RESUMO
The association between the immune status within the tumor microenvironment and prognosis in synovial sarcoma is not well understood. We aimed to investigate the tumor immune microenvironment and analyze its prognostic impact for patients with synovial sarcoma. A total of 36 primary patients who were treated in our institution were retrospectively evaluated. Infiltration of lymphocytes (CD4+, CD8+, and FOXP3+), CD163+ macrophages, and expression of human leukocyte antigen (HLA) class I and programmed death ligand 1 (PD-L1) were evaluated by immunohistochemistry. Moreover, we investigated PD-L1 and programmed death ligand 2 (PD-L2) mRNA expression in 19 of the 36 cases, using real-time PCR. The Kaplan-Meier method was used to estimate overall survival and progression-free survival. Infiltration of lymphocytes and macrophages varied among the patients. Furthermore, the expression of HLA class I was negative or downregulated in 11 specimens. No PD-L1 expression was observed using immunohistochemistry. Moreover, although PD-L1 mRNA expression was observed in 18 of 19 specimens, the expression level was low. A higher infiltration of CD8+ or FOXP3+ lymphocytes in patients was associated with a favorable overall survival. In addition, a higher infiltration of CD163+ macrophages indicated a significantly worse overall and progression-free survival. Infiltration of CD4+ lymphocytes, HLA class I, PD-L1, and PD-L2 expression were not associated with patient prognosis. This represents the first report investigating the tumor immune microenvironment as a prognostic factor in synovial sarcoma, indicating that CD163+ macrophages are associated with tumor progression. Our results underscore the clinical significance of the tumor immune microenvironment in synovial sarcoma.
Assuntos
Biomarcadores Tumorais/imunologia , Linfócitos do Interstício Tumoral/imunologia , Macrófagos/imunologia , Sarcoma Sinovial/imunologia , Microambiente Tumoral/imunologia , Adolescente , Adulto , Idoso , Antígeno B7-H1/imunologia , Antígeno B7-H1/metabolismo , Biópsia , Linhagem Celular Tumoral , Criança , Intervalo Livre de Doença , Feminino , Antígenos de Histocompatibilidade Classe I/imunologia , Antígenos de Histocompatibilidade Classe I/metabolismo , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prognóstico , Estudos Retrospectivos , Sarcoma Sinovial/mortalidade , Sarcoma Sinovial/patologia , Sarcoma Sinovial/cirurgia , Adulto JovemRESUMO
BACKGROUND: A solitary fibrous tumour (SFT) is an unusual neoplasm typically found in soft tissues. Although SFTs can arise in the bones, they very rarely arise in the vertebral arch. Here, we describe a case of a SFT that arose in the vertebral arch of the first lumbar (L1) spinal vertebrae and mimicked osteosarcoma. CASE PRESENTATION: A 49-year-old woman presented with a 2-month history of lower back pain and a lumbar region mass. Magnetic resonance imaging demonstrated a heterogeneously enhanced mass in the L1 vertebral arch. The patient received neoadjuvant chemotherapy, followed by a surgical procedure comprising an anterior spinal fusion and en bloc resection. Histologically, our initial diagnosis was osteosarcoma. The postoperative course was uneventful, and the patient received adjuvant chemotherapy. However, the tumour metastasised to the lung 5 years after the first surgery, and a second surgery was performed for lung tumour resection. The histology of the metastatic lung tumour appeared similar to that of the malignant SFT, and the specimen from the first surgery was re-examined. Immunohistochemically, the tumour was positive for STAT6. Reverse transcription-polymerase chain reaction revealed a NAB2-STAT6 fusion gene, thus confirming our final diagnosis of malignant SFT. The patient died of disease progression 8 years after the first surgery; however, there was no evidence of local recurrence. CONCLUSIONS: Malignant SFT in the vertebral arch is extremely rare and very difficult to distinguish histologically an osteoid from lace-like collagen. STAT6 immunostaining is useful for distinguishing malignant SFTs from other neoplasms. Although it is difficult to completely resect a SFT arising from the spine, we demonstrated the feasibility of an en bloc resection of spinal tumours arising from posterior elements, without local recurrence.
