Intraosseous schwannoma of the femur in a patient with monoclonal gammopathy of undetermined significance
| Autor: | Darren Patrick Moloney, Nazia Faheem, Andrew J. Hughes, Kevin Clesham, Khalid Merghani, Timothy McAleese |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
medicine.medical_specialty
Femoral schwannoma medicine.medical_treatment Schwannoma Article Lesion 03 medical and health sciences 0302 clinical medicine Primary bone tumour hemic and lymphatic diseases Case report medicine otorhinolaryngologic diseases Femur neoplasms business.industry Intraosseous schannoma Soft tissue medicine.disease Curettage nervous system diseases Primary bone 030220 oncology & carcinogenesis Monocloncal gamopathy of underdetermined significance (MGUS) 030211 gastroenterology & hepatology Surgery Radiology Differential diagnosis medicine.symptom business Monoclonal gammopathy of undetermined significance Neurilemmoma |
| Zdroj: | International Journal of Surgery Case Reports |
| ISSN: | 2210-2612 |
| Popis: | Highlights • Intraosseous schwannoma are a rare but important lytic bone lesion on imaging. • There may be an association between primary intraosseous schwannoma and monoclonal gammopathy of undetermined significance (MGUS). • Definitive diagnosis of intraosseous schwannoma is based on a classical histological appearance. • Intraosseous schwannoma is benign and can be treated by local surgical excision alone including in the setting of MGUS. Introduction Schwannomas are slow-growing, benign tumours normally originating from the schwann cells of the nerve sheath. Intraosseous schwannomas account for 0.175% of primary bone tumours and are extremely rare, especially outside the axial skeleton. Monoclonal gammopathy has been associated with soft tissue schwannomas but never with the intraosseous variety. Presentation of case A 55-year-old woman with a background of monoclonal gammopathy of undetermined significance (MGUS) presented with a 2-year history of right thigh pain. CT scan showed a well defined, lytic lesion with a thin peripheral rim of sclerosis in the midshaft of the femur. MRI displayed a hyperintense, well marginated and homogenous lesion. Definitive diagnosis was made based on the classical histopathological appearance of schwannoma. We managed our patient with local curettage and prophylactic cephalomedullary nailing based on her high mirel score. Discussion Intraosseous schwannomas are poorly understood but most commonly reported in middle-aged women. Radiologically, their differential diagnosis includes malignant bone tumours, solitary bone cysts, aneurysmal bone cysts and giant cell tumours. As a result, they are usually diagnosed incidentally on histology. Although malignant transformation is possible in soft tissue schwannomas, all intraosseous schwannomas reported to date have been benign. Conclusion This case demonstrates the importance of suspecting intraosseous schwannoma as a differential diagnosis for lytic bone lesions to avoid the overtreatment of patients. We also highlight monoclonal gammopathy of undetermined significance as a potential risk factor for a poorly understood disease and make recommendations about the appropriate management of these lesions. |
| Databáze: | OpenAIRE |
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