Objective To evaluate the efficacy of hypofractionated 3DCRT for primary liver cancer(PLC) with portal vein tumor thrombosis(PVTT).Methods Between April 1999 and August 2003,34 PLC patients with PVTT received hypofractionated 3DCRT.The severity of hepatic cirrhosis was 23 in Child-Pugh gradeA and 11 gradeB.The median value of GTV was 773?cm~3(105-2097?cm~3).The radiotherapy regimen consisted of 38-63?Gy in 7-15 fractions with 4-8?Gy per fraction(median value 5?Gy),the treatment was delivered 3 times per week during every other day.Results Having response rate(CR+PR) of 76%(26/34),the overall 1-,2-,and 3-year survival rate at was 36%,19% and 13%,respectively.Conclusion Hypofractionated three-dimensional conformal radiotherapy is effective for primary liver cancer with portal vein tumor thrombosis.

Objective To introduce a surgical procedure resulting in less scars for unilateral microform cleft lip.Methods A Z-plasty incision was designed at suffered agger nasi,and then subcutaneours dissection of suffered upper lip and basis nasi was performed.Releasing suffered alar nasi base from maxillary bone by partly severing the mesculus compressor naris,an orbicularis oculi flap was formed and fixed to the hypodermis of columella base for advancing to superior medial position and overlaying orbicularis oculi.The labial frenulum extension and vermillion submucoustissue flap plicature for filling incisura were carried out to correct the deformitiy of vermillion incisura.Results After 6-18 months follow-up,the wounds of 7 cases (aged 7-19 years) healed at the first grade.The deformity of unilateral subcutaneous celft lip,such as the agger nasi and vermillion incisura,widened nostril,and deficient abial height,was significantly improved.Conclusions Unilateral subcutaneous celft lip could be satisfactorily reconstructed by the surgical technique consisting of Z-plasty incision at agger nasi,orbicularis oculi flap advancing,labial frenulum extension and vermillion submucous tissue flap plicature,with less scar formation.

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 study one male patient with cirrhosis for spironolactone induced gynecomastia and promote clinical pharmacists providing better pharmaceutical cares .Methods Based on the patient′s diseases and medication history , clinical pharmacists searched related literature and analyzed the cause of gynecomastia for the patient .Results The patient′s breast pain was relieved and finally disappeared after termination of spironolactone .Therefore ,spironolactone was suspected to be the drug to cause gynecomastia .Conclusion Clinical pharmacists can improve pharmaceutical cares by carefully monitoring the adverse reactions and selecting appropriate medications .

Obiective:To investigate the design, the key points of surgery and the outcome of short-term follow-up of hemi-elbow-arthroplastic bionic total humeral prosthesis by using 3D-print.Methods:The clinical data of 8 patients with humerus malignant tumor who underwent total humerus resection and a 3D-printed auxiliary hemi-elbow-arthroplastic total humeral prosthesis replacement from March 2018 to March 2020 at Peking University People's Hospital were retrospectively analyzed. The operative data, oncological outcome, complication profiles of prosthesis and functional status of all 8 patients were also analyzed. There were 3 males and 5 females with a mean age of 5-37 years. Histological diagnosis included 5 cases of osteosarcoma, 2 cases of Ewing's sarcoma and 1 case of chondrosarcoma.Results:The time for production of the prosthesis was (9.8±2.7) d, the operation time was (209±23) min and intraoperative hemorrhage was (569±173) ml. All the prostheses were implanted successfully and no patients experienced intraoperative complications. While 2 patients had postoperative complications, 1 case of temporary palsy of radial nerve and 1 case of local recurrence. LARS artificial ligament or hernia patch was used to reconstruct joint capsule and tendon-ligament attached around the elbow and shoulder joint. The flexion and extension of the elbow was (118±15)° (100-140°) and (11±9)° (0-25°), and the abduction and anteflexion of the shoulder was (28±12)° (15-50°) and (26±9)° (15-40°), respectively. The postoperative Musculoskeletal Tumor Society (MSTS)-93 scale score was (24.1±1.5) scores. The median follow-up time was 17 months (12-32 months), 7 patients had disease-free survival and 1 patient survived with tumor.Conclusions:The novel 3D-printed total humeral prosthesis with hemi-elbow-arthroplasty has a good perioperative safety, which is effective in restoring the function of elbow joint, solving the problem of stress concentration of ulnar marrow lever of total elbow joint prosthesis and lowering long-term wear rate and loosening rate of prosthesis.