Assuntos
Neoplasias Ósseas/diagnóstico , Vértebras Lombares/patologia , Osteossarcoma/diagnóstico , Tumores Fibrosos Solitários/diagnóstico , Neoplasias Ósseas/cirurgia , Diagnóstico Diferencial , Feminino , Humanos , Vértebras Lombares/cirurgia , Pessoa de Meia-Idade , Osteossarcoma/cirurgia , Tumores Fibrosos Solitários/cirurgiaRESUMO
BACKGROUND: Diagnosing adipocytic tumors can be challenging because it is often difficult to morphologically distinguish between benign, intermediate and malignant adipocytic tumors, and other sarcomas that are histologically similar. Recently, a number of tumor-specific chromosome translocations and associated fusion genes have been identified in adipocytic tumors and atypical lipomatous tumors/well-differentiated liposarcomas (ALT/WDL), which have a supernumerary ring and/or giant chromosome marker with amplified sequences of the MDM2 and CDK4 genes. The purpose of this study was to investigate whether quantitative real-time polymerase chain reaction (PCR) could be used to amplify MDM2 and CDK4 from total RNA samples obtained from core-needle biopsy sections for the diagnosis of ALT/WDL. METHODS: A series of lipoma (n = 124) and ALT/WDL (n = 44) cases were analyzed for cytogenetic analysis and lipoma fusion genes, as well as for MDM2 and CDK4 expression by real-time PCR. Moreover, the expression of MDM2 and CDK4 in whole tissue sections was compared with that in core-needle biopsy sections of the same tumor in order to determine whether real-time PCR could be used to distinguish ALT/WDL from lipoma at the preoperative stage. RESULTS: In whole tissue sections, the medians for MDM2 and CDK4 expression in ALT/WDL were higher than those in the lipomas (P < 0.05). Moreover, karyotype subdivisions with rings and/or giant chromosomes had higher MDM2 and CDK4 expression levels compared to karyotypes with 12q13-15 rearrangements, other abnormal karyotypes, and normal karyotypes (P < 0.05). On the other hand, MDM2 and CDK4 expression levels in core-needle biopsy sections were similar to those in whole-tissue sections (MDM2: P = 0.6, CDK4: P = 0.8, Wilcoxon signed-rank test). CONCLUSION: Quantitative real-time PCR of total RNA can be used to evaluate the MDM2 and CDK4 expression levels in core-needle biopsies and may be useful for distinguishing ALT/WDL from adipocytic tumors. Thus, total RNA from core-needle biopsy sections may have potential as a routine diagnostic tool for other tumors where gene overexpression is a feature of the tumor.
Assuntos
Quinase 4 Dependente de Ciclina/genética , Neoplasias Lipomatosas/genética , Neoplasias Lipomatosas/patologia , Proteínas Proto-Oncogênicas c-mdm2/genética , Reação em Cadeia da Polimerase em Tempo Real , Adulto , Idoso , Biomarcadores Tumorais/genética , Biópsia com Agulha de Grande Calibre , Quinase 4 Dependente de Ciclina/metabolismo , Análise Citogenética , Feminino , Proteína HMGA2/genética , Humanos , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Neoplasias Lipomatosas/diagnóstico , Proteínas de Fusão Oncogênica/genética , Proteínas Proto-Oncogênicas c-mdm2/metabolismo , Adulto JovemRESUMO
This study investigated the pathway underlying the antitumor activity of telomelysin, a telomerase-dependent, replication-selective oncolytic adenovirus, in soft tissue sarcoma cells. Treatment with telomelysin alone resulted in simultaneous induction of apoptosis and autophagy, whereas cotreatment with telomelysin and 3-methyladenine significantly reduced cell viability and increased apoptosis and the cellular ATP level compared to treatment with telomelysin alone, indicating that telomelysin-mediated autophagy is a death-protective but not death-promoting process. Cotreatment with Z-Val-Ala-Asp-CH2F significantly increased cellular ATP depletion compared to telomelysin-alone treatment while inhibiting telomelysin-induced apoptosis and having no significant effect on cell viability, indicating that it promotes transition from apoptotic to necrotic cell death.
