giant-cell tumor of bone

image:Localisation of giant-cell tumor of bone.jpg

Patients usually present with pain and limited range of motion caused by tumor's proximity to the joint space. Swelling may occur, 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. They usually originate from the epiphysis of long bones, but in rare cases, they may arise from anterior arc of the ribs.Dehghan A, Moaddab AH, Eskandarlou M, Moeeni A. Anterior chest wall giant cell tumor. Gen Thorac Cardiovasc Surg. 2010 Jan;58(1):39-41.

The symptoms may include muscular aches and pains in arms or legs and abdominal pain. Patients may also experience nerve pain which feels like an electric shock due to weight bearing.{{citation needed|date=October 2020}}

Image:Giant cell tumour of bone - very high mag.jpg of giant cells in a giant-cell tumor of bone, H&E stain]]

The diagnosis of giant-cell tumors is based on biopsy findings. The key histomorphologic feature is, as the name of the entity suggests, (multinucleated) giant cells with up to a hundred nuclei that have prominent nucleoli. Surrounding mononuclear and small multinucleated cells have nuclei similar to those in the giant cells; this distinguishes the lesion from other osteogenic lesions which commonly have (benign) osteoclast-type giant cells. Soap-bubble appearance is a characteristic feature.{{citation needed|date=October 2020}}

Image:Giant cell tumor of bone08.JPG

On X-ray, giant-cell tumors (GCTs) are lytic/lucent lesions that have an epiphyseal location and grow to the articular surface of the involved bone.{{cite journal |vauthors=Murphey M, Nomikos G, Flemming D, Gannon F, Temple H, Kransdorf M |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=Radiographics |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi=10.1148/radiographics.21.5.g01se251283|doi-access=free }} Radiologically the tumors may show characteristic 'soap bubble' appearance.{{cite book |url=https://archive.org/details/yochumrowesessen0001_3edyoch |title=essentials of skeletal radiology |publisher=Lippincott Williams & Wilkins |year=2005 |isbn=978-0-7817-3946-7 |pages=[https://archive.org/details/yochumrowesessen0001_3edyoch/page/1 1]– |id=GGKEY:29STUY0DQ70 |access-date=21 June 2010 |url-access=registration}} They are distinguishable from other bony tumors in that GCTs usually have a nonsclerotic and sharply defined border. About 5% of giant-cell tumors metastasize, usually to a lung, which may be benign metastasis.{{cite web |url=http://www.wheelessonline.com/ortho/giant_cell_tumor_of_bone |title=Giant-cell tumor of bone - Wheeless' Textbook of Orthopaedics |date=22 July 2020 }}

Plain radiograph is still the main method of diagnosing giant cell tumour. But MRI and CT scans are useful for staging and surgical planning. CT is more superior than plain radiograph in delineating the tumour outline, its extension into structures outside the bone, determining the involvement of bony cortex and tumour recurrence. CT scans show features such as thin and expanded bony cortex, with or without bony matrix calcifications, and fluid levels due to aneurysmal bone cyst or bleeding within tumour. However, axial views of the subarticular bone (bony area adjacent to the articular cartilage) is not accurate due to voxel signal averaging. MRI is superior than CT on delineating the tumour because of its better contrast resolution and imaging in multiple planes. MRI is also useful in determining the extension outside the bone and evaluating the involvement articular surface, skip lesions within bony matrix, and medullary cavity. However, bony cortex destruction is better demonstrated on CT scan.{{Cite journal |last1=Chakarun |first1=Corey J. |last2=Forrester |first2=Deborah M. |last3=Gottsegen |first3=Christopher J. |last4=Patel |first4=Dakshesh B. |last5=White |first5=Eric A. |last6=Matcuk |first6=George R. |date= January 2013 |title=Giant Cell Tumor of Bone: Review, Mimics, and New Developments in Treatment |url=http://pubs.rsna.org/doi/10.1148/rg.331125089 |journal=RadioGraphics |language=en |volume=33 |issue=1 |pages=197–211 |doi=10.1148/rg.331125089 |pmid=23322837 |issn=0271-5333}}

On MRI, the mass generates intermediate to reduced signal intensity on T1-weighted images, and increased signal intensity on fluid sensitive MRI images such as short tau inversion recovery (STIR). The mass enhances after intravenous gadolinium administration.

