ORAL PATHOLOGY II: CONNECTIVE TISSUES(NEURAL NEOPLASMS)

NEURAL NEOPLASMS

 Granular cell tumors

 Schwannoma

 Neurofibroma

 Mucosal neuroma of Mutiple Endocrine Neoplasia

Type III

 Palisaded encapsulated neuroma (Solitary

circumscribed neuroma)

 Malignant peripheral nerve sheath tumor

 Olfactory neuroblastoma

GRANULAR CELL TUMOR

Formerly called granular cell myoblastoma

GRANULAR CELL TUMOR

Uncommon benign, nonrecurring submucosal neoplasm of Schwann cells

GRANULAR CELL TUMOR

Clinical features:

 Presents as an

uninflamed

asymptomatic mass less than 2 cm in diameter

with a yellowish surface

 Seen in a wide age range from children to the elderly (peaks in middle adulthood)

 More common among

GRANULAR CELL TUMOR

Most common

intraoral location:

tongue

GRANULAR CELL TUMORS

Histopathology:

 Unencapsulated sheets

of large polygonal cells

that have a grainy or

granular cytoplasm

 Overlying epithelium

may exhibit

pseudopepitheliomatous

hyperplasia

 Mmicroscopically

mimics carcinoma

GRANULAR CELL TUMORS:Congenital gingival granular cell tumor

 AKA congenital epulis

 Related lesion believed to be the infant

counterpart

GRANULAR CELL TUMORS: Congenital gingival granular cell tumor

 Occurs on anterior gingiva (maxilla more common than the mandible) as an uninflamed, pedunculated, or broad-

based mass

 Affects the maxilla more

commonly than the mandible

 More common among

GRANULAR CELL TUMORS:Congenital gingival

granular cell tumor

 Histopathologically: No

pseudoepitheliomatous

hyperplasia

GRANULAR CELL TUMORS

Treatment:

 Surgical excision

 Does not recur

SCHWANNOMA

AKA neurilemmoma

SCHWANNOMA

Benign neoplasm of

Schwann cells of the

neurilemmal or nerve

sheath

SCHWANNOMA

Clinical features:

 Presents as an

asymptomatic encapsulated

submucosal lump (bony

lesions however may

present with pain or

paresthesia

 Usually slow growing but

may undergo a sudden

increase in size

 Solitary; not syndrome

related

 Affects patients of any age

SCHWANNOMA

Clinical features:

 Site of predilection:

tongue

SCHWANNOMA

Radiographic feature: if it occurs in bones, it

appears as a well-defined radiolucency with a

corticated periphery

SCHWANNOMA

Histopathology: encapsulated lesion where

spindles cells have two different patterns:

Antoni A tissue

Antoni B tissue

SCHWANNOMA: Antoni A tissue

 Spindle cells

organized in

palisaded whorls and

waves.

 Surrounding an

acellular eosinophilic

zone called Verocay

bodies

SCHWANNOMA: Antoni B tissue

 Spindle cells are

haphazardly

distributed in a

delicate fibrillar

microcystic matrix

SCHWANNOMA: Ancient schwannoma:

 Microscopic variant

 Represents

degenerative changes

in a long-standing

schwannoma

 Fibrosis, inflammatory

cells, and hemorrhage

may be seen

SCHWANNOMA

Treatment:

 Surgical excision

 Recurrence is unlikely

NEUROFIBROMA

A common peripheral

nerve neoplasm

Arises from Schwann

cells and perineural

fibroblasts.

Cause: unknown

NEUROFRIBROMA

Inherited as an

autosomal dominant

trait

May exist as solitary

lesions or as part of

neurofibromatosis

type I or von

Recklinghausen’s

disease

NEUROFIBROMA:Solitary neurofibroma

 Presents as an

uninflamed

asymptomatic,

submucosal mass

 Affects any age

Most common intraoral

sites of solitary neurofibroma

 Tongue

 Buccal mucosa

 Vestibule

Solitary neurofibroma Treatment

surgical excision

NEUROFIBROMA: Von recklinghausen’s

disease

AKA

neurofibromatosis 1

NEUROFIBROMA: Von recklinghausen’s

disease

Clinical features:

 Multiple neurofibromas

- Potential for

disfigurement and

malignant

transformation in 5-15%

of pxs

NEUROFIBROMA: Von recklinghausen’s

disease

 Clinical features:

 Six or more café-au-

lait macules → brown

macules on the skin

typically measuring

>1.5 cm in adults, and

>0.5 cm in children

NEUROFIBROMA: Von recklinghausen’s

disease

 Clinical features:

 Axillary freckling

(Crowe’s sign)

 Iris freckling (Lisch

spots)

NEUROFIBROMA: Von recklinghausen’s

disease

Intraoral

manifestations:

 Neurofibromas

 Enlarged fungiform

papillae

NEUROFIBROMA: Bone lesions:

 Cortical erosion from

adjacent soft tissue

tumors or medullary

resorption from

intraosseous lesions.