Assuntos
Adenoviridae/metabolismo , Antineoplásicos/metabolismo , Apoptose/efeitos dos fármacos , Morte Celular/efeitos dos fármacos , Terapia Viral Oncolítica/métodos , Vírus Oncolíticos/fisiologia , Sarcoma/terapia , Sarcoma/virologia , Adenina/análogos & derivados , Adenina/farmacologia , Trifosfato de Adenosina/metabolismo , Infecções por Adenoviridae/metabolismo , Autofagia/efeitos dos fármacos , Linhagem Celular Tumoral , Sobrevivência Celular/efeitos dos fármacos , Células HEK293 , Células HeLa , Humanos , Sarcoma/tratamento farmacológico , Sarcoma/metabolismo , Telomerase/metabolismoRESUMO
Secondary rhabdomyosarcoma (RMS) after treatment of osteosarcoma (OS) is rare. Reported here is the case of a metachronous RMS in the nasal cavity, developing 12 years after successful treatment of non-metastatic OS. The patient was diagnosed as having OS of the femur at 2 years of age. Chemotherapy for OS included doxorubicin (cumulative dose, 488 mg/m(2) ). No radiotherapy was given. There was no family history suggestive of cancer predisposition syndrome. At 14 years of age, alveolar RMS was diagnosed on histopathology. PAX3-FKHR fusion transcripts were detected on reverse transcription-polymerase chain reaction. Germline TP53 mutation was not seen on standard DNA sequencing. The occurrence of secondary sarcomas, in the Children's Cancer Survivor study conducted in North America, has been associated with high cumulative doses of anthracyclines, which may also have played a role in the development of RMS in the present case. In the future, novel molecular technologies might uncover genetic cancer predisposition in patients with metachronous cancers.
Assuntos
Antineoplásicos/uso terapêutico , Neoplasias Ósseas/tratamento farmacológico , Segunda Neoplasia Primária , Neoplasias Nasais/diagnóstico , Osteossarcoma/tratamento farmacológico , Rabdomiossarcoma Alveolar/diagnóstico , Biópsia , Neoplasias Ósseas/diagnóstico , Pré-Escolar , DNA de Neoplasias/análise , Diagnóstico Diferencial , Feminino , Humanos , Mutação , Neoplasias Nasais/genética , Osteossarcoma/diagnóstico , Rabdomiossarcoma Alveolar/genéticaRESUMO
We established a dedifferentiated liposarcoma cell line (NDDLS-1) that produces interleukin-6 (IL-6) and granulocyte-colony stimulating factor (G-CSF). The parental tumor showed high leukemoid reactions. The NDDLS-1 cell line was established from a pleural effusion associated with a lung metastasis. Pleomorphic tumor cells arranged in a haphazard growth pattern were seen in xenograft tumors. Numerous inflammatory cells including neutrophils or eosinophils were present throughout the tumor cells. This finding resembled the dedifferentiated area of the parental tumor. The mice bearing NDDLS-1 showed marked leukocytosis. In addition, the NDDLS-1 cells expressed IL-6 and G-CSF at both the mRNA and protein levels, while the NDDLS-1 cells produced near normal levels of tumor necrosis factor alpha (TNF-α). In the cytogenetic analysis, both the parental tumor and the NDDLS-1 cells showed a ring or giant marker chromosomes. The NDDLS-1 cell line demonstrated the amplification and expression of both MDM2 and CDK4 by fluorescence in situ hybridization and immunohistochemical analysis. The NDDLS-1 cell line is consistent with the parental dedifferentiated liposarcoma, and it should therefore be useful for further investigations of human dedifferentiated liposarcomas.
