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Giant Cell Lesions - A review
Giant Cell Lesions - A review
Introduction
 Giant cell lesions of bones represent a broad
category of entities.
 These include, giant cell lesions of the bones,
central and peripheral giant cell granulomas,
aneurysmal bone cysts, brown tumors of
hyperparathyroidism and cherubism.
Introduction
 There is a longstanding controversy regarding the
relationship between giant cell tumors of the axial
skeleton and giant cell granulomas of the jaws.
Controversies : Giant Cell Lesions
 Until the mid 1950’s giant cell tumors of
the extragnathic skeleton were considered
closely related to giant cell lesions which
occurred in the jaws.
Controversies : Jaffe’s recommendations
 In 1953, Jaffe separated the giant cell
lesions occurring in the jaws from the giant
cell tumors of the long bones, based on the
differences in the clinical presentation,
histopathology, radiography and treatment
response between these two lesions.
Controversies : Jaffe’s recommendations
 Jaffe called these giant cell lesions of the jaws as
“giant cell reparative granulomas”.
 He indicated that the jaw lesions tend to occur in
younger patients (10 to 25), where as giant cell
tumors of the axial skeleton usually appeared after
the age of 20.
Controversies : Jaffe’s recommendations
 He suggested that giant cell tumors of long bones
were far more aggressive, with higher rates of
recurrence with potential to undergo malignant
transformation.
Controversies : Giant Cell Lesions
 In 1966, Waldron and Shafer argued that the
“giant cell tumors” of the long bones and “giant
cell reparative granulomas” of the jaws are one
and the same entity but may differ in presentation
and histology.
Controversies : Giant Cell Lesions
 Currently there are two schools of thought
regarding the existence of giant cell tumors in the
facial skeleton.
Controversies : Giant Cell Lesions
 Some like Jaffe, firmly believe that true giant cell
tumors either do not exist or occur very rarely in
the maxillofacial region.
 However other pathologists and clinicians remain
convinced that giant cell tumors do occur within
the jaws.
Controversies : Giant Cell Lesions
 Abrams and shear in 1974, consolidated these two
positions, and suggested that certain jaw lesions
may be true giant cell tumors and certain giant cell
lesions outside the jaws maybe giant cell
granulomas.
Giant cell lesions of the long bones
Giant cell lesions of the long bones: Clinical
features
 The females have a slight predilection.
 The peak incidence is in the third decade with the
tumor occurring in over 80% of cases after the age
of 20.
Giant cell lesions of the long bones: Clinical
features
 Most long bone lesions are located at the
epiphyseal region of the bone.
 Most of these lesions appear as a solitary lesion,
and multifocal disease is very rare.
 Patients present with pain, localized swelling and
tenderness at the site of the affected bone.
Giant cell lesions of the long bones: Clinical
features
 These lesions present with a wide range of
aggressiveness, ranging from the more
conventional giant cell tumor to true malignancies
with a high potential for recurrence and
metastasis.
 Incidence of truly malignant giant lesion ranges
from 3% to 30%.(Franklin et al).
Giant cell lesions of the long bones:
Clinical features
Giant cell lesions of the long bones:
Histopathology
 There are large number of multinucleated giant
cells of varying density separated by stromal cells.
 This type of histologic pattern is similar in many
other bone lesions like (browns tumor, non
ossifying fibroma, Aneurysmal bone cyst, e.t.c.,)
Giant cell lesions of the long bones
Therefore, the clinician must also correlate the
histological findings with the clinical and
radiographic presentation to make the correct
diagnosis.
Giant cell lesions of the long bones: Treatment
 The surgical treatment is either curettage or
resection and is performed based on the type and
extension of the tumor which affects the bone.
Central Giant cell lesions of the Jaws
Central Giant cell lesions of the Jaws
 The literature regarding giant cell lesions of the
jaw is confusing because some authors have
included both central and peripheral lesions in
their series.
Central Giant cell lesions of the Jaws
 It is now well recognized that the peripheral giant
cell lesion differs in clinical presentation and
behavior from the central lesion.
Central Giant cell lesions of the Jaws: Clinical
features
 These lesions occurs between the age of 2 to 81
years, but the average age is within the second
decade of life.
 Sex: 2:1 female predilection.
 The mandible is clearly the preferred site.
