RESUMO
INTRODUCTION: Grade 3 solitary fibrous tumor, previously known as anaplastic hemangiopericytoma, is a rare and highly malignant intracranial tumor with a limited understanding of its natural history and treatment outcomes. METHODS: We conducted a retrospective analysis using the Surveillance, Epidemiology, and End Results (SEER) database spanning 2000-2019 to evaluate the clinical characteristics and treatment modalities that influence overall survival in this tumor entity. A cohort of 249 patients with intracranial grade 3 solitary fibrous tumors was identified. Univariate and multivariable Cox proportional hazard models were employed to determine significant prognostic factors for overall survival. Kaplan-Meier models were used to visualize survival curves, and a nomogram was constructed to predict survival probabilities at 6- and 12-month following diagnosis. RESULTS: Our findings indicated that patient age (<65 years), localized or regional disease burden, surgical resection, and radiation therapy were significant predictors of better overall survival. Combination therapies showed improved survival, with surgery and radiation therapy having the most significant impact. However, chemotherapy alone or in combination did not demonstrate a significant survival benefit, likely due to the limited sample size. The nomogram provided personalized prognostic predictions based on significant clinical factors. CONCLUSIONS: These data emphasize the importance of surgical resection and radiation therapy in the management of grade 3 solitary fibrous tumors, supporting the use of combination therapies to improve overall survival in this rare and aggressive intracranial neoplasm.
Assuntos
Hemangiopericitoma , Programa de SEER , Tumores Fibrosos Solitários , Humanos , Estudos Retrospectivos , Masculino , Feminino , Tumores Fibrosos Solitários/terapia , Tumores Fibrosos Solitários/mortalidade , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/epidemiologia , Pessoa de Meia-Idade , Hemangiopericitoma/terapia , Hemangiopericitoma/mortalidade , Hemangiopericitoma/patologia , Hemangiopericitoma/epidemiologia , Idoso , Prognóstico , Adulto , Neoplasias Encefálicas/terapia , Neoplasias Encefálicas/mortalidade , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/epidemiologia , Nomogramas , Gradação de Tumores , Estimativa de Kaplan-Meier , Adulto Jovem , Idoso de 80 Anos ou mais , Terapia CombinadaRESUMO
PURPOSE: Solitary fibrous tumors (SFT) are a rare entity of in majority benign neoplasms. Nevertheless, up to 20% of cases show a malignant tendency with local infiltration or metastasis. Commonly arising in the thoracic cavity, only few cases of SFT of the mesorectal tissue have been reported in the literature. Complete surgical resection, classically by posterior approach, is the treatment of choice. The purpose of this review is to demonstrate the safety and suitability of transanal minimally invasive surgery (TAMIS) as a surgical approach for the resection of benign pararectal solid tumors. METHODS: We report the case of a 52-year-old man who was diagnosed incidentally with SFT of the distal mesorectum. Resection by TAMIS was performed. Based on this case, we describe the steps and potential benefits of this procedure and provide a comprehensive review of the literature. RESULTS: Histopathology confirms the completely resected SFT. After uneventful postoperative course and discharge on day four, follow-up was recommended by a multidisciplinary board by clinical examination and MRI, which showed a well-healed scar and no recurrence up to 3 years after resection. CONCLUSION: SFT of the mesorectum is a very rare entity. To our knowledge, this is the first report on a TAMIS resection for SFT, demonstrated as a safe approach for complete resection of benign pararectal solid tumors.