Assuntos
Técnicas de Cultura de Células , Linhagem Celular Tumoral/patologia , Lipossarcoma/patologia , Neoplasias de Tecidos Moles/patologia , Idoso de 80 Anos ou mais , Animais , Linhagem Celular Tumoral/metabolismo , Linhagem Celular Tumoral/transplante , Aberrações Cromossômicas , Quinase 4 Dependente de Ciclina/genética , Quinase 4 Dependente de Ciclina/metabolismo , Fator Estimulador de Colônias de Granulócitos/metabolismo , Humanos , Hibridização in Situ Fluorescente , Interleucina-6/metabolismo , Leucocitose/patologia , Lipossarcoma/genética , Lipossarcoma/metabolismo , Neoplasias Pulmonares/secundário , Masculino , Camundongos , Camundongos SCID , Proteínas Proto-Oncogênicas c-mdm2/genética , Proteínas Proto-Oncogênicas c-mdm2/metabolismo , RNA Neoplásico/análise , Neoplasias de Tecidos Moles/genética , Neoplasias de Tecidos Moles/metabolismo , Cariotipagem Espectral , Transplante HeterólogoRESUMO
Podoplanin is known as a lymphatic marker because its expression is detected in lymphatic but not vascular endothelium. Podoplanin is also expressed in several normal tissues including osteocytes or osteoblasts. A systematic examination of the podoplanin expression was conducted in normal skeletal tissues and some bone tumor cell lines, and the diagnostic value determined in primary bone tumors. Podoplanin mRNA was expressed at a high level in bone marrow tissue and cartilage, and was upregulated with differentiation to osteoblasts in bone marrow cells. Strong podoplanin expression was seen in osteocytes, chondrocytes, and osteoblasts on immunohistochemistry. Podoplanin mRNA was expressed at a high level in several osteosarcoma and chondrosarcoma cell lines, whereas podoplanin was expressed at a low level in a Ewing's/primitive neuroectodermal tumor cell line. In the clinical samples, osteosarcomas (22/26) expressed podoplanin at various levels. In small cell osteosarcomas (2/2), podoplanin was expressed strongly, although the tissue samples included few diagnostic osteoids. Chondrosarcomas (10/10) expressed podoplanin strongly, and chondroblastomas (5/5) expressed podoplanin moderately, while podoplanin was absent or expressed at low levels in Ewing's sarcomas (0/5), chordomas (0/6) and giant cell tumors of bone (1/7). Therefore, podoplanin may be a sensitive immunohistochemical marker of osteogenic and chondrogenic bone tumors.
Assuntos
Neoplasias Ósseas/metabolismo , Osso e Ossos/metabolismo , Condrossarcoma/metabolismo , Glicoproteínas de Membrana/metabolismo , Osteossarcoma/metabolismo , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Neoplasias Ósseas/genética , Osso e Ossos/citologia , Diferenciação Celular , Linhagem Celular Tumoral , Células Cultivadas , Condroblastoma/genética , Condroblastoma/metabolismo , Condrossarcoma/genética , Tumores de Células Gigantes/genética , Tumores de Células Gigantes/metabolismo , Humanos , Imuno-Histoquímica , Glicoproteínas de Membrana/genética , Osteossarcoma/genética , RNA Mensageiro/genética , RNA Mensageiro/metabolismo , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Sarcoma de Ewing/genética , Sarcoma de Ewing/metabolismo , Sarcoma de Células Pequenas/genética , Sarcoma de Células Pequenas/metabolismoRESUMO
BACKGROUND: A giant cell tumor (GCT) of bone is a locally aggressive tumor with a propensity for local recurrence. A characteristic pattern of peripheral bone formation has been described in GCT recurrence in soft tissue, and in some pulmonary metastases from benign GCT. Although the bone formation in GCT in supposedly due to bone morphogenetic proteins (BMPs), the expression pattern of BMPs in GCT has not been well investigated. MATERIALS AND METHODS: The expression of BMPs in GCT tissues, cultured stromal cells from GCT, and osteoclast-like giant cells harvested by laser microdissection (LM), as well as from control osteosarcoma (NOS-1) cells was analyzed using reverse transcriptional-semiquantitative PCR. RESULTS: BMP 2, 3, 4, 5 and 6 were expressed in the GCT tissue. The cultured GCT cells expressed BMP 2, 4, 5 and 6. The osteoclast-like giant cells expressed BMP 2, 3, 5 and 6 and BMP 5 was expressed at the highest level. CONCLUSION: Both stromal cells and osteoclast-like cells in GCT expressed several kinds of BMPs.
