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ABOMS Pathology (Johnston) > Benign Nonodontogenic Tumors > Flashcards

Benign Nonodontogenic Tumors Flashcards

1
Q

CGCL Typical Age

A

1st to 2nd decade

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2
Q

What are two types of CGCL?

A

Nonaggresive Aggressive

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3
Q

Which typically has aggressive CGCL? Older or younger?

A

Younger

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4
Q

What are diagnosis criteria for aggressive CGCL

A

At least 1 major - >5cm - Recurrent At least 3 minor -Rapid Growth -Teeth loose, resorbed, displaced -Cortical perforation or thinning

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5
Q

What are symptoms for nonaggresive CGCL?

A

Asymptomatic

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6
Q

What are symptoms of aggressive CGCL?

A

Pain, Swelling, Malocclusion

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7
Q

What is radiographic appearance of CGCL?

A

Uni or Multilocular Can cross midline

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8
Q

Treatment for CGCL?

A

Depends if aggressive vs nonaggresive

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9
Q

Tx for aggressive CGCL

A

Option 1: Resection with 1cm margins Option 2: E&C plus Pharm - intralesional steroid (anti-angiogenic) - Calcitonin (inhibits bone resorption) - Interferon B (both above and stimulates osteoblasts

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10
Q

Tx for nonaggresive CGCL

A

E&C

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11
Q

Calcitonin: Regulated by? Counteracts effects of what hormones? Source for exogenous calcitonin? Effects of calcitonin? What conditions are treated with calcitonin?

A
  • Regulated by serum Ca2+c Gastrin - counteracts parathyroid hormone, vitamin D - salmon calcitonin - inhibits osteoclasts (major effect), decreases reabsorption of Ca and PO4 in renal tubule (minor effect until kidney resistant, then no effect) -tx for osteoporosis (DM2, post menopause)
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12
Q

Desmoplastic fibroma

  1. Age/gender
  2. Clinical
  3. Radiographic
  4. Histo
  5. Tx
A
  1. <30 yrs, avg 15yrs, M=F
  2. Expansion, may be painful, loose teeth
  3. Ill defined radiolucency
  4. Benign appearing short fibroblasts, no atypia
  5. 1cm margin resection with anatomic barrier
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13
Q

McCune-Albright Syndrome

  1. Classic Triad?
  2. What endocrinopathy is present?
  3. Tx
A
  1. Classic Triad

Polyostotic fibrous dysplasia (expansion, ground glass)

Precocious puberty

Irregular cafe-au-lait

  1. Psuedohypoparathyroidism
  2. Treat with Vitamin D and calcium to manage hypocalcemia/hyperphosphatemia
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14
Q

Fibrous Dysplasia

  1. Classification
  2. Age/Gender
  3. Clinical
  4. Radiograph
  5. Histo
  6. Tx
A
  1. Mono-ostotic (80%), poly-ostotic, poly-ostotic with endocrinopathies (Albright)
  2. 2nd decade
  3. Painless swelling
  4. Ground glass
  5. Normal cancellous bone replaced with fibrous tissue, trabeculation “chinese characters”
  6. Observe, resolves after puberty, surgical contouring for pysch, function, vital structures
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15
Q

Cemento-Osseous Dysplasia

  1. Tissue of origin
  2. Classification
  3. Age/Gender/Race
  4. Clinical
  5. Radiographic
  6. Histo
  7. Tx
A
  1. Arise from PDL
  2. Periapical, Focal, Florid
  3. 4th decade, AA females (except Focal=White females)
  4. Usually asymptomatic unless infected, expansion rare except florid
  5. Early: well-defined, irregular, mixed RL/RD. Late: RD with RL rim.
  6. Fibroblasts and cementum
  7. Observe, ABX if symptomatic, debride if lesions exposed
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16
Q

Ossifying Fibroma - Conventional

  1. Age/Gender/Location
  2. Clinical
  3. Radiographic
  4. Histo
  5. Tx
A
  1. 3-4th decade, F>M, mand premolar
  2. Painless expansion
  3. Early: RL / Intermediate: Mixed / Late: RD with lucent border
  4. Identical to Fibrous Dysplasia, fibrous tissue replacing bone
  5. E&C
17
Q