Assuntos
Tumores Fibrosos Solitários , Humanos , Masculino , Pessoa de Meia-Idade , Tumores Fibrosos Solitários/cirurgia , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/diagnóstico por imagem , Neoplasias Retais/cirurgia , Neoplasias Retais/patologia , Neoplasias Retais/diagnóstico por imagem , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Canal Anal/cirurgia , Canal Anal/patologia , Cirurgia Endoscópica Transanal/métodos , Imageamento por Ressonância MagnéticaRESUMO
BACKGROUND: Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal tumor that mostly involves the pleura and infrequently involves extra-pleural sites. De novo SFT of the kidney is uncommon, and malignant SFT is extremely rare. CASE PRESENTATION: We report a case of a 51-year-old man with a large malignant SFT in the left kidney. Pathological examination confirmed the diagnosis of SFT based on typical morphology, nuclear STAT6 expression, and NAB2-STAT6 gene fusion. The malignant subtype was determined by a large tumor size (≥ 15 cm) and high mitotic counts (8/10 high-power fields). KRAS mutation was identified by DNA sequencing. Insulin-like growth factor 2 (IGF2) was diffusely and strongly expressed in tumor cells, however, hypoglycemia was not observed. Hyperglycemia and high adrenocorticotropic hormone (ACTH) concentration were observed one month after surgery. Hormone measurements revealed normal blood cortisol and aldosterone levels, and increased urinary free cortisol level. A pituitary microadenoma was identified using brain magnetic resonance imaging, which may be responsible for the promotion of hyperglycemia. CONCLUSIONS: We report a case of renal malignant SFT with a KRAS mutation, which was previously unreported in SFT and may be associated with its malignant behavior. Additionally, we emphasize that malignant SFT commonly causes severe hypoglycemia due to the production of IGF2. However, this effect may be masked by the presence of other lesions that promote hyperglycemia. Therefore, when encountering a malignant SFT with diffuse and strong IGF2 expression and without hypoglycemia, other lesions promoting hyperglycemia need to be ruled out.
Assuntos
Hipoglicemia , Fator de Crescimento Insulin-Like II , Neoplasias Renais , Proteínas Proto-Oncogênicas p21(ras) , Tumores Fibrosos Solitários , Humanos , Fator de Crescimento Insulin-Like II/metabolismo , Fator de Crescimento Insulin-Like II/genética , Masculino , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/cirurgia , Tumores Fibrosos Solitários/metabolismo , Tumores Fibrosos Solitários/genética , Tumores Fibrosos Solitários/diagnóstico , Pessoa de Meia-Idade , Neoplasias Renais/patologia , Neoplasias Renais/cirurgia , Neoplasias Renais/metabolismo , Neoplasias Renais/genética , Neoplasias Renais/diagnóstico , Hipoglicemia/metabolismo , Hipoglicemia/etiologia , Hipoglicemia/patologia , Hipoglicemia/diagnóstico , Proteínas Proto-Oncogênicas p21(ras)/genética , Prognóstico , MutaçãoRESUMO
PURPOSE: Meningeal solitary fibrous tumour (SFT) and haemangiopericytoma (HPC) are uncommon tumours that have been merged into a single entity in the last 2021 WHO Classification of Tumors of the Central Nervous System. To describe the epidemiology of SFT/HPC operated in France and, to assess their incidence. METHODS: We processed the French Brain Tumour Database (FBTDB) to conduct a nationwide population-based study of all histopathologically confirmed SFT/HPC between 2006 and 2015. RESULTS: Our study included 399 SFT/HPC patients, operated in France between 2006 and 2015, in one of the 46 participating neurosurgical centres. The incidence reached 0.062, 95%CI[0.056-0.068] for 100,000 person-years. SFT accounted for 35.8% and, HPC for 64.2%. The ratio of SFT/HPC over meningioma operated during the same period was 0.013. SFT/HPC are about equally distributed in women and men (55.9% vs. 44.1%). For the whole population, mean age at surgery was 53.9 (SD ± 15.8) years. The incidence of SFT/HPC surgery increases with the age and, is maximal for the 50-55 years category. Benign SFT/HPC accounted for 65.16%, SFT/HPC of uncertain behaviour for 11.53% and malignant ones for 23.31%. The number of resection progresses as the histopathological behaviour became more aggressive. 6.7% of the patients with a benign SFT/HPC had a second surgery vs.16.6% in case of uncertain behaviour and, 28.4% for malignant SFT/HPC patients. CONCLUSION: Meningeal SFT and HPC are rare CNS mesenchymal tumours which both share common epidemiological characteristics, asserting their merging under a common entity. SFT/HPC incidence is less that one case for 1 billion per year and, for around 100 meningiomas-like tumours removed, one SFT/HPC may be diagnosed. SFT/HPC are equally distributed in women and men and, are mainly diagnosed around 50-55 years. The more aggressive the tumour, the higher the probability of recurrence.