Assuntos
Proteínas Morfogenéticas Ósseas/genética , Neoplasias Ósseas/genética , Tumor de Células Gigantes do Osso/genética , Adulto , Proteínas Morfogenéticas Ósseas/metabolismo , Neoplasias Ósseas/metabolismo , Neoplasias Ósseas/patologia , Feminino , Tumor de Células Gigantes do Osso/metabolismo , Tumor de Células Gigantes do Osso/patologia , Humanos , Lasers , Masculino , Microdissecção , Pessoa de Meia-Idade , Osteossarcoma/genética , Osteossarcoma/metabolismo , Osteossarcoma/patologia , RNA Mensageiro/genética , RNA Mensageiro/metabolismo , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Células Estromais/metabolismo , Células Estromais/patologia , Células Tumorais Cultivadas , Adulto JovemRESUMO
Background: Musculoskeletal sarcomas (MSSs) are rare cancers and often aggressive tumors that originate from mesenchymal tissue. Patients with advanced MSS often report difficulties with symptom burden, which can reduce their health-related quality-of-life. Objective: The aim of this study was to describe the patterns of the physical symptoms of MSS patients in the palliative setting and to detail the palliative treatment used in the last two weeks of life. Design: Retrospective study using the electronic patient records from a single institution. Setting/Subjects: A retrospective study was carried out in a sample of 46 consecutive MSS patients with locally advanced/metastatic disease, who were hospitalized and died in our department. The median age of these patients was 56 years at death. Measurements: Symptom burden and medical intervention during the last two weeks of life were collected. Results: The most frequent physical symptoms were pain and dyspnea in 93% and 78% of patients, respectively, while only 17% of patients suffered from nausea. A total of 98% of patients required opioids, and most patients were treated with morphine through either subcutaneous or intravenous continuous injection. Nonsteroidal anti-inflammatory drugs and acetaminophen were administered to 79% of patients. Corticosteroids were administered for the relief of dyspnea to 83% of patients. Of the patients, 46% received palliative chemotherapy within the last two weeks of life, and the oral treatment was continued until a median of 5.6 days before death. In addition, 39% of patients received a sedative treatment during the last two weeks of life for uncontrolled refractory symptoms. Conclusions: The symptom burden experienced by advanced MSS patients is profound at the end of life for all palliative approaches. Therefore, palliative medicine is an important and even crucial component of the continuum of care, allowing for aggressive symptom management with a variety of medical interventions, including palliative sedation.
Assuntos
Dispneia/tratamento farmacológico , Hipnóticos e Sedativos/uso terapêutico , Doenças Musculoesqueléticas/psicologia , Doenças Musculoesqueléticas/terapia , Cuidados Paliativos/psicologia , Sarcoma/psicologia , Sarcoma/terapia , Assistência Terminal/psicologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
BACKGROUND: Despite accumulating knowledge of chimeric genes derived from fusion of the HMGA2 gene with multiple partners in lipomas, the different clinicopathological features of lipomas depending on different gene aberrations have not been well documented. The purpose of this study was to examine the clinical significance of the expression of fusion genes in lipomas. PATIENTS AND METHODS: The expressions of three previously reported gene fusion transcripts, including HMGA2/LPP, HMGA2/RDC1 and HMGA2/NFIB, were analyzed in 102 tumors from patients with lipomas. RESULTS: There were 23 cases (22.5%) expressing HMGA2/LPP, 2 cases (1.9%) expressing HMGA2/RDC1 and no cases of HMGA2/NFIB expression (0%). There were no significant intergroup differences in age, gender, body mass index, tumor size or location. The magnetic resonance images and pathological features were also not different in regard to the status of fusion gene expression. CONCLUSION: There were no significant differences of clinicopathological features in patients with lipoma with or without these fusion gene transcripts.
