Disastrous evolution of ollier disease: a rare case report.

Ollier disease is a rare genetic disorder characterized by the development of multiple enchondromas. The clinical manifestations of the disease vary widely, but patients often present with bone deformities and an increased risk of developing chondrosarcoma. Here, the authors present a case report of a 25-year-old male patient with a devastating and historic evolution of Ollier disease. Case presentation: At the age of 10, the patient developed a sub-centimeter mass in the first phalanx of the left middle finger, which subsequently grew in size. A biopsy was performed at the age of 14, which confirmed the diagnosis of chondroma. At the age of 14, the patient developed multiple large masses on the left hand, resulting in the amputation of his left hand. At 25 years old, the patient developed new masses in his contralateral hand and left foot. Discussion: Ollier disease is caused by somatic mutations in the PTH/PTHrP receptor gene, leading to the formation of multiple enchondromas. Patients with Ollier disease are at an increased risk of developing chondrosarcoma, which can be life-threatening. The diagnosis of Ollier disease is usually made based on clinical and radiographic findings, and genetic testing can confirm the diagnosis. Treatment is typically focused on managing the symptoms and preventing the development of chondrosarcoma. Conclusion: The authors presented a case report of a patient with a devastating and historic evolution of Ollier disease. This case highlights the importance of early diagnosis and management of this disease to prevent the development of chondrosarcoma and minimize the risk of complications. Further research is needed to better understand the underlying mechanisms of the disease and develop effective treatments.

A remarkable and aggressive aneurysmal bone cyst ravaging the femoral shaft, advancing to lung metastases: An extremely rare case.

INTRODUCTION AND IMPORTANCE: Aneurysmal bone cysts (ABCs) are relatively uncommon tumor-like lesions that require careful management. Reporting such cases is crucial to highlight the importance of understanding the physiopathology and treatment options associated with ABCs. Literature-based writing emphasizes the significance of reporting cases like the one presented here. The aneurysmal bone cyst, classified as a Tumor Rich in Giant Cells according to the latest World Health Organization (WHO) classification of soft tissue and bone tumors, has posed several hypotheses and challenge. This aggressive form and rare localization of this lesion necessitates thorough discussions regarding its management. CASE PRESENTATION: We present a compelling case of a 37-year-old patient, without any specific pathological history, with a giant diaphyseal aneurysmal bone cyst located in the right femur. Remarkably, the lesion had been progressing for a decade, leading to extensive destruction of the entire femoral diaphysis despite undergoing previous surgical interventions. The patient's unique circumstances highlight the unpredictable behavior and destructive potential of aneurysmal bone cysts in rare anatomical locations. DISCUSSION AND CONCLUSION: This case underscores the need for a comprehensive understanding of aneurysmal bone cysts and their management. The utilization of denosumab, an inhibitor of the RANK/RANKL system, as a medical treatment in conjunction with surgery resulted in a favorable therapeutic response, including a reduction in tumor mass. The current WHO classification, recognizing ABCs as Tumors Rich in Giant Cells, consolidates previous hypotheses and enables the development of new therapeutic protocols. The integration of surgery and medical treatment holds promise for improving outcomes in patients with aneurysmal bone cysts. In conclusion, advancements in understanding the physiopathology and treatment options are crucial for developing effective therapeutic protocols to these aggressive forms of ABCs.

A solitary giant osteochondroma of the femur in the shape of a devil's head pushing back the superficial femoral artery: Case report and literature review.

INTRODUCTION: The most common benign bone tumors are osteogenic exostoses or osteochondromas. They occur during growth and are rarely the cause of vascular or nervous complications. CASE PRESENTATION: We present the case of a young 34-year-old patient who consulted for a swelling in his right thigh. The X-ray revealed a giant, exuberant bony tumor in the lower third of the femur. The CT angiography allowed us to see the repression of the superficial femoral artery without interfering with blood flow. By resecting a giant tumor resembling a devil's head, the obstacle on the vascular structures was removed. Histology confirmed the diagnosis of solitary osteogenic exostosis. The patient made a complete recovery and there has been no recurrence after one year of follow-up. DISCUSSION AND CONCLUSION: A solitary femoral diaphysis exostosis causing arterial compression is a rare complication. Resection and relief of artery compression should be considered early, before serious vascular sequelae develop, which can be irreversible and result in amputation. Better recognition and more comprehensive evaluation of these rare cases should be emphasized.

A rare case and unusual localization of a poorly differentiated giant synovial sarcoma of the ankle: Case report and literature review.

INTRODUCTION: Synovial sarcoma is a rare and aggressive soft tissue tumor that affects the para-articular regions of the large joints. It originates from mesenchymal cells with synovial differentiation. Its management remains surgical with wide excision. CASE PRESENTATION: We report a case of synovial sarcoma with rare localization that occurred in a 37-year-old man on the inner side of the right ankle, and we recall the clinical, radiological, and histological signs that allowed us to suggest the diagnosis as well as multidisciplinary management. DISCUSSION: Synovial sarcoma is a highly aggressive soft tissue tumor with a high risk of spreading. It is a deceptive tumor in some clinical and morphological aspects that may indicate benignity. Although non-specific, MRI is critical for guiding diagnosis, identifying prognostic criteria, and ensuring post-treatment follow-up. CONCLUSION: This rare case underlines the importance of evoking a synovial sarcoma in front of a mass of soft parts of the benign aspect of the ankle, as well as adapting the treatment in order to prevent possible metastases, especially in the lungs.