Giant cell tumor of bone: Difference between revisions

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==Pathophysiology==
==Pathophysiology==
Giant cell tumours are believed to result from an over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>  
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>  

Revision as of 16:15, 22 March 2016

For patient information, click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

Overview

Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.[1] On gross pathology, hemorrhage, presence of co-existent aneurysmal bone cyst, and fibrosis are characteristic findings of giant cell tumor of bone.

Pathophysiology

The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.

Location

Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: [2]

  • Around the knee: distal femur and proximal tibia: 50-65%
  • Distal radius: 10-12%
  • Sacrum: 4-9%
  • Vertebral body
  • Multiple locations: 1%

Gross Pathology

Microscopic Pathology

  • Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
  • The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
  • Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
  • It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

Epidemiology and Demographics

Prevalence

  • Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.[1].

Age

  • Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak incidence between 20 and 30.[3]

Gender

  • Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.[4]

Differentiating Giant cell tumor of bone from other Diseases

Giant cell tumor of bone must be differentiated from:

Natural History, Complications and Prognosis

Complications

Common complications of giant cell tumor include:

  • Malignant transformation
  • Malignant transformation is far more common in men (M:F of ~3:1)
  • Sarcomatous transformation is observed, especially in radiotherapy treated inoperable tumors.
  • Recurrence
  • Local recurrence rate of giant cell tumor of bone is 10 to 40%.
  • Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as spine and sacrum.
  • Metastasis
  • Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.[4] Hence, this entity has been called benign metastasising giant cell tumor.

Prognosis

  • The prognosis of giant cell tumor is generally excellent.

Diagnosis

History and Symptoms

  • Patients usually present with pain and limited range of motion caused by tumor's proximity to the joint space.
  • There may be swelling as well, if the tumor has been growing for a long time.
  • Some patients may be asymptomatic until they develop a pathologic fracture at the site of the tumor.

Physical Examination

Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

Extremities

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:

  • Size
  • Location
  • Involvement of adjacent structures (such as peripheral nerves or veins)
  • Edema

X Ray

General radiographic features include:

  • Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
  • No surrounding sclerosis: 80-85%
  • Overlying cortex is thinned, expanded or deficient
  • Periosteal reaction is only observed in 10-30% of cases
  • Soft tissue mass is not infrequent
  • Pathological fracture may be present
  • No matrix calcification/mineralisation

On x-ray, giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone [5]. They are distinguishable from other bony tumors in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or CT may be needed.

(Images courtesy of RadsWiki)

MRI

Typical signal characteristics on MRI of giant cell tumor of bone include:

T1:

  • Low to intermediate solid component
  • Low signal periphery
  • Solid components enhance, helping distinguish giant cell tumor with aneurysmal bone cyst from pure aneurysmal bone cyst
  • Some enhancement may also be observed in adjacent bone marrow

T2:

  • Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
  • If an aneurysmal bone cyst component present, then fluid-fluid levels can be observed
  • High signal in adjacent bone marrow thought to represent inflammatory edema

T1 C+ (Gd):

  • Solid components will enhance, helping differentiate from aneurysmal bone cyst

Scintigraphy: Bone Scan

  • Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
  • Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

Treatment

The treatment of giant cell tumor is directed towards local control without sacrificing joint function.[6] Surgery is the mainstay of treatment for giant cell tumor.

Surgery

  • Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
  • Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
  • Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
  • Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

References

  1. 1.0 1.1 Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS (2003). "Identification of markers of possible prognostic value in 57 giant cell tumors of bone". Oncology Reports. 10 (2): 351–6. PMID 12579271. Retrieved 2012-01-18.
  2. Shrivastava, Sandeep; Nawghare, Shishir P; Kolwadkar, Yogesh; Singh, Pradeep (2008). "Giant cell tumour in the diaphysis of radius – a report". Cases Journal. 1 (1): 106. doi:10.1186/1757-1626-1-106. ISSN 1757-1626.
  3. Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016
  4. 4.0 4.1 Muheremu, Aikeremujiang; Niu, Xiaohui (2014). "Pulmonary metastasis of giant cell tumor of bones". World Journal of Surgical Oncology. 12 (1): 261. doi:10.1186/1477-7819-12-261. ISSN 1477-7819.
  5. Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ (2001). "From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation". Radiographics : a Review Publication of the Radiological Society of North America, Inc. 21 (5): 1283–309. PMID 11553835. Retrieved 2012-01-18.
  6. Puri, Ajay; Agarwal, Manish (2007). "Treatment of giant cell tumor of bone: Current concepts". Indian Journal of Orthopaedics. 41 (2): 101. doi:10.4103/0019-5413.32039. ISSN 0019-5413.


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