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BACKGROUND: Few studies have compared the clinical outcomes of patients with pelvic bone sarcomas treated surgically and those treated with particle beam therapy. This is a multicenter retrospective cohort study which compared the clinical outcomes of patients with pelvic bone sarcoma who underwent surgical treatment and particle beam therapy in Japan. METHODS: A total of 116 patients with pelvic bone sarcoma treated at 19 specialized sarcoma centers in Japan were included in this study. Fifty-seven patients underwent surgery (surgery group), and 59 patients underwent particle beam therapy (particle beam group; carbon-ion radiotherapy: 55 patients, proton: four patients). RESULTS: The median age at primary tumor diagnosis was 52 years in the surgery group and 66 years in the particle beam group (P < 0.001), and the median tumor size was 9 cm in the surgery group and 8 cm in the particle beam group (P = 0.091). Overall survival (OS), local control (LC), and metastasis-free survival (MFS) rates were evaluated using the Kaplan-Meier method and compared among 116 patients with bone sarcoma (surgery group, 57 patients; particle beam group, 59 patients). After propensity score matching, the 3-year OS, LC, and MFS rates were 82.9% (95% confidence interval [CI], 60.5-93.2%), 66.0% (95% CI, 43.3-81.3%), and 78.4% (95% CI, 55.5-90.5%), respectively, in the surgery group and 64.9% (95% CI, 41.7-80.8%), 86.4% (95% CI, 63.3-95.4%), and 62.6% (95% CI, 38.5-79.4%), respectively, in the particle beam group. In chordoma patients, only surgery was significantly correlated with worse LC in the univariate analysis. CONCLUSIONS: The groups had no significant differences in the OS, LC, and MFS rates. Among the patients with chordomas, the 3-year LC rate in the particle beam group was significantly higher than in the surgery group.
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Chondroid lipoma is a rare benign adipose tumor characterized by a recurrent ZFTA::MRTFB fusion. Herein, we report an unusual liposarcoma that partly exhibited overlapping features with those of chondroid lipoma and harbored a ZFTA::RELA fusion. A 59-year-old man presented with a shoulder mass that had existed for approximately 8 years and with increasing pain due to a pelvic mass. The 5.8-cm resected shoulder tumor partly consisted of nests and strands of variably lipogenic epithelioid cells within a hyalinized or focally chondromyxoid stroma, indistinguishable from chondroid lipoma. The histological pattern gradually transitioned to highly cellular, stroma-poor, diffuse sheets of cells with greater nuclear atypia and mitotic activity. Vascular invasion and necrosis were present. The metastatic pelvic tumor revealed a similar histology. Despite multimodal treatment, the patient developed multiple bone metastases and succumbed to the disease 14 months after presentation. Targeted RNA sequencing identified an in-frame ZFTA (exon 3)::RELA (exon 2) fusion, which was confirmed by reverse transcription-polymerase chain reaction, Sanger sequencing, and break-apart fluorescent in situ hybridization assays. The tumor showed a different histology from that of ependymoma, no brain involvement, and no match with any sarcoma types or ZFTA::RELA-positive ependymomas according to DNA methylation analysis. p65 and L1CAM were diffusely expressed, and a CDKN2A/B deletion was present. This is the first report of an extra-central nervous system tumor with a ZFTA::RELA fusion. The tumor partly displayed an overlapping histology with that of chondroid lipoma, suggesting that it may represent a hitherto undescribed malignant chondroid lipoma with an alternative ZFTA fusion.
