Objective To discuss the importance of early di agnosis and treatment of primary hy-perparathyroidism in multiple bone resorptions.Methods From April 1997to April 2001,5patie nts were regarded as having multiple bone resorptions.There were 4females and 1male.The age ranged from 25to 46years(average 37years).The mean interval between symptom o nset and final diagnosis was 19.5mon ths(from8to 36months).All patients were suffering from se vere bone pain in need of strong antalgic,and the symptoms including fatigue,depression,loss of appetite and malaise,e tc.Abnormality on bone scan indicated multiple areas of skeletal involvem ent,bone radiographs showed multip le bone resorptions in skull,rib,pelvis and limbs.The range of serum calcium level was 2.27-3.22mmol /L and that o f PTH was 168-214ng /ml.Palpable neck mass was found i n three patients.Results All patients had undergone resectio n of the abnormal parathyroid.Three were parathyroid adenomas and two defined a s hyperplastic glands.All of them had pain relief after operation.The average follow-up was 15months(3-27months);the bone lesions have been repaired partially or totally s hown in X -ray examination during follow-up.Conclusion Clinically,in younger patients with multiple bone resorptions,hyperparathyroidism should be highly suspected,so that timely parathyroid surgery can be ca rried out for the treatment.[

Objective To investigate the clinical manifestation and diagnosis of osteitis deformans. Methods Three male patients with characteristic manifestations of osteitis deformans were reviewed in this study. The ages of the patients were 51, 49 and 43 respectively. Two patients with pelvic lesions on X ray films presented symptomatic pain and malaise localized in hip, one complained of tibial bowing deformity. The typical radiographic characteristics were bone enlarged size, increased bone mineral density with uneven distribution; the bone trabeculae were usually coarse and arrayed irregularly like a grid. All patients underwent the diagnostic biopsy. Under microscopic observation, the bone structures were arranged disorderly, the trabeculae were thickened and enriched with osteoclasts and osteoblasts, irregular blue cement line were also seen in thickened trabeculae, the bone marrow were replaced by the fibrovascular connective tissue. All patients were given calcitonin and diphosphonates as medical treament. Results In the follow up period of an average of 16 months(range 3-26 months), one patients had pain relief at lesioned hip but functional limitation remained, the others did not have any sign of malignant progression. The serum alkaline decreased dramatically and the involved bone did not present with deteriorated change. They were working as usual. Conclusion Osteitis deformans is a disorder of bone architecture resulting from disturbance in the rate of bone turnover; typical radiographic and pathologic feature is the important points for definite diagnosis of osteitis deformans.

SUMMARY To describe the first case of leiomyosarcoma of the tibia with multiple skeletal metastases in China. A 31-year-old woman was referred to Peking University People’s Hospital for evaluation of a lesion on the proximal left tibia. The radiographic examination showed the lesion had a moth-eaten destructive appearance with ill-defined border and soft tissue extension. The tumor is isointense to muscle on T1-weighed images and heterogeneous and of high signal on T2-weighed images. Technetium-99m bone scintigraphy demonstrated multiple markedly increased skeletal radioisotope uptake. The tibia lesion was surgically removed. Microscopically, tumor cells consisted of spindle shaped cells, arranged in bundles, with cigar-shaped and blunt-ended nuclei. Immunohistochemistry revealed that the tumor cells were positive for smooth muscle antigen (SMA) and caldesmon. It should be cautious to make the diagnosis of leiomyosarcoma arising in bone. After excluding metastasizing leiomyosarcoma of gastrointestinal tract, urinary system and uterus, the final diagnosis relies on immunohistochemical analysis, however, other spindle cell sarcomas have to be differentially diagnosed.

