RESUMO
BACKGROUND: Solitary fibrous tumors (SFT) of the central nervous system are rare and treatment options are not well established. The aim of this study was to evaluate the clinical outcomes of radiotherapy (RT) and re-radiotherapy (re-RT) for de novo intracranial SFT and recurrent intracranial SFT. METHODS: This retrospective study analyzed efficacy and toxicity of different RT modalities in patients who received radiotherapy (RT) for intracranial SFT at Heidelberg University Hospital between 2000 and 2020 following initial surgery after de novo diagnosis ("primary group"). We further analyzed the patients of this cohort who suffered from tumor recurrence and received re-RT at our institution ("re-irradiation (re-RT) group"). Median follow-up period was 54.0 months (0-282) in the primary group and 20.5 months (0-72) in the re-RT group. RT modalities included 3D-conformal RT (3D-CRT), intensity-modulated RT (IMRT), stereotactic radiosurgery (SRS), proton RT, and carbon-ion RT (C12-RT). Response rates were analyzed according to RECIST 1.1 criteria. RESULTS: While the primary group consisted of 34 patients (f: 16; m:18), the re-RT group included 12 patients (f: 9; m: 3). Overall response rate (ORR) for the primary group was 38.3% (N = 11), with 32.4% (N = 11) complete remissions (CR) and 5.9% (N = 2) partial remissions (PR). Stable disease (SD) was confirmed in 5.9% (N = 2), while 41.2% (N = 14) experienced progressive disease (PD). 14% (N = 5) were lost to follow up. The re-RT group had 25.0% CR and 17.0% PR with 58.0% PD. The 1-, 3-, and 5-year progression-free survival rates were 100%, 96%, and 86%, respectively, in the primary group, and 81%, 14%, and 14%, respectively, in the re-RT group. Particle irradiation (N = 11) was associated with a lower likelihood of developing a recurrence in the primary setting than photon therapy (N = 18) (OR = 0.038; p = 0.002), as well as doses ≥ 60.0 Gy (N = 15) versus < 60.0 Gy (N = 14) (OR = 0.145; p = 0.027). Risk for tumor recurrence was higher for women than for men (OR = 8.07; p = 0.014) with men having a median PFS of 136.3 months, compared to women with 66.2 months. CONCLUSION: The data suggests RT as an effective treatment option for intracranial SFT, with high LPFS and PFS rates. Radiation doses ≥ 60 Gy could be associated with lower tumor recurrence. Particle therapy may be associated with a lower risk of recurrence in the primary setting, likely due to the feasibility of higher RT-dose application.
Assuntos
Radioterapia com Íons Pesados , Hemangiopericitoma , Tumores Fibrosos Solitários , Masculino , Humanos , Feminino , Prótons , Recidiva Local de Neoplasia/radioterapia , Estudos Retrospectivos , Hemangiopericitoma/radioterapia , Hemangiopericitoma/patologia , Hemangiopericitoma/cirurgia , Tumores Fibrosos Solitários/radioterapia , Tumores Fibrosos Solitários/patologia , Radioterapia com Íons Pesados/efeitos adversosRESUMO
BACKGROUND: Intracranial solitary fibrous tumors (SFTs), formerly hemangiopericytomas (HPCs), are rare, aggressive dural-based mesenchymal tumors. While adjuvant radiation therapy has been suggested to improve local tumor control (LTC), especially after subtotal resection, the role of postoperative stereotactic radiosurgery (SRS) and the optimal SRS dosing strategy remain poorly defined. METHODS: PubMed, EMBASE, and Web of Science were systematically searched according to PRISMA guidelines for studies describing postoperative SRS for intracranial SFTs. The search strategy was defined in the authors' PROSPERO protocol (CRD42023454258). RESULTS: 15 studies were included describing 293 patients harboring 476 intracranial residual or recurrent SFTs treated with postoperative SRS. At a mean follow-up of 21-77 months, LTC rate after SRS was 46.4-93% with a mean margin SRS dose of 13.5-21.7 Gy, mean maximum dose of 27-39.6 Gy, and mean isodose at the 42.5-77% line. In pooled analysis of individual tumor outcomes, 18.7% of SFTs demonstrated a complete SRS response, 31.7% had a partial response, 18.9% remained stable (overall LTC rate of 69.3%), and 30.7% progressed. When studies were stratified by margin dose, a mean margin dose > 15 Gy showed an improvement in LTC rate (74.7% versus 65.7%). CONCLUSIONS: SRS is a safe and effective treatment for intracranial SFTs. In the setting of measurable disease, our pooled data suggests a potential dose response of improving LTC with increasing SRS margin dose. Our improved understanding of the aggressive biology of SFTs and the tolerated adjuvant SRS parameters supports potentially earlier use of SRS in the postoperative treatment paradigm for intracranial SFTs.