 Arises intraorally near

the mandibular nerve

resulting in pain or

paresthesia

NEUROFIBROMA: Bone lesions:

 Radiographic feature:

BLUNDERBUSS

FORAMEN - flaring of

the inferior alveolar

foramen

NEUROFIBROMA

Histopathology:

spindle-shaped cells,

with fusiform or wavy

nuclei in a myxoid

tissue matrix

NEUROFIBROMA:Plexiform

neurofibroma

 Microscopic variant

 Extensive interlacing

masses of nerve tissue

are supported by a

collagen matrix

NEUROFIBROMA

Treatment:

 Solitary neurofibroma

 Surgical excision

 Recurrence is rare

 Neurofibromatosis 1

 Excision is impractical due to the multiple lesions

MUCOSAL NEUROMAS OF MULTIPLE

ENDOCRINE NEOPLASIA TYPE III

MEN III is an autosomal dominant condition

that is potentially fatal

Etiology: defect in neuroectodermal tissue

MUCOSAL NEUROMAS OF MULTIPLE

ENDOCRINE NEOPLASIA TYPE III: Mucosal neuromas

 Clinical features:

 Prominent feature

 May be the first sign of the condition - early detection is very important in establishing the diagnosis or giving attention to other

syndrome components

 Appear early in life as early in life as small, discrete nodules on the conjunctive,labia, larynx or oral cavity

MUCOSAL NEUROMAS OF MULTIPLE

ENDOCRINE NEOPLASIA TYPE III

Mucosal neuromas Intraoral sites:

 Tongue

 Lips

 Buccal mucosa

MUCOSAL NEUROMAS OF MULTIPLE

ENDOCRINE NEOPLASIA TYPE III: Medullary carcinoma

of the thyroid

Clinical features

 Progressive malignancy

that is locally invasive

and metastasizes to local

lymph nodes and distant

organs

 Has a 50% 5-year survival

rate

MUCOSAL NEUROMAS OF MULTIPLE

ENDOCRINE NEOPLASIA TYPE III: Pheochromocytoma

of the adrenal

Clinical features:

 Benign neoplasm

that produces

catecholamines

causing significant

hypertension and

other cardiovascular

conditions

MUCOSAL NEUROMAS OF MULTIPLE

ENDOCRINE NEOPLASIA TYPE III

Histopathology of neuromas: serpiginous

bands of nerve tissue with proliferating axons

surrounded by normal connective tissue

MUCOSAL NEUROMAS OF MULTIPLE

ENDOCRINE NEOPLASIA TYPE III

Treatment for neuromas:

 Surgical excision

 No recurrence potential

PALISADED ENCAPSULATED

NEUROMA

AKA solitary

circumscribed

neuroma

PALISADED ENCAPSULATED

NEUROMA

A dome-shaped oral

tumor of neural origin

Site of predilection:

palate

PALISADED ENCAPSULATED

NEUROMA

Histopathology:

encapsulated tumor

composed of Schwann

cells exhibiting a

fascicular pattern with

nuclear palisading

PALISADED ENCAPSULATED

NEUROMA

Treatment:

 Surgical excision

 Does not recur

MALIGNANT PERIPHERAL

NERVE SHEATH TUMOR

 AKA Neurosarcoma

MALIGNANT PERIPHERAL NERVE SHEET TUMOR

 Rare malignancy that develops either from a

preexisting neurofibroma or de novo.