Objective To evaluate the functional outcomes of different limb salvage procedures in patients with bone and soft tissue sarcomas of the shoulder girdle.Methods From July 2001 to July 2008,16 patients with limb salvage for sarcomas of shoulder girdle were respectively analyzed,including 11 males and 5 females with an average age of 38.4 years (range,17-67).Localizations of the tumors were 8 in the scapula(including 4 chondrosarcomas,1 Ewing sarcoma,and 3 metastases),5 soft tissues of the shoulder girdie(including 2 synoviosarcomas,1 fibrosarcoma,1 hemangioperieytoma,and 1 well-differentiated liposarcoma),and 3 proximal humerus (including 1 osteosarcoma and 2 metastases).Twelve patients were treated with classical Tikhoff-Linberg procedures,and 4 with improved procedures.Results The mean surgical time duration was 3 hours.The mean blood loss was 1600 ml.The mean follow-up time was 40 months.Major complications included 2 cases of delayed wound healing,and 1 ulnar nerve injury.One patient had local recurrence and died of pulmonary metastases 18 months after second operation of interscapulothoracal amputation.The patient with fibrosarcoma also died of pulmonary metastases 23 months later.One patient with synoviosarcoma was alive with pulmonary metastases in 9 months.Four of 5 patients with carcinoma metastases died during 11 to 23 months later.The 5-year cumulative survival rate was 34.6%.Functions were preserved in the whole hand and elbow.The MSTS functional score of the patients receiving classical Tikhoff-Linberg procedures was 14.7,while improved Tikhoff-Linberg procedures was 19.5.Conclusion The Tikhoff-Linberg procedure not only provides a wide resection of tumors in the shoulder girdle but also preserve the whole hand and elbow functions.The shoulder function was poor in patients receiving classical Tikhoff-Linberg procedures.

Objective To identify prognostic factors and imply the appropriate management of pulmonary metastases from osteosarcoma. Methods Data were obtained retrospectively from 99 osteosarcoma patients with pulmonary metastases who received systemic treatments and followed up in Department of Orthopedic Oncology of Peking University People's Hospital from January 2000 to July 2008. The mean followup time was 24.45 months (range, 13-91 months). The average age of the patients at diagnosis was 20.39 years (range, 7-68 years); the male female ratio was 65:34. All the patients had undergone resection of tumors, and 93 of them had received neoadjuvant chemotherapy. Pulmonary metastases were found in 16 patients at diagnosis of osteosarcoma, in 47 patients during chemotherapy and 36 patients after the whole treatment. The age of patients, the number of pulmonary metastases, the time of appearance of pulmonary metastases,extrapulmonary metastases, the response of primary tumor to chemotherapy and management of pulmonary metastases were analyzed. Results The interval between the diagnosis of osteosarcoma and pulmonary metastases ranged from 0 to 74 months with the mean time of 9.05 months. The 1-year, 2- and 5-year cumulative survival rates were 87.4%, 56.8% and 23.4% respectively in the study, with the median survival time of 25 months. Univariate analysis showed the significant factors included the time of appearance and the management of pulmonary metastases. Cox regression analysis revealed that the time of appearance of pulmonary metastases was the only prognostic factor. There was no significant difference between age, number of pulmonary metastases, extrapulmonary metastases and response of primary tumor to chemotherapy.Conclusion Characteristics related to the time of appearance of pulmonary metastases and surgical management especially as pulmonary resection appear to be more predictive of a successful outcome.

Objective To explore the recurrence rate, the complications and functional status of 25 patients with grade I chondrosarcoma of long bones treated by intralesional curettage and electrocauterization, and to determine the feasibility and effi?cacy of this method for grade IA chondrosarcoma of long bones. Methods Twenty?five eligible patients treated in our hospital from May 2003 to December 2011, were collected in this study with a mean age of 49 years (range, 28-72 years). According to En?neking staging system, all the lesions were staged as IA. Patients received surgery of the lesion before were excluded. The involved bones were femur (13 patients), tibia (4 patients), and humerus (8 patients). During the operation, a large elliptical cortical window about the size of the longest dimension of the lesion was made to ensure the thorough exposure of the lesion and avoid inadequate curettage. The lesion was curettaged thoroughly after the cortical window was made, then the high speed bur drill was applied to clear away a thin layer of the reactive bone shell. After a thorough lavage of the cavity, electrocauterization was done alongside the cavity wall slowly twice. Allograft or artificial bone was used to fill in the cavity to enhance bone healing. If mechanical property of the long bone was endangered by the cortical window and the surgical procedure, plate and screws were applied to strengthen the bone to avoid post?operative fracture. Results All the 25 patients were followed up regularly. The period of follow?up was from 38 months to 142 months, with a mean time of 80 months. Calcification was observed clearly in all the lesions. The length of the le?sions varied from 4 cm to 11 cm, with an average length of 7 cm. Nineteen patients received a plate and screws fixation because the mechanical property of the affected bone, while the rest received no internal fixation. Deep infection occurred in 1 patient (4%, 1/25) two months after operation. Thorough debridement and packing of bone cement with antibiotics was done to control the infec?tion. The infection has been controlled till the latest follow?up at 62 months after operation. Local recurrence occurred in one pa?tient thirteen months after the primary operation. Tumor resection, mega?prosthesis replacement was applied to the patient. The pa?tient has been disease free till now for 67 months. Mean Musculoskeletal Tumor Society (MSTS) 93 functional score of all patients was 93%(range, 83%-100%). The five?year survival rate of these twenty?five patients was 100%. Conclusion Intralesional cu? rettage together with the application of high?speed bur drill and electrocauterization is an applicable method for grade I chondrosar?coma of long bones. The excellent functional result and low recurrence rate make it a favorable option for the selected cohort of pa?tients.