OBJECTIVE: To explore the clinical and genetic characteristics of a patient with 17-hydroxylase/17,20-lyase deficiency. METHODS: The patient was infertile without contraception. Laboratory examination showed her chromosomal karyotype to be 46, XX. DNA sequencing was performed to detect variants of CYP17A1 gene in the patient and her family members. RESULTS: Sanger sequencing revealed that the patient has carried homozygous variant c.1486C>T in the exon 8 of the CYP17A1 gene, which resulted in substitution of arginine by cysteine (p.Arg496Cys). Her family members were all heterozygotes for the same variant. CONCLUSION Homozygous variant of the CYP17A1 gene c.1486C>T probably underlay the 17-hydroxylase deficiency in this patient. Above finding has enabled accurate genetic counseling and prenatal diagnosis for her family.

Objective To evaluate the safety and efficacy of denosumab in treatment of patients with pelvic giant cell tumor of bone (GCTB) during perioperative period. Methods This is a retrospective observational study. Twenty-three patients diagnosed with pelvic GCTB undergoing perioperative denosumab treatment in Musculoskeletal Tumor Center of Peking University People's Hospital from January 2014 to December 2016 were reviewed. The subjective adverse reactions and mandibular X-ray films were used to assess the drug safety. As for efficacy, imaging findings (including X-ray, CT, magnetic resonance imaging) were reviewed. MSTS-93 scoring system was applied in the postoperative functional assessment. Histological response rate, objective response rate, clinical benefit rate and event-free survival rate were all used to deficit the efficacy of denosumab in the treatment of pelvic GCTB combined with surgery. All the results of postoperative were compared statistically with pelvic GCTB patients who underwent surgery in the same hospital from 1999 to 2009. Results All the patients were firstly diagnosed as classic GCTB except for one case which was malignant pelvic GCTB. All patients received denosumab preoperatively and/or postoperatively, and the average number of medications was 8.43. According to the surgical patterns, patients were divided into intralesional surgery group (13 cases) and wide resection group (10 cases). The follow-up was 5-47 months(mean:27.30 months),recurrence was observed in 2 cases in the intralesional surgery group, none in the wide resection group. After drug administration, 13 cases were partial response, 7 cases were stable disease, the objective response rate was 65.0 % (13/20), and the histologically clearance rate of giant cells was 85.0 % (17/20). No case of osteonecrosis of the jaw was observed in this study, and all laboratory indicators were normal. The average postoperative MSTS-93 score was 26.87. Compared with pelvic GCTB patients who underwent surgical treatment from 1999 to 2009, in the intralesional surgery group, there was no significant difference in the recurrence rate [15.4 % (2/13) vs. 30.8 % (4/13), P = 0.514], but the limb function was significantly increased (P= 0.002). Conclusions Denosumab combined with surgery plays an important role in the multidisciplinary treatment of pelvic GCTB. The neoadjuvant strategy can reduce patient's intraoperative blood loss by shrinking the tumor size which makes the intralesional curettage surgery possible, and also diminishing the recurrence rate. But more attention should be paid to secondary malignant GCTB during the use of denousmab.