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Neoplasias , Humanos , Pessoa de Meia-Idade , Hibridização in Situ Fluorescente , Fator de Transcrição RelARESUMO
BACKGROUND: Image-guided percutaneous core needle biopsy (CNB) has been an important diagnostic procedure for musculoskeletal lesions. Here we surveyed the variety of diagnostic strategies available and assessed the clinical usefulness and limitations of image-guided CNB carried out by a multidisciplinary team comprising specialists in various fields. METHODS: We conducted a retrospective study of 284 image-guided CNBs among 1899 consecutive biopsy procedures carried out at our institution for musculoskeletal tumorous conditions, focusing on their effectiveness including diagnostic accuracy and utility for classification of specimens according to malignant potential and histological subtype as well as their correlation with biopsy routes. RESULTS: Among the 284 studied biopsies, 252 (88.7%) were considered clinically "effective". The sensitivity for detection of malignancy was 94.0% (110/117) and the specificity was 95.3% (41/43). The diagnostic accuracy for detection of malignancy was 94.4% (151/160) and that for histological subtype was 92.3% (48/52). The clinical effectiveness of the procedure was correlated with the complexity of the biopsy route (P = 0.015); the trans-pedicular, trans-retroperitoneal and trans-sciatic foramen approaches tended to yield ineffective results. Repeat biopsy did not have a significant impact on the effectiveness of image-guided CNB (P = 0.536). CONCLUSIONS: The diagnostic accuracy rates of image-guided CNB performed at multidisciplinary sarcoma units were usable even for patients who have variety of diagnostic biopsy procedures. It is important to establish and implement diagnostic strategies based on an understanding that complicated routes, especially for spine and pelvic lesions, may be associated with ineffectiveness and/or complications.
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Sarcoma , Neoplasias de Tecidos Moles , Biópsia com Agulha de Grande Calibre/métodos , Humanos , Biópsia Guiada por Imagem/métodos , Estudos Retrospectivos , Sensibilidade e Especificidade , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/patologiaRESUMO
Previous studies reported the critical role of the brefeldin A-inhibited guanine nucleotide exchange protein 3-prohibitin 2 (BIG3-PHB2) complex in modulating estrogen signaling activation in breast cancer cells, yet its pathophysiological roles in osteosarcoma (OS) cells remain elusive. Here, we report a novel function of BIG3-PHB2 in OS malignancy. BIG3-PHB2 complexes were localized mainly in mitochondria in OS cells, unlike in estrogen-dependent breast cancer cells. Depletion of endogenous BIG3 expression by small interfering RNA (siRNA) treatment led to significant inhibition of OS cell growth. Disruption of BIG3-PHB2 complex formation by treatment with specific peptide inhibitor also resulted in significant dose-dependent suppression of OS cell growth, migration, and invasion resulting from G2/M-phase arrest and in PARP cleavage, ultimately leading to PARP-1/apoptosis-inducing factor (AIF) pathway activation-dependent apoptosis in OS cells. Subsequent proteomic and bioinformatic pathway analyses revealed that disruption of the BIG3-PHB2 complex might lead to downregulation of inner mitochondrial membrane protein complex activity. Our findings indicate that the mitochondrial BIG3-PHB2 complex might regulate PARP-1/AIF pathway-dependent apoptosis during OS cell proliferation and progression and that disruption of this complex may be a promising therapeutic strategy for OS.
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Neoplasias Ósseas/patologia , Proliferação de Células/fisiologia , Sobrevivência Celular/fisiologia , Fatores de Troca do Nucleotídeo Guanina/fisiologia , Mitocôndrias/metabolismo , Osteossarcoma/patologia , Proteínas Repressoras/fisiologia , Animais , Apoptose/fisiologia , Fator de Indução de Apoptose/metabolismo , Neoplasias Ósseas/metabolismo , Neoplasias Ósseas/terapia , Linhagem Celular Tumoral , Movimento Celular/efeitos dos fármacos , Proliferação de Células/efeitos dos fármacos , Peptídeos Penetradores de Células/farmacologia , Bases de Dados Factuais , Regulação para Baixo , Pontos de Checagem da Fase G2 do Ciclo Celular , Inativação Gênica , Fatores de Troca do Nucleotídeo Guanina/efeitos dos fármacos , Fatores de Troca do Nucleotídeo Guanina/metabolismo , Humanos , Pontos de Checagem da Fase M do Ciclo Celular , Proteínas de Membrana/metabolismo , Camundongos , Camundongos Nus , Membranas Mitocondriais/metabolismo , Invasividade Neoplásica , Transplante de Neoplasias , Osteossarcoma/metabolismo , Osteossarcoma/terapia , Poli(ADP-Ribose) Polimerase-1/metabolismo , Proibitinas , RNA Interferente Pequeno/metabolismo , Proteínas Repressoras/efeitos dos fármacos , Proteínas Repressoras/metabolismoRESUMO
BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of soft-tissue sarcoma, derived from a peripheral branch or the sheath of the sciatic nerve, brachial plexus, or sacral plexus. The clinical outcomes for MPNST patients with unresectable or metastatic tumors are dismal, and novel therapeutic strategies are required. Although patient-derived cancer cell lines are vital for basic research and preclinical studies, few MPNST cell lines are available from public cell banks. Therefore, the aim of this study was to establish cancer cell lines derived from MPNST patients. METHODS: We used tumor tissues from five patients with MPNSTs, including one derived from a rare bone tissue MPNST. The tumor tissues were obtained at the time of surgery and were immediately processed to establish cell lines. A patient-derived xenograft was also established when a sufficient amount of tumor tissue was available. The characterization of established cells was performed with respect to cell proliferation, spheroid formation, and invasion. The mutation status of actionable genes was monitored by NCC Oncopanel, by which the mutation of 114 genes was assessed by next-generation sequencing. The response to anti-cancer agents, including anti-cancer drugs approved for the treatment of other malignancies was investigated in the established cell lines. RESULTS: We established five cell lines (NCC-MPNST1-C1, NCC-MPNST2-C1, NCC-MPNST3-C1, NCC-MPNST4-C1, and NCC-MPNST5-C1) from the original tumors, and also established patient-derived xenografts (PDXs) from which one cell line (NCC-MPNST3-X2-C1) was produced. The established MPNST cell lines proliferated continuously and formed spheroids while exhibiting distinct invasion abilities. The cell lines had typical mutations in the actionable genes, and the mutation profiles differed among the cell lines. The responsiveness to examined anti-cancer agents differed among cell lines; while the presence of an actionable gene mutation did not correspond with the response to the anticipated anti-cancer agents. CONCLUSION: The established cell lines exhibit various characteristics, including proliferation and invasion potential. In addition, they had different mutation profiles and response to the anti-cancer agents. These observations suggest that the established cell lines will be useful for future research on MPNSTs.
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AIMS: Ewing sarcoma is a small round cell tumour that affects bone and soft tissues. Although the detection of the specific fusion gene is a robust method of its diagnosis, immunohistochemistry may serve as a practical surrogate. Recent tissue microarray studies suggested that PAX7 is a novel marker, because it was expressed consistently in Ewing sarcoma, in addition to rhabdomyosarcoma and synovial sarcoma. Here, we evaluated the utility of PAX7 immunohistochemistry in whole-tissue sections of an expanded array of round cell malignancies with adequate molecular characterisation. METHODS AND RESULTS: We stained 30 molecularly confirmed Ewing sarcomas, one EWSR1-NFATC2 sarcoma and 141 non-Ewing round cell tumours by a monoclonal antibody against PAX7. Staining was considered positive if at least 5% of tumour cells were stained. PAX7 was expressed in 27 of 30 Ewing sarcomas (90%), mainly in a diffuse and strong manner. Although NKX2-2 showed similar sensitivity, PAX7 showed more extensive and strong reactivity. One EWSR1-NFATC2 sarcoma co-expressed PAX7 and NKX2-2. PAX7 was also expressed in 24 of 141 non-Ewing tumours, including alveolar rhabdomyosarcomas (seven of 10), poorly differentiated synovial sarcomas (seven of 10), BCOR-CCNB3 sarcomas (eight of 10), small-cell osteosarcoma (one of five) and desmoplastic small round cell tumour (one of 10), one-third of which showed diffuse strong reactivity. CONCLUSIONS: Although we confirmed that PAX7 is a sensitive marker for Ewing sarcoma, anti-PAX7 antibody also stained several Ewing sarcoma mimics, whose spectrum was distinct from NKX2-2-positive non-Ewing entities. Further studies are required to determine how PAX7 could be integrated into practice to classify small round cell tumours efficiently.
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Biomarcadores Tumorais/análise , Fator de Transcrição PAX7/análise , Fator de Transcrição PAX7/biossíntese , Sarcoma de Ewing/diagnóstico , Diagnóstico Diferencial , Proteína Homeobox Nkx-2.2 , Proteínas de Homeodomínio , Humanos , Imuno-Histoquímica , Proteínas Nucleares , Neoplasias de Tecidos Moles/diagnóstico , Fatores de TranscriçãoRESUMO
We encountered a case of an accessory bone in the foot in the distal portion of the tarsal navicular and the proximal portion of the intermediate cuneiform, namely an os intercuneiform. The patient presented with a history of pain on the dorsal aspect of the left foot, with tenderness and swelling. Perioperative findings revealed a synfibrotic connection between the accessory bone and the navicular and intermediate cuneiform. After unsuccessful conservative treatment, the accessory bone was excised, leading to postoperative symptomatic relief.
