MAXILLOFACIAL SKELETON AND SOFT TISSUE PATHOLOGY IN HEAD
AND NECK REGION.
BENIGN LESIONS OF MAXILLOFACIAL SKELETON
-a large diversity of lesions may involve the maxillofacial bones. Some
occur exclusively at this site.
Other lesions at this location have features that are different from similar
lesions occurring elsewhere
-histologically, the benign lesions occurring in the jaw as well as in the
bones of the skull can be divided to 3 major groups:
-fibro-osseous lesions
-giant cell lesions
-bone forming lesions
1. FIBRO-OSSEOUS AND GIANT CELL LESIONS.
fibro-osseous lesions of head and neck-encompass a diverse group of
lesions, it includes skeletal diseases, such as fibrous dysplasia, and other lesions
peculiar to the jaws, such as cemento-osseous dysplasia, and cemento-ossifying
fibroma
 1.) Fibrous dysplasia-occurs in two variant- monostotic fibrous dysplasia is
characterized by focal but poorly circumscribed fibro-osseous replacement of
the bone, mostly the maxilla, is mainly seen in young adults-mean age of 25
years
-polyostotic fibrous dysplasia is more uncommon, several or many bones are
affected, lesions are typically unilateral- Albright syndrome-comprises
polyostotic fibrous dysplasia, skin pigmentation, and sexual precoxity,
polyostotic variant often includes head and neck region, polyostotic FD is more
common in children
histologically-fibrous dysplasia show replacement of normal bone by moderately
cellular fibrous tissue, containing evenly distributed irregularly shaped bone
trabeculae, there may be myxoid areas in the stroma, and occasionally giant
cells, the bone trabeculae mostly consist of woven bone without rimming
osteoblasts, sometimes, tiny calcified spherules may be present
-although the woven bone is a hallmark of the lesion, the jaw lesion can also
contain mature lamellar bone, or transition of both
treatment- some lesions are self-limited and no treatment is required, cases
with clinical complications (local increase in size, deformities, etc.) should be
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treated by resection, recurrence has to be expected at about 30% of cases, in
some cases- sarcomatous change, higher risk in polyostotic variant
 2.) Cemento-ossifying fibroma- this tumor appears to be peculiar to the jaw,
can affect patients in wide age range, mostly appears between 20 and 40
years of age, the tumor is well circumscribed, has well-defined capsule
histologically- may be very similar to fibrous dysplasia, composed of cellular
fibroblastic proliferation with trabeculae of woven bone with prominent
osteoblastic rims, more often affects mandible, calcifications and globular
deposits of cementum-like material common
two subtypes of ossifying fibroma should be separated:

Juvenile active (aggressive) ossifying fibroma- mainly found in the
maxilla of young patients, they are locally aggressive with tendency to
recur, histologically- more cellular stroma, mitoses are prezent, the
lesion may be confused with LG sarcoma, but the lesion is well
demarcated from the surroundings and is benign
 psammomatoid ossifying fibroma- is characterized by a fibroblastic
stroma containing small ossicles resembling psammoma bodies, the
stroma is loose, predilection site- bony walls of paranasal sinuses
 3.) Cemento-osseous dysplasia
-osseous dysplasia typically occurs in the tooth-bearing jaw in the immediate
vicinity of the tooth apex- periapical COD
-affects more commonly women, particularly middle-aged,
-histologically resembles cemento-ossifying fibroma- in early stage, the lesion
consists of cellular fibrous tissue with foci of cementum-like material, later
they fuse to form solid bone-like masses
-no histological distinguishing features are reliable, should be separated
clinically and by radiological appearance- osseous dysplasia is a mixed radiolucent
and radiodense lesion, while osiifying fibroma is well demarcated
2. GIANT CELL LESIONS OF THE JAW
 Cherubinism- (familial fibrous dysplasia of the jaw) -is an autosomal dominant
disease with variable expressivity- more severe forms twice common in males,
