Soft Tissue Tumors

Reactive and Benign lesions of Fibroblastic and Histiocytic OriginIrritation FibromaGiant Cell fibroma

Inflammatory Fibrous HyperplasiaInflammatory Papillary Hyperplasia

Fibrous HistiocytomaFibromatosis and Myofibromatosis

Oral Focal MucinosisPyogenic Granuloma

Peripheral Giant Cell GranulomaPeripheral Ossifying Fibroma

Benign Tunors of Fat tissue originLipoma

Benign Tumors of Neural Origin Traumatic Neuroma

Palisaded Encapsulated NeuromaSchwannomaNeurofibroma

Granular Cell TumorCongenital Epulis

Melanotic Neuroectodermal Tumor of Infancy

Benign Tumors of Vascular OriginHemangioma

Lymphangioma

Benign Tumors of Muscle OriginLeiomyoma

Rhabdomyoma

Osseous and Cartilaginous Choristomas

Malignant Tumors of Connective TissueFibrosarcoma

Malignant Fibrous HistiocytomaLiposarcoma

NeurofibrosarcomaAngiosarcoma

Kaposi’s SarcomaLeiomyosarcoma

Rhabdomyosarcoma

Metastases to Oral Soft Tissues

Irritation Fibroma (Traumatic Fibroma)

Clinical Features• Reactive hyperplasia of fibrous connective tissue

• Can occur anywhere in the oral cavity that is susceptible to constant trauma – like buccal mucosa and tongue due to biting

• Color is similar to surrounding mucosa and is pedunculated or sessile

• Symptoms present only if ulcerated

• 4th to 6th decades of life

Treatment: Conservative surgical excision

Giant Cell Fibroma

Clinical Features• Occurs at a much younger age compared to fibroma and presents as asymptomatic sessile/pedunculated nodule <1cm

• Not associated with trauma

• More than half the cases occurs on the gingiva and has a papillary surface; Mandible>Maxilla

• Similar to retrocuspid papilla

Treatment: Conservative surgical excision Recurrence is rare

Histology• Vascular and loosely arranged fibrous connective tissue

• Hallmark is the presence of large, stellate shaped fibroblasts which are multinucelated

• Rete ridges are narrow and elongated

Epulis Fissuratum (Inflammatory Fibrous hyperplasia; Denture Injury Tumor)

Clinical Features• Tumor-like hyperplasia of fibrous connective tissue that develops in association with the flange of an ill-fitting denture

• Presents as single or multiple folds of tissue in the alveolar mucosa; usually presents as two folds with denture flanges in between

• The size varies from < 1 cm to large lesions involving the entire length of the vestibule

• Appears as firm, fibrous tissue with variable ulcerations and erythema

• Most common location is facial aspect of alveolar ridges;anterior portions of jaws and older adults with female predilection

Treatment: Surgical removal and denture should be relinedor remade

Histology:• Fibrous connective tissue hyperplasia

• Overlying epithelium is hyperkeratotic and shows hyperplasia of rete ridges

• Pseudoepitheliomatous hyperplasia

• Ulceration and chronic inflammation is also seen frequently

Epulis Fissuratum

Inflammatory Papillary Hyperplasia• Reactive lesion that most commonly develops under a denture

an ill-fitting denturepoor oral hygienewearing the denture 24 hours

• Usually occurs on the hard palate beneath a denture base

• Starts at the palatal vault but advanced lesions can cover the entire palate

• Candidiasis can also be seen associated with the lesion

• Mucosa is pebbly or papillary and appears erythematous

Treatment: Removal of denture Surgical removal with altering the denture

Histology• Papillary growths surfaced by hyperplastic startified squamous epithelium

•Pseudoepitheliomatous hyperplasia

• Chronic inflammation

Inflammatory Papillary Hyperplasia

Fibrous Histiocytoma

• Group of tumors which have both fibroblastic and histiocytic differentiation

• Most common in the skin called dermatofibroma

• Oral cavity – rare; buccal mucosa and vestibule

• Middle aged and older adults

• Painless nodular mass of varying size

Treatment: Local surgical excision

Fibromatosis and Myofibromatosis• Group of fibrous proliferations that have intermediate biologic behavior

