# Case Report: Malignant transformation of maxillary giant cell tumor of bone from EURACAN reference center

**Authors:** Federica Riva, Sabrina Vari, Concetta Elisa Onesti, Renato Covello, Silvia Scuderi, Serena Ceddia, Maria Rosaria Fiore, Vincenzo Anelli, Sabino Strippoli, Virginia Ferraresi

PMC · DOI: 10.3389/fonc.2025.1604056 · Frontiers in Oncology · 2025-07-17

## TL;DR

This case report describes a rare malignant transformation of a maxillary giant cell tumor of bone and the treatment challenges faced.

## Contribution

The report highlights the use of local carbon ion therapy in managing malignant transformation of GCTB.

## Key findings

- Malignant transformation of maxillary GCTB resulted in disease progression despite standard treatments.
- Local carbon ion therapy achieved marked radiological and clinical response in this case.
- Multidisciplinary approaches at specialized centers are crucial for managing such rare transformations.

## Abstract

Giant cell tumor of bone (GCTB) is a benign but locally aggressive neoplasm that can rarely undergo malignant transformation, with a poor prognosis. The most frequent histotypes of the sarcomatous transformation of GCTB are osteosarcoma, fibrosarcoma, and undifferentiated pleomorphic sarcoma, and the treatment approach mirrors that of high-grade sarcomas. This case report describes the malignant transformation of a maxillary GCTB treated with standard systemic treatments for bone tumors and local treatment, resulting in a progression of disease until the patient’s death. Nevertheless, a marked radiological and clinical response was achieved with local carbon ion therapy. This case highlights the diagnostic and therapeutic challenges of malignant transformation of GCTB, emphasizing the importance of a multidisciplinary approach at specialized centers and the potential role of local therapies in selected cases.

## Linked entities

- **Diseases:** Giant cell tumor of bone (MONDO:0005674), osteosarcoma (MONDO:0002623), fibrosarcoma (MONDO:0002676), undifferentiated pleomorphic sarcoma (MONDO:0002142)

## Full-text entities

- **Genes:** SATB2 (SATB homeobox 2) [NCBI Gene 23314] {aka C2DELq32q33, DEL2Q32Q33, GLSS}, H3-3A (H3.3 histone A) [NCBI Gene 3020] {aka BRYLIB1, H3.3A, H3F3, H3F3A}, TNFSF11 (TNF superfamily member 11) [NCBI Gene 8600] {aka CD254, ODF, OPGL, OPTB2, RANKL, TNLG6B}
- **Diseases:** pneumonia (MESH:D011014), hearing loss (MESH:D034381), death (MESH:D003643), facial mass (MESH:C536030), undifferentiated pleomorphic sarcoma (MESH:D002277), bone marrow toxicity (MESH:D001855), lung metastases (MESH:D009362), McCune-Albright syndrome (MESH:D005359), synovial sarcoma (MESH:D013584), acute renal failure (MESH:D058186), MPNST (MESH:D018319), jaw swelling (MESH:D007571), angiosarcoma (MESH:D006394), inflammation (MESH:D007249), Malignant (MESH:D009369), ulcerated lesion (MESH:D014456), epistaxis (MESH:D004844), maxillary giant cell tumor (MESH:D005870), GCTB (MESH:D018212), osteosarcoma (MESH:D012516), bone tumors (MESH:D001859), sarcomatous (MESH:D018316), maxillary lesions (MESH:D008439), bleeding (MESH:D006470), hard palate lesion (MESH:D018804), sarcoma (MESH:D012509), fibrous dysplasia (MESH:D005357), breathing (MESH:D004417), bone (MESH:D001847), fibrosarcoma (MESH:D005354)
- **Chemicals:** HE (MESH:D006371), regorafenib (MESH:C559147), cisplatin (MESH:D002945), pembrolizumab (MESH:C582435), Adriamycin (MESH:D004317), denosumab (MESH:D000069448), ifosfamide (MESH:D007069), lenvatinib (MESH:C531958), carbon (MESH:D002244), NCT04784247 (-)
- **Species:** Homo sapiens (human, species) [taxon 9606]
- **Mutations:** G34W, G34R, G34V

## Full text

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## Figures

4 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12310670/full.md

## References

28 references — full list in the complete paper: https://tomesphere.com/paper/PMC12310670/full.md

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Source: https://tomesphere.com/paper/PMC12310670