony
• Late ndings/poor prognosis = parotid mass, CN palsies, lymphadenopathy (LAD)
• Sleeve resection
– T1 tumors, limited to cartilaginous canal
– Adjunct RT if bony, cartilaginous, or soft tissue invasion
• Lateral temporal bone (LTB) resection
– Resection includes entire EAC, TM, malleus, and incus
– Indicated for early-stage (T1 and T2) tumors, tumor involvement, or abutment
against osseous EAC
– Adjunct RT if bony, cartilaginous, or soft tissue invasion
• Subtotal temporal bone (STTB) resection
– Resection includes EAC, TM, middle ear contents, mastoid, otic capsule, and
medial wall of the middle ear
– Indicated for tumors involving or abutting osseous EAC with mesotympanic
extension, <1cm dural involvement
– LTB can be combined with subtotal petrosectomy
• Total temporal bone (TTB) resection
– Resection includes EAC, TM, middle ear contents, mastoid, otic capsule,
medial wall of the middle ear, petrous apex, and neurovascular bundle
– Indicated for tumors extending beyond the labyrinth and cochlea and into the
petrous apex (extremely rare)
• Radiation
– Adjuvant RT
All T3 and T4, or if indicated by pathologic behavior (perineural invasion,
LN mets, extracapsular spread)
50–60Gy (T3 and T4, consider for T2)
– Palliative RT
Indications: internal carotid artery encasement, petrous apex extension,
>1cm dural involvement, intraparenchymal invasion
– Outcomes of radical surgery and postoperative XRT for SCC of TB
Stage I–II: 100%
Stage III: 100%
Stage IV: 34.3%

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K. Wong et al.
Overall for entire series: 43.2%
Node positive, poorly differentiated, brain involvement, and salvage surgery=poorer outcome
Improved survival in de novo therapy vs. salvage surgery
• Chemotherapy
– Consider preoperative chemotherapy in borderline resectable tumors
– Postoperative treatment given concurrently as radiosensitizer during
adjuvant RT
– Retrospective series
50% of patients treated with 5-FU or uoropyrimidine complex during
external beam radiation (40Gy) were disease-free at 24–47months
Further Reading
1. Amin MB, etal. AJCC cancer staging manual. 8th ed. Springer: American Joint Commission
on Cancer; 2017.
2. Barona CG, Frank RG, Ruzicka T, Megahed M, Tebbs V, Owens M, Stampone P, Gollnick
H. Recurrence rate of supercial basal cell carcinoma following successful treatment with
imiquimod 5% cream: interim 2-year results from an ongoing 5-year follow-up study in
Europe. Eur J Dermatol. 2005;15:374–81.
3. Berner A. Actinic keratosis and development of squamous cell carcinoma of the skin. J
Norwegian Med Assoc. 2005;125:1653–4.
4. D’Sousa J, Clark J.Management of the neck in metastatic cutaneous squamous cell carcinoma
of the head and neck. Curr Opin Otolaryngol Head Neck Surg. 2011;19(2):99–105.
5. Cohen LM. Lentigo maligna and lentigo maligna melanoma. J Am Acad Dermatol.
1995;33(6):923–40.
6. Margoob A, Keonig K, Bittencourt F, Kopf A, Bart R. Breslow thickness and clark level in
melanoma: support for including level in pathology reports and in American Joint Committee
on Cancer Staging. Cancer. 2000;88(3):589–95.
7. Flaherty KT, Fecher LA.Where are we with adjuvant therapy of stage III and IV melanoma in
2009? J Natl Compr Cancer Netw. 2009;7(3):295–304.
8. Gidley PW.Managing malignancies of external auditory canal. Expert Rev Anticancer Ther.
2009;9(9):1277–82.
9. Goh YH, Chan YM, Chong VF, Low WK, Lim LH.Malignancy of the temporal bone and
external auditory canal. Otolaryngol Head Neck Surg. 2000;122(6):882–6.
10. Walvekar RR, Arriaga MA, Dileo MD, Nuss DW, Pou AM, Hagan J, Lin J, Gaudet
JE.Applicability of the Pittsburgh staging system for advanced cutaneous malignancy of the
temporal bone. Skull Base. 2010;20(6):409–14.