Objective To determine the feasibility of limb salvage with major vascular reconstruction for sarcomas of extremities, focusing on the early complications, oncological and functional outcomes.Methods Between August 2004 and June 2009, 13 patients (mean age 38 years, range 14-63 years) underwent wide resection of upper and lower extremity sarcomas to include the involved arterial and venous segments. 4 patients had bone sarcomas and 9 soft tissue sarcomas, 6 patients only had underwent artery replacement and the other 7 had underwent both artery and vein. 8 resected vessels were reconstructed with vascular graft and 5 with ipsilateral or contralateral saphenous vein. Results No patient suffered from reperfusion injury and infection. The patient with osteosarcoma in proximal humerus had suffered acute arterial graft thrombosis at first day postoperatively; however, the limb was salvaged after successful thrombectomy. 5 of 8 patients with synthetic graft developed minor delayed wound healing and hematoma, but the other 5 patients with autologous saphenous vein replacement did not. The mean follow-up was 19.4 months (7-45months). One patient died of pulmonary metastasis; the patient with osteosarcoma in proximal humerus had developed local recurrence, but refused amputation and was alive with metastasis. No arterial occlusion was observed at final follow-up or at the time of death, but the patency of artery and vein was 100% and 28.6%respectively. One patient who has pulmonary metastases before operation died of metastases 7.5 months after operation. Four patients developed pulmonary metastases, therefore, Kaplan-Meier survival analysis showed that 2-year overall and metastasis-free survival rate was 90.9% and 63.6% respectively. Functional status were judged as good or excellent (mean MSTS score 72%) in 12 of 13 patients. Conclusion The study indicate that malignant involvement of major vessels is not a contraindication for limb-salvage. Vascular reconstruction is a feasible option in limb salvage surgery. Wide resection with vascular reconstruction provide acceptable oncological and functional outcome of limb salvage.

Objective To evaluate the clinical and functional outcome of biological reconstruction by using pasteurized autograft and massive allograft after en-bloc resection of primary femoral diaphyseal sarcomas.Methods Retrospectively reviewed 19 consecutive patients with primary femoral diaphyseal sarcomas between Feb.2005 and Dec.2013.There were 11 males and 8 females with the mean age of 18 (2-38) years old.Thirteen patients were diagnosed as osteogenic sarcoma (OS),while five Ewing' s sarcoma (EWS) and one malignant fibrous histocytoma (MFH).All patients were treated with wide local excision,and 9patients were reconstructed by intercalary femur segmental allograft and 10 by pasteurized autograft.The median length of the resected bone was 16.9 (9-24) cm.15 segmental grafts were fixed by using plates including 10 intramedullary free vascularised fibular graft constructs,the other 4 segmental grafts were fixed by intramedullary nails.The average operation time for pasteurized autograft construct was 5.1 hours,while the time for intercalary allograft construct was 4.22 hours.Of 38 host-donor junctions,there are 28 diaphyseal junctions and 10 metaphyseal junctions.Results The average operation time for pausterized autograft construct tended to be longer than intercalary allograft (5.1h Vs 4.22h),although the difference did not reach the significance.Bone union occurred at a median of 10.3 months and 7.25 months at diaphyseal and metaphyseal junction for pasteurized autograft-host construct; 13.8 months at the diapyhseal junction and 11.5 months at the metaphyseal junction for allograft-host construct.Bone healing time of diaphyseal junction and metaphyseal junction between these two constructs were significant difference.Eight of 19patients (42.1％) developed complications:5 bone unnunion/fracture (including 1 subsequently developed local recurrence),1deep infection and 2 local recurrence (including 1 soft tissue recurrence).The mean overall follow-up was 33.5 months (3-107),five patients died of lung metastases,the cumulative patient survival was 76.5％ at 2 years and 61.2％ at 5 years determined by Kaplan-Meier method.All living patients except the MFH patients who received amputation,had a mean MSTS score of 83.7％ (70％-95％).Conclusion Although the reconstructive procedure with pasteurized autograft is more complicated and needs longer operation time than allograft reconstruction,the bone healing time with autograft is significant shorter than allograft.Our observations suggest the pausterized autograft shell with intramedullary free fibular graft is strongly recommeded.