Objective:To evaluate the safety and short-term clinical postoperative functional outcomes of a novel 3D-printed porous prosthesis of the distal tibia for the bone defect after tumorectomy.Methods:From December 2017 to December 2019, a total of eight patients diagnosed with malignant bone tumor of the distal tibia were enrolled in this study. All cases received standard preoperative chemotherapy, after which osteosarcoma resection was performed and ankle arthrodesis was reconstructed using a 3D-printed prosthesis developed by our medical center. The contact surface between the distal part of the prosthesis and the talus is a 3D-printed porous surface, which is conducive to ankle fusion. The length of the prosthesis is adjusted by the conical mounting part of the modular prosthesis. The proximal part of the prosthesis can be fixed either biologically or with bone cement. At postoperative follow-up, the function of the fused ankle was assessed by radiographs and the monthly Musculoskeletal Tumor Society (MSTS) score.Results:Of the 8 patients, 5 were male and 3 were female, aged 8-29 years (mean 16.1±7.4 years), including 7 osteoblastic osteosarcomas and 1 telangiectatic osteosarcoma. Among the procedures, the mean length of osteotomy was 16 cm (11-20 cm). The method of fixation of the proximal part of the prosthesis included one case with 3D-printing of trabecular metal bone, one case with autogenous fibular graft, and six cases with bone cement. All patients were followed up for 7-39 months (mean 15.6±10.5 months). The distal prosthesis and talus were completely fused in all cases. The mean fusion duration was 4.3±0.7 months. The mean MSTS score was 84.2%±3.0% (mean 80%-90%). No tumor recurrence, wound complications, or prosthesis loosening were observed during the follow-up period.Conclusion:The novel 3D-printed distal tibial prosthesis is a safe and effective technique for reconstruction of a massive bone defect after tumorectomy of a malignant bone tumor, with high fusion rate, few complications, and satisfactory postoperative function.

Objective:To investigate the outcome of surgical treatment of malignant tumor at the distal tibial after reconstruction with modular hinged ankle prosthesis.Methods:The data of 9 patients with malignant tumor at the distal tibia at Musculoskeletal Tumor Center of PKUPH from June 2020 to November 2021 were analyzed retrospectively. They were male patients with age of 17 (14, 24) years (range 11-56 years). There were five tumors at the left sides and four at the right sides. There were eight patients with osteosarcoma who received the neo-chemotherapy. Among eight osteosarcomas, one was Enneking IIA and seven Enneking IIB. Furthermore, there was only one patient with renal carcinama and with solitary metastatic lesion at the distal tibia. After the resection of tumor at the distal tibia, talus cartilage was removed and talus component was fixed by lag screws. The proper tibia component was used to restore the defect of tibia and the reduction of tibia and talus components were performed. The following clinical data were collected: baseline demographic features, surgical and follow-up data. The baseline demographic features included gender, age, side, lung or/and other metastasis at initial diagnosis, Enneking stage or systematic progression for renal carcinama, histological type. The surgical data included: surgery time, blood loss, length of bone involved by the tumor, prosthesis type. Monitoring data was also recorded: complications (ankle pain when loading, talar collapse, component loosing, infection and wound dehiscence), local recurrence, pulmonary and systematic metastasis, radiological image and the function at the last follow-up (MSTS and VAS evaluation).Results:Among these nine patients, the average blood loss was 245.6±103.9 ml (range 100-400 ml) and the mean surgery time was 178.9±56.9 mins (range 120-300 min). No patient was lost during the follow-up period and the average follow-up was 21.4±5.6 months (range 12.5-27.2 months). The excision length of tibia was 14 (11, 17) cm (range 11-28 cm). There were one case with 2# prosthetic base, three cases with 3# and five cases with 4#. Five had cement fixation of prosthetic stem and four had the pressing fixation. No pulmonary and other organ metastasis occurred among eight patients with osteosarcoma and one patient with distal tibia matastasis of renal carcinama. One patient with OShad the local recurrence and received the resection. One sustained the deep infection after four months and received the removal of prosthesis and spacer implant. At the final follow-up, except one with deep infection and receiving the removal of prosthesis and spacer implant, eight patients were assessed for the function. The average MSTS was 97.1%±3.3% (range 93%-100%). The VAS of all patients was 0. One patient with prosthesis removal had no functional evaluation. At the final follow-up, all patients walked without crutch. No breakage and loosening of prosthetic stem, talar collapse, prosthetic sinking and ankle pain occurred at the final follow-up.Conclusion:The early satisfactory outcome can be obtained for patients with segmental defect after resection of malignant tumor at the distal tibia, who received the newly designed modular hinged ankle prosthesis. Meanwhile, it's worth promoting in the reconstruction of large segmental defect at the distal tibia.