Assuntos
Proteína HMGA2/genética , Lipoma/genética , Proteínas de Fusão Oncogênica/genética , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Fusão Gênica , Proteínas HMGA/genética , Humanos , Lipoma/patologia , Masculino , Pessoa de Meia-Idade , Fatores de Transcrição NFI/genética , RNA Mensageiro/genética , Receptores CXCR/genéticaRESUMO
The value of routine blood tests in malignant soft tissue tumors remains uncertain. To determine if these tests can be used for screening, the routine pretreatment blood test findings were retrospectively investigated in 359 patients with benign and malignant soft tissue tumors. Additionally, the prognostic potential of pretreatment blood abnormalities was evaluated in patients with soft tissue sarcomas. We compared clinical factors and blood tests findings between patients with benign and malignant soft tissue tumors using univariate and multivariate analysis. Subsequently, patients with malignant tumors were divided into two groups based on blood test reference values, and the prognostic significance of each parameter was evaluated. In the univariate analysis, age, tumor size, and tumor depth were significant clinical diagnostic factors. Significant increases in the granulocyte count, C-reactive protein (CRP) level, erythrocyte sedimentation rate (ESR), and γ-glutamyl transpeptidase (γ-GTP) levels were found in patients with malignant soft tissue tumors. Multiple logistic regression showed that tumor size and ESR were independent factors that predicted malignant soft tissue tumors. The Kaplan-Meier survival analysis revealed that granulocyte counts, γ-GTP levels, and CRP levels correlated significantly with overall survival. Thus, pretreatment routine blood tests are useful diagnostic and prognostic markers for diagnosing soft tissue sarcoma.
Assuntos
Biomarcadores Tumorais/sangue , Proteína C-Reativa/análise , Granulócitos/patologia , Doenças Hematológicas/fisiopatologia , Sarcoma/patologia , gama-Glutamiltransferase/sangue , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Sedimentação Sanguínea , Criança , Pré-Escolar , Feminino , Seguimentos , Testes Hematológicos/métodos , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Sarcoma/sangue , Sarcoma/classificação , Sarcoma/metabolismo , Taxa de Sobrevida , Adulto JovemRESUMO
Bone leiomyosarcoma is a rare primary osseous malignant tumor with a high metastatic potential. Similar to other bone sarcomas, high histological grade and tumor stage are predictive of a poor outcome. We herein present our experience with treating a 64-year-old woman with bone leiomyosarcoma accompanied by multiple bone metastases. A biopsy revealed occasional osteoclast-like giant cells. In addition to radiation therapy, the osteoclastogenesis inhibitor denosumab was administered but the patient did not undergo adjuvant chemotherapy or surgery. Good clinical and short-term radiological responses to denosumab have been observed for 2 years. Therefore, denosumab may represent a viable treatment option without the need for adjuvant chemotherapy.
RESUMO
Dedifferentiated chondrosarcoma is a rare, highly malignant variant of chondrosarcoma in which a high-grade sarcoma coexists with a low-grade chondroid tumor. We herein review a case of dedifferentiated chondrosarcoma with an osteosarcoma omit component that occurred in the distal femur of a 38-year-old man. We established the cell line (NDCS-1) from a pleural effusion of the metastatic lung tumor. The cell line was characterized by a the G-banded karyotype, polymerase chain reaction (PCR) single-strand conformation polymorphism analysis, spectral karyotyping, and reverse transcriptase PCR (RT-PCR). The tumor exhibited complex karyotypes and a high frequency of chromosomal amplication with p53 mutation. This tumor revealed an osteoblastic and chondroblastic character in vitro and in severe combined immunodeficiency mice. The expression and phosphorylation of platelet-derived growth factor receptor-beta, which seemed to play a major role in the malignant phenotype of chondrosarcoma, was confirmed by RT-PCR and Western blotting. To our knowledge, this is the first report of the establishment of a human dedifferentiated chondrosarcoma.
