1. Soft Tissue Tumors Flashcards
(90 cards)
1
Q
Describe the general features of Soft Tissue (Mesenchymal) Tumors.
A
- Submucosal lesions
- Appear similar to Salivary Gland Tumors
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)
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
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
5
Q
What is a Neurofibroma composed of?
A
- Perineural fibroblasts
- Schwann cells
- Axons
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
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
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)
9
Q
What are the Skin Lesions of Neurofibromatosis Type 1?
A
- Cafe-au-lait spots
- Multiple Neurofibromas
- Axillary Freckling (Crowe’s Signs)
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
11
Q
What are Lisch Nodules?
A
- Associated lesion of Neurofibromatosis 1
- Pigmented (melanocytic) iris hamartomas
- aka brown macules (freckles) in the iris
12
Q
What are other accompanying but non-diagnostic features of Neurofibromatosis 1? (5)
A
- Hypertension
- Mental Deficiency
- Seizures
- Macrocephaly
- Short Stature
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
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
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
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
17
Q
What is the Treatment and Prognosis for Granular Cell Tumors?
A
- Conservative Excision - usually curative
- Prognosis is Excellent
18
Q
What is the Pathogenesis of MEN 2B?
A
- Autosomal Dominant; 50% spontaneous mutations
- Mutation of RET proto-oncogene (chromosome #10)
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%)
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
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
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
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
24
Q
What are the lab values associated with MEN 2B?
A
- Inc. Calcitonin
- Urinary Vanillylmandelic Acid (VMA) with pheochromocytomas
25
What is the Prognosis of **MEN 2B**?
* Early diagnosis
* Prophylactic **thyroidectomy** at early age
* Die by 21 w/o
* Monitor for **pheochromocytomas**
26
What are the Highly Distincitve Clinical Features of **Melanotic Neuroectodermal Tumor of Infancy (MNTI)**?
* 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
27
What is the Characteristic Radiographic Appearance of **MNTI**? (3)
* **RL anterior maxilla**
* **"tooth floating in space"**
* Deciduous maxillary incisor is pushed labially
* Occasional *osteogenic rxn **mimicking Osteosarcoma***
* **"Sun Ray"** radiographic appearance
28
What is the Characteristic Histology of **MNTI**? (2)
* Proliferation of **small, dark,** **neuroectodermal-appearing cells** that are nested aggregates
* Surrounded by **plump, epithelioid cells** that produce **melanin**
29
What are the lab values associated with **MNTI**?
* **VMA elevated in the urine**
* Neural crest-derived tissues make NE-like hormones that are metabolized to VMA
* Concentration **normalizes with tumor excision**
30
What is the Prognosis of **MNTI**?
* **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
31
What is the etiology/pathogenesis of MPNST (Neurogenic Sarcoma - Neurofibrosarcoma)?
May arise **spontaneously** OR in **association with NFT**
32
What are the Clinical Features of **Malignant Peripheral Nerve Sheath Tumor (MPNST)**?
* **Enlarging** mass, **pain/paresthesia** is common
* **Mandible**, lips, buccal mucosa
33
What is the Histology of **MPNST**? (3)
* 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**
34
What is the radiographic appearance of Neurofibrosarcoma (MPNST)?
Ill-defined, **pushing teeth out of the way**
35
What is the Treatment for **MPNST**?
* **Radical surgical excision** +/- adjuvant **Radtx and Chemotx**
36
What is the Prognosis for **MPNST**?
* **Sporadic Cases** (not assoc with NF) --\> **50% 5-yr survival**
* Thoose developing in the setting of **NF1** --\> may have **worse prognosis**
37
What are the _Distinctive_ Clinical Features of **Congenital Epulis**? (5)
* 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
What is the Histology of **Congenital Epulis**?
* 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
What is the Treatment for **Congenital Epulis**?
* **Conservative Excision**
* No tendency to recur
* Some reports of **spontaneous involution without surgery**
40
What are the Clinical Features of **Lipoma**? (7)
* 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
What is the Histology of **Lipomas**?
* Demarcated or **encapsulated** collection of **mature fat cells**
* Thin fibrous **septae** divide the fat cells
* Will **float in formalin** due to fat
42
What is the Treatment and Prognosis of **Lipomas**? (3)
* Enucleation or **conservative excision**
* Little or no tendency to recur
* No evidence of malignant transformation
43
What are the Clinical Features of **Hemangiomas**? (7)
* 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
What is the Treatment for **_Deep_** **Hemangioma**?
* **Steroids, IV vincristine** (chemo drug, if unresponsive)
* If cosmetically unacceptable:
* Surgical excision or **cryotherapy**
* **Embolization** or **Sclerosing agents**
45
What is the Histology of **Early Hemangioma**?
* **Plump endothelial cells** with **indistinct vascular lumina**
* Looks like granulation tissue
46
What is the Histology of **Developed Hemangioma**?
* **Endothelial cells flatten**, due to deposition of collagen between them
* **Small capillaries** more evident
47
What is the Histology of **Involution of Hemangiomas**?
* Vascular spaces regress
* Replaced by **fibrous CT**
48
What are the key features of Vascular Malformations? (2)
* 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
What are the Clinical Features of **Port Wine Stain (Nevus Flammeus)**?
* A **capillary** vascular malformation affecting superficial and deeper tissues in region of **CN V**
* Usually **flat, unilateral** (stops at midline)
* *Hemangioma is raised*
50
What are the 2 conditions/syndromes that are **High Flow** **Vascular Malformations**?