Central Giant cell lesions of the Jaws: Clinical
features
 These lesions mainly occur in the anterior portion
of the maxilla and mandible.
 Posterior maxilla or mandible is rarely involved.
Central Giant cell lesions of the Jaws: Clinical
features
 Multiple central giant cell lesions in a single
patient are exceedingly rare.
 A painless local swelling is the most commonest
presenting symptom although some patients may
have pain or anesthesia.
Central Giant cell lesions of the Jaws:
Radiographic features
 The most characteristic X-ray feature is a radiolucency
within bone and can either have an multilocular or
unilocular configuration.
Central Giant cell lesions of the Jaws: Radiographic
features
 Thin, wispy septa.
 Resorption of teeth very common.
 “Salt & Pepper “ calcification can be seen.
Central Giant cell lesions of the Jaws: Radiographic
features
 V-shaped bony ridges separating the locules.
Central Giant cell lesions of the Jaws:
Histopathology
 Histologically a central giant cell lesion is
characterized by numerous multinucleated giant
cells within a loose fibrous connective tissue
Stroma.
Central Giant cell lesions of the Jaws: Clinical
behaviour
 Small,
slow growing, asymptomatic
responds well to curettage.
mass
 However, the large and aggressive mass produces
pain and recurs frequently, especially in younger
patients.
 True malignant giant cell tumors of the jaw may
produce distant and local metastases.
Central Giant cell lesions of the Jaws: Clinical
behaviour
 There are no definitive histological parameter to
predict the clinical behavior.
 Therefore, the clinician must use his judgment,
appropriate treatment strategies and long-term
follow-up in order to properly manage these
lesions.
Central Giant cell lesions of the Jaws:
Treatment
Surgical curettage is the most commonest
procedure.
 Large aggressive lesions of the maxilla and
mandible may require an en-bloc resection.
Central Giant cell lesions of the Jaws:
Treatment
 In some lesions both the overlying mucosa and
periosteum must be removed.
 Non surgical modalities of treatment include
systemic
calcitionin
administration,
antiangiogenic therapy with interferon, and intralesional corticosteroids.
Central Giant cell lesions of the Jaws:
Treatment
 Harris (1992) reported regression of these masses
with systemic calcitonin therapy. Two of the four
patients in his study exhibited complete
regression, while the other required additional
surgery to eradicate the lesion.
Central Giant cell lesions of the Jaws:
Treatment
 Lange et. al. (1999) had obtained significant
regression of the lesions(central giant cell
granuloma) in all his four patients following
cacitonin treatment.
 The efficacy of intra-lesional steroids use in the
treatment of giant cell lesions remains to be
proven.
Peripheral Giant cell lesions of the
Jaws
Peripheral Giant cell lesions of the Jaws
 In the past these lesions were referred to as “giant
cell epulis”.
Peripheral Giant cell lesions of the Jaws :
Etiology
 The exact etiology for these lesions is not known.
 However, it is believed that these lesions are
reactive in nature and develop as a result of
localized trauma or irritation ( e.g., tooth extraction,
calculus deposits, ill fitting dentures and poor
restorations).
Peripheral Giant cell lesions of the Jaws:
Clinical features
Although the peripheral giant cell lesion occurs at
a much higher frequency than the central type, it
remains relatively uncommon (accounting for only
0.3% to 0.5% of all oral biopsies).
 They are usually seen in patient’s between the
third and fifth decades of life.
Peripheral Giant cell lesions of the Jaws:
Clinical features
 There is a significant female predilection, suggesting the
role of hormone levels in the development of these lesions.
 Most lesions occur on the gingiva or the edentulous
alveolar ridge in either a posterior or anterior portion on
the jaw.
 The mandible is affected slightly more than the maxilla .
Peripheral Giant cell lesions of the Jaws:
Clinical features
The lesions can be either pedunculated or sessile.
 They rarely exceed 2 cm in size and typically
present with a bluish – purple color.
Peripheral Giant cell lesions of the Jaws:
Radiographic features
 As most of these lesions are usually manifestations within
the gingiva, no radiological finding is reported other than
an occasional ovoid surface erosion of the underlying
bone.
Peripheral Giant cell lesions of the Jaws:
Histopathology
 The histological features demonstrate the characteristic
giant cells in a fibrous connective tissue stroma which is
covered by a surface epithelium.