Juvenile Ossifying Fibroma

  1. Age/Location
  2. Clinical
  3. Radiographic
  4. Histo
  5. Classification
  6. Tx
A
  1. 1-2 decades, Mand/Max
  2. Expansion, may be symptomatic
  3. Early: RL / Intermediate: Mixed / Late: RD with lucent border. Large lesions loose lucent border (capsule)
  4. Two types: Trabecular and psammomatoid (worse prognosis)
  5. Resection 0.5cm margin, consider E&C if lucent border (capsule)
18
Q

Tumors of neural origin

A
  1. Neurofibroma (most common)
  2. Schwanoma, aka neurilemoma (uncommon)
  3. Granular Cell Tumor
  4. Melanotic neuro-ectodermal tumor of infancy
  5. Traumatic Neuroma (is reactive, not a true neoplasm)
19
Q

Main epidemiologic, clinical presentation, histology difference between Schwanoma and Neurofibroma

A

Age (neurofibroma only young)

  • Clinical (neurofibroma always asymptomatic, neurofibroma located skin, tongue, buccal mucosa, whereas schwannoma tongue and IAN)
  • Histology (schwannoma has pseudocapsule, Antoni A and Antoni B formations, Verocay bodies)
20
Q

Lesion

  • One liner
  • Age/Gender
  • Location
  • Malignant potential
  • Clincal presentation
  • Histology keys
  • Tx
A
21
Q

Describe histology keys schwannoma

A

Verocay Bodies (collection of Antoni A and B forms)

Two columns of pallisading spindle cells (A) + acellular area (B)

23
Q

Neurofibromatosis Type 1

  • Inheritance
  • Chromosome
  • Incidence
  • Malignant potential
  • Treatment
  • Associated tumors
  • Associated clinical findings
A
  • AD, diagnosed in childhood
  • Chromosome 17
  • 1:3000, type 1 most common (85%)
  • Malignant schwanoma in 5% of cases
  • Excision due to painful inpingement on nerve or comesis
  • Pheochromocytoma (10% rule), Wilms tumor (most common pediatric kidney cancer), optic glioma (CN II), malignant nerve sheath tumors (poor prognosis, 5 year survival 16%)
  • Cafe au lait (smooth), lisch nodules (iris hamartomas), Crowe sign (armpit freckles)
24
Q

Malignant nerve sheath tumor

  • One liner
  • Age/Gender
  • Location
  • Malignant potential
  • Clincal presentation
  • Histology keys
  • Tx
A
  • Tumor of bone/soft tissue that can arrise from neurofibroma (50% occur in NF-1), de novo, post radiation sarcoma.
  • Any age, any gender
  • IAN, mandible
  • Paresthesia, tooth mobility, bone expansion
  • Wide excision
  • 5-year survival 16% in NF-1
  • 5-year survival 50% other
25
Q

Neurofibroma

  • One liner
  • Age/Gender
  • Location
  • Malignant potential
  • Clincal presentation
  • Histology keys
  • Tx
A
  • Neoplasm of schwann cells and fibroblasts
  • younger
  • skin, tongue, buccal mucosa
  • Slow growing, soft, painless lesion
  • Spindle shape cells with wavi nuclei in myxoid stroma and many mast cells
  • excision
26
Q

Traumatic Neuroma

  • One liner
  • Age/Gender
  • Location
  • Malignant potential
  • Clincal presentation
  • Histology keys
  • Tx
A
  • Reactive neural tissue proliferation, not a neoplasm
  • Mental foramen, lip, tongue
  • Smooth surface, possible pain
  • Haphazard mature nerve bundles in fibrous stroma
  • Excision
27
Q

Inflammatory Papillary hyperplasia

  • One liner
  • Age/Gender
  • Location
  • Malignant potential
  • Clincal presentation
  • Histology keys
  • Tx
A
  • Reactive hyperplastic epithelium on palate
  • Peppled, asymptomatic, erythema
28
Q