Assuntos
Radiocirurgia , Febre Grave com Síndrome de Trombocitopenia , Tumores Fibrosos Solitários , Humanos , Radiocirurgia/métodos , Seguimentos , Estudos Retrospectivos , Resultado do Tratamento , Tumores Fibrosos Solitários/radioterapia , Tumores Fibrosos Solitários/cirurgiaRESUMO
The knowledge of optimal treatments for patients with intracranial solitary fibrous tumor (SFT) is limited, with inconclusive results from previous studies. In this study, we conducted a meta-analysis of relevant studies to identify the prognostic impact of the extent of resection (EOR) and postoperative radiotherapy (PORT) on survival outcomes of patients with intracranial SFT. We searched the Medline, Embase, and Cochrane Central Register of Controlled Trials (CENTRAL) to identify relevant studies published till April 2022. Progression-free survival (PFS) and overall survival (OS) were the outcomes of interest. Differences between two cohorts (gross total resection [GTR] vs. subtotal resection [STR] and PORT vs. surgery only) were estimated by calculating hazard ratios. Twenty-seven studies were selected for the meta-analysis, including data of 1348 patients (GTR, n = 819 vs. STR, n = 381 and PORT, n = 723 vs. surgery only, n = 578). Pooled hazard ratios of PFS (1, 3, 5, and 10 years) and OS (3, 5, and 10 years) revealed that the GTR cohort showed sustained superiority over the STR cohort. In addition, the PORT cohort was superior to the surgery-only cohort with respect to all PFS periods. Although the 10-year OS between the two cohorts was not statistically different, PORT showed significantly better 3- and 5-year OS than surgery only. The study findings suggest that GTR and PORT provide significant benefits for PFS and OS. Aggressive surgical resection of tumors to achieve GTR followed by PORT should be implemented as optimal treatments for all patients with intracranial SFT when feasible.
Assuntos
Hemangiopericitoma , Tumores Fibrosos Solitários , Humanos , Estudos Retrospectivos , Tumores Fibrosos Solitários/radioterapia , Tumores Fibrosos Solitários/cirurgia , Prognóstico , Hemangiopericitoma/cirurgia , Intervalo Livre de ProgressãoRESUMO
BACKGROUND/AIM: The curative treatment for solitary fibrous tumors (SFTs) consists of surgery. However, surgery for SFTs in the skull base is difficult due to the anatomy and curative surgery may not be possible. Carbon-ion radiotherapy (C-ion RT) might be useful in the treatment of inoperable SFTs in the skull base because of its biological and physical nature. This study presents the clinical outcomes of C-ion RT for an inoperable SFT of the skull base. CASE REPORT: A 68-year-old female patient experienced hoarseness, deafness on the right side, right facial nerve paralysis, and dysphagia. Magnetic resonance imaging revealed a tumor located in the right cerebello-pontine angle with destruction of the petrous bone, and immunohistochemical studies of the biopsy specimen showed a grade 2 SFT. Firstly, the patient underwent tumor embolization and surgery. However, 5 months after surgery, magnetic resonance imaging revealed regrowth of residual tumor. Subsequently, the patient was referred to our hospital for C-ion RT because curative surgery was unsuitable. The patient received 64 Gy (relative biological effectiveness) of C-ion RT in 16 fractions. Two years after C-ion RT, the tumor showed a partial response. The patient was still alive at the last follow-up without evidence of local recurrence, distant metastasis, or late toxicities. CONCLUSION: These findings suggest that C-ion RT is a suitable treatment option for inoperable SFTs of the skull base.
Assuntos
Hemangiopericitoma , Febre Grave com Síndrome de Trombocitopenia , Tumores Fibrosos Solitários , Feminino , Humanos , Idoso , Base do Crânio , Cabeça , Tumores Fibrosos Solitários/diagnóstico , Tumores Fibrosos Solitários/radioterapia , Tumores Fibrosos Solitários/cirurgia , CarbonoRESUMO
Spinal solitary fibrous tumour (SFT) is an uncommon tumour with few cases reported in the literature. It rarely originates at the craniocervical junction. To our knowledge, only eight cases of spinal SFT located at the craniocervical junction have been reported in the literature. We presented a patient with a craniocervical junction SFT and discussed its clinical presentation, radiological features, pathology, management and outcome. This was the first patient reported in the literature with a recurrent craniocervical junction SFT treated with Gamma Knife radiosurgery. The treatment reduced the tumour volume by more than 85% within 12 months.