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Deformidades Congênitas do Pé/diagnóstico , Deformidades Congênitas do Pé/cirurgia , Ossos do Tarso/anormalidades , Ossos do Tarso/cirurgia , Feminino , Deformidades Congênitas do Pé/complicações , Humanos , Radiografia , Ossos do Tarso/diagnóstico por imagem , Adulto JovemRESUMO
BACKGROUND: Staphylococcus aureus (S. aureus), including MRSA, is considered to be the leading cause of surgical site infection (SSI) after orthopedic surgery. We screened for nasal carriers of S. aureus among patients who were scheduled to undergo orthopedic surgery at our hospital to reveal the effect of nasal S. aureus carriage on SSI. Our study design clearly has the intent of finding S. aureus nasal carriage and eradicating MRSA when found, and this strategy is to verify whether it's effective for preventing orthopedic surgical infections. METHODS: Subjects were 4148 patients who underwent preoperative screening for nasal carrier and subsequently underwent orthopedic surgery during a 7-year period between April 2007 and March 2014. The incidence of SSI among patients who were operated in our department was investigated, and the rates were compared between patients with and without nasal carriage to reveal the effect of preoperative nasal carriage on SSI. RESULTS: In total, 1036 patients were nasal carriers of S. aureus (carriage rate, 25.0%), whereas 140 patients carried MRSA (carriage rate, 3.4%). SSI developed in 24 patients [incidence, 0.58% (24/4148)] consisting of 12 non-carriers [0.39% (12/3112)] and 12 carriers [1.16% (12/1036)] with a significant difference in the incidence between the groups. Among 24 cases of SSI, more than half (13 cases) were caused by bacterial species other than S. aureus or those that could not be detected by the tests used. Only 7 patients out of 24 SSI patients, S. aureus was the bacterium detected in preoperative nasal cultures and the causal bacterium for SSI (concordance rate of 29.2%). CONCLUSIONS: It was difficult to reduce the incidence rate of SSI in eradication group to the same level as nasal culture negative group. However, nasal carriage of S. aureus or MRSA may be a risk factor for SSI in orthopedic surgery.
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Portador Sadio/microbiologia , Cavidade Nasal/microbiologia , Procedimentos Ortopédicos/efeitos adversos , Infecções Estafilocócicas/epidemiologia , Staphylococcus aureus/isolamento & purificação , Infecção da Ferida Cirúrgica/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Infecções Estafilocócicas/diagnóstico , Infecção da Ferida Cirúrgica/microbiologiaRESUMO
AIMS: The common treatment for an undisplaced femoral neck fracture is osteosynthesis. Two major complications of osteosynthesis are non-union and late collapse of the femoral head. We speculated that femoral head perfusion is one of the most important factors that affect the outcome of osteosynthesis after femoral neck fracture. We have preoperatively estimated femoral head perfusion by dynamic MRI positive enhancement integral color mapping (PEICM). The purpose of this study was to evaluate the outcomes of undisplaced femoral neck fractures based on PEICM. PATIENTS AND METHODS: Sixty-eight patients participated in this prospective study. All patients underwent PEICM in a 1.5-Tesla MRI machine using coronal fast spoiled gradient echo imaging sequences with gadopentetate dimeglumine as the contrast agent. Femoral head perfusion was displayed via color mapping using PEICM. Three types were distinguished. For type A, the color was identical to unaffected side indicated normal perfusion. For type B, the color was darker than unaffected side indicated decreased perfusion. For type C, the color was black indicated complete absence of perfusion. All patients underwent osteosynthesis with three cannulated screws. The rates of non-union and late collapse for each type were calculated. RESULTS: Sixteen patients were classified as Type A, 43 as Type B, and 6 as Type C. The non-union rates were 0% for Type A, 6.7% for Type B, and 50.0% for Type C. The late collapse rates were 0% for Type A, 4.4% for Type B, and 0% for Type C. CONCLUSION: PEICM precisely detected femoral head perfusion. Primary prosthetic replacement should be considered for older patients with Type C to minimize the chances of revision surgery, even in undisplaced femoral neck fractures.