Assuntos
Hemangiopericitoma , Neoplasias Meníngeas , Tumores Fibrosos Solitários , Humanos , França/epidemiologia , Hemangiopericitoma/epidemiologia , Hemangiopericitoma/patologia , Hemangiopericitoma/cirurgia , Hemangiopericitoma/diagnóstico , Feminino , Masculino , Pessoa de Meia-Idade , Neoplasias Meníngeas/epidemiologia , Neoplasias Meníngeas/patologia , Neoplasias Meníngeas/cirurgia , Neoplasias Meníngeas/diagnóstico , Tumores Fibrosos Solitários/epidemiologia , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/cirurgia , Tumores Fibrosos Solitários/diagnóstico , Adulto , Idoso , Incidência , Adulto Jovem , Meningioma/epidemiologia , Meningioma/patologia , Meningioma/cirurgia , Meningioma/diagnóstico , Adolescente , Idoso de 80 Anos ou mais , CriançaRESUMO
Synovial sarcoma (SS) and solitary fibrous tumor (SFT) are entities with considerable morphological and immunohistochemical similarities that sometimes show a non-confirmatory profile (TLE1 negative, CD34 and focal or negative STAT6 and lack of specific fusion IHC markers), in which the utility ultrastructure is unknown. A cross-sectional, retrospective, analytical, nonexperimental study was carried out by the Department of Pathology of the National Cancer Institute of Mexico (INCan) e from January 1, 2009 to December 31, 2018. With 17 SFT cases with diffuse or focal CD34 and STAT6 positivity and 18 cases of SS with positive FISH molecular test t(X:18) breakapart were studied by electron microscopy of fresh glutaraldehyde fixed or paraffin-embedded tissue. The ultrastructural findings with a significant difference present in the SS were tandem tight junctions, desmosomes and abundance of dilated rough endoplasmic reticulum (RER) cisternae (p < 0.001, 0.003, and 0.001, respectively); while in the (SFT) the presence of abundant glycogen, basal lamina, long and slender cytoplasmic processes, pinocytic vesicles, hemidesmosomes, and/or dense plaques, collagen skein, and microvilli-like buds (p = 0.028, 0.005, and <0.001 for the last five). We then infer that the five distinctive markers of the SFT are the collagen skeins intermingled with cellular processes in a shape of "squid can," and the pinocytic vesicles as they were not observed in any case of SS. Conversely, tandem junctions were not found in any SFT case. Although the presence of multivesicular buds in the SFT was not significant, it had not been previously described.
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Sarcoma Sinovial , Tumores Fibrosos Solitários , Humanos , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/ultraestrutura , Sarcoma Sinovial/ultraestrutura , Sarcoma Sinovial/patologia , Adulto , Masculino , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , México , Estudos Transversais , Biomarcadores Tumorais , Idoso , Adulto Jovem , Diagnóstico DiferencialRESUMO
PURPOSE: To delineate specific imaging characteristics of solitary fibrous tumors, schwannomas, cavernous venous malformations, and well-circumscribed orbital lymphoma. METHODS: Patients undergoing excisional biopsy of solitary fibrous tumor, schwannomas, cavernous venous malformations, or well-circumscribed orbital lymphoma with preoperative MRIs available for review were identified at 3 academic centers in the United States and Australia. An exploratory statistical analysis was performed to identify important radiologic features, which were subsequently included in a random forest model. Histopathologic correlates were evaluated in representative cases. RESULTS: A total of 91 cases were included with a mean age of 52.9 ± 17.2 years. Nearly all solitary fibrous tumors were located in the anterior or mid orbit (87.5%) and they more commonly demonstrated intralesional heterogeneity on T2-weighted imaging (45.5%) ( p < 0.01). Compared with the other tumors, schwannomas tended to be intraconal (66.7%) and were often in the mid or posterior orbit (83.4%) ( p < 0.01). Cavernous venous malformations characteristically demonstrated progressive contrast enhancement (93.9%; p < 0.01). Most lesions in all 4 groups were hypointense on T1-weighted imaging (80%-100%; p = 0.14) and only well-circumscribed orbital lymphoma tended to also be hypointense on T2 (81.8%) ( p < 0.01). Finally, cases of lymphoma had significantly lower apparent diffusion coefficient ratios (0.9 ± 0.2) ( p < 0.001), while the other 3 groups were not significantly different from one another (cavernous venous malformations: 1.8 ± 0.4; schwannomas: 1.8 ± 0.5; and solitary fibrous tumor: 1.6 ± 0.6) ( p = 0.739). CONCLUSIONS: Key features that aid in the differentiation of these 4 tumors from one another include T2 intensity and homogeneity, early contrast-enhancement pattern, and ADC ratio.