• Named based on clinicopathologic features: juvenile aggressive fibromatoses, extrabdominal desmoids

• Myofibromatosis is similar but less aggressive

• Painless mass occurring in children or young adults

• Most common site: Paramandibular soft tissues

•Tumor can grow to considerable size and can cause significant facial disfigurement

• Destruction of adjacent bone can be seen in radiographs

Histology:• Cellular proliferation of spindle-shaped cells arranged in fascicles

• Poorly circumscribed and infiltrates adjacent tissues

• Cells should be uniform with NO pleomorphism and hyperchromatism

Treatment: Wide excision 23% recurrence rate

Metastasis does not occur

Fibromatosis and Myofibromatosis

Myofibroma (Myofibromatosis)

• Rare spindle cell neoplasm that consists of myofibroblasts

• The multicentric disease affects infants and young children and this is called myofibromatosis

• Predilection to the head and neck; occurs in the first 4 decades of life with most lesions occurring in neonates and infants

• Most common oral site is the mandible followed by lips, cheek, and tongue

• Painless mass in dermis or subcutaneous tissue and intrabony cases are radiolucent

Treatment: Local excision; can spontaneously regress; lesionsaffecting vital or visceral organs are aggressive and can be fatal

Oral Focal Mucinosis

• Uncommon tumor-like mass of unknown cause.

• Maybe due to overproduction of hyaluronic acid • Commonly seen in young adults with a 2:1 female-to-male ratio

• Most commonly seen in the gingiva followed by hard palate

• Sessile painless nodule of normal color

• Size varies from a few mm to 2 cm

Histology: Well-demarcated loose myxomatous tissuesurrounded by dense collagenous tissue

Treatment: Surgical excision and recurrence is rare

Pyogenic Granuloma• Common tumor-like growth of the oral cavity

• Exuberant response to irritation or trauma; periodontal irritation could be a major source

• Smooth or lobulated pedunculated mass which appears pink to red in color and is commonly ulcerated

• Range from a few mm to several cm – hormone dependent• GINGIVA however other sites also affected

• Most common in children and young adults with females>males

• Develops in pregnant women during first trimester and increases through 7th months - Pregnancy tumors; Some will resolve after delivery

Histology• In spite of name, not a true granuloma

• Vascular proliferation that resembles granulation tissue

• Surface is usually ulcerated

• Mixed inflammatory infiltrate

• Younger lesions are very vascular, but older lesions mature and are fibrous

Treatment: Conservative surgical excision. Recurs if incompletely excised; Irritation also

has to be removed.

Pyogenic Granuloma

Peripheral Giant Cell Granuloma

• Reactive lesion due to local irritation or trauma

• Resembles central giant cell granuloma

• GINGIVA or edentulous alveolar ridge

• Red or reddish-blue nodular mass which is usually < 2 cm

• Appearance similar to pyogenic granuloma

• 5th to 6th decades; F > M (60% occurs in females)

• Mandible > Maxilla

• Although occurs in soft tissues a “cupping” resorption of bone

Histology:• Proliferation of multinucleated giant cells in a background of plump ovoid and spindle-shaped cells

• Abundant hemorrhage is observed

Treatment:• Local surgical excision down to the underlying bone• Scaling of the adjacent teeth of any source of irritation• Rarely, lesions similar to this are seen in hyperparathyroidism (however these are mostly intraosseous)

Peripheral Giant Cell Granuloma

Peripheral Giant Cell Granuloma

Peripheral Ossifying Fibroma• Reactive growth of the gingiva with uncertain histogenesis

• Believed to be a matured pyogenic granuloma that ultimately undergoes calcifications

• It does not represent central ossifying fibroma

• Occurs exclusively on the GINGIVA

• Nodular mass that is either pedunculated or sessile usually of the interdental papillae and appears red to pink and frequently ulcerated

• Younger adults and teens with F > M

• Maxilla > Mandible; >50% cases occur in the incisor/canine area

Histology: • Fibrous proliferation associated with formation of mineralized product