Chapter 25
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Odontogenic Cysts andTumors
RobertN.Sharobiem, ToddR.Wentland, BrettA.Miles,
andMohemmedKhan
Pearls
• Dentigerous cysts are the most common odontogenic cysts and arise from the
dental follicle.
• Keratocysts have a high rate of recurrence and most commonly occur in the
mandible.
• Ameloblastomas are the most common of odontogenic tumors.
Odontogenic Cysts
• All odontogenic cysts consist of a central lumen, an epithelial lining of odonto-
genic origin, and a connective tissue wall.
R. N. Sharobiem
Advanced Dentistry of Alhambra, Alhambra, CA, USA
T. R. Wentland (*)
Department of Oral and Maxillofacial Surgery, John Peter Smith Hospital,
Ft. Worth, TX, USA
B. A. Miles
Otolaryngology Head and Neck Surgery, Oral and Maxillofacial Surgery, Northwell Health
System, New Hyde Park, NY, USA
e-mail: bmiles4@northwell.edu
M. Khan
Department of Otolaryngology - Head and Neck Surgery, Icahn School of Medicine at the
Mount Sinai Hospital, New York, NY, USA
e-mail: mohemmed.khan@mountsinai.org
© Springer Nature Switzerland AG 2023
F. Y. Lin, Z. M. Patel (eds.), ENT Board Prep,
https://doi.org/10.1007/978-3-031-26048-3_25
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R. N. Sharobiem et al.
Dentigerous Cyst
• Clinical features
– Most common developmental odontogenic cyst.
– Peak incidence during the teenage years and 20s, male predilection of 1.6:1.
– Most common in posterior mandible or maxilla and usually associated with
third molars. Other common teeth are maxillary canines and mandibular sec-
ond premolars.
– Arises from the dental follicle of an unerupted tooth.
– May present as an incidental nding or as an asymptomatic bony expansion
as these can be as large as 15cm causing facial asymmetry. These cysts are
thought to enlarge due to increased osmotic pressure in their lumen.
– Asymptomatic unless secondarily infected.
• Radiographic features
– Well-dened, unilocular radiolucency associated with the crown of an
unerupted tooth. The tooth may be signicantly displaced by cyst expansion.
• Histopathologic features
– Surrounds the crown of a tooth and is attached at the cemento-enamel junction.
– Grossly there may brownish uid or semisolid cystic material.
– The epithelial cyst lining resembles reduced enamel epithelium and has two
to three rows of cuboidal or attened nonkeratinizing cells.
– Cholesterol clefts may be seen.
• Treatment and prognosis
– Enucleation is the treatment of choice and no recurrence is expected.
– Marsupialization is an option for larger cysts. Consider marsupialization
when it will allow the tooth to erupt or when surgical removal presents a risk
to damaging developing teeth, the inferior alveolar (IA) nerve, or other
structures.
– Prognosis excellent, but malignant transformation of lining has been
reported 1–2%.
Odontogenic Keratocyst
• Clinical features
– Twice as common in the mandible and more common in posterior body/
ramus region.
– Most aggressive of all odontogenic cysts with high rates of recurrence.

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– Primordial origin—60% arise from dental lamina rests or from the basal cells
of oral epithelium.
– Dentigerous origin—40% arise from reduced enamel epithelium of the dental
follicle.
– Tumors extend anteroposteriorly as they progress through medullary bone
although cortical expansion and perforation may be observed. Usually does
not inltrate soft tissues.
– Associated with the nevoid basal cell carcinoma (Gorlin) syndrome—multi-
ple basal cell carcinomas of the skin, multiple odontogenic keratocysts, intra-
cranial calcications, epidermal cysts of the skin, palmar/plantar pits, enlarged
head circumference, hypertelorism, and rib and vertebral anomalies.
Prevalence is 1:60,000.
– Malignant transformation possible but exceedingly rare.
• Radiographic features
– May present as a well-dened, small or large unilocular radiolucency or as a
multilocular radiolucency. Those of dentigerous origin are associated with
a tooth.
• Histopathologic features
– Epithelial lining is 6–8 cells thick of stratied squamous epithelium.
– Presence of thin brous wall which may have epithelial islands, cysts, or
cords with central keratinization and cyst formation. These are daughter or
satellite cysts and are present in 7–26% of cases. Some authors regard these
as the source of recurrence.
– The cyst lumen contains varied amount of keratinaceous debris that grossly
appears as a caseous/cheesy material.
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• Treatment and prognosis
– Treatment options include enucleation and curettage, marsupialization, and
resection.
– Enucleation is the best option for small lesions, especially if cyst can be
removed without rupturing the lining. If unable, then curettage of bony cavity
is necessary. Curettage may be physical with a rotary bur (peripheral ostec-
tomy), hypothermal with cryotherapy, or chemical with Carnoy’s solution
(xative composed of 60% ethanol, 30% chloroform, and 10% glacial acetic
acid, also can include ferric chloride).
– Marsupialization can bring an associated tooth into functional position or
when surgical removal presents a risk to damaging developing teeth, the IA
nerve, or other vital structures. This is also thought to thicken the cyst lining
making subsequent removal easier.
– Resection is indicated if there have been multiple recurrences after enucle-
ation and curettage. Resection can be subperiosteal as there is generally no
soft tissue invasion and osseous margin should be 1.0cm. Involved soft tissue,
such as site of initial biopsy, should be excised with specimen.

