Surgical treatment outcome of giant cell tumor of distal ulna: En bloc resection vs. curettage and bone graft.

Background: Giant cell tumor (GCT) of the bone is a benign neoplasm with local aggressive behavior. Distal ulna is a very rare place for GCT. Published studies have mainly focused on case reports, and thus there is no consistent treatment strategy for this tumor at this location. This retrospective study was conducted to evaluate the oncological and functional results of 2 different surgical treatment methods for GCT in distal ulna. Methods: In this study, 9 patients with GCT of distal ulna were followed after surgical treatment of GCT of distal ulna. Of the patients, 2 had local recurrence after surgery in other hospitals and 7 had primarily been admitted to our hospital. Four patients, all with grade 2 Enneking and Campanacci's classification, were treated by extended curettage and bone grafting, while 5 patients with grade 3 were managed by distal ulnar resection. Minimum follow-up time was 24 months. Results: Patients with grade 3 GCT of distal ulna, who had extended curettage, had a local recurrence up to one year after surgery. Local recurrence was never seen in those with grade 3 disease with resection or in those with grade 2 disease with extended curettage as the treatment of GCT in this location. Conclusion: Based on our study, which is the largest cohort of GCT of distal ulna, extended curettage, and en bloc resection can be suggested as valuable methods of treatment for grades 2 and 3 tumors, respectively.

Intra-articular osteoid osteoma of the capitulum: A diagnostic challenge.

Here, we describe the first case of intra-articular osteoid osteoma of the capitulum, which is presented as elbow pain, extension lack, and sensation of click in joint flexion. Surgical treatment either arthroscopic or open is more in use in this location of the tumor than cortical osteoid osteoma.

Multiple Schwannomas in Sciatic Nerve: A Rare Case Report.

Schwannomas are the most common nerve sheath tumors which are usually solitary enucleated lesions. Multiple schwannomas are a rare circumstance which may occur with or without other manifestations of neurofibromatosis. In this study, we report the case of a 45-year-old man presenting with pain and swelling in the thigh and popliteal space without any manifestation of neurofibromatosis or neurologic problems. Surgery was performed and revealed four separated tumors in the sciatic nerve which was characteristic of schwannoma and confirmed by pathologic findings. Although multiple schwannomas are frequently associated with neurofibromatosis, the current case demonstrated a patient with multiple schwannomas without any manifestation of neurofibromatosis.

Foot Metastasis: Review of 38 Cases.

Acrometastases are rare and account for approximately 0.1% of metastases. The most common primary cancer site is the lung, followed by colorectal and genitourinary system. We searched PubMed (www. pubmed.com), Google scholar (www.scholar.google.com), Science Direct (http://www.sciencedirect.com), and Springer (http://link.springer.com) databases, using a combination of controlled vocabulary and text word terms and reviewed the last 10 years literature in order to describe demographic trends, anatomical distribution, the most common primary sources of malignancy, and survival rates in the reports of foot metastases. In conclusion 38 cases were included in this review analysis. Lung and genitourinary system were the most frequent primary sites. Forefoot was involved in 71% of all metastases to foot either alone or in combination with other areas of the foot. Calcaneus was involved in about 23% of patients either alone or in combination with other foot bones.

Evaluation of local and circulating osteopontin in malignant and benign primary bone tumors.

PURPOSE: The development of novel and efficient biomarkers for primary bone cancers is of grave importance. METHODS: The expression pattern of osteopontin (OPN) was investigated in the 153 patients with benign (n = 72) and malignant (n = 81) primary bone cancers. Both local and circulating OPN mRNA expression levels and their protein concentration in serum and tumor site were assessed using real-time qRT-PCR, ELISA, and immunohistochemistry techniques, respectively. As a control, 29 healthy individuals were considered. The number of 153 tumor tissue specimens and the 153 paired margins were taken on surgical resection from the patients. 153 blood samples were also drained from all participants, then peripheral blood mononuclear cells (PBMC) and sera were separated. RESULTS: The mean mRNA expression was significantly higher in all of the cancerous tissues than the paired margins and the PBMC of the patients than the controls. Consistently, the protein concentrations of OPN in serum and tumor tissues were significantly higher in the patients. Furthermore, the malignant cases had significantly elevated the mRNA levels and the protein compared to the benign cases. OPN could potentially differentiate the patients from the controls with 100% sensitivity and specificity in serum. Moreover, OPN could predict some of the malignant cases' clinicopathological features, including metastasis, recurrence, grade, and response to chemotherapy. CONCLUSIONS: In conclusion, OPN might be involved in the pathogenesis of primary bone tumors and can be considered as a potential biomarker to bone cancer diagnosis.

The local and circulating SOX9 as a potential biomarker for the diagnosis of primary bone cancer.