Peripheral Giant cell lesions of the Jaws:
Treatment
 The treatment of choice is a surgical excision.
 In lesions involving the gingival mucosa around
the teeth, care must be taken to debride the
surrounding teeth and surgical site, so as to
remove all sources of possible irritation.
Extraction is no longer recommended.
Peripheral Giant cell lesions of the Jaws:
Recurrence
 Recurrence rate ranges between 5% and 10%.
 These recurrences are most often due to failure in
removing the entire lesion or the source of any
local irritation.
Brown’s Tumor
Of Hyperparathyroidism
Hyperparathyroidism (Brown’s tumor)
 Brown’s
tumor of hyperparathyroidism is
clinically, histologically and radiographically
indistinguishable from other types of giant cell
lesions.
Hyperparathyroidism: Types
 Primary hyperparathyroidism is caused by an
overproduction of parathormone by the
parathyroid gland affected by adenoma or
hyperplasia.
 Secondary hyperparathyroidism also results in
increased production of parathormone and is
found to occur in various disease states where
there is resistance to the metabolic actions of the
parathormone.
Hyperparathyroidism: Clinical features
 The typical hyperparathyroidism patient presents
without any significant symptoms.
 Some patients present with renal calculi or some
skeletal manifestations.
 The disease is easily diagnosed by measuring the
patient’s serum calcium and parathormone levels.
Hyperparathyroidism: Radiographic features
Hyperparathyroidism: Treatment
 Treatment
is directed towards normalizing
calcium/phosphate/parathyroid homeostasis.
 If a parathyroid adenoma is the cause of elevated
hormone level, surgical excision of this tumor
produces a complete recovery.
 In secondary hyperparathyroidism, the treatment
efforts is on correcting the underlying metabolic
disturbance.
Aneurysmal bone cyst
Aneurysmal bone cyst
 In 1942, Jaffe and Lichenstein, introduced the
term aneurysmal bone cyst.
 Other names for this lesion include, aneurysmal
giant cell tumor, subperiosteal giant cell tumor
e.t.c.
Aneurysmal bone cyst : Etiology
 There is controversy regarding the etiology of this lesion.
 For the moment there are no strong evidences to prove
that these lesions develop primarily, or to say that they
develop secondarily due to another underlying disease
process.
Aneurysmal bone cyst: Etiology
 Lichenstein indicates that this lesion results from a
locallised vascular disturbance such as a venous
thrombosis or arteriovenous malformation.
Aneurysmal bone cyst: Etiology
 Others believe that this lesion can either be a separate
reactive process, arising from another primary bone lesion
such as fibrous dyplasia, central giant cell granuloma, or
pagets disease.
Aneurysmal bone cyst : Clinical features
 This lesion occurs in younger patients usually
below 20 years of age.
 The mandible appears to be more commonly
affected than the maxilla.
Aneurysmal bone cyst: Clinical features
 The posterior molar-bearing segments of the maxilla and
mandible seem to be more commonly affected region.
 Clinically these lesions are characterized by a non-pulsatile
swelling of variable duration.
Aneurysmal bone cyst: Clinical features
 50% of patients present with pain in the affected region.
 Tooth displacement and external root resorption may also
be seen.
 There is a slight female predilection in both the long bones
and jaw lesions.
Aneurysmal bone cyst: Radiographic features
 This lesion is classically described as an expanded,
cystically transformed, eccentric ballooning of the bone.
 Fine bony trabeculations in the lesion give a “soap bubble
appearance”.
Aneurysmal bone cyst: Histopathology
 Microscopically this lesion is not a true cyst, as it does not
have a epithelial lining, and is characterized by large,
blood filled cavities contained within a thin, bony
framework.
 The histologic pattern of the stroma between the sinusoids
is very similar to that of other giant cell lesions.
Aneurysmal bone cyst: Recurrence
 The recurrence rate of these lesions in th long
bones is about 21% to 40%, and in the jaws is
about 19%.
Aneurysmal bone cyst: Treatment
 Despite the recurrence rates, a thorough curettage
is the most commonest treatment modality for this
lesion.
Aneurysmal bone cyst: Treatment
 In
some lesions with associated vascular
malformations,
preoperative
super-selective
embolization of the feeding vessels to the lesion is
mandatory before surgery.