Assuntos
Imageamento por Ressonância Magnética , Neoplasias Orbitárias , Tumores Fibrosos Solitários , Humanos , Neoplasias Orbitárias/patologia , Neoplasias Orbitárias/diagnóstico , Neoplasias Orbitárias/diagnóstico por imagem , Pessoa de Meia-Idade , Feminino , Masculino , Adulto , Idoso , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/diagnóstico , Tumores Fibrosos Solitários/diagnóstico por imagem , Estudos Retrospectivos , Linfoma/diagnóstico , Linfoma/patologia , Linfoma/diagnóstico por imagem , Neurilemoma/patologia , Neurilemoma/diagnóstico , Neurilemoma/diagnóstico por imagem , Idoso de 80 Anos ou mais , Adulto Jovem , Adolescente , BiópsiaRESUMO
Solitary fibrous tumour is a relatively rare soft tissue fibroblastic tumour, accounting for approximately 2% of soft tissue tumours. It has been described primarily as a tumour of the pleural cavity; however, up to 70% of cases occur elsewhere, in any anatomical location, which can make diagnosis difficult. If this is the diagnosis being considered, the STAT6 antibody is currently available with high sensitivity and specificity. In this paper we describe the case of a 72-year-old female patient, followed up and treated by an outpatient endocrinologist for a multinodular euthyroid goitre for several years. Due to complete nodular remodelling of the left lobe of the thyroid gland and sonographic findings of several small nodules in the right lobe of the thyroid gland, total thyroidectomy was recommended to the patient. The operation was performed at the ENT department in Jindrichuv Hradec Hospital. Material from the operation was subsequently sent for histopathological examination. Several hyperplastic colloid nodules and a small oncocytic adenoma were detected microscopically in the right lobe of the thyroid gland. In the left lobe, an imprecisely delineated, greyish-white lesion measuring 2 x 1.8 x 1.5 cm was observed on the section. Microscopically, the tumour consisted of spindle-shaped cells in a focally hyalinised stroma. In the immunohistochemical examination, tumour cells reacted positively with the CD34 antibody, and negatively with antibodies against thyroglobulin, cytokeratins (CK AE1/AE3) and S100 protein. Further immunohistochemical examinations (Bcl2, CD99, STAT6) with positive results were supplemented upon consultation at a higher facility. Based on morphology and the results of the immunohistochemical examinations, the tumour was diagnosed as a solitary fibrous tumour of the thyroid gland. This is a relatively unusual finding in this location; according to literature, only a few dozen cases have been described.
