Soft Tissue Tumors- Chapter 12

Objectives: The student clinician should be able to describe the most common location for various soft tissue tumors
and provide the rationale for occurrence in that location.

Soft tissue tumors are derived from mesenchymal proliferations. The majority of the oral soft tissue masses are reactive
processes. That is, irritation, commonly low-grade and chronic, stimulates cellular hyperplasia. Recall from your general
pathology lectures that one method of cellular adaptation is hyperplasia. Hyperplasia is a response to increased
functional demand, neural or hormonal signals, or irritation.
This should not be confused with neoplasia – a cellular proliferation that has escaped growth control. Neoplastic lesions
are discussed under the benign and malignant categories.

 Soft tissue masses:

o Reactive-common intraorally due to bite trauma and hygiene practices (fibroma, IFH, IPH, the 4 P’s of
the gingiva)
o Benign-slow-growing, non-metastasizing, mesenchymal neoplasms, remain localized
o Malignant-sarcomas, most often metastasize hematogenously (lungs, liver, bone), these are rare
intraorally.
 Often the masses present as dome-shaped elevations that are mucosal-colored, but shape, color, and texture
depend upon the tissue that is proliferating. The following can cause dome-shaped swellings:
o Cyst or cyst-like entities (have a fluctuant feel)
o Benign proliferations of the underlying connective tissue
 Collagen, fat, vessels, nerve, muscle
o Structures invested by connective tissue
 Minor salivary glands, sebaceous glands

The cysts, cyst-like entities, and glands have or will be discussed in other lectures, and you should recognize the tissues
native (anatomically distinctive) to an area that can give rise to masses.
Irritation Fibroma – can occur anywhere in the oral cavity

 Most common intraoral soft tissue mass, reactive lesion in response to local trauma
 Adults: lips, tongue, buccal mucosa along the bite line (most common location)
 Pyogenic granulomas can mature over time and become fibromas
 Dome-shaped, mucosal colored, soft or “rubbery”
o Well circumscribed, exophytic, pink, smooth, buccal mucosa, 2-3cm
 Histopathology: hyperkeratotic surface epithelium with or without rete peg formation
 Abundant, mature loose or dense, irregular interlacing collagen with few scattered spindle-shaped fibroblasts,
largely hypocellular, scant, if any, chronic inflammatory cells
 Leaf fibromas are common under poorly fitted dentures
 Treatment (Tx): surgical excision (continuously grow, can make hygiene difficult)

Giant Cell Fibroma

 Fibrous tumor that is not associated with traumatic irritation (unlike traumatic fibroma)
 Young persons (developmental?), tongue, mandibular lingual gingiva, domed, mucosal-colored mass, soft
 Has a rough, dented, puckered appearance
 Retrocuspid Papillae – seen on lingual gingiva of mandibular canine
 Histo: few multinucleated fibroblasts in close proximity to epithelium
 Elongated rete pegs, stellate, “manta ray” shaped fibroblasts, abundant collagen
 Tx: local excision – do not regress due to bulk of connective tissue
Inflammatory Fibrous Hyperplasia (Epulis Fissuratum)

 Associated with ill-fitting denture (over-extended flanges, chronic trauma)

 Occur in maxillary or mandibular vestibule, redundant folds of “rubbery” tissue
 Linear troughs may show healing ulcers
 Histo: hyperplastic surface epithelium overlying mature fibrous connective tissue, variable chronic inflammatory
cells (lymphoplasmacytic infiltrate), dysplastic or metaplastic calcification, osteoid or bone formation
 Tx: excision, remake dentures

Inflammatory Papillary Hyperplasia (IPH)

 Occurs on palatal vault under full or partial denture or appliance

 Cobblestone or reddish “mushrooms”
 Reactive lesion associated with 24hr. denture wear, candidiasis, high palatal vault
 Histology: domed nodules, 50:50 epithelium: connective tissue
 Hyperplastic epithelium, pseudoepitheliomatous hyperplasia(PEH
 Dense, well-vascularized connective tissue, inflammatory cells, rare fungal hyphae are found
 Tx: caught early can remove denture for relief
o excision-blade, acrylic bur, electro-surgery, or laser; remake denture
Fibrous Histiocytomas
 Group of true neoplasm, fibrohistiocytic proliferation, origin from “facultative” fibroblasts
 Most common on skin (dermatofibroma), firm feeling
 Histo: swirling pattern of fibrous CT and abundant histiocytes (storiform or “mat-like” pattern)
 Tx: local excision

Fibromatoses

 Aggressive benign masses, young persons, firm feeling, painless

 May occur centrally or in soft tissue over bony prominences, can undergo spontaneous regression
 Very cellular, mitotically active, rapid growth, pseudosarcomatous
 Desmoplastic fibroma, myofibromatosis, nodular fasciitis
 Tx: wide excision, ~20% recurrence
Gingival growths: The three P’s