• The surface if ulcerated, shows a fibrinopurulent membrane

• The mineralized component varies from bone, cementum-like material or dystrophic calcifications Treatment: • Local surgical excision down to the periosteum

• Scaling of the adjacent teeth to remove irritants

• Recurrence rate of 16%

Peripheral Ossifying Fibroma

Peripheral Ossifying Fibroma

The Four “P”s

• Peripheral Fibroma

• Pyogenic Granuloma

• Peripheral Giant Cell Granuloma

• Peripheral Ossifying Fibroma

Lipoma

• Benign tumor of fat

• It represents the most mesenchymal tumor, however most of them occur in the trunk and extremities – Head and Neck are less common

• Oral lipomas are soft nodular masses that is sessile or pedunculated with yellow color

• Asymptomatic and present for several years

• Buccal mucosa and vestibule are the most common sites

• >40 years; female = male

Treatment: conservative local excision

Traumatic Neuroma

• Reactive proliferation of neural tissue after damage to nerve bundle

• Smooth nodules most common in mental foramen, tongue and lower lip with a history of trauma; intraosseous lesions appear as radiolucencies

• Any age but mostly middle-age, with F>M

• Hallmark is PAIN which could be intermittent or constant and mild or severe; Mental nerve neuromas are painful especially with denture flange impingement

Histology: Haphazard proliferation of mature, myelinatednerve bundles within a fibrous connective tissue

• Mild chronic inflammation is also seen sometimes

Treatment: Surgical excision along with a small portion of theinvolved nerve; low recurrence rate

Traumatic Neuroma

Palisaded Encapsulated Neuroma

• Benign neural tumor common in the head and neck area

• Trauma is considered as a major etiological factor

• Face: 90% of cases with majority occurring on the nose and cheek• Oral cavity: hard palate and maxillary labial mucosa

• Smooth, PAINLESS nodules; More common in adults; F=M

Histology: Well-circumscribed and encapsulated with interlacingfascicles of spindle cells (Schwann cells); wavy nuclei with nomitotic activity or pleomorphism; parallel oriented cells

Treatment: Conservative surgical excision

Palisaded Encapsulated Neuroma

Schwannoma (Neurilemoma)

• Benign neural neoplasm of Schwann cell origin

• Relatively uncommon, however 25-48% of all cases occur in the Head and Neck area

• Usually painless; slow-growing that arises in association with a nerve trunk; Asymptomatic and pushes the nerve aside

• Younger and middle-aged adults

• Tongue is the most common location

• Intraosseous appears as unilocular or multilocular radiolucency in posterior mandible

• Pain and paresthesia seen in intrabony tumors

Histology: Encapsulated tumor with varying amounts of Antoni A and Antoni B cellsAntoni A: Streaming fascicles of spindle-shaped Schwann cells;These cells are often palisaded around acellular eosinophilicareas called Verocay bodies (which are reduplicated basementmembrane and cytoplasmic processes)Antoni B: is less cellular and organizedDegenerative changes are seen in older lesions•Treatment: Surgical excision

Schwannoma (Neurilemoma)

Neurofibroma

• MOST COMMON type of peripheral nerve tumors arising from a mixture of Schwann cells and perineural fibroblasts

• Can be solitary or associated with Neurofibromatosis

• Solitary are more common and present as slow-growing, soft, painless nodule, most common in the skin

• Oral cavity lesions are seem mostly in tongue and buccal mucosa

• Intraosseous lesions also seen as poorly defined unilocular or multilocular radiolucencies

Histology: Not well-demarcated and consists of interlacing bundles of spindle-shaped cells that exhibit wavy nuclei

Numerous mast cells are present

Treatment: local surgical excision; If multiple lesions are present, patients should be evaluated for Neurofibromatosis

Neurofibroma

Granular Cell Tumor

• Benign tumor that shows predilection to oral cavity

• Derived from Schwann cells or neuroendocrine cells

• Dorsal surface of TONGUE – most common site; followed by buccal mucosa

• 4th to 6th decades of life and 2:1 (F:M) ratio

• Asymptomatic sessile nodule that is <2 cm and appears pink or yellow in color

• Usually solitary but multiple sometimes seen in black patients

Treatment: Conservative local excision

Histology:• Large polygonal cells with abundant pale eosinophilic, granular cytoplasm and pale nuclei