Assuntos
Radiocirurgia , Tumores Fibrosos Solitários , Humanos , Radiografia , Tumores Fibrosos Solitários/diagnóstico por imagem , Tumores Fibrosos Solitários/radioterapia , Tumores Fibrosos Solitários/cirurgiaRESUMO
PURPOSE: This study aimed to evaluate the role of postoperative radiotherapy (PORT) in intracranial solitary fibrous tumor/hemangiopericytoma (SFT/HPC). MATERIALS AND METHODS: A total of 133 patients with histologically confirmed HPC were included from eight institutions. Gross total resection (GTR) and subtotal resection (STR) were performed in 86 and 47 patients, respectively. PORT was performed in 85 patients (64%). The prognostic effects of sex, age, performance, World Health Organization (WHO) grade, location, size, Ki-67, surgical extent, and PORT on local control (LC), distant metastasis-free survival (DMFS), progression-free survival (PFS), and overall survival (OS) were estimated by univariate and multivariate analyses. RESULTS: The 10-year PFS, and OS rates were 45%, and 71%, respectively. The multivariate analysis suggested that PORT significantly improved LC (p < 0.001) and PFS (p < 0.001). The PFS benefit of PORT was maintained in the subgroup of GTR (p=0.001), WHO grade II (p=0.001), or STR (p < 0.001). In the favorable subgroup of GTR and WHO grade II, PORT was also significantly related to better PFS (p=0.028). WHO grade III was significantly associated with poor DMFS (p=0.029). In the PORT subgroup, the 0-0.5 cm margin of the target volume showed an inferior LC to a large margin with 1.0-2.0 cm (p=0.021). Time-dependent Cox proportion analysis showed that distant failures were significantly associated with poor OS (p=0.003). CONCLUSION: This multicenter study supports the role of PORT in disease control of intracranial SFT/HPC, irrespective of the surgical extent and grade. For LC, PORT should enclose the tumor bed with sufficient margin.
Assuntos
Neoplasias Encefálicas/radioterapia , Hemangiopericitoma/radioterapia , Cuidados Pós-Operatórios/métodos , Tumores Fibrosos Solitários/radioterapia , Adolescente , Adulto , Idoso , Neoplasias Encefálicas/patologia , Criança , Intervalo Livre de Doença , Feminino , Hemangiopericitoma/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Intervalo Livre de Progressão , Estudos Retrospectivos , Tumores Fibrosos Solitários/patologiaRESUMO
INTRODUCTION: A meningeal solitary fibrous tumor (SFT), also called hemangiopericytoma, is a rare mesenchymal malignancy. Due to anatomic constrains, even after macroscopic complete surgery with curative intent, the local relapse risk is still relatively high, thus increasing the risk of dedifferentiation and metastatic spread. This study aims to better define the role of postoperative radiotherapy (RT) in meningeal SFTs. PATIENTS AND METHODS: A retrospective study was performed across seven sarcoma centers. Clinical information was retrieved from all adult patients with meningeal primary localized SFT treated between 1990 and 2018 with surgery alone (S) compared to those that also received postoperative RT (S + RT). Differences in treatment characteristics between subgroups were tested using independent samples t-test for continuous variables and chi-square tests for proportions. Local control (LC) and overall survival (OS) rates were calculated as time from start of treatment until progression or death from any cause. LC and OS in groups receiving S or S + RT were compared using Kaplan-Meier survival curves. RESULTS: Among a total of 48 patients, 7 (15%) underwent S and 41 (85%) underwent S + RT. Median FU was 65 months. LC was significantly associated with treatment. LC after S at 60 months was 60% versus 90% after S + RT (p = 0.052). Furthermore, R1 resection status was significantly associated with worse LC (HR 4.08, p = 0.038). OS was predominantly associated with the mitotic count (HR 3.10, p = 0.011). CONCLUSION: This retrospective study, investigating postoperative RT in primary localized meningeal SFT patients, suggests that combining RT to surgery in the management of this patient population may reduce the risk for local failures.