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Fraturas do Colo Femoral/diagnóstico por imagem , Fraturas do Colo Femoral/cirurgia , Fixação Interna de Fraturas , Imageamento por Ressonância Magnética , Idoso , Idoso de 80 Anos ou mais , Meios de Contraste , Feminino , Cabeça do Fêmur/irrigação sanguínea , Cabeça do Fêmur/diagnóstico por imagem , Gadolínio DTPA , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Falha de TratamentoRESUMO
BACKGROUND: Adverse tissue reaction to metal debris (ARMD) as a secondary complication of Metal-on-metal total hip replacement (MoM THR) has been of concern. We have been performing cementless MoM THR using an Asian-type anatomic medullary locking (AML) stem. The purpose of this study is to examine the incident rate of ARMD, and the implant survival rate. METHODS: The study included 187 patients (211 hip joints) who underwent MoM THR between February 2007 and November 2009 at our hospital and who were followed up for a minimum of 6 years. The cases included 174 female joints and 37 male joints. The average age at the time of surgery was 68.4 years. The average postoperative follow-up period was 87.5 months. RESULTS: ARMD was observed in 23 joints postoperatively, and the incidence rate was 10.9%. Most of the instances occurred within the first 30 months postoperatively. Revision surgery was performed for 14 joints after conservative treatment failed, and we changed the bearing surface. During the intraoperative observation, black-colored deposition of metal debris on the head-neck junction was observed in 13 cases. Kaplan-Meier analysis using the replacement surgery as the end point showed that this implant has a survival rate of 93.8% 7 years after the primary surgery. CONCLUSIONS: We conclude that the major cause of failure of Asian-type AML stemmed MoM THR is likely the breakage of the fixation between the taper neck and metal head at the head-neck junction.
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Artroplastia de Quadril/efeitos adversos , Prótese de Quadril , Próteses Articulares Metal-Metal/efeitos adversos , Desenho de Prótese/métodos , Falha de Prótese , Adulto , Idoso , Idoso de 80 Anos ou mais , Artroplastia de Quadril/métodos , Povo Asiático , Estudos de Coortes , Intervalos de Confiança , Análise de Falha de Equipamento , Feminino , Seguimentos , Humanos , Incidência , Japão , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Medição de Risco , Resultado do TratamentoRESUMO
INTRODUCTION AND IMPORTANCE: Denosumab is a new standard treatment for bone disease caused by multiple myeloma. There are a few reports of atypical femoral fracture in patients with multiple myeloma, all of which were associated with long-term use of bisphosphonate. Here, we report the first case of denosumab-induced atypical femoral fracture in a patient with multiple myeloma. CASE PRESENTATION: A 71-year-old woman with multiple myeloma developed dull pain in her right thigh 8 months after restarting high-dose denosumab following its initial administration for 4 months and subsequent withdrawal for 2 years. Fourteen months later, complete atypical femoral fracture occurred. Osteosynthesis was achieved using an intramedullary nail and she was switched to oral bisphosphonate 7 months after cessation of denosumab. There was no exacerbation of the multiple myeloma. Bone union was achieved and she recovered to her pre-injury level of activities. The oncological outcome was alive with disease at 2 years after surgery. CLINICAL DISCUSSION: Prodromal symptoms such as thigh pain and radiographical finding of thickening of the lateral cortex in the subtrochanteric region of the femur were attributed to denosumab-induced atypical femoral fracture in the case. A unique aspect of this case worth highlighting is that the fracture occurred after short-term denosumab use. This may be associated with multiple myeloma or other medication including dexamethasone and cyclophosphamide. CONCLUSION: Atypical femoral fracture may occur in patients with multiple myeloma who receive denosumab, even for a short period. Attending physicians should be cognizant of the early symptoms and signs of this fracture.