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Tumores Fibrosos Solitários , Neoplasias da Glândula Tireoide , Humanos , Feminino , Idoso , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/diagnóstico , Neoplasias da Glândula Tireoide/patologia , Neoplasias da Glândula Tireoide/diagnóstico , Neoplasias da Glândula Tireoide/cirurgiaAssuntos
Tumores Fibrosos Solitários , Neoplasias da Bexiga Urinária , Humanos , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/metabolismo , Tumores Fibrosos Solitários/genética , Tumores Fibrosos Solitários/cirurgia , Neoplasias da Bexiga Urinária/patologia , Neoplasias da Bexiga Urinária/metabolismo , Neoplasias da Bexiga Urinária/genética , Neoplasias da Bexiga Urinária/cirurgia , Antígenos CD34/metabolismo , Diagnóstico Diferencial , Fator de Transcrição STAT6/metabolismo , Fator de Transcrição STAT6/genética , Proteínas Repressoras/genética , Proteínas Repressoras/metabolismo , MasculinoRESUMO
Solitary fibrous tumor (SFT) is a relatively rare mesenchymal fibroblastic tumor occurring most commonly in adults with no gender predilection. Although the pathological diagnosis of SFT is usually straightforward, some difficulties may occasionally arise mainly due to the wide morphological spectrum exhibited by this tumor. In the present paper we aimed to evaluate the unusual clinicopathological features in a series of 31 SFTs arising from parenchymal organs, superficial soft tissues and deep soft tissues. Our results emphasize that SFTs may occur anywhere, including unusual sites such as periosteum of the thoracic spine, mesorectal tissue, hepatic hilum, paravescial space, kidney and breast. Moreover, a wide morphological spectrum was observed in tumors included in our series. The most striking morphological features observed included: extensive lipomatous component, myxoid stromal changes, epithelioid cell component, metaplastic mature bone, neurofibroma-like, myxofibrosarcoma-like and pseudoalveolar-like areas. Additionally, multinucleated giant cells and sarcomatous dedifferentiation were also identified. Our paper emphasizes that SFT may occur in unusual anatomical locations and exhibits a wide morphological spectrum. Pathologists must be aware of these features to avoid confusion with other benign and malignant neoplasms that may show overlapping morphological features.
Assuntos
Hemangiopericitoma , Sarcoma , Febre Grave com Síndrome de Trombocitopenia , Tumores Fibrosos Solitários , Humanos , Adulto , Biomarcadores Tumorais , Tumores Fibrosos Solitários/patologia , Hemangiopericitoma/patologiaRESUMO
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
Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms constituting less than 2% of all soft tissue tumors. They typically originate in the thoracic cavity, mainly in the pleura, but can also occur in other various sites such as lung parenchyma, pericardium, and bronchus. In this study, a 49-year-old non-smoking female with a history of allergies presented to our pulmonary clinic with a chronic cough. An explorative bronchoscopy revealed an intrabronchial mass in the left superior bronchi, and a 68 Ga-DOTATOC positron emission computed tomography suggested a carcinoid tumor. Subsequent pulmonary segmentectomy unveiled a well-circumscribed polypoid lesion diagnosed as a low-grade bronchus SFT through histopathological and immunohistochemical assessments. The patient was asymptomatic after surgical excision and showed no other lesion during the 6-month follow-up. The endobronchial location of SFT is uncommon, with only a few reported cases in the literature, underscoring the necessity of considering various differential diagnoses, including carcinoid, mucoepidermoid carcinoma, endobronchial pleomorphic adenoma, hamartoma, leiomyoma, and metastasis, depending on location and imaging features. This report underscores the importance of careful histological and immunohistochemical evaluation in understanding and appropriately stratifying the risk associated with polypoid lesions.