Assuntos
Hemangiopericitoma , Neoplasias Meníngeas , Tumores Fibrosos Solitários , Adulto , Hemangiopericitoma/radioterapia , Hemangiopericitoma/cirurgia , Humanos , Neoplasias Meníngeas/radioterapia , Neoplasias Meníngeas/cirurgia , Recidiva Local de Neoplasia , Estudos Retrospectivos , Tumores Fibrosos Solitários/radioterapia , Tumores Fibrosos Solitários/cirurgiaRESUMO
BACKGROUND: Solitary fibrous tumor (SFT) is a rare mesenchymal malignancy. Although surgery is potentially curative, the local relapse risk is high after marginal resections. Given the lack of prospective clinical trial data, the objective of the current study was to better define the role of perioperative radiotherapy (RT) in various SFT presentations by location. METHODS: This was retrospective study performed across 7 sarcoma centers. Clinical information was retrieved from all adult patients with extrameningeal, primary, localized SFT who were treated between 1990 and 2018 with surgery alone (S) compared with those who also received perioperative RT (S+RT). Differences in treatment characteristics between subgroups were tested using analysis of variance statistics and propensity score matching. Local control and overall survival rates were calculated from the start of treatment until progression or death from any cause. RESULTS: Of all 549 patients, 428 (78%) underwent S, and 121 (22%) underwent S+RT. The median follow-up was 52 months. After correction for mitotic count and surgical margins, S+RT was significantly associated with a lower risk of local progression (hazard ratio, 0.19: P = .029), an observation further confirmed by propensity score matching (P = .012); however, this association did not translate into an overall survival benefit. CONCLUSIONS: The results from this retrospective study investigating perioperative RT in patients with primary extrameningeal SFT suggest that combining RT with surgery in the management of this patient population is significantly associated with a reduced risk of local failures, especially in patients who have less favorable resection margins and in those who have tumors with a high mitotic count.
Assuntos
Tumores Fibrosos Solitários/radioterapia , Tumores Fibrosos Solitários/cirurgia , Análise de Variância , Terapia Combinada/estatística & dados numéricos , Progressão da Doença , Extremidades , Feminino , Seguimentos , Neoplasias de Cabeça e Pescoço/mortalidade , Neoplasias de Cabeça e Pescoço/radioterapia , Neoplasias de Cabeça e Pescoço/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Índice Mitótico , Intervalo Livre de Progressão , Pontuação de Propensão , Neoplasias Retroperitoneais/mortalidade , Neoplasias Retroperitoneais/radioterapia , Neoplasias Retroperitoneais/cirurgia , Estudos Retrospectivos , Tumor Fibroso Solitário Pleural/mortalidade , Tumor Fibroso Solitário Pleural/radioterapia , Tumor Fibroso Solitário Pleural/cirurgia , Tumores Fibrosos Solitários/mortalidade , Taxa de Sobrevida , TroncoRESUMO
BACKGROUND: Malignant solitary fibrous tumor (MSFT) arising from the spinal cord is extremely rare and poorly understood mesenchymal neoplasms: only a few MSFT in the spinal canal has been described. We describe the clinical course of the patient with MSFT arising from the thoracic spinal cord. CASE REPORT: We describe the clinical course of the patient and the radiological and pathological findings of the tumor. The tumor had been resected by microscopic posterior approach and video-assisted thoracic surgery, but local recurrence was observed by MRI at 1-year follow-up period. No metastatic lesion was confirmed. Then, carbon ion radiotherapy (CIRT) was administered to the recurrent lesion. Local suppression has been observed for 40 months after irradiation. CONCLUSION: Dumbbell-shaped MSFT arising from thoracic spinal cord is a highly unusual presentation. CIRT might be effective for treatment of recurrent tumors.
Assuntos
Radioterapia com Íons Pesados/métodos , Recidiva Local de Neoplasia/radioterapia , Tumores Fibrosos Solitários/radioterapia , Neoplasias da Coluna Vertebral/radioterapia , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/diagnóstico , Tumores Fibrosos Solitários/diagnóstico , Neoplasias da Coluna Vertebral/diagnóstico , Vértebras TorácicasRESUMO
BACKGROUND AND PURPOSE: The role of radiotherapy (RT) in the treatment of hemangiopericytoma/solitary fibrous tumor (HPC/SFT) is still under debate. We aimed at investigating whether radiotherapy can improve the results in patients operated for extracranial HPC/SFT. MATERIALS AND METHODS: Data from patients with HPC/SFT, treated from 1982 to 2012, were retrospectively reviewed within the Rare Cancer Network framework. Actuarial local control (LC), disease-free survival (DFS), metastasis-free survival (MFS) and overall survival (OS) were calculated with Kaplan-Meyer method. Patient and tumor parameters were analyzed by univariate and multivariate analysis. RESULTS: Of 114 HPC/SFT, 58 (50.9%) occurred in the extremities/superficial trunk and 56 (49.1%) in intra-thoracic/retroperitoneum. Seventy-eight patients (68.4%) underwent surgery only (Sx), and 36 (31.6%) Sx and RT (Sx + RT). Median RT dose was 60 Gy (range 45-68.4 Gy) in 1.6-2.2 Gy fractions. In the extremities/superficial trunk group of patients, actuarial 5-year LC rates were 50.4% after Sx and 91.6% after Sx + RT (p < 0.0001) for LC, and 50.4% after Sx and 83.1% after Sx + RT (p = 0.008) for DFS. In the intra-thoracic/retroperitoneum group of patients, actuarial 5-year rates were 89.3% after Sx and 77.8% after Sx + RT (p = 0.99) for LC, and 73.8% after Sx and 77.8% after Sx + RT (p = 0.93) for DFS. At multivariate analysis, the addition of RT resulted in better LC and DFS in the whole series. The advantage was confirmed for LC in the group of patients affected by extremity/superficial trunk tumors. CONCLUSION: Addition of RT to Sx could improve the prognosis, in terms of LC and DFS, essentially in patients with extremities/superficial trunk tumor locations.
