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Oral Pathology Exam 2 > 1. Soft Tissue Tumors > Flashcards

1. Soft Tissue Tumors Flashcards

1
Q

Describe the general features of Soft Tissue (Mesenchymal) Tumors.

A
  • Submucosal lesions
  • Appear similar to Salivary Gland Tumors
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2
Q
  • Benign tumor of schwann cell origin, no axons.
  • Associated with a nerve trunk
  • H/N - tongue most common
  • Slow-growing, solitary, encapsulated, rubbery-firm, most often non-tender mass
  • May be seen in the Mandible, often involving the Mandibular Canal (odontogenic)
  • Pain and paresthesia may occur in bony lesions
A

Neurilemoma (Schwannoma)

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3
Q

What is the Histologic Pattern assocaited with Neurilemoma (Schwannoma)?

A
  • Antoni A
    • ​Palisaded nuclei arranged around Verocay bodies
  • Antoni B
    • ​Loose arrangement of haphazard schwann cells, reticulin fibers and cystlike foci
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4
Q

What is Neurofibromatosis Type II?

A
  • Bilateral schwannomas of CN VIII vestibulocochlear nerve
    • Tumor of the Ears
  • Leading to progressive deafness, dizziness and tinnitus
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5
Q

What is a Neurofibroma composed of?

A
  • Perineural fibroblasts
  • Schwann cells
  • Axons
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6
Q

What are the clinical features of a Neurofibroma? (4)

A
  • Soft, dome-shaped, non-tender, superficial nodule, affecting skin most commonly
  • If oral –> tongue, buccal mucosa
  • Demarcated, but unencapsulated
  • 90% are solitary, but 10% are multiple and associated with Neurofibromatosis
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7
Q

What is the Histology of a Neurofibroma? (3)

A
  • Collection of spindle-shaped cells with wavy nuclei (comma-shaped)
  • Mast cells are often seen
  • Lesional tissue tends to mingle with the adjacent normal tissue
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8
Q
  • Most common type (1/3,000 live births)
  • Mutation of Chromosome 17-NFL gene (neurofibromin)
  • 50% autosomal dominant; 50% new mutations
  • Highly variable gene expression
    • Some cases mild and others severe
A

Neurofibromatosis Type 1

(von Recklinghausen Ds of the skin)

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9
Q

What are the Skin Lesions of Neurofibromatosis Type 1?

A
  • Cafe-au-lait spots
  • Multiple Neurofibromas
  • Axillary Freckling (Crowe’s Signs)
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10
Q

What is a pathognomonic skin lesion of Neurofibromatosis Type 1?

A
  • Plexiform Neurofibroma
    • “bag of worms”
    • A tortuous mass of expanded nerve branches embedded in a backdrop of neurofibromatous tissue
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11
Q

What are Lisch Nodules?

A
  • Associated lesion of Neurofibromatosis 1
  • Pigmented (melanocytic) iris hamartomas
    • aka brown macules (freckles) in the iris
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12
Q

What are other accompanying but non-diagnostic features of Neurofibromatosis 1? (5)

A
  • Hypertension
  • Mental Deficiency
  • Seizures
  • Macrocephaly
  • Short Stature
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13
Q

What are the Oral Lesions associated with Neurofibromatosis 1? (3)

A
  • Neurofibroma affecting the: tongue, gingiva, bone
  • Enlarged fungiform papillae
  • Bone Lesions:
    • Enlargement or branching of the mandibular foramen
    • Increased dimension of coronoid notch
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14
Q

What is the Prognosis of of Neurofibromatosis Type 1?

A
  • Reduced life expectancy due to:
    • Vascular ds
    • Variety of malignancies
  • ~5% of pts develop malignant transformation of a neurofibroma –> malignant peripheral nerve sheath tumor
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15
Q
  • Benign neoplasm possibly derived from schwann cells
  • Develops on any cutaneous or mucosal surface, but 40% occur on the tongue (dorsal)
    • Also on buccal mucosa and FOM
  • Slow-growing, demarcated, unencapsulated, non-tender, firm, submucosal nodule
A

Granular Cell Tumor

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16
Q

What is the Histology of Granular Cell Tumor?

A
  • Collection of mesenchymal cells with a granular-appearing cytoplasm
  • Poorly circumscribed
  • PEH (pseudoepitheliomatous hyperplasia) in up to 50%​
    • May be mistaken for SCCA microscopically
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17
Q

What is the Treatment and Prognosis for Granular Cell Tumors?

A
  • Conservative Excision - usually curative
  • Prognosis is Excellent
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18
Q

What is the Pathogenesis of MEN 2B?