 Following embolization either curettage or en-bloc
resection can be performed.
Cherubism
Cherubism
 Jones reported on the clinical entity known as Cherubism
in the year 1933.
 He coined the term cherubism due to these patients
resemblance to the cherubs portrayed in renaissance art.
Cherubism: Clinical features
 Classic cherubic appearance is due to a characteristic
bilateral enlargement of the maxillae, causing retraction of
the lower eyelids and exposure of the sclera, giving an
“eyes raised to heaven” appearance.
Cherubism: Clinical features
 In contrast to other giant cell lesions, cherubism has a
hereditary predilection which helps to differentiate it from
other similar entities.
Cherubism: Clinical features
 Cherubism is an autosomal dominant disorder with
variable expressivity.
 Cherubism may also result from a spontaneous mutation
with no apparent familial involvement.
 The disease occurs with a 2 : 1 male preponderance.
Cherubism: Clinical features
 Cherubism is not found to occur very commonly.
 Typically, it appears in young children before the age of
five, most commonly prior to the second birthday.
Cherubism: Clinical features
 The lesion occurs almost exclusively in the mandible and
maxilla, but in some cases have occurred in extragnathic
sites.
 They are most often seen in the mandible, with the
ascending rami and the retromolar area being most
commonly affected.
Cherubism: Clinical features
 In rare cases the mandibular condyles can also be
involved.
 The lesions are classically bilateral, although
unilateral involvement has been reported.
Cherubism: Clinical features
 Maxilla
involvement
is
less
frequent.
However,when it occurs it involves the maxillary
tuberosity region.
 These lesions may also involve the orbital floor
and anterior maxilla.
Cherubism: Grading
 The diverse clinical presentation has prompted
some clinicians to adopt a grading system which
categorizes these lesions according to extent of
involvement.
Cherubism: Grading
 Grade 1 : Involvement of both mandibular ascending rami.
 Grade 2 : Involvement of both rami and both maxillary
tuberosities.
 Grade 3 : Diffuse bilateral involvement of the entire
maxilla and mandible with sparing of the condyle.
 Grade 4 : Lesions with the features of grade 3 but with
orbital floor involvement.
Cherubism: Clinical behavior
 These lesions present as a progressively enlarging, painless
swelling with marked bony expansion.
 The dentition in the affected region is often displaced.
Cherubism: Clinical behavior
 The deciduous teeth are usually exfoliated by the age of
three and the permanent tooth follicles are displaced,
resulting in delayed eruption, multiple impacted teeth or
both.
Cherubism: Clinical behavior
 Uniform, bilateral mandibular involvement can
commonly result in gross malocclusion in addition
to facial disfigurement.
 In the most severe cases, speech, swallowing, and
respiration can be affected.
Cherubism: Radiographic features
 The typical radiographic presentation is a diffuse osteolytic
radiolucent process with diffuse cortical expansion and
thinning of the involved bone.
 In the tooth-bearing segments of the mandible, the teeth
are described as “floating in cyst-like spaces”.
Cherubism: Histopathology
 The histologic profile of cherubism is similar to
other giant cell lesions and shows numerous
multinucleated giant cells are contained within
highly vascular fibrous stroma.
Cherubism: Treatment
 The prognosis for cherubism remains good, given
the fact that these lesions usually are self-limiting
and tend to regress with time.
 The lesions become static as a patient approaches
puberty and thereafter begins to slowly regress.
Cherubism: Treatment
 Most cases undergo a surgical procedure for
cosmetic or functional needs.
 Curettage and re-contouring is advocated by some
to arrest the disease process and stimulate bone
deposition.
 Few others recommend no treatment other than
following the patient.
Giant Cell Lesions: Discussion
Miles et al (1991) provided the following comments
regarding their understanding of giant cell lesions:
 A central giant cell lesion may consists of several related
reactive lesions which result from trauma or vascular insult
which produces intramedullary bleeding.
Giant Cell Lesions: Discussion - Miles et al (1991)
 If the blood supply is cut off completely, no giant cell
reaction occurs and the traumatic bone cyst occurs.
 Conversely, if the blood supply is maintained fully, an A-V
malformation develops.
 However, if the blood supply is maintained only partially,
then an aneurysmal bone cyst or central giant cell lesion
could result.
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