Fibroma

  • One liner
  • Age/Gender
  • Location
  • Malignant potential
  • Clincal presentation
  • Histology keys
  • Tx
A
  • Reactive hyperplasia of fibrous connective tissue
  • Female 2:1
  • Anywhere, especially buccal mucosa
  • No malig potential
  • Sessile, smooth, +/- keratosis
  • Connective tissue with inflammation
  • Excision
29
Q

Melanotic neuro ectodermal tumor of infancy

  • One liner
  • Age/Gender
  • Location
  • Malignant potential
  • Clincal presentation
  • Histology keys
  • Tx
A
  • Rapidly expanding pigmented mass in anterior maxilla of infants with bone destruction
  • Infants
  • Anterior maxilla
  • Hyperchromatic small round cells
  • Excision with 5mm margin
30
Q

Lesion

  • One liner
  • Age/Gender
  • Location
  • Malignant potential
  • Clincal presentation
  • Histology keys
  • Tx
A
31
Q

Paraganglioma

  • One liner
  • Age/Gender
  • Location
  • Malignant potential
  • Clincal presentation
  • Histology keys
  • Tx
A
  • Carotid body tumor on medial aspect of carotid bifurcation
  • adventitia
  • Fontaine sign. Moves AP, doesn’t mover vertically.
  • Symptoms may be due to compression of surrounding nerves: Tongue paresis, hoarseness, Horner syndrome,
    dysphagia
  • Histology: Chief Cells
  • Tx: excision with or without embolization. Radiation is option for non-surgical candidates.
32
Q

Peripheral CGCL

  • Soft tissue version of CGCL
  • Etiology
  • One of three “P’s”
  • Age
  • Clinical
  • Radio
  • Histo
  • Tx
A

Etiology: Irritation and/or trauma

One of three P’s

  • Peripheral Giant Cell Lesion
  • Pyogenic Granuloma
  • Peripheral Ossifying Granuloma

6-7th decade

Red/Blue pedunclated lesion, Mandibular Gingiva, Hemorrhage and/or ulceration

Bone cupped out below lesion

Excision to bone. Recurrence not likely, 10%

33
Q

Aneurysmal Bone Cyst

  • Age
  • Clinical
  • Radio
  • Histo
  • Tx
A
  • 3rd decade
  • Painful expansion, paresthesia
  • Unilocu/multiloc
    *
34
Q

LCH Langerhans Cell Histiocytosis

  • 3 Severity Types / Ages
  • H&N Clinical
  • Radiographic
  • Histo
  • Tx
A
  • Severity Types/Ages
    • Unifocal Eosinophilic granuloma (Adolescent/Adult)
    • Multifocal unisystem Hand-Schuller-Christian (<10yrs)
    • Multifocal multisystem Letterer-Siwe (<2yrs)
  • Local bone pain, loose teeth
  • Floating teeth in air
  • Staining markers: CD1a, S100
  • Tx:
    • Unifocal: Observe vs E&C, good prognosis
    • Multifocal unisystem: E&C if accesible vs Low-dose XRT if non-accessible (50-60Gy), intermediate prognosis
    • Multifocal multisystem: Low risk organs = excision and low-dose XRT
    • Multifocal multisystem: High risk organs = Chemo + excision + XRT. 5 year survival 50%
35
Q

Multifocal unisystem or Multifocal multisystem

  • Lesions in jaws
  • Exophthalmos (pituitary gland involved)
  • Diabetes insipidus (pituitary gland involved)
A

Multifocal unisystem

36
Q

High risk organs LCH

A
  • Bone marrow
  • Lung
  • Liver
  • Spleen
37
Q

Multiple Endocrine Neoplasia (MEN)

  • Which type is associated with oral and/or intestinal neuromas?
  • What else is associated with this type?
  • What tests should be ordered for workup?
A
  • MEN 2b
    • Autosomal dominant
    • RET gene mutation
  • Medullary thyroid carcinoma, pheochromocytoma, Oral and/or intestinal neuromas
  • Genetic testing very sensitive. Urine catecholamines for pheochromocytoma. Thyroid nodule +

ABOMS Pathology (Johnston) (16 decks)

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