 Cell origin: Schwann cell

MALIGNANT PERIPHERAL

NERVE SHEATH TUMOR: SOFT TISSUES

Clinical features:

 In soft tissues:

presents as an

asymptomatic

expansile mass

MALIGNANT PERIPHERAL

NERVE SHEATH TUMOR: IN BONE

Clinical features:

 In bone:

 Site of predilection:

inferior alveolar nerve

 Presents as a dilation of

the mandibular canal or as

a diffuse lucency

accompanied by pain or

paresthesia

MALIGNANT PERIPHERAL

NERVE SHEATH TUMOR

Histopathology:

abundant spindle cells

with variable numbers

of abnormal mitotic

figures

MALIGNANT PERIPHERAL

NERVE SHEATH TUMOR

Treatment:

 Wide surgical excision

 Recurrence common

 Metastases frequently seen

Prognosis: fair to good

OLFACTORY

NEUROBLASTOMA

AKA

esthesioneuroblastoma

OLFACTORY NEUROBLASTOMA

A rare malignant lesion

arising from olfactory

tissue found in the

superior part of the

nasal cavity

OLFACTORY

NEUROBLASTOMA

 Clinical features:

 Presents as polyps in the

roof of the nasal cavity

 Results in epistaxis,

rhinorrhea, nasal

obstruction, a

nasopharyngeal mass or

an invasive maxillary

sinus lesion.

 Common among young

adults

OLFACTORY

NEUROBLASTOMA

Histopathology:

 Small, undifferentiated,

round cells with little

visible cytoplasm

 Compartmentalization

and pseudorosette and

rosette formations are

often seen

OLFACTORY

NEUROBLASTOMA

Treatment:

 Surgery or radiation

 Recurrence is common

 May metastasize to local nodes or lung

MUSCLE NEOPLASMS

Leiomyoma and leiomysarcoma

Rhabdomyoma and rhabdomyosarcoma

LEIOMYOMA

Rare benign neoplasm

of smooth muscle

origin

Most commonly arises

in the muscularis layer

of the gut and in the

body of the uterus

LEIOMYOMA: Oral leiomyoma

 Rare

 Present as slow-

growing, asymptomatic

submucosal masses

with a diameter of 1 to

2 cm

 Affects any age

 Sites of predilection:

tongue, hard palate, or

buccal mucosa

LEIOMYOMA

Treatment:

 Surgical excision

 Does not recur

LEIOMYOSARCOMA

Rare malignant

neoplasm of SMOOTH MUSCLE ORIGIN

Most commonly arise

in the retroperitoneum,

mesentery, omentum,

or subcutaneous and

deep tissues of the

limbs

LEIOMYOSARCOMA

Oral leiomyosarcoma

Reported in all age-

groups and most

intraoral regions.

LEIOMYOSARCOMA

Treatment:

 Wide surgical excision

 Metastasizes to lymph nodes and lung

RHABDOMYOMA

Very rare, benign

neoplasm of skeletal

muscle origin

RHABDOMYOMA

Have a predilection for

the soft tissues of the

head and neck.

RHABDOMYOMA

Common intraoral

sites:

 Floor of the mouth

 Soft palate

 Tongue

 Buccal mucosa

RHABDOMYOMA

Clinical features:

Presents as an

asymptomatic, well-

defined submucosal

mass.

Affects children and

adults (mean age of

50

RHABDOMYOMA VARIANTS

ADULT TYPE AND FETAL TYPE

RHABDOMYOMA- ADULT TYPE

neoplastic

cells closely mimic their

normal counterpart

RHABDOMYOMA: FETAL TYPE

cells are

elongated, less

differentiated and

exhibit fewer cross-

striations and may be

confused with

rhabdomyosarcoma

RHABDOMYOMA

Treatment:

 Excision

 Recurrence is rare

RHABDOMYOSARCOMA

 Rare malignant

neoplasm of skeletal

muscle origin

 When seen in the head

and neck region, it is

more common among

children

 When seen outside the

head and neck, it is more

common among adults

RHABDOMYOSARCOMA

 Most common intraoral sites:

 Tongue

 Soft palate

RHABDOMYOSARCOMA

 Presents as a rapidly

growing mass that may

cause pain or

paresthesia if there is

jaw involvement

RHABDOMYOSARCOMA: Three microscopic

variant:

1. Embryonal

2. Alveolar

3. Pleomorphic

RHABDOMYOSARCOMA: Embryonal Variant

 Seen in children

 Type most commonly seen

in the head and neck

region

 Composed of primitive

round cells with rare

striations

RHABDOMYOSARCOMA: EMBRYONAL SUBTYPES

Three microscopic

variant:

2 subtypes:

 Spindle cell type

 Botryoid type

 Prognosis: excellent

RHABDOMYOSARCOMA: Alveolar

 Composed of round cells

but in a

compartmentalized

pattern.

 Predominant in children

RHABDOMYOSARCOMA: Pleomorphic

 Most well differentiated

 Composed of strap or

spindle cells that often

exhibit cross-striations

 Seen in adults

RHABDOMYOSARCOMA

Treatment: combination of surgery, radiation

and chemotherapy