A
  • Autosomal Dominant; 50% spontaneous mutations
  • Mutation of RET proto-oncogene (chromosome #10)
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19
Q

What are the Clinical Features of MEN 2B? (4)

A
  • Marfanoid Build
    • Thin elongated limbs, with muscle wasting
    • Narrow face
    • Thick, protuberant lips
    • Eversion of upper eyelid
    • Neuromas on eye
  • Oral Mucosal Neuroma
  • Medullary Carcinoma of the Thyroid (90%)
  • Adrenal Pheochromocytomas (50%)
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20
Q

What are the Clinical Features of Oral Mucosal Neuromas associated with MEN 2B?

A
  • Often 1st sign of ds
  • Lips, anterior tongue, buccal mucosa, gingiva, palate
  • Bilateral neuromas of commissure - highly suggestive
  • Soft, yellowish
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21
Q

What are the Clinical Features of Medullary Carcinoma of the Thyroid (90%) associated with MEN 2B?

A
  • Aggressive C Cell Malignancy (inc. calcitonin)
  • Develops during childhood or adolescence
  • Without Tyroidectomy - death ~21 yo
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22
Q

What are the Clinical Features associated with Adrenal Pheochromocytomas (50%) associated with MEN 2B?

A
  • Frequently bilateral
  • Catecholamin production
    • Diffuse sweating, diarrhea, headaches, flushing, palpitations, sever hypertension
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23
Q

What is the Characteristic Histopathology of MEN 2B?

A
  • Mucosal Neuroma
    • Hyperplasia of schwann cells and axons
    • Surrounded by a thickened perineural sheath
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24
Q

What are the lab values associated with MEN 2B?

A
  • Inc. Calcitonin
  • Urinary Vanillylmandelic Acid (VMA) with pheochromocytomas
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25
Q

What is the Prognosis of MEN 2B?

A
  • Early diagnosis
  • Prophylactic thyroidectomy at early age
    • Die by 21 w/o
  • Monitor for pheochromocytomas
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26
Q

What are the Highly Distincitve Clinical Features of Melanotic Neuroectodermal Tumor of Infancy (MNTI)?

A
  • Rare tumor most often (91%) seen in the 1st year of life
  • Neural Crest Origin
  • Most develop in the bone of the anterior maxilla
  • Rapid Growth
  • May be brown or black in color
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27
Q

What is the Characteristic Radiographic Appearance of MNTI? (3)

A
  • RL anterior maxilla
  • “tooth floating in space”
    • Deciduous maxillary incisor is pushed labially
  • Occasional osteogenic rxn mimicking Osteosarcoma
    • “Sun Ray” radiographic appearance
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28
Q

What is the Characteristic Histology of MNTI? (2)

A
  • Proliferation of small, dark, neuroectodermal-appearing cells that are nested aggregates
  • Surrounded by plump, epithelioid cells that produce melanin
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29
Q

What are the lab values associated with MNTI?

A
  • VMA elevated in the urine
    • Neural crest-derived tissues make NE-like hormones that are metabolized to VMA
  • Concentration normalizes with tumor excision
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30
Q

What is the Prognosis of MNTI?

A
  • Conservative excision is usually curative
  • 20% recurrence with possible malignant behavior (metastasis) in a very small %
  • May create a significant bone defect and failure of premanent teeth to develop
  • Follow-up is warranted to fix defect
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31
Q

What is the etiology/pathogenesis of MPNST (Neurogenic Sarcoma - Neurofibrosarcoma)?

A

May arise spontaneously OR in association with NFT

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32
Q

What are the Clinical Features of Malignant Peripheral Nerve Sheath Tumor (MPNST)?

A
  • Enlarging mass, pain/paresthesia is common
  • Mandible, lips, buccal mucosa
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33
Q

What is the Histology of MPNST? (3)

A
  • Invasive cellular proliferation of spindle-shaped cells with wavy nuclei
    • ​similar to Neurofibroma
  • Similar appearance to several other tumors with H-E stain
  • S-100 positivity
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34
Q

What is the radiographic appearance of Neurofibrosarcoma (MPNST)?

A

Ill-defined, pushing teeth out of the way

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35
Q

What is the Treatment for MPNST?

A
  • Radical surgical excision +/- adjuvant Radtx and Chemotx
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36
Q

What is the Prognosis for MPNST?