 Pyogenic granuloma (reactive)

 Peripheral giant cell granuloma (reactive)
 Peripheral ossifying fibroma (reactive)
 All appear to be more common in females. Why? – Progesterone

1. Pyogenic Granuloma
a. Common, exuberant (granulation) tissue response to irritation, 75% of oral lesions on gingiva
b. Smooth, lobulated, can be pedunculated, “spongy” pink-red to purple, common in pregnant women
c. Mature into fibromas (vascularity disappears)
i. “epulis granulomatosum” when associated with extraction socket
d. Histo: abundant immature, plump endothelial-lined capillaries in delicate, myxoid connective tissue
e. Tx: excision; in pregnancy, defer until after delivery

2. Peripheral Giant Cell Granuloma (only in tooth bearing area of the jaw)
a. Relatively common, reactive lesion caused by trauma
b. Occurs exclusively on gingiva or edentulous alveolar ridge
c. Bimodal distribution – adults (30’s) and kids in mixed dentition, firm, typically anterior jaw
d. Females MX = MD, anterior to molars
e. Reddish-blue, domed swelling, may loosen teeth (cupping resorption)
f. Histo: abundant, evenly distributed, mono-& multi-nucleated giant cells
g. Variably-sized ovoid and spindled fibroblasts, abundant dilated capillaries, extravasated RBCs,
hemosiderin
h. Tx: surgical excision
 3. Peripheral Ossifying Fibroma (only in tooth bearing area of the jaw)
a. Occurs exclusively on the gingiva
b. F>M, mucosal-colored or pale, may be ulcerated
c. Periosteal or PDL origin (pluripotential mesenchymal cells) cells may be osteogenic
d. Firm to hard; fixed to surrounding tissue; may be radiopaque (collagen begins to ossify)
e. Histo: abundant, plump fibroblasts; hyalinized collagen; “osteoid” from fibroblasts; rarely, mature bone
is present
f. Tx: excision down to periosteum

Lipoma
 Benign tumor of fat
 Adults, equal sex distribution
 Neck, orally – 50% in buccal mucosa, trunk, extremities
 Sessile, yellowish, soft to doughy feel
 Histo: lobules of mature fat with variable amounts of connective tissue (fibrolipoma)
 Tx: excision, recurrence is rare

Traumatic Neuroma
 Reactive proliferation of neural tissue after damage of a nerve bundle
o Axonal ends proliferate to “heal” or re-find distal segment
 Most occur in adults, common in seizure disorder (coordination difficulty)
 Painful; most common in: mental foramen (a source of pain in denture wearers), tongue, lower lip
 Histo: abundant, variably-sized, proliferating nerve fascicles
 Tx: surgical excision
Neurilemoma (Schwannoma)
 Any age, non-painful, benign peripheral nerve sheath tumor
 Firm, slow growing, smooth-surfaced nodule
o Potentially resembles a mucocele
 Dorsal tongue, H and N, Inferior alveolar nerve
 Histo: discrete encapsulated nodule, Antoni A Verocay bodies), Antoni B tissue
 Tx: surgical excision, rare recurrence

Neurofibroma
 Benign nerve sheath tumor in the peripheral nervous system. Usually found in NF1.
 Adults, M=F, assoc. with neurofibromatosis (von-Recklinghausen’s disease – NF1)

 Solitary or multiple, tongue, buccal mucosa

 Lisch nodules – affects iris of patient eye
 Well-demarcated soft or firm nodules orally, feature smooth border
 Histo: varied, diffuse or demarcated, spindle cells with wavy nuclei, neurites variable, abundant fibrous
connective tissue
 Tx: excision
Multiple Endocrine Neoplasia Type III or 2 B
 Genetic disease that causes multiple tumors on the mouth, eyes, and endocrine glands
o Most severe type of MEN
 Mucosal neuromas “lips, tongue, buccal mucosa, gingiva, palate) first sign of disease
 AD, pheochromocytomas (cause hypertension), medullary carcinoma of the thyroid (life-threatening)
 Marfanoid body habitus, thick lips
 MEN III (2B) also called Schimke’s, Williams-Pollack, and Gorlin-Vickers syndrome

Melanotic Neuroectodermal Tumor of Infancy

 Rare, blue-black lesion of anterior maxilla; 1st year of life; bone destruction; most are benign
o Can behave like a malignancy, purely a pediatric disease
 Radiograph may show “floating teeth”, loss of alveolar bone, swelling of the alveolar ridge
 Histo: biphasic, nests of small round cells and larger epithelioid, pigment-producing cells
 Tx: surgical excision or curettage
Granular Cell Tumor
 Adults, can be multiple, firm and fixed, yellow hue
 Most commonly located on the tongue
 Histo: syncytial growth of granular cells, indistinct cell borders, granular cytoplasm, vesicular nuclei,
pseudoepitheliomatous hyperplasia (epithelial hyperplasia that resembles SCCa). The granular cells insinuate
among muscle fibers
 The tumors are S-100 protein positive – signifies neural derivation
 Tx: excision