Objective:To describe the design, manufacture and use of three-dimensional (3D)-printed endoprosthesis for reconstruction after metaphysis-involved intercalary tumor resection and to evaluate its outcome.Methods:Forty-three patients who received metaphysis-involved intercalary tumor resection followed by 3D-printed endoprosthetic reconstruction in Musculoskeletal Tumor Center, Peking University People's Hospital between January 2018 and December 2021 were retrospectively reviewed. There were 25 males and 18 males with an average age of 20.1±15.2 years (range, 4-58 years). The pathological diagnosis included 24 cases of osteosarcomas, 6 cases of Ewing sarcomas, 5 cases of chondrosarcomas, 2 cases of pleomorphic undifferentiated sarcomas, 3 cases of soft-tissue sarcomas (liposarcoma, synovial sarcoma, malignant peripheral nerve sheath tumor for each) and 3 others (adamantinoma, recurrent aneurysmal bone cyst and recurrent osteofibrous dysplasia for each). The tumors located at femur in 25 patients (58%), including 14 lesions involving distal femoral metaphysis and 11 lesions involving both proximal and distal metaphysis; the tumors located at tibia in 11 patients (26%), including 4 lesions involving distal tibial metaphysis, 5 lesions involving proximal tibial metaphysis and 2 lesions involving both proximal and distal tibial metaphysis; the tumors located at humerus in 7 patients (16%), including 1 lesion involving distal humeral metaphysis, 3 lesions involving proximal humeral metaphysis and 3 lesions involving both proximal and distal humeral metaphysis. The endoprosthesis was designed in a semi-modular fashion and consisted of three parts: a diaphysis-fixing component, a semi-modular lap joint component, and a custom-made 3D-printed metaphysis-fixing component which was designed as two types with 3D-printed porous bone-contacting surfaces according to the osteotomy plane (Type I on meta-diaphyseal region, Type II on meta-epiphyseal region). The functional outcome was assessed using Musculoskeletal Tumor Society (MSTS) 93 system.Results:All surgeries were accomplished sucessfully. The median resection length and the distance from osteotomy plane to adjacent joint was 16.0 (13.0, 22.0) cm and 4.5 (3.5, 6.0) cm, respectively. 59 metaphysis-fixing components were installed in 43 patients. Type I components were used in single and dual ends of endoprosthesis in 12 and 6 cases respectively. Type II components were used in single and dual ends in 15 and 5 cases respectively. Hybrid endoprosthesis with Type I and II components were used in 5 cases. The mean follow-up time was 26.0 (17, 37) months (range, 12-54 months). The mean MSTS 93 score was 29.0 (28.0, 30.0) points (range, 21-30 points). Implant failures were found in 5 patients, including 2 cases of aseptic loosening (loosening was observed in the cementing diaphysis-fixing stems while no evidence of loosening in metaphysis-fixing components) and 3 cases of local tumor progression. The 2-year implant survival rate was 90.3% (95% CI: 0.81, 0.99). Conclusion:Using 3D-printed intercalary endoprosthesis for reconstruction after intercalary resection of metaphysis-involved bone tumor shows satisfactory functional outcome and implant survival. Moreover, by assembling endoprosthetic components according to the different osteotomy plane, the semi-modularized endoprosthesis also provids a comprehensive and individualized reconstruction for patients with metaphysis-involved intercalary tumor.