Fan Tang

Advanced alveolar soft part sarcoma responds to apatinib

Alveolar soft part sarcoma (ASPS) is a rare, hypervascular soft tissue sarcoma with a low chemotherapy response rate. Here, we report an ASPS case with multiple lung metastases on initial presentation. The primary tumor, a hypervascular soft tissue mass 4.1×3.2×2.0 cm, located in the right thigh, was resected prior to chemotherapy. The patient suffered disease progression after two cycles of gemcitabine-docetaxel treatment. Immunohistochemical examination of the tumor tissue revealed strong positive staining for vascular endothelial growth factor (VEGF) and VEGF receptor-2 (VEGFR-2). The patient was subsequently treated with apatinib (500 mg/day), a specific VEGFR-2 inhibitor. Treatment was well tolerated, and the patient exhibited a partial response, with the lung metastases reduced in size and number after one month of therapy. To date, 12-month progression-free survival has been achieved. Apatinib may provide an additional treatment option for metastatic ASPS, particularly in cases resistant to other chemotherapeutic options. Furtherstudies with more cases with longer follow-up times will be necessary to determine the clinical efficacy of apatinib for treatment of ASPS.

A case report of apatinib in treating osteosarcoma with pulmonary metastases.

Rationale: Osteosarcoma is the most common malignant bone tumor in children and adolescents. Pulmonary metastases lead to a significantly increased risk of death. Apatinib, a new potent oral small-molecule tyrosine kinase inhibitor targeting the intracellular domain of vascular endothelial growth factor receptor 2 (VEGFR-2), shows survival benefits in treating advanced or metastatic gastric adenocarcinoma, non-squamous non-small cell lung cancer and metastatic breast cancer. However, its efficacy in metastatic osteosarcoma has not been reported yet. Patient concerns: Herein, we presented a 50-year-old man patient who visited hospital due to local bone pain in the left leg. Diagnoses: He was initially diagnosed with osteoblastic osteosarcoma. Interventions: The patient suffered repeated resection surgeries but developed multiple lung metastases. Positive staining for CD31, CD34, and VEGFR-2 were detected in the tumor section. As he refused to receive chemotherapy due to concerns regarding the chemotherapy toxicities and sorafenib due to high cost, apatinib was given at a dose of 500 mg daily. Outcomes: Eleven months following apatinib administration, the patient achieved a partial response according to the RECIST 1.1 standard. No severe toxicity or drug-related side effect was observed during the treatment. Lessons: Therefore, apatinib could be a new option for the treatment of metastatic osteosarcoma. Clinical trials are required to further confirm the efficacy and safety of apatinib in treating pulmonary metastases from osteosarcoma.

Cemented allograft-prosthesis composite reconstruction for the proximal femur tumor.

Introduction Cemented allograft-prosthesis composite (APC) reconstruction is one option following resection of the proximal femur tumor. However, rare studies have focused on the indications and complications. The goal of the present study was to (1) ascertain the indications for cemented APC arthroplasty in the proximal femur; (2) identify the detailed perioperative management; and (3) illustrate our experiences to avoid the complications of cemented APC. Materials and methods A total 28 patients who underwent cemented APC reconstruction of the proximal femur after tumor resection were retrospectively evaluated at a median follow-up of 56 months. Clinical records and radiographs were reviewed to evaluate patients’ outcome. Results In our series, excluding three cases of death that had a short follow-up period, union occurred in 22 (88.0%) patients (range 9–18 months). Nonunion of the greater trochanter was seen in six of the 12 patients (50.0%). Eight (32.0%) hips had resorption. There were two (8.0%) hips that were observed to have asymptomatic wear of the acetabulum. The average Musculoskeletal Tumor Society (MSTS) score was 26.5 points. The average Harris Hip Score (HHS) score was 80.6 points. There were no cases of recurrence, but metastasis was found in two hips. Conclusions Mastering indications, perioperative management, and complication prevention are all very important in the APC reconstruction after resection of the proximal femur.

Limb-salvage treatment of en-block resected distal femoral tumors with endoprosthesis of all-polyethylene tibial component: a 9-year follow-up study

Objective To evaluate the medium-term outcome of limb-salvage surgery using all-polyethylene tibial endoprosthetic replacement following en-block resection for distal femoral tumors. Methods Forty-nine patients with distal femoral tumor were treated between June 2006 and June 2012. The follow-up period was 6–110 months (average 53.4 months). The prosthetic survival was analyzed using the Kaplan–Meier method. The classification of failure of limb salvage after reconstructive surgery for bone tumors was adapted. Limb function was evaluated with the scoring system of the Musculoskeletal Tumor Society (MSTS). Results Complications were observed in six cases (12.2%). Four suffered infection around the prosthesis, of which two cases were treated with debridement, drainage, and antibiotics without removal of the prosthesis, and the other two cases underwent amputation. Two cases were identified as radiographically loose at 7 year follow-up and did not require revision surgery. One patient underwent amputation due to local recurrence. Failure of limb salvage occurred in nine cases (18.4%), of which two cases were of type 1A, two cases of type 2B, three cases of type 4A, one case of type 4B, and one case of type 5A. The mean MSTS score was 84.3%. Twelve cases died due to distant metastases (24.5%), and the average survival time for these patients was 13.5 months. Thirty-seven patients survived (75.5%), for whom the average follow-up time was 66.3 months and the 5-year prosthetic survival rate was 88.2%. Conclusion The outcome of medium-term and long-term clinical follow-up was satisfactory. All-polyethylene tibial endoprosthetic replacement following en-block resection can be an alternative method of limb salvage for distal femoral tumors.

