Non-ossifying fibromas: Case series, including in uncommon upper extremity sites

AIM To investigate non-ossifying fibromas(NOFs) common fibrous bone lesions in children that occur in bones of the lower extremities.METHODS We analyzed 44 cases of NOF including 47 lesions, which were referred with a working diagnosis of neoplastic lesions. Lesions were located in the upper extremities(1 proximal humerus, 1 distal radius) and the lower extremities(25 distal femurs, 12 proximal and 4 distal tibias, and 4 proximal fibulas).RESULTS Three cases had NOFs in multiple anatomical locations(femur and fibula in 1 case, femur and tibia in 2 cases). Overall, larger lesions > 4 cm and lesion expansion at the cortex were seen in 21% and 32% of cases, respectively. Multiple lesions with bilateral symmetry in the lower extremities suggest that these NOFs were developmental bone defects. Two patients suffered from fracture and were treated without surgery, one in the radius and one in the femur. Lesions in the upper extremities(i.e., humerus of a 4-year-old female and radius of a 9-year-old male) expanded at the cortex and lesion size increased with slow ossification.CONCLUSION NOFs in the lower extremity had fewer clinical problems, regardless of their size and expansiveness. In these two upper extremity cases, the NOFs had aggressive biological features. It seems that there is a site specific difference, especially between the upper extremity and the lower extremity. Furthermore, NOFs in the radius are predisposed to fracture because of the slender structure of the radius and the susceptibility to stress.

Jaffe-Campanacci syndrome resulted in amputation: A case report

BACKGROUND Jaffe-Campanacci syndrome(JCS)is a very rare syndrome.The treatment of JCS is more conservative,and most authors recommend that no surgery should be done in asymptomatic patients.The conventional concept holds that the natural course of non-ossifying fibromas(NOFs)grows with the development of bones,and the osteolytic region gradually stops expanding and self-healing through bone ossifying around the lesion and ossification within the lesion.But in this case,the bone lesions were potentially biologically aggressive,which led to severe limb deformities and pain.CASE SUMMARY We present the case of a 5-year-old girl with JCS presenting with not only NOF sand café-au-lait macules,but also showed features not mentioned before,severe limb pain,and at last resulted in amputation.She was admitted to our hospital after presenting with claudication and mild pain over her right thigh,which worsened when stretching or being touched.Skin examination revealed multiple café-au-lait macules on the neck,arm,axilla,and torso,including the nipples and perineum.Radiographs revealed multiple lytic lesions in the proximal part of the right humerus,distal part of the right clavicle,proximal and distal parts of the right femur,and proximal parts of the right tibia and fibula.Curettage and biopsy were performed on the distal part of the right femur.At the age of 7,the girl was re-admitted to our hospital for a pathological fracture in the middle in the right femur and underwent Intralesional excision,internal fixation,bone grafting,and spica casting.At the age of 10,the girl came to our hospital again for severe pain of the right leg.Amputation from the middle level of the right femur was performed.We present the case of a 5-year-old girl with JCS presenting with not only NOFs and café-au-lait macules,but also showed features not mentioned before,severe limb pain,and at last resulted in amputation.She was admitted to our hospital after presenting with claudication and mild pain over her right thigh,which worsened when stretching or being touched.Skin examination revealed multiple café-au-lait macules on the neck,arm,armpit,and torso,including the nipples and perineum.Radiographs revealed multiple lytic lesions in the proximal part of the right humerus,distal part of the right clavicle,proximal and distal parts of the right femur,and proximal parts of the right tibia and fibula.Curettage and biopsy were performed on the distal part of the right femur.At the age of 7,the girl was re-admitted to our hospital for a pathological fracture in the middle in the right femur and underwent Intralesional excision,internal fixation,bone grafting,and spica casting.At the age of 10,the girl came to our hospital again for severe pain of the right leg.Amputation from the middle level of the right femur was performed.CONCLUSION In our opinion,education on preventing pathological fractures and explaining the consequent serious consequences to the parents is a matter of prime significance.At the same time,prophylactic treatment(restricted exercise,support,or surgery)is also considerable for JSC.

Enchondromas of Long Bones and Other Skeletal Lesions Found Incidentally Need Critical Evaluation, But Rarely Systematic Follow-Up

Purpose: Incidental bone lesions are a challenge for the specialist, who has to give recommendations for further management. This review of our cases will assist in the decision whether the lesion can be “neglected”, needs further active follow-up or direct initiation of treatment. Patients and Methods: 153 cases of incidental bone findings were presented to our musculoskeletal tumor service for evaluation from July 2008 through June 2021. 73 of them were cartilaginous tumors and 63 of these were diagnosed as enchondroma of a long bone based on X-Ray and MRI. Results: Follow-up imaging of the enchondroma patients was available for 35 patients at 1 to 13 years (mean 4.3 y), with no change in size except for one femoral diaphyseal enchondroma with increasing diameter from age 18 to 20 years. 14 additional patients answered written contact stating that they remained asymptomatic at 2 to 12 years (mean 5.6 y). None of the patients has been reported to the Swiss Confoederation Cancer Registry to have developed malignancy. Among the 10 other cartilaginous tumors were one chondrosarcoma grade II exhibiting different imaging, 3 non-long-bone localizations (pelvis, scapula and rib), 2 Ollier-type enchondromas, and 2 osteochondromas. Incidental findings other than cartilaginous tumors were fibrous dysplasia (n = 31), non-ossifying fibroma (n = 31) and 18 other “sporadic” entities. Conclusions: Incidentally found enchondromas not exhibiting aggressive features need no systematic follow-up and patients can be “discharged” with the advice to present, if symptoms would develop. This also applies to fibrous dysplasia and the other sporadic lesions. 6 cases with other diagnoses needed specific treatment.