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BACKGROUND: Synovial sarcoma (SS) has limited treatment options and there is an urgent need to develop a novel therapeutic strategy to treat SS. Blue light (BL) has been shown to inhibit the growth of several cancer cells. However, the efficacy of BL in soft tissue sarcomas such as SS has not been demonstrated, and the detailed mechanism underlying the antitumor activity of BL is not fully understood. In this study, we investigated the antitumor effect of BL on SS. METHODS: Human SS cell lines were continuously irradiated with BL using light-emitting diodes (LEDs) in an incubator for in vitro analysis. The chicken chorioallantoic membrane (CAM) tumors and xenograft tumors in mice were subjected to daily BL irradiation with LEDs. RESULTS: BL caused growth inhibition of SS cells and histological changes in CAM tumors. BL also suppressed the migration and invasion abilities of SS cells. The type of cell death in SS cells was revealed to be apoptosis. Furthermore, BL induced excessive production of reactive oxygen species (ROS) in mitochondria, resulting in oxidative stress and malfunctioned mitochondria. Reducing the production of ROS using N-acetylcysteine (NAC), a ROS scavenger, attenuated the inhibitory effect of BL on SS cells and mitochondrial dysfunction. In addition, BL induced autophagy, which was suppressed by the administration of NAC. The autophagy inhibitor of 3-methyladenine and small interfering RNA against the autophagy marker light chain 3B facilitated apoptotic cell death. Moreover, BL suppressed tumor growth in a mouse xenograft model. CONCLUSION: Taken together, our results revealed that BL induced apoptosis via the ROS-mitochondrial signaling pathway, and autophagy was activated in response to the production of ROS, which protected SS cells from apoptosis. Therefore, BL is a promising candidate for the development of an antitumor therapeutic strategy targeting SS.
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Sarcoma Sinovial , Humanos , Camundongos , Animais , Espécies Reativas de Oxigênio/metabolismo , Sarcoma Sinovial/terapia , Sarcoma Sinovial/patologia , Apoptose , Autofagia , Mitocôndrias , Linhagem Celular TumoralRESUMO
BACKGROUND: Dedifferentiated liposarcoma occurs predominantly in the retroperitoneum. Given the paucity of cases, information on the clinical characteristics of this entity in the extremities and trunk wall is quite limited. In particular, the significance of preoperative evaluation and principles of intraoperative management of the different components, i.e., well-differentiated and dedifferentiated areas, are still to be defined. METHODS: Clinical characteristics, treatment outcomes, and risk factors for poor oncological outcomes in cases of dedifferentiated liposarcoma in the extremity or trunk wall were analyzed by a retrospective, multicentric study. RESULTS: A total of 132 patients were included. The mean duration from the initial presentation to dedifferentiation was 101 months in dedifferentiation-type cases. The 5-year local recurrence-free survival, metastasis-free survival, and disease-specific survival rates were 71.6%, 75.7%, and 84.7%, respectively. Among 32 patients with metastasis, 15 presented with extrapulmonary metastasis. A percentage of dedifferentiated area over 87.5%, marginal/intralesional margin, and R1/2 resection in the dedifferentiated area were independent risk factors for local recurrence. Dedifferentiated areas over 36 cm2, French Federation of Cancer Centers Sarcoma Group grade III, and intralesional or marginal resection were independent risk factors for metastasis. A dedifferentiated area over 77 cm2 and lung metastasis were independent risk factors for disease-specific mortality. CONCLUSIONS: The typical clinical characteristics of dedifferentiated liposarcoma in the extremity and trunk wall were reconfirmed in the largest cohort ever. The evaluation of the dedifferentiated area in terms of grade, extension, and pathological margin, together with securing adequate surgical margins, was critical in the management of this entity.
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População do Leste Asiático , Lipossarcoma , Humanos , Estudos Retrospectivos , Lipossarcoma/patologia , Extremidades/patologia , Resultado do TratamentoRESUMO
Soft tissue myoepithelial carcinoma (MEC) is an extremely rare mesenchymal tumor that has a poor prognosis unless complete surgical resection is achieved. The present study reported a case of a 38-year-old woman with a tumor in the left paraspinal region at L2 to L3 with vertebral destruction. MEC was diagnosed based on molecular pathological examination of a biopsy specimen. Because curative surgery was expected to be difficult, a combination of chemotherapy with doxorubicin and ifosfamide and proton beam therapy as local therapy was performed, resulting in long-term survival for at least 7.8 years. To the best of our knowledge, this is the first case of soft tissue MEC for which classical cytotoxic chemotherapy and proton beam therapy were effective. Although surgical resection with negative margins is the mainstay of treatment for MEC, adequate doxorubicin-based systemic therapy and high-dose radiation therapy may be a feasible alternative in patients with unresectable or advanced MEC. Future studies on the relationship between molecular pathological features, including biomarkers, and the selection of therapeutic agents are warranted.