Assuntos
Neoplasias de Tecido Conjuntivo e de Tecidos Moles , Neoplasias de Tecidos Moles , Tumores Fibrosos Solitários , Humanos , Feminino , Pessoa de Meia-Idade , Tumores Fibrosos Solitários/diagnóstico , Tumores Fibrosos Solitários/patologia , Diagnóstico Diferencial , Neoplasias de Tecidos Moles/diagnóstico , Brônquios/patologia , Neoplasias de Tecido Conjuntivo e de Tecidos Moles/diagnósticoRESUMO
BACKGROUND: As the form of World Health Organization Central Nervous System (WHO CNS) tumor classifications is updated, there is a lack of research on outcomes for intracranial combined solitary-fibrous tumor and hemangiopericytoma (SFT/HPC). This study aimed to explore conditional survival (CS) pattern and develop a survival prediction tool for intracranial SFT/HPC patients. METHODS: Data of intracranial SFT/HPC patients was gathered from the Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute. The patients were split into training and validation groups at a 7:3 ratio for our analysis. CS is defined as the likelihood of surviving for a specified period of time (y years), given that the patient has survived x years after initial diagnosis. Then, we used this definition of CS to analyze the intracranial SFT/HPC patients. The least absolute shrinkage and selection operator (LASSO) regression and best subset regression (BSR) were employed to identify predictive factors. The Multivariate Cox regression analysis was applied to establish a novel CS-based nomogram, and a risk stratification system was developed using this model. RESULTS: From the SEER database, 401 patients who were diagnosed with intracranial SFT/HPC between 2000 and 2019 were identified. Among them, 280 were included in the training group and 121 were included in the internal validation group for analysis. Our study revealed that in intracranial SFT/HPC, 5-year survival rates saw significant improvement ranging from 78% at initial diagnosis to rates of 83%, 87%, 90%, and 95% with each successive year after surviving for 1-4 years. The LASSO regression and BSR identified patient age, tumor behavior, surgery and radiotherapy as predictors of CS-based nomogram development. A risk stratification system was also successfully constructed to facilitate the identification of high-risk patients. CONCLUSION: The CS pattern of intracranial SFT/HPC patients was outlined, revealing a notable improvement in 5-year survival rates after an added period of survival. Our newly-established CS-based nomogram and risk stratification system can provide a real-time dynamic survival estimation and facilitate the identification of high-risk patients, allowing clinicians to better guide treatment decision for these patients.
Assuntos
Hemangiopericitoma , Tumores Fibrosos Solitários , Humanos , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/patologia , Hemangiopericitoma/cirurgia , Tumores Fibrosos Solitários/diagnóstico , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/cirurgia , Análise de Sobrevida , Prognóstico , NomogramasRESUMO
Fat-forming solitary fibrous tumor is a rare and specific subtype of solitary fibrous tumor. In this case, a mass of 8.3 cm in diameter was found in a 59-year-old male patient's right retroperitoneum, as revealed by abdominal contrast-enhanced computed tomography (CT) images. The tumor exhibited a well-circumscribed nature and histological features characterized by a combination of hemangiopericytomatous vasculature and mature adipose tissue, comprising around 70% of the total tumor composition. Immunohistochemistry staining revealed diffuse positive expression of STAT6 and CD34 in the tumor cells. Based on these findings, the final diagnosis was determined to be a fat-forming solitary fibrous tumor located in the retroperitoneum. It is important to consider other potential differential diagnoses, including angiomyolipoma, dedifferentiated liposarcoma, spindle cell lipoma, and atypical lipomatous tumor/well-differentiated liposarcoma.
Assuntos
Lipoma , Lipossarcoma , Tumores Fibrosos Solitários , Humanos , Masculino , Pessoa de Meia-Idade , Tecido Adiposo/metabolismo , Lipoma/diagnóstico , Lipoma/genética , Lipossarcoma/genética , Lipossarcoma/patologia , Tumores Fibrosos Solitários/diagnóstico , Tumores Fibrosos Solitários/genética , Tumores Fibrosos Solitários/patologia , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Solitary fibrous tumors can manifest at various anatomical sites, predominantly occurring at extrapleural sites with a peak incidence between 40 and 70 years. SFT necessitates long-term follow-up owing to its tumor characteristics. However, comprehensive reports covering the period from initial diagnosis to the patient's demise are lacking. Herein, we present a case of a malignant SFT of the buttocks that was treated at our hospital from the time of initial diagnosis to the end of life, with a literature review. METHODS: A 54-year-old woman had a T1 low-to-isobaric and T2 isobaric-to-hyperintense mass in the psoas muscle on magnetic resonance imaging, diagnosed as an SFT. Wide excision was performed, followed by postoperative radiotherapy and chemotherapy. Multiple lung metastases were treated, while bone metastases appeared in the left femur. Multiple spinal metastases developed, causing respiratory distress due to pleural effusion. Best support care was initiated; however, a thrombus appeared in the inferior vena cava. Despite anticoagulant therapy, the patient died 11 years and 6 months after the initial surgery. Herein, marginal resection resulted in a relatively short operative time and average blood loss. The radiotherapy dose was 66 Gy; no complications occurred, and local recurrence was prevented. Tumor arthroplasty was performed to stabilize the affected limbs, and the patient required careful follow-up. RESULTS: Despite the poor prognosis, the patient survived >11 years after surgery and had a favorable outcome. CONCLUSION: Long-term monitoring for potential complications remains necessary.
