FPC3: Pathology Week 3

Soft tissue tumors are derived from _________ prolilferations

Mesenchymal

The major of soft tissue tumors are ______

reactive

low grade chronic irritation

Fibrosarcoma

Malignant tumor of fibroblasts

10-19% occur in H&N--more common extremities

What are the clinical presentations of fibrosarcoma?

Slow growing masses

can present at ANY age

can present ANYWHERE

Fibrosarcoma histology

Liposarcoma

Sarcoma of adipocytes

adults ages 40-60

What are the clinical features of liposarcoma?

Slow growing, soft lesion

pain is a late feature

Where are common locations for liposarcoma?

Thigh

Retroperitoneum

inguinal area

H&N is rare---> 3% is neck and cheek

Liposarcoma histology

Struge-Weber Angiomatosis Syndrome

Port wine stain of skin- follows distribution of TRIGEMINAL N.

10% of port wine stains are bilateral

Patients with Sturge-Weber Angiomatosis are more likely to also have these disorders:

Seizure disorders

cognitive disabilities

parallel calcifications in the brain's cortex (Tram-Line)

Intraoral hypervascularization

What is the treatment for Sturge-Weber Angiomatosis?

Depends on severity

lasors

Surgical excision (may need more extensive surgery)

intra-oral lesions surgery

Angiosarcoma

Sarcoma of vascular endothelium

Seen in elderly patients

What is a common site for angiosarcoma?

Scalp & forehead

can present orally --rare

What are the clinical features of angiosarcoma?

Early lesions appear as a bruise

Enlarges to create an ulcerated, nodular elevated surface

Angiosarcoma histology

Are there malignant soft tissue pathologies of the lymphatic tissue?

No

Neurofibromatosis Type 1 (syndrome)

Most common + presents with oral lesions

Heredity condition

Autosomal dominant

Mapped on Chromosome 17

--NF1 gene

What are the clinical features of neurofibromatosis Type 1?

Multiple fibromas on the body

Cafe au lait pigmentation

Axillary freckling

Lisch nodules

Neurofibromas occur at a young age

What is the treatment of neurofibromatosis Type 1?

No specific treatment

Removal of neurofibromas

**Neurofibromas may undergo malignant transformation

Patients with neurofibromatosis Type 1 can undergo malignant transformation into what cancer?

Malignant peripheral nerve sheath tumor

Neurofibromatosis Type 1 patients are more susceptible to what other tumors?

Leukemia

Wilms Tumor

CNS tumors

Neurofibromatosis Type 2 characteristics

Autosomal dominant -NF2 gene

Bilateral Schwannomas (CN VIII)

Café au lait pigmentation (less

common than in NF1)

Cutaneous neurofibromas are

uncommon

MEN type 2B

Genetic mutation -- autosomal dominant

Mucosal neuromas on lip, tongue, BM, gingiva, & palate (can be 1st sign of disease)

Pheochromocytomas

Marfanoid appearance

MEN type 2b patients are almost 100% likely to get what type of malignant soft tissue pathology?

Medullary thyroid carcinoma

what is the treatment for MEN type 2B?

Thyroid removal ASAP

Lab values show serum or urinary calcitonin

Pheochromycytomas may result in increased levels of urinary vanillymandellic acid (VMA)

Malignant Peripheral Nerve Sheath Tumor

Common in people with neurofibromas (50%)

10% of all soft tissue sarcomas

What are the clinical features of malignant peripheral nerve sheath tumor?

Asymptomatic

Expansile mass

Young patients

radiographs can show widening of mandibular canal or mental foramen

Malignant peripheral nerve sheath tumor histology

What is the most common site for an angiosarcoma?

(video question)

Scalp

rhabdomyosarcoma

Malignant tumor of skeletal muscle

What are the clinical features of rhabdomyosarcoma?

Common in young children (1st decade)

2-5% in adults

Presents as an infiltration mass

What are the common sites for rhabdomyosarcoma?

Eye and nasal cavity

Rhabdomyosarcoma histology

Leiomyosarcoma

Derived from vascular smooth muscle

7% of all soft tissue sarcomas

RARE in oral cavity

What are the clinical features of leiomyosarcoma?

Common in middle aged and older adults

Appear as enlarging mass

Can be painful or ulcerated

Leiomyosarcoma histology

What is the inheritance pattern for neurofibromatosis type I?

Autosomal Dominant

Metastases to the oral soft tissue accounts for ____% of oral malignancies

1

What are the clinical features of metastases to the oral soft tissues?

Gingiva is most common site** (mimic 4 P's)

Resembles hyperplastic/reactive growths

Can be ulcerated

Histology matches PRIMARY tumor

What is the most common site for metastasic tumors to the oral cavity?

(video question)

Gingiva

Cell division and proliferation is necessary and normal... Why is that?

Its needed for growth & injury repair

Its controlled by GENES

Proto-oncogenes

Encourage growth

Suppressor genes

Restrict growth

What is malignancy?

Its uncontrolled growth, damaging the growth of cells

So, What is cancer?

Uncontrolled growth with invasion +/- metastasis

Most cancers form tumors

ANY age affected

Genetic abnormalities

--cancer promoting oncogenes= ON

--tumor supressor genes = OFF

What cancer does NOT form a tumor?

Leukemia

What are 2 histological components of cancer?

Dysplasia

Anaplasia

Dysplasia

Disordered growth

Anaplasia

Cells assume a bizarre shape

OR lack of differentiation

Oral SCCa presentation

Variable pain

Red/white lesion

Non-healing ulcer

Exophytic growth or endophytic growth

HIGH risk locations --->

-Floor of mouth

-Posterior lateral border of tongue

What are adjuncts to oral cancer diagnosis?