A number of tumors have giant cells, but are not true benign giant-cell tumors. These include aneurysmal bone cyst, chondroblastoma, simple bone cyst, osteoid osteoma, osteoblastoma, osteosarcoma, giant-cell reparative granuloma, Giant-cell tumor of the tendon sheath and brown tumor of hyperparathyroidism.{{citation needed|date=September 2021}}

General treatment regimens have not changed much in the past 30 years, in part due to the lack of randomized clinical trials. Surgery is the treatment of choice if the tumor is determined to be resectable. Curettage is a commonly used technique.{{cite journal |vauthors=Balke M, Schremper L, Gebert C, etal |title=Giant cell tumor of bone: treatment and outcome of 214 cases |journal=J. Cancer Res. Clin. Oncol. |volume= 134|issue= 9|pages= 969–78|date=March 2008 |pmid=18322700 |doi=10.1007/s00432-008-0370-x|s2cid=2971150 }} The situation is complicated in a patient with a pathological fracture. It may be best to immobilize the affected limb and wait for the fracture to heal before performing surgery.

Patients with tumors that are not amenable to surgery are treated with radiation therapy.{{cite journal |vauthors=Mendenhall W, Zlotecki R, Scarborough M, Gibbs C, Mendenhall N |title=Giant cell tumor of bone |journal=Am J Clin Oncol |volume=29 |issue=1 |pages=96–9 |year=2006 |pmid=16462511 |doi=10.1097/01.coc.0000195089.11620.b7|s2cid=31907929 }} However caution is employed since a majority of recurrent tumors with transformations to the malignant sarcoma phenotype have been in patients receiving radiotherapy for their primary benign lesion.{{cite journal | author = Mendenhall WM, Zlotecki RA, Scarborough MT, Gibbs PC, Mendenhall NP | year = 2006 | title = Giant Cell Tumor of Bone | journal = American Journal of Clinical Oncology | volume = 29 | issue = 1| pages = 96–99 | doi = 10.1097/01.coc.0000195089.11620.b7 | pmid = 16462511 | s2cid = 31907929 }} Pharmacotherapy for GCTOB, includes bisphosphonates such as Zoledronate, which are thought to induce apoptosis in the MNGC fraction, preventing tumor-induced osteolysis. Indeed, in vitro studies have shown zoledronate to be effective in killing osteoclast-like cells. More recently, humanized monoclonal antibodies such as Denosumab targeting the RANK ligand have been employed in treatment of GCTOB in a phase II study. This is based on the notion that increased expression of RANK-ligands by stromal cells plays a role in tumor pathogenesis.

Giant-cell tumor of the bone accounts for 4-5% of primary bone tumors and about 20% of benign bone tumors.{{cite journal |vauthors=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi M |title=Identification of markers of possible prognostic value in 57 giant-cell tumors of bone |journal=Oncol Rep |volume=10 |issue=2 |pages=351–6 |year= 2003 |pmid=12579271 |doi=10.3892/or.10.2.351}} However, significantly higher incidence rates are observed in Asia, where it constitutes about 20% of all primary bone tumors in China.{{cite journal | author = Thomas D. M., Skubitz T. | year = 2009 | title = Giant-cell tumour of bone | journal = Current Opinion in Oncology | volume = 21 | issue = 4| pages = 338–344 | doi = 10.1097/cco.0b013e32832c951d | pmid = 19444102 | s2cid = 41807503 }} It is slightly more common in females, has a predilection for the epiphyseal/metaphyseal region of long bones,{{cite journal | author = Dickson B. C., Li S.-Q., Wunder J. S., Ferguson P. C., Eslami B., Werier J. A. | display-authors = etal | year = 2008 | title = Giant-cell tumor of bone express p63 | journal = Modern Pathology | volume = 21 | issue = 4| pages = 369–375 | doi = 10.1038/modpathol.2008.29 | pmid = 18311114 | doi-access = free }} and generally occurs in the third to fourth decade. Although classified as a benign tumor, GCTOB has been observed to metastasize to the lungs in up to 5% of cases, and in rare instances (1-3%) can transform to the malignant sarcoma phenotype with equal disease outcome.

• Large cell
• Aneurysmal bone cyst

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{{Medical resources

| DiseasesDB = 9337

| ICD10 = {{ICD10|C|40||c|40}}, {{ICD10|C|41||c|40}}

| ICD9 =

| ICDO = 9250/1

| OMIM =

| MedlinePlus =

| eMedicineSubj = radio

| eMedicineTopic = 307

| MeshID = D018212

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{{Osseous and chondromatous tumors}}

{{DEFAULTSORT:Giant Cell Tumor Of Bone}}

Category:Osseous and chondromatous neoplasia