characteristic abnormalities are multiple, symmetric massess in the jawscyst-like radiographically, more frequently involved-mandible, early onset of
disease- at the age of 6 months to 5 years
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histologically-soft tissue masses consist of highly cellular and vascular fibrous
tissue containing many giant cells, blood vessels are surrounded by hyalinized
perivascular collagen, scanty bone trabeculae
clinical behavior- in many cases spontaneous resolution, exceptionally surgical
treatment is necessary

Central giant cell granuloma - uncommon lesion, that affects mandible,
painless swelling sometimes with displacement of teeth, mean age is 20
histologically- composed of giant cells, proliferating highly vascularized fibrous
tissue, with areas of hemorhage and deposits of hemosiderin, prominent osteoid
and bone formation- very similar to giant cell tumors of long bones
prognosis- benign tumor with tendency to recur, the lesion is not encapsulated
treatment- curettage appears to be adequate
 Brown tumors of hyperparathyroidism
- brown tumor is a bone lesion that develops as a component of metabolic bone
disease known as “osteitis fibrosa cystica”- or von Recklinghausen disease of
bone
-the lesion is solitary or multifocal, involvement of skull bones is common
treatment: is aimed at correction of hyperparathyroidism, complete resolution
of bone lesions follows within 6 months after decrease of serum level of
parathormon
 Aneurysmal bone cyst
-is an intraosseous pseudocystic lesion filled with blood surrounded by fibrous
connective tissue without endthelial or epithelial lining
-most patients are younger than 20 yrs of age
-clinically there is sudden increase in size, often accompanied by pain, surgical
curretage is the treatment of choice, recurrences are common
the overall prognosis is good
radiologically: the lesion presents as unilocular radiolucent space with soapbubble appearance
3.BENIGN BONE FORMING LESIONS
 Osteoblastoma
Is not uncommon in jaw, OB most commonly seen in vertebral column and the
femur, followed by jaws…
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histologically: anastomosing spicules of woven bone are rimmed by a single layer
of plump uniform osteoblasts separated by loose fibrovascular stroma
tumor is benign, but sometimes it exhibits more aggressive behavior
 Cementoblastoma
-is benign neoplasms occurring in vicinity to roots of the teeth
histologically: the lesion is identical to osteoblastoma, the lesions may be heavily
mineralized, in addition, cementoblastoma is defined by its relation to tooth,
thus the presence of root dentin is needed for diagnosis
2. MESENCHYMAL TUMORS
 1. Fibromatoses and fasciites- are proliferative lesions of connective tissue
that infiltrate, have tendency to recur, but are non-neoplastic and do not
metastasize
-overall, fibromatoses mainly affect adults, but oral and perioral f.-ses appear
between 0-9 years of age, clinically f. form slow-growing painless poorly
circumscribed tumor-like masses
-histologically- consist of proliferating fibrous tissue, appearance range from
mature collagen to highly cellular tissue composed of fibroblasts and
myofibroblasts
-surgical control is in maxillofacial region difficult- close proximity to vital
structures- likelihood of recurrences is high
 desmoplastic fibroma of the jaw- form of orofacial fibromatosis, affects
patients between 10 and 20 years of age, gradual expansion of the jaw,
aggressive behavior, progressive, but without tendency to metastasize
 gingival fibromatosis- is composed of dense collagenous fibrous tissue, can be
familial (with autosomal dominant trait) ar drug-induced
 fasciitis- benign proliferative lesion of fibroblasts- tend to affect limbs, and
is rare in mouth region, treatment of choice is limited excision
 2.Olphactory neuroblastoma- rare malignant neoplasm that originates from
the olphactory membrane of the upper nasal cavity, composed of closely
packed small round blue cells with little cytoplasm, treatment is surgical
excision + radiotherapy, tumor is slow-growing, but can metastasize (brain,
lymph nodes, lung, bone)
 3.Melanotic neuroectodermal tumor of infancy- uncommon tumor that