A
  • Sporadic Cases (not assoc with NF) –> 50% 5-yr survival
  • Thoose developing in the setting of NF1 –> may have worse prognosis
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37
Q

What are the Distinctive Clinical Features of Congenital Epulis? (5)

A
  • Rare lesion of undetermined histogenesis
  • Found at birth on the maxillary (x2) alveolar ridge
  • Female (9:1)
  • Smooth-surfaced, swelling of soft tissue (differentiates from MPNST), often pedunculated
  • Vary in size
38
Q

What is the Histology of Congenital Epulis?

A
  • Benign proliferation of cells having granular cytoplasms
  • Compared to Granular Cell Tumor:
    • No PEH
    • No S-100 positivity
    • Different location from dorsal tongue of GCT
39
Q

What is the Treatment for Congenital Epulis?

A
  • Conservative Excision
  • No tendency to recur
  • Some reports of spontaneous involution without surgery
40
Q

What are the Clinical Features of Lipoma? (7)

A
  • Benign tumor of adipose tissue
  • Adult pts
  • Slow-growing, non-tender, soft, doughy, usually encapsulated
  • Common in H/N
    • Soft swelling in the neck of adult
  • Occasionally found intraorally, most commonly on buccal mucosa/vestibule
  • Yellow if close to the surface, Pink if deep
  • Often though to be a salivary gland tumor or fibroma
41
Q

What is the Histology of Lipomas?

A
  • Demarcated or encapsulated collection of mature fat cells
  • Thin fibrous septae divide the fat cells
  • Will float in formalin due to fat
42
Q

What is the Treatment and Prognosis of Lipomas? (3)

A
  • Enucleation or conservative excision
  • Little or no tendency to recur
  • No evidence of malignant transformation
43
Q

What are the Clinical Features of Hemangiomas? (7)

A
  • Most common tumor of infancy
  • Females
  • H/N is most common site (60%), superficial skin
  • Tongue is common intraoral site
  • Rarely present at birth, usually arise shortly after birth and
  • Show rapid growth for ~1yr
  • Then involute over time (90% by 9yo)
44
Q

What is the Treatment for Deep Hemangioma?

A
  • Steroids, IV vincristine (chemo drug, if unresponsive)
  • If cosmetically unacceptable:
    • Surgical excision or cryotherapy
    • Embolization or Sclerosing agents
45
Q

What is the Histology of Early Hemangioma?

A
  • Plump endothelial cells with indistinct vascular lumina
  • Looks like granulation tissue
46
Q

What is the Histology of Developed Hemangioma?

A
  • Endothelial cells flatten, due to deposition of collagen between them
  • Small capillaries more evident
47
Q

What is the Histology of Involution of Hemangiomas?

A
  • Vascular spaces regress
  • Replaced by fibrous CT
48
Q

What are the key features of Vascular Malformations? (2)

A
  • Are present at birth, though may not be apparent
  • DON’T involute over time
    • Opposite of Hemangiomas, that appear after birth, grow rapidly, and involute over time
49
Q

What are the Clinical Features of Port Wine Stain (Nevus Flammeus)?

A
  • A capillary vascular malformation affecting superficial and deeper tissues in region of CN V
  • Usually flat, unilateral (stops at midline)
    • Hemangioma is raised
50
Q

What are the 2 conditions/syndromes that are High Flow Vascular Malformations?

A
  • Sturge-Weber Angiomatosis
  • Von Hippel-Lindau Sx
51
Q

What are the Clinical Features of Intrabony AV Malformations? (4)

A
  • Ill-defined or cyst-like RL defect,
    • Hint of trabeculation that have a “sun ray” appearance
    • Often Multilocular
  • May detect “bruit” or pulsation on palpataion/ascultation
  • Overlying skin warm to touch
  • Yields bright red blood on aspirate
    • ​Also see aspirate in Traumatic Bone Cyst
52
Q

What is the Treatment for Port Wine Stain (Vascular Malformation)?

A
  • Flash lamp pulsed dye laser
53
Q

What is the Treatment for Large Lesion(Vascular Malformations?

A
  • Sclerosing agents (destroys vessels) + excision
54
Q

What is the Treatment for High Flow AV Malformations?

A
  • Embolization + Excision
    • ​Injecting into feeder artery trying to avoid excessive hemorrhage
55
Q

What is the Pathogenesis of Encephalotrigeminal Angiomatosis (Sturge-Weber Sx/Angiomatosis)?

