Giant Cell-Rich Variant of Extraskeletal Osteosarcoma Above the Patella – A Rare Case Study and Review of the Literature
Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2019, Vol 19, Issue 5
Abstract
Background: The giant cell-rich variant of extraskeletal osteosarcoma is a rare subtype of osteosarcoma, occurring in the extremities of adults prevalently in their sixth decade of life. We report an exceptional giant cell-rich variant of extraskeletal osteosarcoma that was detected above the patella in an adolescent patient. The exact histological criteria for giant cell-rich osteosarcoma is not clearly defined, which makes it very challenging to identify the key characteristics for preoperative discrimination between malignant giant cell-rich osteosarcoma and benign soft tissue giant cell tumors. This exceptional case can be a useful example for differential diagnosis of tumors with similar giant cell morphology for further appropriate choices of treatment. Case Presentation: An 11-year-old Thai girl presented at our hospital with a 10-cm painless swelling in her right knee for three months. On examination, an irregular mass measuring 10x5 cm2 in size was detected above her right patella bone along with two 4-cm right inguinal lymph nodes. Radiological investigations and MRI showed the heterogeneous soft tissue mass beneath the subcutaneous fat without any patella bone invasion. Histopathology displayed numerous scattered multi-nucleated giant cells among the medium-size round cells to spindle cells in the background, together with osteoid formation. Abnormal mitoses, multi-nucleation and bizarre nuclei were found. One inguinal lymph node out of the two was positive for metastatic sarcoma. Our final diagnosis was giant cell-rich variant of extraskeletal osteosarcoma. The patient underwent neoadjuvant chemotherapy (EURAMOS-1) and a wide resection with a rotational flap. Histopathology reported less than 1% tumor necrosis. The patient remains alive without local recurrence or distant metastasis during the past 13 months. Conclusion: Giant cell-rich subtype of extraskeletal osteosarcoma has an extremely rare incidence, as well as considerable variations in histological entities. As a result, it has a very poor prognosis and possesses a high risk of misdiagnosis. Our report illustrates an unprecedented rare case of the giant cell-rich variant of extraskeletal osteosarcoma detected above the patella in an adolescent patient. Using a combination of radiography, magnetic resonance imaging, and histological examination, while carefully noticing all points of the clear cutting-criteria for differential diagnosis proposed in this study, a diagnosis of the giant cell-rich subtype of extraskeletal osteosarcoma can be reached. The preoperative differentiation of this exceedingly rare entity is crucial and needs to be examined by musculoskeletal pathologists for early accurate diagnosis and further effective treatment.
Authors and Affiliations
Thanapon Chobpenthai, Pichaya Thanindratarn
Keywords
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- EP ID EP623336
- DOI 10.26717/BJSTR.2019.19.003369
- Views 180
- Downloads 0
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