Brush biopsy

Velscope

Vizilite

Identafi

What are the 3 types of treatment for oral cancers?

Surgery

radiation therapy

Chemotherapy

Why are dental consultations with your cancer patients important?

The goal is to assist the patient in returning to as near normal life as possible!

Plan ahead: restore lost tissue/teeth

What should your examination consist of in your pretreatment dental exam for your cancer patient?

Radiographs

Examine Salivary glands

Maximum incisal opening

Eliminate foci of infection for your cancer patient, what does this mean?

Restore caries

Address perio

Plan extractions 2-3 weeks PRIOR to starting cancer treatment

What is chemotherapy?

Cytotoxic agents + antimetabolites

What is the goal of chemotherapy?

High kill rates

Target rapidly dividing cells

--tumor shrinkage

--eradicate micrometastases

What are the different types of chemotherapy?

Induction

Concurrent

Adjuvant

What are the side effects of chemotherapy?

Myelosuppression

Mucositis

Alopecia

What is adjuvant chemotherapy?

Additional treatment AFTER srugery

Helps reduce risk of relapse due to occult disease

Aim to improve overall survival

What is neoadjuvant chemotherapy?

BEFORE the main treatment

Reduction of tumor size to help minimize an invasive surgery

What is the goal of ionizing radiation?

Control malignant cells

-usually gamma

What are the uses of ionizing radiation?

Curative (Primary)

Adjuvant

Palliative

Total body irradiation

What are the side effects of radiation therapy?

Mucositis

Dermatitis

Altered pigmentation

Hair loss

damage taste buds

damage salivary glands

delayed bone/tissue necrosis

In radiation therapy, dose fractionation minimizes what?

Minimizes damage to normal tissue

What are complications to radiation therapy treatment?

Alteration of normal tissues

Complications can delay or limit treatment

Effective therapy does NOT eliminate risks for second cancer

What are the acute complications for chemotherapy?

Mucositis

Hemorrhage

What are the acute complications for radiation?

Mucositis

Dermatitis (acute)

Loss of taste (hypogeusia)

Pain

Infection

trismus

What are the chronic complications of cancer treatment?

Xerostomia

Loss of taste

Chronic dermatitis

Dental caries

Trismus

Osteoradionecrosis

Mucositis

inflammation of the mucosa

--thinning and breakdown

What are the symptoms of mucositis?

Pain

Ulceration

Odynophagia

Secondary infections

reduced oral intake

________ can last up the 6 months AFTER cancer therapy

mucositis

Characteristics of grade 1 mucositis?

Soreness

Erythema

Characteristics of grade 2 mucositis

Erythema

ulcers

patient CAN swallow solid food

Characteristics of grade 3 mucositis

Ulcers with extensive erythema

Patient CANNOT swallow food, only liquid diet only

*considered severe mucositis

Characteristics of grade 4 mucositis

Mucositis to the extend that alimentation is not possible

patient cannot eat/drink

LIFE-THREATENING

*considered severe mucositis

What is the treatment for mucositis?

No prevention exists

Increased risks for infections

topical analgesics and coating agents

Trismus

Inability to open the mouth completely

Max. incisal opening <35 mm

Affects quality of life

What is the treatment for trismus?

Exercise therapy

jaw opening devices (splints)

Emphasis on prevention

Xerostomia

Subjective feeling of decreased salivary flow

Hyposalivation

Objective reduction of salivary flow

Most common long term side effect in most patients undergoing head and neck radiation

Cholinergic agonists (parasympathetic) medications for xerostomia

Pilocarpine

Cevineline

Salivary flow stimulants

Sugarless gum

biotene

Xilifresh

Sugarless hard candy

Salix lozenges

What are other dental adjuncts to help xerostomia?

Fluoride

Acid buffer

Antif-ungals

Frequent dental visits

Sialogogues

What are things that interfere with a patient's nutrition once they start receiving treatment?

Mucositis

Loss of taste

Xerostomia

Loss of interest in eating

Comprised nutrition

Osteoradionecrosis

Exposed bone in a previously irradiated area

fail to heal over 3-6 months

NO history of anti-resorptive of metastatic tumors to jaw

What is the etiology of osteoradionecrosis?

Avascular effect of radiation to the bone causing hypoxia, hypovascularity, hypocellularity

T/F: Mucositis is a common side effect of both chemotherapy and radiation therapy

(video question)

True

What is the management of osteoradionecrosis?

Conservative management (routine prophylactic cleaning with 0.12% CHX)

Antibiotics + pain medication

T/F Osteoradionecrosis can develop at any point following radiation therapy?

True

Medication-Related Osteonecrosis of the Jaw (MRONJ)

Pt has a HISTORY of anti-resorptive or anti-angiogenic medications

NO history of radiation or metastatic tumors of jaw

Exposed bone or probed bone >8 weeks

What are initiating events of Medication-Related Osteonecrosis of the Jaw (MRONJ)?

Spontaneous- 25%

Tooth extraction- 38%

Active perio disease-29%

Perio surgery- 11.2%

Dental implants- 3.4%

Apicoectomy - 0.8%

Who is more at risk for developing Medication-Related Osteonecrosis of the Jaw (MRONJ)?

Females > males

Smokers

Obesity

Bisphosphonates

Inhibit bone resorption

Bind to bone surfaces and inhibit osteoblasts-- stays in body for 13yrs

increases bone density

Decreases fractures

excreted by kidneys

IV Bisphosphonates are contain _________ and are ______x more potent than oral Bisphosphonates

nitrogen, 100

Etidronate (Didronel)

IV bisphosphonate

Pamidronate (Aredia)

IV bisphosphonate

Zoledronate (Zometa)

IV bisphosphonate

Zoledronic Acid (reclast)

IV bisphosphonate