• Cells arranged in sheets

• Lesion is not encapsulated and appears to infiltrate into surrounding tissues

• Overlying epithelium shows acanthosis and pseudoepitheliomatous hyperplasia

Treatment: Conservative local excision

Granular Cell Tumor

Granular Cell Tumor

Congenital Epulis

• Occurs exclusively in the alveolar ridge of the newborn

• Histologically similar to granular cell tumor, but ultrastructurally and immunohistochemical different

• Pink-red smooth surfaced mass on the alveolar ridge of newborns

• Size varies from small to over 7.5 cm with multiple tumors also occurring in 10% of cases

• Maxilla > Mandible in the area of lateral incisor and canine

• STRIKING FEMALE PREDILECTION (90% cases)

Treatment: Surgical excision; spontaneous regression also seen

Melanotic Neuroectodermal Tumor of Infancy

• Generally considered a benign tumor despite rapid growth of neural crest origin

• Rare pigmented neoplasm that occurs during the first year of life

• Striking predilection for the anterior maxilla (almost 2/3 of cases)

• Occurs as a rapidly expanding mass that is black or blue in color

• Destroys bone and displaces associated developing teeth

• Can also occur in skull, mandible, brain and testis

Melanotic Neuroectodermal Tumor of Infancy

Histology: Biphasic population of cells that form nests, tubules andalveolar structures within a dense connective tissue

The 2 cell types: cuboidal epithelioid cells and neuroblastic

Melanotic Neuroectodermal Tumor of Infancy

Melanotic Neuroectodermal Tumor of Infancy

Lab Tests: Vanillylmandelic acid (VMA) as in otherneural crest lesions

Treatment: Surgical removal Rapid growth and destruction despite being called benign 15% recurrence rate 6% behave like malignancy and metastasize

Hemangioma and Vascular Malformations

Hemangiomas are considered to be benign tumors of infancythat are characterized by a rapid growth phase with endothelialcell proliferation, followed by gradual involution

Vascular malformations are structural anomalies of bloodvessels without endothelial proliferation

Most hemangiomas are not recognized at birth, but arise duringthe first 8 weeks later of life

Vascular malformations are present at birth and persistthroughout life

Hemangioma

Most common tumors of infancy

More common in females (3:1)

Most common in Head and Neck (60% of cases)

Mostly occurs as single lesions

Red/blue lesions that occur in skin, lips, tongue and buccalmucosa; The lesion blanches when compressed

Intraosseous lesions also occur – Mandible > Maxilla andoccurs as multilocular radiolucency

Vascular Malformations

Present at birth and persist throughout life

PORT-WINE STAINS are common capillary malformationoccurring most commonly on the face particularly in the areaof the trigeminal nerve

Port-wine stains are pink or purple macules that growsproportionally with the patient; Older patients have darkerlesions and becomes nodular

HistologyCellular Hemangioma

Capillary Hemangioma

Cavernous Hemangioma

Treatment: Most congenital lesions will involute (“WatchfulNeglect”)Surgical removal and sclerotherapy with 95% ethanol

Hemangioma

Sturge-Weber Syndrome

Hamartomatous vascular proliferation of the face and brain

Dermal capillary malformation (Port wine stain) in a unilateraldistribution along one or more segments of trigeminal nerve

Leptomeningeal angiomas involving the ipsilateral cortexrevealing “tramline” calcifications on X-rays

Mental retardation and convulsions

Eye involvement: glaucoma and vascular malformations

Intraoral: Vascular involvement of the ipsilateral oral mucosa

Lymphangioma

Benign hamartomatous tumors of lymphatic vessels

Predilection to the head and neck with 50 – 75% occurring

Three types: capillary; cavernous and cystic lymphangiomas

Cavernous lymphangiomas are most common in oral cavity

Most frequent site in the oral cavity - anterior 2/3 of the tonguewhere it causes MACROGLOSSIA

Pebbly surface resembling cluster of translucent vesicles(similar to frog eggs)