Assuntos
Hemangiopericitoma , Tumores Fibrosos Solitários , Hemangiopericitoma/radioterapia , Hemangiopericitoma/cirurgia , Humanos , Prognóstico , Intervalo Livre de Progressão , Estudos Retrospectivos , Tumores Fibrosos Solitários/radioterapiaRESUMO
Hemangiopericytoma and Solitary Fibrous Tumor are tumors with low incidence. They have a tendency to recur locally and to metastasize. The WHO integrated both tumors into a new entity but one of the pending issues is to demonstrate the effectiveness of surgery plus complementary radiotherapy (RT) and standardize the use of it. We reviewed the data from 10 years. We assessed pathologic and radiologic characteristics. The operation records were evaluated to determine the features and extent of tumor resection. We compared the outcomes in patients using or not RT. The mean follow-up was 74.8 months, with a range of 12 and 210 months. The population included 3 males (30%) and 7 females (70%). The most common location was brain convexity (30%), the remaining were cervical and lumbar spine, sacrum, intraventricular, torcular, sphenoid ridge and intraorbital. Postoperative external beam radiotherapy was delivered in 7 patients (70%), the criteria were a partial resection or WHO II and III histological grades. 2 patients developed local recurrences at 12 and 19 months after initial surgery. 1 patient underwent 2 surgeries, and the other, 4 surgeries. The mean recurrence free survival rate was 15.5 months. Distant metastases were found in 4 patients. 3 of the 10 patients died. Five-year overall survival rate was 66% and mean overall survival was 76 months. A safe and complete resection in the first surgery is the most important prognostic factor. Complementary RT can be helpful, even in cases of complete resection in WHO low-grade.
Assuntos
Hemangiopericitoma , Tumores Fibrosos Solitários , Sistema Nervoso Central/fisiologia , Feminino , Hemangiopericitoma/radioterapia , Hemangiopericitoma/cirurgia , Humanos , Masculino , Recidiva Local de Neoplasia/epidemiologia , Prognóstico , Estudos Retrospectivos , Tumores Fibrosos Solitários/radioterapia , Tumores Fibrosos Solitários/cirurgiaRESUMO
OBJECTIVE: In this study, we retrospectively reviewed our experience in the surgical management of solitary fibrous tumor (SFT)/hemangiopericytomas (HPCs) of the spinal cord. METHODS: Sixteen patients with SFT/HPCs of the spinal cord were enrolled in this study. Data on clinical presentation, radiologic findings, histopathologic features, surgical treatment, adjuvant therapy, and prognosis were retrospectively reviewed. Kaplan-Meier curves and log-rank tests were used to identify the prognostic factors for recurrence and overall survival (OS). RESULTS: Our series included 6 men and 10 women, with a male/female ratio of 1:1.7. Magnetic resonance imaging (MRI) showed slightly hyperintense lesions on T2-weighted images for all 16 patients. All tumors showed positive immunohistochemical staining for signal transducer and activator of transcription 6. Statistical analysis of clinical data showed that age, gender, tumor location, tumor size, medullary compartment location, and Ki-67 index were not associated with recurrence and OS (P > 0.05). However, World Health Organization grade III was significantly associated with recurrence (P < 0.01). Gross total resection (GTR) and postoperative radiotherapy significantly reduced recurrence (P < 0.01 and P < 0.05), but only GTR showed remarkable benefits to improve OS (P < 0.05). CONCLUSIONS: SFT/HPCs of spinal cord are rare neoplasms with a propensity to recur. Hyperintensity on T2-weighted magnetic resonance imaging combined with positive immunohistochemical staining for signal transducer and activator of transcription 6 are important clues for classification and differentiation of these tumors. The extent of resection, World Health Organization grade, and postoperative radiotherapy might be predictive factors for recurrence. Complete tumor resection should be sought whenever possible, and adjuvant radiotherapy is recommended after surgical resection. Moreover, regular and long-term follow-up is mandatory to monitor recurrence.