Assuntos
Diferenciação Celular , Linhagem Celular Tumoral , Condrossarcoma/patologia , Neoplasias Femorais/patologia , Osteoblastos/patologia , Adulto , Animais , Western Blotting , Divisão Celular , Condrossarcoma/genética , Aberrações Cromossômicas , Neoplasias Femorais/genética , Genes p53/genética , Humanos , Cariotipagem , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/secundário , Masculino , Camundongos , Camundongos SCID , Mutação , Transplante de Neoplasias , Derrame Pleural/citologia , Polimorfismo Conformacional de Fita Simples , Receptor beta de Fator de Crescimento Derivado de Plaquetas/genética , Receptor beta de Fator de Crescimento Derivado de Plaquetas/metabolismo , Reação em Cadeia da Polimerase Via Transcriptase ReversaRESUMO
We report on a case of a solitary fibrous tumor that developed in the thigh of an 82-year-old woman. The tumor was composed of areas of high-grade sarcoma and typical solitary fibrous tumor. Its karyotype was: 70,XXX,+X[4],+1[2],add(1)(p36)[4],add(1)[2],+2[4],-3[4],+6[4],add(6)(p11)x2[4],+7[4],+9[3],-11[4],-12[4],-13[4],add(13)(p11)x2[4],-14[4],+15[4],-16[3],-17[4],-19[4],+20,[4],+21[4],+22[2],+mar1x2[4][cp4].
Assuntos
Neoplasias de Tecido Fibroso/patologia , Neoplasias de Tecidos Moles/patologia , Idoso , Idoso de 80 Anos ou mais , Análise Citogenética , Feminino , Humanos , Neoplasias de Tecido Fibroso/genética , Neoplasias de Tecidos Moles/genética , Coxa da PernaRESUMO
BACKGROUND: Reconstruction of the extensor mechanism after resection of the proximal tibia is challenging, and several surgical procedures are available. The purpose of this study was to determine the outcome of the fibular transposition technique for reconstruction of the extensor mechanism of the knee after proximal tibial resection. METHODS: We retrospectively reviewed five consecutive patients who underwent resection of the proximal tibia with prosthetic reconstruction and reconstruction of the extensor using fibular transposition between 1997 and 2011. There were two female and three male patients with a mean age of 50years (range, 27 to 76years). A follow-up evaluation included both passive and active range of motion, extensor lag, the MSTS score and complications. RESULTS: Patients were followed up for 93months (range, 44 to 160months). The mean extensor lag and active flexion were four degrees (range, 0 to 10°) and 103° (range, 85 to 110°), respectively. The mean MSTS score was 80% (range, 73 to 90%). All patients were able to ambulate without crutches at the latest follow-up. CONCLUSIONS: The utilization of the fibular transposition technique is a simple, reliable, and successful procedure for extensor reconstruction after proximal tibial resection.
Assuntos
Neoplasias Ósseas/cirurgia , Fíbula/cirurgia , Osteotomia , Ligamento Patelar/cirurgia , Tíbia/cirurgia , Adulto , Idoso , Feminino , Humanos , Articulação do Joelho/cirurgia , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
The prognosis of alveolar soft part sarcoma is poor, despite the slow growth of the tumor. A number of cases with spontaneous regression of this rare tumor have been reported. Although the mechanisms underlying spontaneous regression remain uncertain, local immune reaction may be a possible contributing factor. Immunohistochemical expression of human leukocyte antigen (HLA) class I, cluster of differentiation (CD) 3, CD4, CD8, CD20, CD45, CD56, CD68, CD138 and CD163 were assessed in a series of 10 alveolar soft part sarcomas, and the expression profiles were associated with patients' clinicopathological parameters. Expression of HLA class I was observed in almost all the tumor cells of all cases. CD8(+) cells were identified in all tumors with varying densities. Moderate infiltration of CD8(+) cells was detected in three patients; one of these patients survived with long-term tumor remission. Infiltration of CD10(+), CD20(+), CD56(+) or CD138(+) cells was not revealed in all tumors. Moderate-diffuse infiltration of CD163(+) cells was observed in all tumors. To the best of our knowledge, the present study represents the first report of intratumoral immune cells in alveolar soft part sarcoma. Frequent expression of HLA class I in tumor cells was observed. CD8(+) cells were identified at various densities and CD163(+) cells were observed in alveolar soft part sarcoma. Moderate infiltration of CD8(+) cells in patients with a good prognosis may indicate the antitumor effects of immune cells in alveolar soft part sarcoma.