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– Recurrences occur in 5–62% of cases and are either due to failure to remove
all original cyst lining or a new primary cyst formation from activated rests or
oral basal epithelium. Most recur within 5 years, but may recur later than
10 years.
R. N. Sharobiem et al.
Calcifying Odontogenic Cyst
• Clinical features
– Also known as a Gorlin’s cyst. Asymptomatic jaw expansion and usually an
incidental radiographic nding.
– Average size is about 3.0cm, but can be as large as 12.0cm.
– The more inltrative or even malignant neoplasms are referred to as dentino-
genic ghost cell tumors and occur in older patients.
– Extraosseous calcifying odontogenic cysts (COCs)—25% of all COCs occur
anterior to rst molar in people older than 50 years. Appear on interdental
papilla or alveolar mucosa as a rm, soft tissue mass. Also may show calci-
cations on radiographs.
• Radiographic features
– Early on, the cysts are completely radiolucent, but as they mature develop
calcications and are well-dened, mixed radiolucent-radiopaque lesions.
– Three patterns of radiopacity: salt-and-pepper pattern of ecks, uffy cloud
appearance, and a crescent-shaped pattern on one side of the radiolucency
appearing moon-shaped. Radiopacities are present in 33–50% of cases.
– One-third of cases are associated with an unerupted tooth, with the canine
being most common. Root resorption or divergence is commonly seen.
• Histopathologic features
– Unilocular cysts with a distinct odontogenic, ameloblast-like basal cell lining
consisting of cuboidal to columnar cells with hyperchromatic nuclei, which
may show reverse polarization away from the basal membrane. These cells
are loose in arrangement (similar to stellate reticulum) and include the pres-
ence of ghost cells, which are eosinophilic cells with degenerated nuclei (only
a clear space remains).
• Treatment and prognosis
– Enucleation and curettage are curative and they rarely recur.
– Ameloblastomas may have ghost cell differentiation and should be treated as
an ameloblastoma as they have no relation to COC.
– Malignant odontogenic ghost cell carcinomas, although rare, can occur and
have a 5-year survival rate of 73%.

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Glandular Odontogenic Cyst
• Clinical features
– Middle-aged adults with a mean age of 48 years, 75% occur in the mandible.
Predilection for anterior region of jaw and may cross the midline.
– May present as a small asymptomatic lesion or a large destructive lesion with
clinical expansion, pain, and paresthesia.
• Radiographic features
– Well-dened, unilocular or multilocular, radiolucent lesions especially in the
anterior mandible.
• Histopathologic features
– Multilocular with stratied squamous epithelial lining of varying thickness
and a at epithelium–connective tissue interface. The epithelium has a dis-
tinctive surface layer of cuboidal to columnar cells with eosinophilic cyto-
plasm and cystic spaces.
• Treatment and prognosis
– Enucleation and curettage are treatment, but recurrence rates are as
high as 30%.
– Higher recurrences in multilocular lesions and some authors advocate for en
bloc resection, especially of multilocular lesions.
– Surveillance is recommended to follow up for malignant transformation,
including low-grade mucoepidermoid carcinoma, which has been misdiag-
nosed as a glandular odontogenic cyst (GOC).
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Odontogenic Tumors: Odontogenic Epithelium
• Ameloblastoma
– Most common true odontogenic benign neoplasm.
Invasive Ameloblastoma: Conventional Solid/Multicystic
Ameloblastoma (86%)
• Clinical features
– Asymptomatic expansion of the jaw—mandible 75–80%, maxilla 20–25%.
Posterior third molar-ascending ramus region most common.
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