Assuntos
Hemangiopericitoma/cirurgia , Tumores Fibrosos Solitários/cirurgia , Neoplasias da Medula Espinal/cirurgia , Adulto , Idoso , Feminino , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/mortalidade , Hemangiopericitoma/radioterapia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Prognóstico , Radioterapia Adjuvante , Estudos Retrospectivos , Tumores Fibrosos Solitários/diagnóstico , Tumores Fibrosos Solitários/mortalidade , Tumores Fibrosos Solitários/radioterapia , Medula Espinal/patologia , Medula Espinal/cirurgia , Neoplasias da Medula Espinal/diagnóstico , Neoplasias da Medula Espinal/mortalidade , Neoplasias da Medula Espinal/radioterapia , Análise de Sobrevida , Adulto JovemRESUMO
PURPOSE: Solitary fibrous tumors (SFTs) are extremely rare mesenchymal malignancies. Given the lack of large prospective studies on radiation therapy (RT) with definitive and/or palliative intent in SFT patients, this retrospective study aimed to better define the benefit of RT in this disease. METHODS AND MATERIALS: A retrospective observational study was performed across 7 sarcoma centers. Clinical information was retrieved from all patients receiving RT without surgery over the course of their locally advanced and/or metastatic disease. Differences in treatment characteristics between subgroups were tested using analysis-of-variance tests. Local control (LC) and overall survival (OS) rates were calculated as time from the start of RT until local progression and death from any cause, respectively. RESULTS: Since 1990, a total of 40 patients were identified. RT was applied with definitive intent in 16 patients and with palliative intent in 24. The median follow-up period was 62 months. In patients treated with definitive RT (receiving approximately 60 Gy), the objective response rate was 67%. At 5 years, the LC rate was 81.3%, and the OS rate was 87.5%. In the case of palliative RT (typically 39 Gy), the objective response rate was 38%. The LC and OS rates at 5 years were 62.5% and 54.2%, respectively. In both subgroups, RT-associated toxicities were mild with predominantly grade 1 acute and late side effects. CONCLUSIONS: This retrospective study suggests a clinically meaningful benefit for RT given with either definitive or palliative intent without surgery in SFT management. Prospective registries potentially in collaboration with patient advocacy groups are warranted to further assess the role of RT in patients with this rare malignancy.
Assuntos
Sarcoma/mortalidade , Sarcoma/radioterapia , Tumores Fibrosos Solitários/mortalidade , Tumores Fibrosos Solitários/radioterapia , Adulto , Idoso , Progressão da Doença , Intervalo Livre de Doença , Europa (Continente) , Feminino , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Metástase Neoplásica , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Tempo , Resultado do TratamentoRESUMO
OBJECTIVE: Solitary fibrous tumours (SFTs) occurring in the spine are rare. Herein, we review the clinical and imaging data of spinal SFT. METHODS: We retrospectively analysed eight cases of pathologically confirmed spinal SFT imaging and clinical data, pathological manifestations, surgical methods, and follow-up results. RESULTS: Five SFTs cases occurred in the cervical spine, two in the thoracic spine, and one in the lumbosacral spine. Five cases showed a dumbbell-shaped or lobulated soft tissue mass that grew across the intervertebral foramen, two cases showed an expansive intraosseous mass formation in the vertebral body and/or posterior element, and one case showed a long-spindle shaped intraspinal canal mass growing along the spinal canal. Seven caused local invasion and destruction of the vertebral body and posterior element. Benign SFTs displayed a good prognosis, whereas malignant SFTs were prone to recurrence and metastasis (3/4). CONCLUSION: Spinal SFTs are difficult to characterise with imaging and required pathological and immunohistochemical investigation. Prolonged follow-up is recommended once a diagnosis of spinal SFTs has been established because of the unclear biology. KEY POINTS: ⢠Spinal solitary fibrous tumours are extremely rare. ⢠SFTs should be showed the differential of masses developing though the foramen. ⢠Combing imaging with pathology and immunochemistry assesses the diagnosis and establish nature.
