Abstract
Purpose
The purpose of this study was to review the clinical presentation, imaging, pathology and outcome of patients with giant cell-rich osteosarcoma (GCRO) of long bones.
Materials and methods
Radiography (n=9), magnetic resonance imaging (MRI) (n=6), computed tomography (CT) (n=3) and clinical course of nine patients (five males and four females; mean age, 26 years) with pathologically confirmed GCRO were retrospectively reviewed. Specific imaging findings, including size, eccentricity, ossification, lysis, cystic change, expansile growth, periosteal reaction, cortical destruction, soft tissue extension and joint involvement were documented.
Results
Presenting symptoms were pain in six patients and pain and palpable mass in three. An ill-defined margin surrounding a predominantly osteolytic lesion was detected at the proximal tibia (n=7) or femur (n=2) on imaging studies. Seven cases showed limited ossification. Three cases had tumours in the metaphysis and six in the metaepiphysis. The average maximum tumour dimension was 4.7 cm×5.2 cm×7.8 cm. Microscopically, tumours were composed of atypical cells with scanty osteoid formation and multinucleated giant cells. All patients received chemotherapy, and surgery was performed in eight patients. Three patients were dead and six were alive at the last follow-up.
Conclusions
GCRO is a rarer variant that has very close resemblance to giant cell tumour. Patients usually present nonspecific symptoms of pain and palpable mass. It usually shows an osteolytic lesion with locally spared new bone formation in the metaphysis and/or metaepiphysis on imaging. Histologically, the atypical tumour cells with osteoid formation and multinucleated giant cells are the key factor in the diagnosis and differential diagnosis.
Riassunto
Obiettivo
Lo scopo di questo studio è stato di rivedere la presentazione clinica, le caratteristiche all’imaging, la patologia e gli esiti di pazienti con osteosarcoma ricco in cellule giganti delle ossa lunghe
Materiali e metodi
Radiografia (n=9), imaging con risonanza magnetica (MRI) (n=6), tomografia computerizzata (CT) (n=3) e decorso clinico di nove pazienti (cinque maschi e quattro femmine; età media 26 anni) con GCRO confermato anatomo-patologicamente sono stati esaminati retrospettivamente. Specifici rilievi di imaging, inclusi dimensione, eccentricità, ossificazione, lisi, evoluzione cistica, crescita espansiva, reazione periostale, distruzione corticale, estensione ai tessuti molli e coinvolgimento articolare sono stati documentati.
Risultati
I sintomi di presentazione sono stati il dolore in sei pazienti e sia il dolore che la massa palpabile in tre pazienti. All’imaging è stato riscontrato un margine indefinito che circonda una lesione prevalentemente osteolitica a livello della tibia prossimale (n=7) o del femore (n=2). Sette dei casi hanno mostrato ossificazione limitata. In 3 casi il tumore era localizzato alla metafisi ed in 6 nella meta-epifisi. La massima dimensione tumorale è stata in media di 4,7 cm×5,2 cm×7,8 cm. Microscopicamente, i tumori erano composti di cellule tumorali atipiche con scarsa formazione di osteoide e cellule giganti multinucleate. Tutti i pazienti hanno ricevuto chemioterapia e un intervento chirurgico è stato eseguito su 8 pazienti. Tre pazienti erano morti e 6 erano in vita all’ultimo follow-up.
Conclusioni
GCRO è una variante più rara che assomiglia molto al tumore a cellule giganti. I pazienti di solito presentano sintomi aspecifici come dolore e massa palpabile. Di solito si dimostra una lesione osteolitica con neoformazione ossea localmente conservata nella metafisi e/o meta-epifisi all’imaging. Istologicamente, le cellule tumorali atipiche con formazione di osteoide e le cellule giganti multinucleate sono i fattori chiave nella diagnosi e nella diagnosi differenziale.
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Wang, CS., Yin, QH., Liao, JS. et al. Giant cell-rich osteosarcoma in long bones: clinical, radiological and pathological features. Radiol med 118, 1324–1334 (2013). https://doi.org/10.1007/s11547-013-0936-9
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DOI: https://doi.org/10.1007/s11547-013-0936-9
Keywords
- Bone neoplasms
- Giant cell tumour of bone
- Osteosarcoma
- Tomography
- X-ray
- Computed tomography
- Magnetic resonance imaging