Giant%20Cell%20Lesions%20-%20A%20review

1
Giant Cell Lesions - A review

2

• Giant Cell Lesions - A review

3
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.

4
Introduction

• There is a longstanding controversy regarding the
  relationship between giant cell tumors of the
  axial skeleton and giant cell granulomas of the
  jaws.

5
Controversies Giant Cell Lesions

• Until the mid 1950s giant cell tumors of the
  extragnathic skeleton were considered closely
  related to giant cell lesions which occurred in
  the jaws.

6
Controversies Jaffes 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.

7
Controversies Jaffes 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.

8
Controversies Jaffes 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.

9
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.

10
Controversies Giant Cell Lesions

• Currently there are two schools of thought
  regarding the existence of giant cell tumors in
  the facial skeleton.

11
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.

12
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.

13

• Giant cell lesions of the long bones

14
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.

15
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.

16
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).

17
Giant cell lesions of the long bones Clinical
features
18
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.,)

19
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.

20
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.

21

• Central Giant cell lesions of the Jaws

22
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.

23
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.

24
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 21 female predilection.
• The mandible is clearly the preferred site.

25
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.

26
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.

27
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.

28
Central Giant cell lesions of the Jaws
Radiographic features

• Thin, wispy septa.
• Resorption of teeth very common.
• Salt Pepper calcification can be seen.

29
Central Giant cell lesions of the Jaws
Radiographic features

• V-shaped bony ridges separating the locules.

30
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.

31
Central Giant cell lesions of the Jaws Clinical
behaviour

• Small, slow growing, asymptomatic mass responds
  well to curettage.
• 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.

32
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.

33
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.

34
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,
  anti-angiogenic therapy with interferon, and
  intra-lesional corticosteroids.

35
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.

36
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.

37

• Peripheral Giant cell lesions of the Jaws

38
Peripheral Giant cell lesions of the Jaws

• In the past these lesions were referred to as
  giant cell epulis.

39
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).

40
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 patients between the
  third and fifth decades of life.

41
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 .

42
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.

43
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.

44
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.

45
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.

46
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.

47

• Browns Tumor
• Of Hyperparathyroidism

48
Hyperparathyroidism (Browns tumor)

• Browns tumor of hyperparathyroidism is
  clinically, histologically and radiographically
  indistinguishable from other types of giant cell
  lesions.

49
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.

50
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
  patients serum calcium and parathormone levels.

51
Hyperparathyroidism Radiographic features
52
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.

53

• Aneurysmal bone cyst

54
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.

55
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.

56
Aneurysmal bone cyst Etiology

• Lichenstein indicates that this lesion results
  from a locallised vascular disturbance such as a
  venous thrombosis or arteriovenous malformation.

57
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.

58
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.

59
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.

60
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.

61
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.

62
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.

63
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.

64
Aneurysmal bone cyst Treatment

• Despite the recurrence rates, a thorough
  curettage is the most commonest treatment
  modality for this lesion.

65
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.

66

• Cherubism

67
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.

68
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.

69
Cherubism Clinical features

• In contrast to other giant cell lesions,
  cherubism has a hereditary predilection which
  helps to differentiate it from other similar
  entities.

70
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.

71
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.

72
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.

73
Cherubism Clinical features

• In rare cases the mandibular condyles can also
  be involved.
• The lesions are classically bilateral, although
  unilateral involvement has been reported.

74
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.

75
Cherubism Grading

• The diverse clinical presentation has prompted
  some clinicians to adopt a grading system which
  categorizes these lesions according to extent of
  involvement.

76
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.

77
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.

78
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.

79
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.

80
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.

81
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.

82
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.

83
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.

84
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.

85
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.

86

• Thank you