RESUMO
Prominent osteoconductive activity and the biodegradable nature of commercially available beta-tricalcium phosphate (beta-TCP, OSferion) have been documented in animal experiments. We analyzed four cases of involving grafted OSferion in human bone with respect to histological features by routine hematoxylin and eosin staining, silver impregnation, immunohistochemistry and in situ hybridization. OSferion affords early bioresorption by osteoclasts, vascular invasion of macropores and osteoblastic cell attachment on the surface on the ceramic surface 14 days after grafting. Prominent bone formation and direct bone connection between preexisting bone and OSferion were evident 28 days after grafting. Nearly the entire TCP surface was covered by lamellar bone; additionally, active osteoblastic lining and attachment of the osteoclast-like giant cells were not observed 72 weeks after grafting. Silver impregnation revealed the presence of collagen fibrils within probable micropores of OSferion.
Assuntos
Materiais Biocompatíveis , Substitutos Ósseos , Osso e Ossos/citologia , Fosfatos de Cálcio , Adolescente , Adulto , Idoso , Materiais Biocompatíveis/isolamento & purificação , Materiais Biocompatíveis/uso terapêutico , Substitutos Ósseos/isolamento & purificação , Substitutos Ósseos/uso terapêutico , Osso e Ossos/patologia , Fosfatos de Cálcio/isolamento & purificação , Fosfatos de Cálcio/uso terapêutico , Durapatita , Feminino , Fraturas do Fêmur/patologia , Fraturas do Fêmur/cirurgia , Fraturas do Quadril/patologia , Fraturas do Quadril/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Osteossarcoma/patologia , Osteossarcoma/cirurgiaRESUMO
BACKGROUND: Activating Gs a mutations have been identified in most instances of fibrous dysplasia (FD). This mutation leads to consistently elevated intracellular cyclic adenosine monophosphate (cAMP) levels, with various biological consequences. The development of secondary sarcoma in FD is a rare but well-established phenomenon. This finding raised the possibility that a common gene mutation exists in these tumors. MATERIALS AND METHODS: The expression of the Gs a mutation was examined in 16 cell lines and 173 musculoskeletal tumor tissues, including 13 cases of FD, via RT-PCR and sequence analysis. RESULTS: No expression of a Gs a mutation was detected in any cell line or clinical tissue sample, excluding FD tissues. Direct sequence analysis demonstrated results identical to those of RT-PCR. CONCLUSION: Activating Gs a mutation rarely occurs in musculoskeletal tumors other than FD. The occurrence of most sarcomas displays no correlation with Gs a mutations.
Assuntos
Neoplasias Ósseas/genética , Displasia Fibrosa Óssea/genética , Subunidades alfa Gs de Proteínas de Ligação ao GTP/genética , Neoplasias Musculares/genética , Mutação , Osteossarcoma/genética , Sarcoma/genética , Linhagem Celular Tumoral , Humanos , Reação em Cadeia da Polimerase Via Transcriptase ReversaRESUMO
A giant-cell tumor of the bone (GCTB) is a benign but locally aggressive bone tumor. Recently, the receptor activator of nuclear factor κB (RANK) ligand inhibitor, denosumab, has demonstrated activity against giant-cell tumors. The current study reports a case of a sacral GCTB with lung metastasis. A 19-year-old male patient presented with right buttock pain and right lower leg pain, and a sacral GCTB was diagnosed based on the histological analysis of a biopsy specimen. The patient was successfully treated with neoadjuvant denosumab therapy, which allowed curettage. In addition, the pulmonary nodule reduced in size following denosumab administration, and surgical resection was performed. Since the operation, the patient has been managed with the continued use of denosumab with no sign of recurrence. Microscopic findings from the surgical specimen following denosumab treatment revealed that the giant cells had disappeared and woven bone had formed. The specimen from the pulmonary nodule exhibited similar findings to the surgical specimen. It was reported that denosumab treatment was able to reduce the number of giant cells and RANK-positive stromal cells, and cause the formation of new bone in the primary lesion. The present study reports the first case to demonstrate the efficiency of denosumab in treating pulmonary metastasis of GCTB.