PURPOSE: The status of the local and circulating SOX9, a master regulator of the tumor fate, and its relevance to tumor types, severity, invasion feature, response to therapy, and chemotherapy treatment were surveyed in bone cancer in the current study. METHODS: The SOX9 expression level was evaluated in tissue and peripheral blood mononuclear cells from patients with different types of malignant and benign bone tumors also tumor margin tissues using Real-Time PCR. The protein level of SOX9 was assessed using immunohistochemistry and western blot analysis. Also, the correlations of the SOX9 expression level with the patient's clinical and pathological features were considered. RESULTS: The remarkable overexpression of SOX9 was detected in bone tumors compared to tumor margin tissues (P < 0.0001). Malignant bone tumors revealed a higher expression of SOX9 compared to benign tumors (P < 0.0001) while osteosarcoma tumors showed higher expression levels compared to Ewing sarcoma, and chondrosarcoma. Overexpression of SOX9 was observed in high grade, metastatic, recurrent tumors also tumors with poor response to therapy. Besides, the patients under the chemotherapy treatment demonstrated higher levels of SOX9 compared to the rest of malignant tumors (P = 0.02). The simultaneous up-regulation of circulating SOX9 in the patients with bone cancer was observed compared to healthy individuals (P < 0.0001) accompanying with overexpression of SOX9 in malignant tumors compared to benign tumors (P < 0.0001). The circulating SOX9 expression was up-regulated in the patients with malignant bone tumors who receive chemotherapy treatment also patients with high grade, metastatic, recurrent tumors. The protein level of SOX9 was in line with our data on the SOX9 gene expression. CONCLUSION: The simultaneous overexpression of local and circulating SOX9 in bone cancer besides its positive correlation with tumor severity, malignancy, size, and chemotherapy may deserve receiving more attention in bone cancer diagnosis and therapy.

Chondrosarcoma in Metachondromatosis: A Rare Case Report.

Metachondromatosis which was first described in 1971 by Maroteaux is a rare genetic disease consisting of osteochondromas and enchondromas, caused by loss of function of the PTPN11 gene. It is distinct from other cartilaginous tumors such as multiple osteochondromas and hereditary multiple exostosis by the distribution and orientation of lesions, and pattern of inheritance. In Metachondromatosis osteochondromas typically occur in hands, feet, femur, and tibia while enchondromas commonly affect the pelvic bones and femurs. Both tumors are generally reported to regress in adulthood. To the best of our knowledge only one case of Chondrosarcoma has been reported, and our case is the second reported case of Chondrosarcoma in metachondromatosis.

Long-term Results of Osteoarticular Allograft Reconstruction in Children with Distal Femoral Bone Tumors.

BACKGROUND: There is no consensus regarding the best method of reconstruction in pediatric population following the wide resection of malignant bone tumors. More exploration of the complications of osteoarticular reconstruction leads to less existing controversy of this type of reconstruction, which is the main point of this article. METHODS: Long-term outcomes and complications of osteoarticular allograft reconstruction of primary distal femoral bone sarcomas in 22 children with mean age of 10.7 years old were reviewed in this study. Musculoskeletal Tumor Society (MSTS) scoring system was used for functional evaluation of the allografts. RESULTS: With an average follow-up time of 81 months, the outcomes of 16 patients with allografts at the final follow up were evaluated. As expected, Limb length discrepancy (LLD) was observed in all patients (mean LLD= 2.73cm), which was significantly correlated to allograft survival time (P<0.001). Degenerative joint disease (DJD) was also seen in all patients and its grade was also significantly correlated to allograft survival time (P<0.001). The mean MSTS-score was 74% at the latest follow-up, ranging from 60% to 90%. Five and 10 year survival rate of allografts were found to be 93.3% and 62.2%, respectively. CONCLUSION: Osteoarticular allograft reconstruction could result in several complications including DJD. Despite its considerable biologic advantage over endoprosthesis, osteoarticular allograft reconstruction is a long-lasting but still a temporary solution before performing megaprosthesis. This allows patients to preserve their remaining physis for limb growth and become old enough for an adult megaprosthesis.

Giant Cell Tumor of the Sacrum: Series of 19 Patients and Review of the Literature.

There are still some debates regarding the best treatment of Giant Cell Tumor (GCT) of the sacrum. Since GCT of this location is rare, therapeutic strategies are mainly based on the treatment of GCT in other anatomic locations. The objective of this study was to evaluate the oncologic and clinical results of surgical management of sacral GCT with and without local adjuvant therapy. Medical records of 19 patients diagnosed with GCT of the sacrum, were retrospectively reviewed. Sixteen patients were treated by intralesional curettage and three patients with marginal resection. Musculoskeletal tumor society (MSTS) score was used for the evaluation of functional outcome. Prolonged pain was the most common complication after treatment. Mean Pre and post-operative pain based on visual analogue scale (VAS) was 6.1 ± 1.99 and 3.05 ± 1.64, respectively. Postoperative neurologic deficit appeared in six patients. In addition, infection occurred in five patients. One case of spinopelvic instability was also observed after surgery. At average follow up of 158.5 ± 95.9 months (25 to 316 months), recurrence was seen in eight (42.7%) out of seventeen patients treated by intralesional curettage. The size of the tumor significantly correlated with the tumor recurrence (r=0.654, P=0.001). Mean MSTS score was 74.7 ± 16.78. Those patients, in whom sacral nerve roots remained intact before and after surgery, had better functional outcome. Preservation of sacral nerve roots is associated with better functional outcome and less pain. Although an acceptable surgical outcome was observed in our cohort, the problem of local recurrence still warrants further investigations for better local control of the tumor.