Assuntos
Tumores Fibrosos Solitários/diagnóstico por imagem , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Adulto , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica , Metástase Neoplásica , Recidiva Local de Neoplasia , Estudos Retrospectivos , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/radioterapia , Tumores Fibrosos Solitários/cirurgia , Neoplasias da Coluna Vertebral/patologia , Neoplasias da Coluna Vertebral/radioterapia , Neoplasias da Coluna Vertebral/cirurgia , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: To report on the outcome of patients diagnosed with central nervous system haemangiopericytoma (HPC) or solitary fibrous tumours (SFT) and identify factors that may influence recurrence and survival. MATERIAL AND METHODS: Between January 1977 and December 2016, a retrospective search identified 22 HPCs/SFTs. The patients underwent a total of 40 surgical resections and 63.6% received radiotherapy. Median follow-up was 7.8 years. RESULTS: Six patients (27.3%) were re-operated for tumour recurrence. At the end of the study, 15 patients (68.2%) had no residual tumour on the last imaging. Surgical recurrence-free survival at 5 years was 77.4%, [95% CI: 60.1-99.8]. None of the investigated variables was associated with recurrence. At the end of the study, 5 patients were deceased (22.7%) and only 10 patients (45.5%) had no residual tumour on the last imaging and were alive. Overall survival at 5 years was 95%, [95% CI: 85.9-100]. None of the investigated variables was associated with overall survival. Patients who received radiotherapy demonstrated neither a reduced risk of surgical recurrence (P=0.378) nor a longer overall survival (P=0.405). CONCLUSION: SFTs/HPCs are associated with a significant risk of recurrence that may reduce the survival. Even if we could not demonstrate their benefit in this limited series, we believe that tailored maximal tumour resection on initial surgery is beneficial and that adjuvant RT is useful for tumours displaying grade II or III, even in case of complete removal.
Assuntos
Neoplasias do Sistema Nervoso Central/terapia , Hemangiopericitoma/terapia , Neoplasias Meníngeas/terapia , Tumores Fibrosos Solitários/terapia , Adulto , Idoso , Neoplasias do Sistema Nervoso Central/mortalidade , Neoplasias do Sistema Nervoso Central/radioterapia , Neoplasias do Sistema Nervoso Central/cirurgia , Feminino , Hemangiopericitoma/mortalidade , Hemangiopericitoma/radioterapia , Hemangiopericitoma/cirurgia , Humanos , Masculino , Neoplasias Meníngeas/mortalidade , Neoplasias Meníngeas/radioterapia , Neoplasias Meníngeas/cirurgia , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Prognóstico , Estudos Retrospectivos , Tumores Fibrosos Solitários/mortalidade , Tumores Fibrosos Solitários/radioterapia , Tumores Fibrosos Solitários/cirurgia , Análise de Sobrevida , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVES: This study aims to evaluate the clinical characteristics and treatment results of patients with a diagnosis of solitary fibrous tumor localized in extremity. PATIENTS AND METHODS: Clinical findings, treatment methods, clinical outcomes and treatment failures of seven patients (3 males, 4 females; mean age 49.7±20.7 years; range 22 to 79 years) diagnosed as solitary fibrous tumor localized in extremity between January 2005 and December 2016 were evaluated retrospectively. RESULTS: Most frequent localization was the thigh (42.8%, n=3). Mean tumor size was 10.8±2.4 cm (range 8-15 cm). All patients applied with painless mass and all had primary tumor. All patients had localized disease at the time of diagnosis. Postoperative mean follow-up duration was 44.4 months (range 13-121 months). Marginal resection was performed in two patients at low risk group while wide resection was performed in other patients at moderate and high risk groups. Patients who underwent marginal resection were performed adjuvant radiotherapy. No recurrence or metastasis was detected in any of the patients during follow-up. CONCLUSION: In the treatment of solitary fibrous tumors localized in extremity, marginal resection together with radiotherapy may be an alternative treatment method for patients in low risk group according to risk stratification model, for whom wide resection is not possible. Wide resection is recommended in moderate and high risk groups to decrease local recurrence and metastasis risks.
Assuntos
Dissecação , Extremidades , Recidiva Local de Neoplasia , Radioterapia Adjuvante/métodos , Neoplasias de Tecidos Moles , Tumores Fibrosos Solitários , Adulto , Idoso , Dissecação/efeitos adversos , Dissecação/métodos , Extremidades/diagnóstico por imagem , Extremidades/patologia , Extremidades/cirurgia , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/etiologia , Recidiva Local de Neoplasia/prevenção & controle , Planejamento de Assistência ao Paciente , Risco Ajustado , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/radioterapia , Neoplasias de Tecidos Moles/cirurgia , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/radioterapia , Tumores Fibrosos Solitários/cirurgia , Tomografia Computadorizada por Raios X/métodos , Resultado do TratamentoRESUMO
Solitary fibrous tumours (SFTs) are relatively rare tumours that were originally thought to arise from the pleura but have thereafter been demonstrated as occurring anywhere in the body. These tumours are generally considered benign but have frequently been noted for recurrence and local invasion. Furthermore, their indolence is controversial due to increasing evidence implicating the existence of a spectrum that includes hemangiopericytoma (HPC). Stereotactic radiosurgery (SRS) has been well characterised in the treatment of benign, malignant and vascular conditions, and it appears to be a reasonable option as adjuvant or recurrent treatment for intracranial SFTs. We present in this case the first complete description of an SFT of the orbit treated by SRS as well as a systematic review of available English literature for intracranial SFTs treated by SRS. We report effective local tumour control in our case and conclude that SRS is a reasonable treatment option for recurrent SFT.