* Sturge-Weber Angiomatosis
* Von Hippel-Lindau Sx
51
What are the Clinical Features of **Intrabony AV Malformations**? (4)
* 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
What is the Treatment for **Port Wine Stain** (Vascular Malformation)?
* Flash lamp pulsed **dye laser**
53
What is the Treatment for **Large Lesion(Vascular Malformations**?
* **Sclerosing agents** (destroys vessels) + **excision**
54
What is the Treatment for **High Flow** **AV Malformations**?
* **Embolization + Excision**
* ****Injecting into feeder artery trying to avoid excessive hemorrhage
55
What is the Pathogenesis of **Encephalotrigeminal Angiomatosis** (*Sturge-Weber Sx/Angiomatosis*)?
* **Developmental, Congenital** Condition
* _Not Inherited_
* Due to **G protein mutation**
* Only 10% of people with Port Wine Stain have this condition
56
What are the Clinical Features of **Encephalotrigeminal Angiomatosis**? (3)
* **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
What are the Oral Lesions of **Encephalotrigeminal Angiomatosis**? (4)
* **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
What is the pathogenesis of Von Hippel-Lindau Syndrome?
* Bad **VHL protein**
* Get lots of **bv tumors**
59
What are the Clinical Features of **Hereditary Hemorrhagic Telangiectasia (HHT)**?
* 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
What are the Complications associated wtih **HHT**? (2)
* **GI lesions** may bleed
* **_Iron deficiency anemia_**
* **AV Malformations** of **lungs, liver, or brain**
* May develop **fistulous tracts** or secondary infections (**brain abscesses**)
61
What is the Histopathology of **HHT**?
Multiple superficial **dilated vascular spaces**
62
What is the Treatment of **HHT**?
Laser ablation or surgery
63
What is the Dental Management of **HHT**?
* **Antibiotic Prophy** required until AV malformation is excluded
* To prevent _possibility of brain abscess_
64
What is the Prognosis of **HHT**?
Good in most cases
65
In what population does Nasopharyngeal Angiofibroma occur?
Rare benign neoplasm
**Male adolescents**
66
Where is Nasopharyngeal Angiofibroma presumed to arise?
In the **pterygopalatine fossa** (behind maxilla) with extension to **involve adjacent structures**
67
What are the Distinctive Clinical Features of **Nasopharyngeal Angiofibroma**? (3)
* **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
What is the Histology of **Nasopharyngeal Angiofibroma**?
* Composed of an **unencapsulated**, **infiltrative** proliferation of **dilated vessels** and **fibrous tissue**
* Clinically Aggressive Growth, despite bland histo
69
What is the Treatment and Prognosis for **Nasopharyngeal Angiofibroma**?
* **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
What are the Clinical Features of **Lymphangioma**? (6)
* 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
How are **Lymphangiomas** Classified?
* _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
What are the Clinical Features of **Cystic Hygroma** (Macrocytic Lymphatic Malformations)? (2)
* Affects the:
* Neck
* Mediastinum
* Axilla
* Oral Cavity
* May cause **airway obstruction**, particularly if it becomes _secondarily infected_
73
What is the Histology of **Lymphangioma**? (2)
* 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
What is the Treatment for **Lymphangioma**? (4)
* 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
What is angiosarcoma, and in what population does it occur?
* Rare **malignancy** of **endothelial origin** (blood or lymphatic)
* **Elderly** pts
76
What are the Clinical Features of **Angiosarcoma**? (2)
* **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
What is the treatment and prognosis for Angiosarcoma?
* Tx: **Radical surgical excision +/- radtx**
* **Poor** prognosis
78
What is Kaposi Sarcoma, and what causes it?
* Multifocal Vascular Neoplasm
* Caused by **HHV-8 infection**
79
What are the types of **Kaposi Sarcoma**?
* _Classic_
* _Endemic (African)_
* _Iatrogenic (immunosuppression)_
* __**Organ transplant pts**
* Can affect the **mouth**
* _AIDS Related_
80
What are the Clinical Features of **AIDS-related KS**? (5)
* **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
What is the Treatment for **Kaposi Sarcoma - Skin Lesions**?
Radiation or Chemotx
82
What is the Treatment for **Kaposi Sarcoma - Multiple Lesions**?
* **Single agent chemotx** if no result with HAART
* Tx HIV first
83
What is the Treatment for **Kaposi Sarcoma - Oral Lesions**?
* **Excision**, intralesional vinblastine or sclerotherapy
* **Laser and Electrosurgery** may cause **aerosolization of viral particles**
* NOT RECCOMMENDED
84
What is the Prognosis for each of the different types of **Kaposi Sarcoma**?
* _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
What is the histology of Kapsoi Sarcoma?
* **HHV-8 stain** - stains all cells with virus brown
* See lots of **blood and blood vessels**
86
What are the Clinical Features of **Leiomyoma**? (5)
* 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
What is the Histology and Treatment of **Leiomyoma**?
* 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
What are the Clinical Features and Types of **Rhabdomyoma**?
* 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
What are the Clinical Features, Histology, and Tx of **Rhabdomyosarcoma**? (7)
* 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
What are the Clinical Features of **Leiomyosarcoma**, **Liposarcoma**, and **Fibrosarcoma**? (2)
* Rarely affect the H/N region
* Destructive appearing bone or soft tissue lesion