appears to originate from the neural crest, affects small children-in the first
year of life, the tumor consists of slit-like spaces lined by melanin containing
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small round cells in dense stroma, it is a destructive maxillary tumor,
biologically benign, but can recur,
treatment-radical excision + radiotherapy
 4.Hemangiomas- are very common in head and neck region, most hemangiomas
are malformations or hamartomas of blood vessels, may be congenital, form
flat or prominent lesions, extensive congenital h. of the face- involves the
skin, and underlying soft tissue
 -capillary hemangioma consists of dense mass of neoplastic capillaries,
cavernous h. consists of dilated blood-fille vascular spaces with endothelial
lining
 5. Lymphangiomas- are more uncommon than hemangiomas, may be superficial
or deep (affect tongue, lips, soft tissue of the neck, etc)
cystic hygroma- is a term given to cavernous lymphangioma with unusually large
cyst-like lymphatic spaces- most common in neck
 6. Leiomyoma and angiomyoma- are rare in the mouth, angiomyoma-is a
leiomyoma of apparentyl vasvular origin- bluish color- benign, respond to
conservative excision
 7. Lipomas, spindle cell lipoma- lipomas occasionally form within the mouth
from buccal fat, or from salivary gland tissue
-spindle cell lipoma-consists of variable numbers of adipocytes surrounded by
fibroblast-like cells in myxoid or collagenous matrix, benign, poorly
circumscribed tumor, typicall location- neck and shoulder, in middle aged men
 8. Synovial sarcoma- orofacial region is relatively common site, synovial
sarcoma affects mostly tissue unrelated to joints, histologically- synovial
sarcoma is biphasic, with both spindle cell and epithelial elements
-malignant tumor, locally aggressive, with meta to lungs, treatment - wide
excision + raditherapy
3. TUMORS OF THE JAW (NON-ODONTOGENIC)
 1. Tumors of bone- osteomas and exostoses- both are composed of compact
or cacellous bone, exostosis is a localized bone overgrowth, more common than
osteomas, bones of maxillofacial region are commonly affected
-compact and cancellous osteoma- very slowly growing benign tumor, consists of
dense bone
-osteochondroma- is a bony overgrowth with ossification beneath a cartilaginous
cap- rare in maxillofacial skeleton
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-osteosarcoma- highly malignant tumor of bone, may be found in jaws, mean age
about 30 years, rapidly invasive, lend to metastasize, radical surgery
supplemented by radiotherapy and chemotherapy is necessary
 2. Cartilagineous tumors - chondroma-uncommon in the jaw, more often found
in nose and nasal sinuses, chondroma consists of mature hyaline cartilage,
treatment -wide excision
-chondrosarcoma- malignant tumor with cartilagineous differentiation, occurs
more commonly in jaws than benign chondromas, mean age is 36 years,
maxillofacial chondrosarcomas are aggressive, tumors with invasive behaviorlocal recurrence are common- prognosis is generally worse that that for other
sites- because of difficulties with adequate excision in this area
 3. Multiple myeloma, solitary plasmocytoma- multiple myeloma- is a neoplasm
of plasma cells, causes multifocal bone-destructive lesions- painful and
tender, may affect the jaws, composed of neoplastic plasma cells that are
monoclonal and sho kapa or lambda restriction
- as a result of proliferation of myeloma cells in the bone marrow- anemia and
thrombocytopenia
-solitary extramedullary myeloma- is rare tumor, but about 80% of them form in
the head and neck - nasal cavity and nasopharynx
 4. Langerhans cell histiocytosis- uncommon osteolytic lesion, due to
proliferation of L. cells, LCH has highly variable manifestations and behavior,
ranging from solitary bone lesion to disseminated forms, three main forms are
recognized-solitary eosinophilic granuloma, Hand-Schuller-Christian disease
and Letterer Siwe disease
-solitary eosinophilic granuloma- adults are affected, the bone of jaw is
relatively common site, pain and swelling are common symptoms
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