A
  • Developmental, Congenital Condition
    • Not Inherited
  • Due to G protein mutation
  • Only 10% of people with Port Wine Stain have this condition
56
Q

What are the Clinical Features of Encephalotrigeminal Angiomatosis? (3)

A
  • Nevus Flammeus (Port Wine Stain) in distribution of CN V
  • Involves deeper soft tissues as well as Meninges
    • “Tramline” calcifications seen on skull film
      • May represent calcification of abnormal b.v. walls
  • Seizure Disorders because of brain growth, may lead to mental disability, and contralateral hemiplegia (opposite side of bv)
57
Q

What are the Oral Lesions of Encephalotrigeminal Angiomatosis? (4)

A
  • Flat, red patches
  • Pyogenic Granuloma-like lesions
  • Gingival Hyperplasia-due to increased vascularity and/or anti-convulsive meds
    • Prophy challenging
  • Severe hemorrhage may occur with surgical procedures
58
Q

What is the pathogenesis of Von Hippel-Lindau Syndrome?

A
  • Bad VHL protein
  • Get lots of bv tumors
59
Q

What are the Clinical Features of Hereditary Hemorrhagic Telangiectasia (HHT)?

A
  • aka Osler-Weber Rendu Sx
  • Inherited (Autosomal Dominant) mucocutaneous disorder
  • 2 types showing numerous vascular hamartomas
  • Frequent Epitaxis
  • Numerous 1-2mm red papules of skin and mucosa
    • Positive to Diascopy (blanch)
60
Q

What are the Complications associated wtih HHT? (2)

A
  • GI lesions may bleed
    • Iron deficiency anemia
  • AV Malformations of lungs, liver, or brain
    • ​May develop fistulous tracts or secondary infections (brain abscesses)
61
Q

What is the Histopathology of HHT?

A

Multiple superficial dilated vascular spaces

62
Q

What is the Treatment of HHT​?

A

Laser ablation or surgery

63
Q

What is the Dental Management of HHT​?

A
  • Antibiotic Prophy required until AV malformation is excluded
    • To prevent possibility of brain abscess
64
Q

What is the Prognosis of HHT​?

A

Good in most cases

65
Q

In what population does Nasopharyngeal Angiofibroma occur?

A

Rare benign neoplasm

Male adolescents

66
Q

Where is Nasopharyngeal Angiofibroma presumed to arise?

A

In the pterygopalatine fossa (behind maxilla) with extension to involve adjacent structures

67
Q

What are the Distinctive Clinical Features of Nasopharyngeal Angiofibroma? (3)

A
  • Epitaxis and Nasal Obstruction are the most common presenting signs
  • Anterior bowing of posterior wall of maxilla
  • May present as a mass intraorally, with bulging of the soft palate
68
Q

What is the Histology of Nasopharyngeal Angiofibroma?

A
  • Composed of an unencapsulated, infiltrative proliferation of dilated vessels and fibrous tissue
  • Clinically Aggressive Growth, despite bland histo
69
Q

What is the Treatment and Prognosis for Nasopharyngeal Angiofibroma?

A
  • Surgery, usually with preoperative embolization of the tumor
    • To prevent hemorrhage
  • Recurrent/extensive tumors - Radiation tx
  • Guarded, Recurrence rates = 20-40%
    • Hard to remove in the midface
70
Q

What are the Clinical Features of Lymphangioma? (6)

A
  • Benign, hamartomatous tumor-like growth of lymphatic vessels
  • Developmental malformation, not a true neoplasm
  • 1/2 present at birth; 90% develop by 2 yrs
  • Most in H/N (50-75%)
  • Tongue most common intraoral site –> macroglossia
  • Surface resembles traslucent vesicles “frog eggs”
    • Trauma leading to edema causes red/purple appearance
71
Q

How are Lymphangiomas Classified?

A
  • Macrocytic
    • ​> 2cm, often occur in neck = Cystic Hygroma
    • May cause airway obstruction particularly if it becomes secondarily infected
  • Microcytic
    • ​< 2cm, often in the mouth
  • Mixed
72
Q

What are the Clinical Features of Cystic Hygroma (Macrocytic Lymphatic Malformations)? (2)

A
  • Affects the:
    • Neck
    • Mediastinum
    • Axilla
    • Oral Cavity
  • May cause airway obstruction, particularly if it becomes secondarily infected
73
Q

What is the Histology of Lymphangioma? (2)

A
  • Markedly dilated lymphatic vessels often right beneath the epithelium replacing the CT papillae they stick up causing clear clinical vesicle
  • Vessels often infiltrate more deeply into soft tissue and muscle in irregular fashion
    • No capsule, makes removal more difficult, hard to tell where it ends
74
Q

What is the Treatment for Lymphangioma? (4)

A
  • Surgical Excision or Sclerotherapy
  • Infiltrative nature complicates assured excision leading to high recurrence rate (up to 40%)
  • Just monitor non-enlarging tongue lesions
  • Sclerotherapy most effective with macrocystic lesions (84% success)
75
Q

What is angiosarcoma, and in what population does it occur?

