• The Journal of the Korean bone and joint tumor society
• /
• v.20 no.1
• /
• pp.7-13
• /
• 2014

Purpose: As well as patient survival, the restoration of postoperative function such as ambulation is important in limb salvage operations for treatment of malignant bone tumors involving the proximal femur. The authors analyzed clinical outcomes of limb salvage operations using tumor prostheses for metastatic or primary malignant bone tumors in the proximal femur. Materials and Methods: From February 2005 to January 2014, 20 cases (19 patients) with malignant bone tumor involving the proximal femur with pain or complicated pathologic fracture were treated with segmental resection and limb salvage operations with tumor prostheses. Mean age was 63.1 years (range 35-86). Fourteen patients were male and six ones were female. The mean follow-up period was 20 months (1-94 months). There were 15 cases of metastatic bone tumor, 4 cases of osteosarcoma, and 1 case of multiple myeloma. The primary tumors of the metastatic bone tumors included 4 lung cancers, 3 hepatocellular carcinomas, and 3 renal cell carcinomas. Other primary tumors were breast cancer, thyroid cancer, colon cancer, prostate cancer, and malignant spindle cell tumor, each in 1 case. Modular tumor prostheses were used in all cases; (Kotz's$^{(R)}$ Modular Tumor prosthesis (Howmedica, Rutherford, New Jersey) in 3 cases, MUTARS$^{(R)}$ proximal femur system (Implantcast, Munster, Germany) in 17 cases). Perioperative pain was assessed with Visual Analogue Scales (VAS). Postoperative functional outcome was assessed with Musculoskeletal Tumor Society (MSTS) grading system. Results: Out of 20 cases (19 patients), 11 cases (10 patients) survived at the last follow-up. Average postoperative survival of the 9 deceased patients was 10.1 months (1-38 months). VAS score improved from pre-operative average of 8.40 (5-10) to 1.35 (0-3) after operation. Average postoperative MSTS function score was 19.65 (65.50%, 7-28). The associated complications were 2 local recurrences, 3 hematomas, 3 infections, 2 scrotal swellings, and 1 dislocation. There was no case of periprosthetic fracture or loosening. Conclusion: Limb salvage operation with tumor prosthesis is an appropriate treatment for early pain reduction and functional restoration in malignant bone tumors in the proximal femur with pain an/or complicated pathologic fractures.

• The Journal of the Korean bone and joint tumor society
• /
• v.6 no.2
• /
• pp.53-60
• /
• 2000

Purpose : The purpose of the current study is to evaluate the functional and oncologic results of the limb salvage surgery with intramedullary nailing and cementization in malignant bone tumors of the proximal humerus. Materials and Methods : We reviewed 18 cases of limb salvage surgery of resection and reconstruction with an intramedullary nail and cement-molded humeral head for the malignant bone tumors of the proximal humerus, which performed between August, 1992 through the April, 1998. The diagnoses included the osteosarcoma in 7 patients, chondrosarcoma in 3, the recurrent giant cell tumor in 3, metastatic tumor in 3, multiple myeloma in one patient and the one patient with malignant fibrous histiocytoma. The mean age at the time of surgery was 38 years(range, 15-73 years). The mean follow-up period was 26 months(range, 6-67 months). Results : Average functional score by ISOLS evaluation system was 21.1(70.3%). There were 3 local recurrences and 5 distant metastases. There were one case of shoulder instability and one case of deep infection. The seven patients are continuously disease free state and seven patients are alive with disease. Four patients died from the disease. Conclusion : The limb salvage surgery with intramedullary nailing and cementization in the bone tumors of the proximal humerus may be considered an option for the malignant bone tumors of the proximal humerus in selected patients.

• The Journal of the Korean bone and joint tumor society
• /
• v.13 no.1
• /
• pp.48-54
• /
• 2007

Purpose: To evaluate clinical results of the tumor prosthetic replacement for pathologic fracture of the proximal femur with extensive bony destruction due to metastatic bone tumors. Materials and Methods: From 2005 October to 2006 October, resection of proximal femur and tumor prosthesis replacement was performed for metastatic bone tumors extensively involving trochanteric area in 6 patients. The mean age of overall patients was 61(range, 48~77). Mean clinical follow up was 10.5 months (range, 6~16). Primary tumor consisted with multiple myeloma in 2 patients, lung cancer in 1, breast cancer in 1, transitional cell carcinoma of the renal pelvis in 1, unknown origin in 1 patient. $MUTARS^{(R)}$ proximal femur (Implantcast, Munster, Germany) were used in all cases. The functional evaluation in the lower extremities was performed with Musculoskeletal Tumor Society 1993 scores. The degree of pain relief after surgical treatment was assessed with Visual Analogue Scale (VAS). Results: At the final follow up, all of the patients survived. The mean lower extremities functional score of Musculoskeletal Tumor Society 1993 was 17.8(59.3%)(range, 12~25). VAS was improved to 2.5 from 8.5 after the surgical treatment. Ambulation was encouraged as soon as possible and mean duration after surgery for ambulation was 7.3(range, 3~16) days. Post-operatively, there was no surgical infection, periprosthetic fracture or loosening. Dislocation occurred in one out of six cases. Conclusion: Resection of proximal femur and tumor prosthesis replacement for metastatic bone tumors around proximal femur extensively involving trochanteric area seems to be safe procedure in view of post-operative complication, and effective for functional restoration as well as pain relief. Thus, it is accordant to the treatment principle of the metastatic bone tumors.

• Pediatric Infection and Vaccine
• /
• v.30 no.3
• /
• pp.129-138
• /
• 2023

Purpose: Cancer incidence is known to be higher in patients with inborn errors of immunity (IEI) compared to the general population in addition to traditionally well-known infection susceptibility. We aimed to investigate cancer occurrence in patients with IEI in a single center. Methods: Medical records of IEI patients treated at Samsung Medical Center, Seoul, Korea were retrospectively reviewed from November 1994 to September 2023. Patients with IEI and cancer were identified. Results: Among 194 patients with IEI, seven patients (3.6%) were diagnosed with cancer. Five cases were lymphomas, 4 of which were Epstein-Barr virus (EBV)-associated lymphomas. The remaining cases included gastric cancer and multiple myeloma. The median age at cancer diagnosis was 18 years (range, 1-75 years). Among patients with cancer, underlying IEIs included X-linked lymphoproliferative disease-1 (XLP-1, n=3), activated phosphoinositide 3-kinase delta syndrome (APDS, n=2), and cytotoxic T-lymphocyte antigen 4 (CTLA-4) haploinsufficiency (n=2). Seventy-five percent (3/4) of XLP-1 patients, 40.0% (2/5) of APDS patients, and 50.0% (2/4) of CTLA-4 haplo-insufficiency patients developed cancer. Patients with XLP-1 developed cancer at earlier age (median age 5 years) compared to those with APDS and CTLA-4 (P<0.001). One patient with APDS died during hematopoietic cell transplantation. Conclusions: Cancer occurred in 3.6% of IEI patients at a single center in Korea. In addition to infectious complications and inflammation, physicians caring for IEI patients should be aware of the potential risk of cancer, especially in association with EBV infection.