Cystic Hygroma (Cystic Lymphangiomas)

Most commonly occur in the neck and axilla

Cervical lymphangiomas are most common in theposterior triangle and are soft, fluctuant masses

Occasionally could extend into the mediastinum orupward into oral cavity ; could also extend into theanterior triangle resulting in respiratory difficultiesor dysphagia

Histology

TreatmentIntraoral: Excision and prognosis is good; recurrence does occurCystic: Well circumscribed and have lower recurrence rate

SCLEROSING AGENTS DO NOT WORK AS IN HEMANGIOMAS

Leiomyoma

Benign neoplasms of smooth muscle

Most of these have origin in the vascular smooth muscle

3 types: SOLID, VASCULAR AND EPITHELIOID

75% of oral cases are vascular leiomyomas

Can occur at any age; slow-growing mucosal nodule thatoccasionally can be PAINFUL

Commonly seen in lips, tongue, palate and cheek

Local surgical excision

Rhabdomyoma

Benign neoplasm of skeletal muscle

Adult and Fetal types

Adult: Middle-aged and older patients; M>FIntraoral lesions: FOM, soft palate and base of the tongueNodule or mass that grows for many years

Fetal: Young children with a male predilection; face andperiauricular region

Treatment: local surgical excision

Osseous and Cartilagenous Choristomas

Choristoma is a tumorlike growth of microscopically normaltissue in an abnormal location

Bone, cartilage or both

TONGUE (85% of cases); especially posterior tongue near theforamen cecum

Gagging or dysphagia are common symptoms

Histology: well-circumscribed mass of dense lamellar boneor mature cartilage

Treatment: Surgical excision

DENTAL CHORISTOMA: THE FIRST CASE OF ECTOPICDEVELOPING TOOTH IN THE TONGUE

Soft Tissue Sarcomas

Account for less than 1% of cancers in the oral and maxillofacial area

FibrosarcomaMalignant fibrous histiocytoma

LiposarcomaMalignant peripheral nerve sheath tumor

Olfactory neuroblastomaKaposi’s sarcomaLeiomyosarcoma

RhabdomyosarcomaSynovial sarcoma

Alveolar soft part sarcoma

Rhabdomyosarcoma

Malignant neoplasm of skeletal muscle origin

MOST COMMON SOFT TISSUE SARCOMA IN CHILDREN

HEAD AND NECK IS THE MOST SITE (40% of cases)

Primarily occurs in the first decade, teenagers and young adults

60% of cases occurs in males

Painless infiltrative mass that grows rapidly

Orbit > nasal cavity and nasopharynx

Intraoral: PALATE

3 Histologic TypesEmbryonal, Alveolar and Pleomorphic

The head and neck cases are either embryonal or alveolarEmbryonal: First 10 years of life and 60% of cases

Alveolar: occurs between 10-25 years and accounts for20% - 30% of cases

Treatment: Local surgical excision followed by multiagentchemotherapy (vincristine, actinomycin D andcyclophosphamide)Radiation therapy

Prognosis: 5 year survival rate is 60% to 70%

Metastases to Oral Soft Tissues

Uncommon representing 1% of all oral malignancies

Oral metastases can occur in bone and soft tissues

Lymphatic and blood-borne route

Batson’s plexus: a valveless vertebral venous plexus thatmight allow retrograde spread of tumor cells, bypassingfiltration through the lungs

GINGIVA followed by the tongue

Nodular masses often resembling hyperplastic or reactivegrowths with occasional ulcerations and loosening ofadjacent teeth

Oral metastases is more common in malesMore common in middle-aged and older adultsMale: Primary tumor is seen in lung cancerFemale: Primary tumor is seen in breast cancer (25% of cases)

In most cases, the primary tumor is known before themetastases is discovered; HOWEVER IN SOME CASES THEORAL LESION IS THE FIRST SIGN OF MALIGNANT DISEASE

Histology is similar to the primary tumorMOST CASES TO ORAL CAVITY ARE CARCINOMAS ANDNOT SARCOMAS

Treatment: Poor prognosis; palliative management

Metastases to Oral Soft Tissues