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Introductionâ :â Hemangioblastoma in the spine mainly occurs at the cervical and thoracic levels and is often associated with von Hippel-Lindau (VHL) syndrome. Here, we reported a quite rare case of spinal sporadic hemangioblastoma arising from the cauda equina. Case presentationâ :â A 66-year-old woman presented with a 5-year history of low back and leg pain. Imaging revealed a hypervascular intradural extramedullary tumor in the lumbar region. Preoperative angiography helped to identify the feeding arteries and draining vein, and so facilitated subsequent tumor resection. The pain was dramatically improved but weakness of the left tibialis anterior and left extensor hallucis longus muscles persisted. Discussionâ :â We reported a rare case of spinal hemangioblastoma arising from the cauda equina. Preoperative angiography may be useful for diagnosis and understanding of the anatomy of feeding veins. J. Med. Invest. 69 : 312-315, August, 2022.
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Cauda Equina , Hemangioblastoma , Neoplasias da Medula Espinal , Doença de von Hippel-Lindau , Idoso , Cauda Equina/diagnóstico por imagem , Cauda Equina/patologia , Cauda Equina/cirurgia , Feminino , Hemangioblastoma/diagnóstico por imagem , Hemangioblastoma/cirurgia , Humanos , Imageamento por Ressonância Magnética , Dor , Neoplasias da Medula Espinal/complicações , Neoplasias da Medula Espinal/diagnóstico , Neoplasias da Medula Espinal/cirurgia , Doença de von Hippel-Lindau/complicações , Doença de von Hippel-Lindau/diagnóstico , Doença de von Hippel-Lindau/patologiaRESUMO
This study aimed to retrospectively analyze the clinical outcomes of patients with pelvic and retroperitoneal bone and soft tissue sarcoma (BSTS). Overall, 187 patients with BSTS in the pelvis and retroperitoneal region treated at 19 specialized sarcoma centers in Japan were included. The prognostic factors related to overall survival (OS), local control (LC), and progression-free survival (PFS) were evaluated. The 3-year OS and LC rates in the 187 patients were 71.7% and 79.1%, respectively. The 3-year PFS in 166 patients without any distant metastases at the time of primary tumor diagnosis was 48.6%. Osteosarcoma showed significantly worse OS and PFS than other sarcomas of the pelvis and retroperitoneum. In the univariate analyses, larger primary tumor size, soft tissue tumor, distant metastasis at the time of primary tumor diagnosis, P2 location, chemotherapy, and osteosarcoma were poor prognostic factors correlated with OS. Larger primary tumor size, higher age, soft tissue tumor, chemotherapy, and osteosarcoma were poor prognostic factors correlated with PFS in patients without any metastasis at the initial presentation. Larger primary tumor size was the only poor prognostic factor correlation with LC. This study has clarified the epidemiology and prognosis of patients with pelvic and retroperitoneal BSTS in Japan.
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INTRODUCTION: The specific morphology and differences between patients with cervical spondylotic myelopathy (CSM) and those with normal spines remain unclear. This study aimed to evaluate and determine the features of cervical spine morphology on reconstructive CT. METHODS: We investigated that axial reconstructive CT scans of the cervical spine at C3 to C7 were obtained from 309 individuals (97 CSM patients and 212 controls). Those of the optimal pedicle diameter were selected, and the following parameters were measured: (a) sagittal diameter of the spinal canal (b) transverse diameter of the spinal canal, (c) pedicle width, (d) lateral mass thickness, (e) transverse diameter of the foramen, (f) sagittal diameter of the vertebral body, and (g) transverse diameter of the vertebral body. The following ratios were calculated using these values: the sagittal-transverse ratio and the canal-body ratio. RESULTS: Most parameters differed significantly between the sexes in both groups. The parameters without the mean sagittal diameter of the spinal canal were significantly larger in men than in women. However, the mean sagittal diameter of the spinal canal did not differ significantly between the sexes in CSM patients. The sagittal-transverse ratio and canal-body ratio were significantly smaller in the CSM patients at all levels. According to relative operating characteristic curves of the sagittal diameter of the spinal canal, sagittal-transverse ratio, and canal-body ratio, the sensitivity from C3 to C7 in both sexes was > 60% at the threshold. In men, the specificity from C3 to C7 was also >60% at the threshold. CONCLUSIONS: The morphometry of the sagittal diameter of the spinal canal, sagittal-transverse ratio, and canal-body ratio on axial reconstructive CT images appears useful for distinguishing cervical spinal canal stenosis involving myelopathy.