Objective:We designed and applied a novel, bionic megaprosthesis for distal femur, which substituted the hinged articulation with an artificial ligament and a constrained tibial insert in order to maintain articular stability and reduce stress and the risks of wearing, loosening and breakage.Methods:We reviewed the cases of distal femoral osteosarcoma in children population who were initially treated in our center during 2019. Twelve cases of bionic distal femoral prosthesis (bionic group) and 21 cases of fixed-hinge distal femoral prosthesis (control group) were included. In the bionic group, there were six female and six male with the mean age as 8.8±2.6 y. One patient had pathological fracture. Staging according to Enneking system included 1 case of stage IIA, 10 cases of stage IIB and 1 case of stage III. In the control group, there were seven female and fourteen male with the mean age as 8.6±2.2 y. Pathological fracture was seen in three cases. Staging included 15 cases of stage IIB and 6 cases of stage III.We investigated the peri-operative safety, oncological outcome, complication profiles and post-operative functional status of the bionic prosthesis by comparing the baseline data, operative data, and oncological and prosthetic outcomes between the two groups.Results:The two groups were comparable in terms of baseline data including gender, age, height, weight and onset duration, and operative data including duration of operation (126.7±27.9 min vs 143.3±38.9 min, P=0.203), intra-operative hemorrhage (162.5±212.3 ml vs 247.6±175.6 ml, P=0.224) and duration of wound drainage (6.3±2.4 d vs 6.4±3.4 d, P=0.908). The mean follow-up duration of bionic group was 16.0±4.7 months, during which time three patients had systemic progression and one of them died of disease. Another patient had local recurrence and subluxation of the prosthesis that was treated by amputation. Subluxation might be related to the huge tumor mass and excessive resection of the quadriceps femoris. As for the control group, the mean follow-up duration was 12.7±4.5 months. Three patients had systemic progression and all patients were still alive by last follow-up. Local recurrence was seen in two patients and was treated by excision. No prosthetic complications were seen in the control group. The averaged range of motion (ROM) was greater in the bionic group than that in the control group (120.6°±13.6° vs 92.0°±7.7°, P<0.05), but the MSTS 93 scores were similar between groups (29.1±0.9 vs 29.5±0.6, P=0.337). Conclusion:Compared with the fixed-hinge distal femoral prosthesis, the bionic prosthesis had good peri-operative safety, did not increase the risks of prosthetic complications, and could achieve a better range of motion and a similar functional status based on the results of short-term follow-up.

Objective:To investigate the clinicopathological, immunohistochemical and molecular features of small round cell sarcoma (SRCS) of the bone and soft tissue, and to compare the diagnostic value of different techniques.Methods:Seventy-two cases of SRCS of the bone and soft tissue diagnosed at People′s Hospital, Peking University from January 2016 to March 2020 were recruited and retrospectively analyzed for pathological morphology, immunophenotype and fluorescence in situ hybridization (FISH) data. Next generation sequencing (NGS) was performed on 13 difficult cases.Results:In the study cohort, the patients ranged in age from 4-55 years, with a male predominance. The most Ewing′s sarcomas and osteosarcomas occurred in the bone, while CIC-rearranged sarcomas, BCOR-rearranged sarcoma, synovial sarcoma, extraskeletal myxoid chondrosarcoma and FUS-NFATc2 rearranged sarcoma occurred in soft tissue. Histologically, all cases were composed predominantly of small round cells. Most cases were positive for vimentin and CD99, and showed a variable reactivity for neurogenic markers. Muscle marker and epithelial marker were negative for most cases. Combined with clinical features, histopathologic findings, immunophenotype, FISH and NGS, we diagnosed 46 Ewing sarcomas, 14 osteosarcomas, 3 CIC-rearranged sarcomas, 1 BCOR-rearranged sarcoma, 1 synovial sarcoma, 1 clear cell soft tissue sarcoma, 1 extraskeletal myxoid chondrosarcoma, 1 FUS-NFATc2 rearranged sarcoma, and 4 undifferentiated small round cell sarcomas.Conclusions:SRCS of bone and soft tissue is a group of malignant mesenchymal tumors based on morphological features. Most cases can be diagnosed with a combination of clinical characteristics, morphological features and immunohistochemical phenotype, while some cases require such further tests as FISH and NGS technologies, and NGS can be useful in diagnosing and categorizing SRCS.