Assuntos
Hemangiopericitoma , Tumores Fibrosos Solitários , Humanos , Feminino , Pessoa de Meia-Idade , Nádegas/patologia , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/cirurgia , Tumores Fibrosos Solitários/terapia , Hemangiopericitoma/cirurgia , Hemangiopericitoma/patologia , Hemangiopericitoma/terapia , Evolução Fatal , Imageamento por Ressonância Magnética , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/secundário , Neoplasias Pulmonares/terapia , Neoplasias Pulmonares/cirurgiaRESUMO
PURPOSE: Solitary fibrous tumor (SFT) of the orbit is a rare tumor that was first described in 1994. We aimed to investigate its imaging characteristics that may facilitate the differential diagnosis between SFT and other types of orbital tumors. MATERIAL AND METHODS: Magnetic resonance imaging (MRI) data of patients with immunohistochemically confirmed orbital SFT from 2002 to 2022 at a tertiary care center were retrospectively analyzed. Tumor location, size, morphological characteristics, and contrast enhancement features were evaluated. RESULTS: Of the 18 eligible patients 10 were female (56%) with a mean age of 52 years. Most of the SFTs were oval-shaped (67%) with a sharp margin (83%). The most frequent locations were the laterocranial quadrant (44%), the extraconal space (67%) and the dorsal half of the orbit (67%). A flow void phenomenon was observed in nearly all cases (94%). On the T1-weighted imaging, tumor signal intensity (SI) was significantly lower than that of the retrobulbar fat and appeared predominantly equivalent (82%) to the temporomesial brain cortex, while on T2-weighted imaging its SI remained equivalent (50%) or slightly hyperintense to that of brain cortex. More than half of the lesions showed a homogeneous contrast enhancement pattern with a median SI increase of 2.2-fold compared to baseline precontrast imaging. CONCLUSION: The SFT represents a rare orbital tumor with several characteristic imaging features. It was mostly oval-shaped with a sharp margin and frequently localized in the extraconal space and dorsal half of the orbit. Flow voids indicating hypervascularization were the most common findings.
Assuntos
Imageamento por Ressonância Magnética , Neoplasias Orbitárias , Tumores Fibrosos Solitários , Humanos , Feminino , Pessoa de Meia-Idade , Imageamento por Ressonância Magnética/métodos , Masculino , Tumores Fibrosos Solitários/diagnóstico por imagem , Tumores Fibrosos Solitários/patologia , Neoplasias Orbitárias/diagnóstico por imagem , Estudos Retrospectivos , Adulto , Idoso , Diagnóstico Diferencial , Meios de ContrasteRESUMO
Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm most often arising from the pleura and rarely in extra-pleural locations, including the gastrointestinal tract. We describe two cases of a SFT presenting as submucosal colonic lesion and review the literature on this lesion. One submucosal lesion was localized in the cecum and was 10 mm in size. The second lesion presented as a 17 mm submucosal rectal lesion. Both lesions presented as well-circumscribed submucosal lesions arranged in short fascicles, blending with abundant collagenous stroma. In both cases, the spindle cells were positive for CD34, STAT6 and CD99, and molecular studies showed NAB2:STAT6 fusion supporting the diagnosis of SFT. Both patients are alive and well 10 and 5 years post-excision, respectively. In conclusion, SFT can occur in the colon as a submucosal lesion and should be included in the differential diagnosis of colonic mesenchymal lesions.