A
  • Rare malignancy of endothelial origin (blood or lymphatic)
  • Elderly pts
76
Q

What are the Clinical Features of Angiosarcoma? (2)

A
  • 50% H-N
    • Mostly skin of face and scalp
    • Rare intraorally
  • On skin, looks like a bruise (reddish-purple patch) with an indurated border
    • ​It grows –> elevates –> ulcerates
77
Q

What is the treatment and prognosis for Angiosarcoma?

A
  • Tx: Radical surgical excision +/- radtx
  • Poor prognosis
78
Q

What is Kaposi Sarcoma, and what causes it?

A
  • Multifocal Vascular Neoplasm
  • Caused by HHV-8 infection
79
Q

What are the types of Kaposi Sarcoma?

A
  • Classic
  • Endemic (African)
  • Iatrogenic (immunosuppression)
    • ​Organ transplant pts
    • Can affect the mouth
  • AIDS Related
80
Q

What are the Clinical Features of AIDS-related KS? (5)

A
  • Skin and anal area of male homosexuals with AIDS
  • HHV-8 has tropism for oral/oropharyngeal epithelium
    • Found in saliva (transmission reservoir)
  • 70% will have oral lesions (palate, gingiva, or tongue)
    • Tumors are aggressive in oral cavity
  • Clinical Stages of Evolution: thickens over time
    • Patch (macular) –> Plaque –> Nodular
  • Brown or Reddish/Purple - macule or nodule
    • Negative to Diascopy
81
Q

What is the Treatment for Kaposi Sarcoma - Skin Lesions?

A

Radiation or Chemotx

82
Q

What is the Treatment for Kaposi Sarcoma - Multiple Lesions?

A
  • Single agent chemotx if no result with HAART
    • Tx HIV first
83
Q

What is the Treatment for Kaposi Sarcoma - Oral Lesions?

A
  • Excision, intralesional vinblastine or sclerotherapy
  • Laser and Electrosurgery may cause aerosolization of viral particles
    • NOT RECCOMMENDED
84
Q

What is the Prognosis for each of the different types of Kaposi Sarcoma?

A
  • Classic
    • _​_Fair - pts usually die from something else
  • Endemic
    • _​​_Fair-very poor
  • Transplant-associated
    • _​_Fair-poor
    • May regress with decreased immune suppression
  • AIDS-related
    • _​_depends on tx access
    • In US recent 5yr survival 70%
85
Q

What is the histology of Kapsoi Sarcoma?

A
  • HHV-8 stain - stains all cells with virus brown
  • See lots of blood and blood vessels
86
Q

What are the Clinical Features of Leiomyoma? (5)

A
  • Benign smooth muscle tumor
  • Most common in the uterus
  • Oral tumors of upper lip, tongue, buccal mucosa, and palate
    • probably arise from vascular smooth muscle
  • Reddish/purple if vascular
  • Well-demarcated, rubbery firm, <1cm in diameter
87
Q

What is the Histology and Treatment of Leiomyoma?

A
  • Benign proliferation of spindle-shaped cells with cigar-shaped nuclei that resemble smooth muscle
  • No significant atypia, and no mitotic activity
  • Tx: conservative excision
88
Q

What are the Clinical Features and Types of Rhabdomyoma?

A
  • Very rare, benign tumor of skeletal muscle
  • Most common in the heart (Cardiac rhabdomyoma)
    • Can be assoc with Tuberous Sclerosis
  • H-N is next most common site
    • Fetal Type
      • _​_Face, periauricular
    • Adult Type
      • _​_Pharynx, larynx, and oral cavity
        • Firm, slow-growing mass on jaw
89
Q

What are the Clinical Features, Histology, and Tx of Rhabdomyosarcoma? (7)

A
  • Rare neoplasm of skeletal muscle differentitation
  • Usually affects Children or Adolescents
  • Location
    • H-N is the most common
      • Face, orbit, nasal cavity
    • Intraoral
      • Palate, may break through from maxillary sinus
  • Most often painless, rapid, infiltrative growth
  • Growth into body cavities (mouth, vagina) can look like a bunch of grapes (botryoid rhabdomyosarcoma)
  • Histo: small, round cells that are loosely cohesive
  • Tx: wide surgical excision combined with radtx and chemotx
90
Q

What are the Clinical Features of Leiomyosarcoma, Liposarcoma, and Fibrosarcoma? (2)

A
  • Rarely affect the H/N region
  • Destructive appearing bone or soft tissue lesion

Oral Pathology Exam 2 (7 decks)

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