Safety and efficacy of denosumab in the treatment of pulmonary metastatic giant cell tumor of bone

Background Giant cell tumor (GCT) of bone is an intermittent and locally aggressive tumor with increasing pulmonary metastatic potential. In this study, we evaluated the interim clinical outcome of denosumab in patients with pulmonary metastatic GCT. Materials and methods We retrospectively reviewed seven patients with pulmonary metastatic GCT who received denosumab treatment after local tumor surgery during January 2014 and July 2016. Denosumab treatment for all patients lasted for at least 12 months. Serial chest computerized tomography scan was used to monitor the drug response and RECIST 1.1 standard was used to evaluate the therapeutic efficacy. Results All patients experienced chest pain relief in the first month of treatment. Three patients showed partial response. Four patients got stable disease after denosumab treatment. Adverse events included one patient with hypocalcemia and two patients with fever. No treatment-related deaths were reported. No patient with metastatic disease progression was found during an average of 28.6 months follow-up period. Conclusion We presented a promising interim clinical outcome using denosumab to treat patients with pulmonary metastatic GCT. Denosumab might be considered as the first-line treatment for patients with inoperable metastatic pulmonary GCT. However, Phase II clinical study with larger number of patients and longer follow-up period is needed to detect the further efficacy and safety of this drug for lung metastatic GCT.

Low-grade central osteosarcoma in proximal humerus: a rare entity

Low-grade central osteosarcoma is a rare subtype of tumor with low-grade malignancy. Currently, wide resection with negative resection margin is the standard treatment for this disease. The role of neoadjuvant chemotherapy in low-grade central osteosarcoma was controversial and was mostly considered for tumors containing high-grade focal areas. Local tumor recurrences often exhibited a tumor with higher histologic grade or differentiation with the potential for metastases. In low-grade central osteosarcoma, timely wide resection after definite diagnosis can result in 5-year survival for almost 90%. However, the relatively nonspecific radiological and pathological findings make diagnosis very difficult. MDM2 and CDK4 are specific and provide sensitive markers for the diagnosis of low-grade central osteosarcoma, helping to differentiate low-grade central osteosarcoma from some benign lesions, including fibrous dysplasia, bone giant cell tumor, and chondrosarcoma. Here, we report the case of a 19-year-old woman with low-grade central osteosarcoma located at the proximal humerus. The affected site was rare, but the sensitive biomarkers CDK4 and MDM2 were positive. The patient recovered well after wide tumor resection following a proximal humerus endoprosthesis replacement. Our case highlighted the management strategies in low-grade central osteosarcoma. Being familiar with radiographic features, understanding the biological characteristics, and mastering diagnostic biomarkers can help oncologists avoid embarrassing situations in treatment when this rare tumor is highly suspected, even when located at an uncommon site. The discussion in this report focuses on radiographic and pathological features, advances of biomarkers that help in differential diagnosis, and current treatment options in low-grade central osteosarcoma.

Classic Hodgkin lymphoma in pelvis: A case report highlights diagnosis and treatment challenges

Rationale: Classic Hodgkin lymphoma with pelvic involvement is a rare entity. Diagnosis and treatment for such an uncommon disease are challenging. Here we report a special case of classic Hodgkin lymphoma in pelvis. Patient Concerns: A 20-year-old woman was admitted to our department due to left hip symptoms. The patient reported a history of drenching night sweats, low-grade fever, pruritic rash on the body, and an almost 15% weight loss during the previous 3 months. Diagnoses: Imaging studies revealed osteolytic destruction of the left hemi-pelvic with a huge soft-tissue mass. Open biopsy established the pathological diagnosis of classic Hodgkin lymphoma. Interventions: Considering the B symptom, bulky disease, and high risk of pathological fracture of the patient, we performed limb-salvage surgery and 6 cycles ABVD chemotherapy with 2 cycles before surgery. Outcomes: Up to now, at the 3-year follow-up, there is no sign of disease relapse and metastasis. Besides, her limb function recovered well. Lessons: Based on this case and literature we reviewed, diagnoses for primary bone Hodgkin lymphoma should be cautious. For the treatment, chemotherapy was the main treatment option. Classic Hodgkin lymphoma patients seldom received tumor resection surgery, but for the special bone classic Hodgkin lymphoma individual with a huge tumor volume and high risk of pathological fracture in our study, limb-salvage surgery based on ABVD chemotherapy provided a satisfying clinical outcome.