Congenital Epulis
 Rare alveolar ridges of newborns (birth or first year), pink/red polypoid mass; MX>MD, F>M
 Granular appearance
 Histo: syncytial growth of granular cells w/o the pseudoepitheliomatous hyperplasia (PEH) seen in granular cell
tumor
 Tx: surgical excision, but may spontaneously regress

Hemangiomas and Lymphangiomas

 Hemangiomas are considered to be benign tumors of infancy that display a rapid growth phase with endothelial
cell proliferation, followed by gradual involution
 Lymphangiomas are benign, hamartomatous tumors of lymphatic vessels

 Hamartomatous processes – overgrowth of tissue native to an area (developmental)

 Vascular proliferation, tortuous architecture
 Can be superficial or deep, capillary or cavernous and cystic hygroma
 Often have characteristic hue, hemangiomas are diascopy negative
 Oral lymphangiomas may have a “cobblestone” surface, cause macroglossia
 Sturge-Weber Angiomatosis (Encephalotrigeminal Angiomatosis):
o Port wine stain of skin, follows distribution of trigeminal nerve, 10% bilateral
o Seizure disorder and retardation are common, “tram-line” calcifications on skull films
 Hemangiomas most common in white infants, incidence 10-12 X that of black and Asian infants
o F>M, 3:1
o 30% present at birth, 70% appear in first several weeks of life
o Early rapid growth “proliferation) then slow involution, Earliest sign – blanching of skin, fine
telangiectasis, then red, crimson macule, Involution may be rapid or prolonged
o Rapidly involuting congenital hemangioma (proliferates in utero, fully dev. At birth), involutes by 2nd yr
of life
o 50% of infantile variety complete involution by age 5, 70% by 7 yrs; remainder may take additional 3-5
years to involute
o Of lesions that have involuted by age 6, 38% have residual evidence – scar formation, telangiectasia, or
redundant skin
o 80% of lesions that complete involution >age 6 may show marked cosmetic deformities
o Infants whose mothers had chorionic villus sampling may have increased risk
o Unique relationship between hemangiomaas & placental microvessels
Leiomyoma
 Benign smooth muscle tumor (uterine fibroids), slow growing [only smooth muscle in oral cavity is vascular]
 Histo: spindle cells with truncated, “lit cigar” nuclei
 Rare intraorally, derived from vascular smooth muscle, lip, tongue, BM
 Tx: local excision

Rhabdomyoma
 Benign skeletal muscle tumor, M<F, adult and fetal types
 FOM, soft palate, tongue, sometimes nodular, multicentric
 Histo: lobules of large granular cells
 Tx: local excision

Osseous and Cartilaginous Choristomas

 Normal tissue in an abnormal location, 85% in posterior tongue near foramen cecum
MALIGNANT SOFT TISSUE TUMORS – SARCOMAS

Malignant Peripheral Nerve Sheath Tumor (Neurofibrosarcoma)

 10% of all soft tissue sarcomas, young persons, asymptomatic, expansile mass
 Peripheral nerve malignancy, rare in H & N (MD, lips, BM), widened inferior alveolar canal
 50% occur in patients with neurofibromatosis, pain or nerve deficit are common
 Histo: hypercellular fascicles, resembles fibrosarcoma and leiomyosarcoma
 Tx: wide excision; metastasis and recurrence common

Angiosarcoma
 Sarcoma of vascular endothelium, affects the elderly, scalp and forehead (uncommon in the oral cavity)
 Appears as a red patch, lump on the head – yet no indication of prior trauma
 50% H & N, may resemble bruise, oral lesions are rare, MD>MX
 Tx: surgical excision, RT. Poor prognosis

Leiomyosarcoma
 Malignant smooth muscle tumor, rare intraorally, derived from vascular smooth muscle
 Tx: surgery, chemotherapy, radiation therapy. Poor prognosis

Rhabdomyosarcoma
 Malignant skeletal muscle tumor, Head & neck – common site, most frequently the orbit.
 Symptoms can mimic a toothache
 Most common soft tissue sarcoma of young people – 1st decade, rare after age 45
 3 Histologic patterns – Embryonal accounts for 75%, altering hypercellular and myxoid zones (alveolar,
pleomorphic are other forms)
 Tx: multimodal – Surgical excision-> multiagent chemotherapy-> radiation therapy