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INTRODUCTION: Intertrochanteric fractures are common fragility fractures in elderly patients. The importance of a reliable classification system for these fractures has been increasingly recognized. The aim of this study was to test the hypothesis that three-dimensional classification by CT has better intra- and inter-observer reliability than conventional two-dimensional classification. MATERIAL AND METHODS: Two hundred and three consecutive patients (39 male, 164 female; mean age 84.5 years) with intertrochanteric fracture were included in the study. In each case, the fracture was classified using the two-dimensional Evans-Jensen and AO/OTA systems that rely on plain radiographs and using a three-dimensional fragment-based CT system. The second evaluation was performed 1 month after the first evaluation by the same examiner to determine intra-observer reliability. Another blinded examiner also classified each case to assess inter-observer reliability. The kappa coefficient was used for determination of intra- and inter-observer reliability. RESULTS: The kappa values for the two-dimensional Evans-Jensen and AO/OTA classification systems showed moderate intra-observer reliability (κ=0.65 and κ=0.61, respectively) and slight inter-observer reliability (κ=0.20 and κ=0.19). The intra-observer kappa value for the three-dimensional classification system was 0.88, indicating almost perfect reliability; the inter-observer kappa value was 0.70, indicating substantial reliability. CONCLUSION: The findings of this study confirm that the fragment-based classification system has high reliability. Surgeons should be aware that the three-dimensional fragment-based CT system for classification of intertrochanteric fractures has better intra-observer and inter-observer reliability than the conventional two-dimensional systems.
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Fraturas do Quadril , Tomografia Computadorizada por Raios X , Idoso , Idoso de 80 Anos ou mais , Feminino , Fraturas do Quadril/diagnóstico por imagem , Fraturas do Quadril/cirurgia , Humanos , Masculino , Variações Dependentes do Observador , Radiografia , Reprodutibilidade dos TestesRESUMO
Osteochondroma is a common benign bone tumor that is relatively rare in the spine. Here, we report two cases of symptomatic solitary osteochondroma of the lumbar spine. The first case was a 61-year-old man who presented with a 2-year history of right leg numbness. Imaging findings showed that the cause of the radiculopathy was osteochondroma of the right inferior articular process at L4. The tumor was removed en bloc, and the numbness resolved. The second case was a 62-year-old man with osteochondroma of the right superior articular process at L5 that caused pain and numbness in the right leg. En bloc resection of the osteochondroma with the ligamentum flavum relieved the symptoms. Spinal osteochondroma occurs relatively frequently in elderly individuals compared with peripheral lesions and mimics a degenerative spinal disorder. Careful physical examination and imaging evaluation can reveal this tumor and surgery is effective for relieving the symptoms.
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Alveolar rhabdomyosarcoma (aRMS) is a histological subtype of RMS, which is the most common pediatric and adolescent soft-tissue sarcoma, accounting for 3-4% of all pediatric malignancies. Patient-derived cells are essential tools for understanding the molecular mechanisms of poor prognosis and developing novel anti-cancer drugs. However, only a limited number of well-characterized cell lines for rhabdomyosarcoma from public cell banks is available. Therefore, we aimed to establish a novel cell line of aRMS from the tumor tissue of a patient with aRMS. The cell line was established from surgically resected tumor tissue from a 4-year-old male patient diagnosed with stage III, T2bN1M0 aRMS and was named as NCC-aRMS1-C1. The cells were maintained for more than 3 months under tissue culture conditions and passaged more than 20 times. We confirmed the presence of identical fusion gene such as PAX7-FOXO1 in both the original tumor and NCC-aRMS1-C1. The cells exhibited spheroid formation and invasion. We found that docetaxel, vincristine, ifosfamide, dacarbazine, and romidepsin showed remarkable growth-suppressive effects on the NCC-aRMS1-C1 cells. In conclusion, the NCC-aRMS1-C1 cell line exhibited characteristics that may correspond to the lymph node metastasis in aRMS and mirror its less aggressive features. Thus, it may be useful for innovative seeds for novel therapeutic strategies.