RESUMO
BACKGROUND: Synchronous multicentric osteosarcoma (SMOS) is a rare disease characterized by simultaneous multicentricity of intraosseous osteosarcoma without visceral involvement. SMOS, including a skull lesion, which occurs relatively rarely, and reconstruction using a frozen autograft after the excision of a lesion of SMOS has been infrequently reported previously. CASE PRESENTATION: We report an 18-year-old girl with SMOS, with lesions located in the left distal femur, right proximal humerus, and left occipital bone. Her major complaint was pain and swelling around the left knee joint. Asymptomatic lesions of the humerus and skull bone were detected on a systemic bone scan. No visceral organ metastasis was observed. A biopsy of the distal femoral lesion revealed osteosarcoma. Based on the histological findings, multiple bone lesions, and absence of visceral lesion, the clinical diagnosis of SMOS was made. After five courses of neoadjuvant chemotherapy with a regimen of doxorubicin and cisplatin, reconstruction using a tumor prosthesis following wide excision of the left distal femur was performed, and total necrosis was histologically observed in the retracted specimen. Following three cycles of adjuvant chemotherapy, tumor excision and reconstruction with a frozen autograft treated with liquid nitrogen was conducted for both lesions of the humerus and skull, rather than tumor prosthesis or synthetics, in order to retain a normal shoulder function, and to obtain a good cosmetic and functional outcome after treatment of the skull lesion. Further adjuvant chemotherapy could not be administered after the completion of the surgical treatment for all lesions because the adverse events due to chemotherapy were observed. At over 5 years after the diagnosis, she remains clinically disease-free. CONCLUSIONS: An early correct diagnosis, the proper management of chemotherapy, and surgical treatment for all lesions are essential for achieving a good clinical outcome, even in SMOS including a skull lesion. By performing reconstruction using a frozen autograft for a proximal humeral lesion and a skull lesion after confirming the good histological efficacy of neoadjuvant chemotherapy for the primary lesion, the excellent function of the shoulder joint and a good cosmetic outcome at the site of the skull lesion was acquired without complications or recurrence.
Assuntos
Neoplasias Ósseas , Crioterapia , Úmero , Neoplasias Primárias Múltiplas , Osso Occipital , Osteossarcoma , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Autoenxertos , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Ósseas/cirurgia , Cisplatino/administração & dosagem , Protocolos Clínicos , Terapia Combinada , Crioterapia/métodos , Doxorrubicina/administração & dosagem , Feminino , Neoplasias Femorais/diagnóstico por imagem , Neoplasias Femorais/tratamento farmacológico , Neoplasias Femorais/cirurgia , Humanos , Úmero/diagnóstico por imagem , Úmero/cirurgia , Úmero/transplante , Iodo/uso terapêutico , Terapia Neoadjuvante , Neoplasias Primárias Múltiplas/diagnóstico por imagem , Neoplasias Primárias Múltiplas/tratamento farmacológico , Neoplasias Primárias Múltiplas/cirurgia , Nitrogênio/uso terapêutico , Osso Occipital/diagnóstico por imagem , Osso Occipital/cirurgia , Osso Occipital/transplante , Osteossarcoma/diagnóstico por imagem , Osteossarcoma/tratamento farmacológico , Osteossarcoma/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Solução Salina/uso terapêutico , Neoplasias Cranianas/diagnóstico por imagem , Neoplasias Cranianas/tratamento farmacológico , Neoplasias Cranianas/cirurgia , Transplante Autólogo/métodosRESUMO
Giant-cell tumor of bone (GCTB) is a locally aggressive intermediate bone tumor with a rarely metastasizing disposition. Standard surgical treatment consists of curettage, adjuvant treatment, and augmentation with allograft, autograft, or synthetics. Polymethylmethacrylate (PMMA) has been widely used for augmentation of the bone defect; however, the hyperthermic polymerization of PMMA may cause damage to articular cartilage, and the stiffness of the material may decrease the ability of the joint to absorb shock. These properties were reported to result in secondary osteoarthritis. Calcium phosphate cement has a low degree of thermal reaction and a strength that is similar to cortical bone. The aim of the present study was to investigate the incidence of secondary osteoarthritis around the knee joint following augmentation with calcium phosphate cement. METHODS: We retrospectively evaluated 19 patients with primary GCTB from 2003 to 2012. Curettage, high-speed burring, phenolization, and filling with calcium phosphate cement were performed in all patients. Radiographic evidence of osteoarthritis progression was evaluated with use of the Kellgren-Lawrence grade; the postoperative grade was compared with both the preoperative grade and the grade of the nonoperative contralateral knee at the time of the latest follow-up. The Musculoskeletal Tumor Society score and oncological outcomes at the time of the latest follow-up were evaluated. RESULTS: At a median follow-up period of 131 months, osteoarthritic progression was observed in 5 patients (26%), of which 2 were classified as Kellgren-Lawrence grade 3 and 1 was classified as Kellgren-Lawrence grade 4. The patient with grade-4 osteoarthritis underwent total knee arthroplasty, and 1 of the patients with grade-3 osteoarthritis underwent open-wedge high tibial osteotomy. The 10-year survival rate of joint cartilage with a Kellgren-Lawrence grade of <3 was 83%. The average Musculoskeletal Tumor Society score was 29 points. GCTB recurred in 2 patients, and 1 of these patients developed pulmonary metastasis. CONCLUSIONS: The incidence of secondary osteoarthritis was low, despite the long follow-up period. Prospective investigation comparing PMMA and calcium phosphate cement is warranted to determine the relative rate of secondary osteoarthritis and the outcomes associated with the 2 different types of augmentation. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.
RESUMO
PURPOSE: Radio-hyperthermo-chemo (RHC) therapy, which combines radiotherapy, hyperthermia, and chemotherapy, for malignant soft tissue tumors has been introduced with the aim of decreasing the possibility of local recurrence after surgery. To avoid unnecessary neoadjuvant therapy and to plan the appropriate surgical treatment, surveillance of RHC therapeutic efficacy during treatment is necessary. In this study, we determined the optimal response criteria to evaluate the efficacy of RHC by comparing preoperative images before and after RHC with pathological evaluation of necrosis in the resected tumor. PATIENTS AND METHODS: From 2004 to 2014, 20 patients were enrolled into this study. Needle biopsy revealed 6 cases of myxoid liposarcoma, 6 cases of undifferentiated pleomorphic sarcoma, 4 cases of myxofibrosarcoma, and 4 cases of synovial sarcoma. Based on the Response Evaluation Criteria in Solid Tumors (RECIST) 1.1 or modified RECIST, we calculated the responses to RHC therapy by comparing pre- and post-RHC therapy images. In addition, resected specimens underwent pathological analysis to evaluate response based on tumor necrosis. The correlation between assessment based on preoperative images and resected tumors were evaluated by the Spearman's rank-order correlation coefficient. RESULT: From the surgical specimens, pathological assessment of necrosis in resected tumor were assessed as less than 50% (2 cases), 50-90% (9 cases), 90-99% (6 cases), and total necrosis (3 cases). Use of the RECIST 1.1 underestimated good responders as stable disease (SD) or progressive disease (PD) in 5 out of 15 cases; on the other hand, use of the modified RECIST did not underestimate the pathological assessment of necrosis. The correlations between responses based on preoperative images and those based on histological assessments were 0.23 (RECIST 1.1) and 0.76 (modified RECIST). CONCLUSION: Because pathological responses can be underestimated using the RECIST 1.1, the modified RECIST, which take into consideration tumor viability, as assessed by contrast MRI, should also be considered when evaluating the efficacy of RHC.