Assuntos
Neoplasias do Colo , Tumores Fibrosos Solitários , Humanos , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/cirurgia , Tumores Fibrosos Solitários/diagnóstico , Tumores Fibrosos Solitários/diagnóstico por imagem , Masculino , Neoplasias do Colo/patologia , Neoplasias do Colo/cirurgia , Neoplasias do Colo/diagnóstico , Pessoa de Meia-Idade , Feminino , Fator de Transcrição STAT6/análise , Fator de Transcrição STAT6/metabolismo , Idoso , Antígenos CD34/análise , Antígenos CD34/metabolismo , Colonoscopia , Mucosa Intestinal/patologia , Mucosa Intestinal/diagnóstico por imagem , Diagnóstico Diferencial , Proteínas RepressorasRESUMO
This case report describes a male in his late 40s with a 4 cm pelvic mass compressing the left distal ureter, resulting in left hydroureteronephrosis. Biopsy of the mass was suggestive of a solitary fibrous tumour. The patient underwent a robotic-assisted laparoscopic excision of the left pelvic mass. Intraoperatively, the mass was found to be densely adhered to the ureter, necessitating a left distal ureterectomy and ureteric reimplantation. Subsequent histopathological analysis revealed the mass was a solitary fibrous tumour with no evidence of malignancy.
Assuntos
Laparoscopia , Procedimentos Cirúrgicos Robóticos , Tumores Fibrosos Solitários , Ureter , Obstrução Ureteral , Humanos , Masculino , Procedimentos Cirúrgicos Robóticos/métodos , Tumores Fibrosos Solitários/cirurgia , Tumores Fibrosos Solitários/complicações , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/diagnóstico por imagem , Laparoscopia/métodos , Ureter/cirurgia , Obstrução Ureteral/cirurgia , Obstrução Ureteral/etiologia , Adulto , Neoplasias Pélvicas/cirurgia , Neoplasias Pélvicas/complicações , Neoplasias Pélvicas/patologia , Hidronefrose/etiologia , Hidronefrose/cirurgiaRESUMO
A male in his 20s, a tobacco chewer, presented to the outpatient department with a history of painless, slowly progressive swelling in the floor of the mouth. After a thorough history and clinical examination, MRI was done and the tumour was completely excised. Histopathological examination revealed the mass to be a solitary fibrous tumour, confirmed with immunohistochemical markers. On subsequent follow-ups, the patient was found to be asymptomatic with no clinical signs of recurrence.
Assuntos
Imageamento por Ressonância Magnética , Soalho Bucal , Neoplasias Bucais , Tumores Fibrosos Solitários , Humanos , Masculino , Tumores Fibrosos Solitários/cirurgia , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/diagnóstico por imagem , Tumores Fibrosos Solitários/diagnóstico , Neoplasias Bucais/patologia , Neoplasias Bucais/cirurgia , Neoplasias Bucais/diagnóstico , Neoplasias Bucais/diagnóstico por imagem , Soalho Bucal/patologia , Adulto , Adulto JovemRESUMO
PURPOSE: Fibroblast activation protein (FAP) is a serine integral membrane protease, the expression of which has been confirmed in various cancer types. Solitary fibrous tumors of the pleura (SFTP) are rare mesenchymal fibroblastic neoplasms. We present a case of 18F-labeled FAP inhibitor ([18F]FAPI-74) PET imaging and its correlation with histological FAP expression and review an SFTP series at our institution in relation to the extent of FAP expression. METHODS: This retrospective study included 13 patients who underwent surgery between March 2011 and December 2022 at our institute. One of the patients also underwent [18F]FAPI-74 PET imaging. We semi-quantitatively evaluated FAP expression in SFTPs using immunohistochemical staining and H-scores. RESULTS: Nine of the 13 patients were male, with a median age of 64 years (range, 28-79 years). The median tumor size was 6.6â¯cm (1.1, 16â¯cm). In the pathological findings, expression levels of Ki67 were 1-5% in 12 of 13 cases. Furthermore, FAP expression was observed in all patients, and the median H-score was 160 (range, 10-280). The H-score of FAP expression in two of the 13 patients was low (10 in both), and that in two of the 13 patients was high (240 and 280). The SUVmax value of [18F]FAPI-74 PET was 3.57 in a patient in whom the H-score of FAP expression was 180. CONCLUSIONS: SFTPs expressed FAP to varying degrees in different patients and the [18F]FAPI-74 PET results in one patient reflected FAP expression in the tumor tissue.