Assuntos
Neoplasias Oculares/diagnóstico , Olho/patologia , Radiocirurgia , Tumores Fibrosos Solitários/diagnóstico , Adulto , Olho/diagnóstico por imagem , Neoplasias Oculares/patologia , Neoplasias Oculares/radioterapia , Humanos , Imageamento por Ressonância Magnética , Masculino , Recidiva , Sarcoma , Tumores Fibrosos Solitários/radioterapia , Acuidade VisualRESUMO
Solitary fibrous tumor of the pelvic is an uncommon neoplasm with nonspecific symptoms. Reports of malignant transformation are especially rare. We report a case of solitary fibrous tumor in pelvic. A unique feature of our case compared with previously reported is that this patient relapsed with malignant transformation and had significant response to radiotherapy. The patient was initially treated with surgery, followed by postoperative dimensional conformal intensity modulated radiation therapy (dynamic MLC VRIAN 23EX Linac, inversely optimized by the Eclipse system) to provide a radical cure for residual tumor.In this case, there were no signs of recurrence after six and a half years of further follow-up, indicating that postoperation radiotherapy may be an effective treatment for SFT with malignant transformation in pelvic.
Assuntos
Transformação Celular Neoplásica/patologia , Recidiva Local de Neoplasia/patologia , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/radioterapia , Biópsia por Agulha , Seguimentos , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/radioterapia , Pelve , Radioterapia Adjuvante , Radioterapia de Intensidade Modulada/métodos , Doenças Raras , Medição de Risco , Tumores Fibrosos Solitários/cirurgia , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Ultrassonografia DopplerRESUMO
OBJECTIVE: Solitary fibrous tumor is a rare tumor occurring in almost every anatomic location of human body; however, reports of malignant solitary fibrous tumor in the nasal and paranasal area are especially rare. In this report, we describe a case of non-recurrent malignant solitary fibrous tumor of the nasal and paranasal area. METHODS: The patient was initially treated with nasal and paranasal tumor cytoreductive surgery, followed by post-operative three-dimensional conformal intensity modulated radiation therapy (dynamic MLC Varian 600CD Linac, inversely optimized by the Eclipse system) and stereotactic body radiation therapy to provide a radical cure for residual tumor. RESULTS: The tumor of the nasal and paranasal area was effectively treated and the integrity of the right eye kept. There were no signs of recurrence after four and a half years of further follow-up. CONCLUSIONS: This is the first attempt to successfully combine cytoreductive surgery with intensity modulated radiation therapy and stereotactic body radiation therapy together to treat solitary fibrous tumor of the nasal and paranasal area, which may provide a potential strategy for the treatment of similar cases.
Assuntos
Neoplasias Nasais/radioterapia , Neoplasias Nasais/cirurgia , Radiocirurgia , Radioterapia de Intensidade Modulada , Tumores Fibrosos Solitários/radioterapia , Tumores Fibrosos Solitários/cirurgia , Adolescente , China , Intervalo Livre de Doença , Feminino , Humanos , Imuno-Histoquímica , Imageamento por Ressonância Magnética , Cavidade Nasal , Neoplasias Nasais/diagnóstico por imagem , Neoplasias Nasais/patologia , Período Pós-Operatório , Radioterapia Adjuvante , Tumores Fibrosos Solitários/diagnóstico por imagem , Tumores Fibrosos Solitários/patologia , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
The decision to give neoadjuvant chemotherapy in patients with localized high-risk soft tissue sarcoma is often based on tumor grade evaluated from biopsies, but biopsies can have the inherent issue of sampling bias. Incorporation of SUVmax and heterogeneity assessed by F-FDG PET/CT could be other crucial components in the effort to tailor treatment to an individual patient, providing valuable parameters to guide the selection of the most appropriate management schedule for an individual. We present 1 representative case describing how FDG PET/CT can assist in clinical management decisions for treatment of malignant solitary fibrous tumor of the pelvis.