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1.
Medicine (Baltimore) ; 99(13): e19651, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32221091

RESUMO

RATIONALE: Intracranial solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) are rare spindle cell tumors of mesenchymal origin that include benign and malignant neoplasms. PATIENT CONCERNS: We present a 66-year-old male with a 5-year history of headache and dizziness, with left progressive sensorineural hearing loss over 1 month. DIAGNOSES: WHO grade II SFT/HPC originating from the internal auditory canal in the left cerebellopontine angle. INTERVENTIONS: surgical resection. OUTCOMES: No local recurrence or metastases were observed in the follow-up 3 months after the surgery. LESSONS: Intracranial SFTs/HPCs are rare mesenchymal neoplasms that are challenging to manage. If the imaging characteristics of tumor are not typical, clinicians should depend on tissue biopsy and immunohistochemistry to make a definitive diagnosis.


Assuntos
Neoplasias Cerebelares/patologia , Ângulo Cerebelopontino/patologia , Hemangiopericitoma/patologia , Tumores Fibrosos Solitários/patologia , Idoso , Neoplasias Cerebelares/diagnóstico , Neoplasias Cerebelares/cirurgia , Diagnóstico Diferencial , Tontura/etiologia , Cefaleia/etiologia , Perda Auditiva/etiologia , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/cirurgia , Humanos , Masculino , Gradação de Tumores , Neuroma Acústico/diagnóstico , Tumores Fibrosos Solitários/diagnóstico , Tumores Fibrosos Solitários/cirurgia
2.
Neurocirugía (Soc. Luso-Esp. Neurocir.) ; 31(1): 14-23, ene.-feb. 2020. tab, ilus, graf
Artigo em Inglês | IBECS | ID: ibc-190368

RESUMO

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


El hemangiopericitoma y el tumor fibroso solitario son enfermedades del sistema nervioso central (SNC) con una incidencia baja. Estos tumores también pueden tener algunas características como una tendencia a recurrir localmente y hacer metástasis. La OMS, en su última clasificación de tumores del SNC, integró ambos tumores en una nueva entidad: tumor fibroso solitario/hemangiopericitoma (SFT/HPC), pero uno de los problemas actuales pendientes es demostrar la efectividad de la cirugía y la radioterapia (RT) complementaria, y estandarizar el uso de la misma. Revisamos todos los datos clínicos de nuestro hospital en un período de 10 años, y encontramos 10 pacientes con SFT/HPC. Se evaluaron los tamaños de los tumores, las ubicaciones y las características radiológicas. Los registros quirúrgicos de cada paciente se evaluaron para determinar las características macroscópicas y el alcance de la resección del tumor (EOR). También comparamos los resultados en los pacientes que utilizan o no RT como tratamiento complementario. El seguimiento medio fue de 74,8 meses, con un rango de 12 y 210 meses. La población incluía 3 varones (30%) y 7 mujeres (70%). La localización más frecuente fue la convexidad cerebral (30%). Las distribuciones de las localizaciones restantes del tumor fueron columna cervical y lumbar, sacro, intraventricular, torcular, ala esfenoidal e intraorbitario. La radioterapia postoperatoria (EBRT) se administró en 7 pacientes (70%), los criterios para tratarlos fueron una resección parcial o un grado histológico de la OMS II y III. Dos pacientes desarrollaron recidivas locales a los 12 y 19 meses después de la cirugía inicial. Un paciente se sometió a 2 cirugías, y el otro a 4 cirugías. La tasa media de supervivencia libre de recidiva (RFS) fue de 15,5 meses. Se encontraron metástasis a distancia en 4 pacientes durante el período de seguimiento. Tres de los 10 pacientes fallecieron durante el período de seguimiento. La tasa de supervivencia general a 5 años fue del 66%, y la supervivencia media global fue de 76 meses. Una resección segura y completa en la primera cirugía es el factor pronóstico más importante. Consideramos que la RT complementaria puede ser útil, incluso en casos de resección completa en SFT/HPC de bajo grado. Sería interesante definir previamente la diferenciación entre el SFT/HPC y los meningiomas para las estrategias de tratamiento


Assuntos
Humanos , Masculino , Feminino , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Hemangiopericitoma/radioterapia , Hemangiopericitoma/cirurgia , Neoplasias do Sistema Nervoso Central/radioterapia , Neoplasias do Sistema Nervoso Central/cirurgia , Taxa de Sobrevida , Estudos Retrospectivos , Hemangiopericitoma/diagnóstico por imagem , Eletrofisiologia/métodos , Hemangiopericitoma/patologia , Diagnóstico Diferencial , Período Pós-Operatório
3.
Medicine (Baltimore) ; 98(47): e17888, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31764783

RESUMO

RATIONALE: Hemangiopericytoma (HPC) is a rare pediatric neoplasm with a high risk of bleeding, aggressive growth and high early relapse rates. Surgical excision remains the mainstream treatment, while the functions of chemotherapy and radiotherapy remain controversial. In particular, an infantile giant extracranial HPC located in the forehead has never been reported. PATIENT CONCERNS: A 3-day-old girl was delivered normally with a giant tumor localized mainly in the right frontal region. The surface of the mass was filled with vascularity. DIAGNOSIS: According to the results of imaging and pathological examinations, the diagnosis was HPC grade II. INTERVENTIONS: Gross total resection of the tumor and the invading partial frontal bone followed by skin scalp reconstruction was carried out without any blood transfusion. OUTCOMES: No recurrence was identified during 5 years of follow-up. And better outcomes can be achieved without adjuvant therapy. LESSONS: Multimodality imaging and a collaborative multidisciplinary approach are indispensable for the successful surgical management of infantile HPC, especially for giant tumors and their potential risk of life-threatening bleeding. Gross total resection is the optimal option for infantile HPC, and even without adjuvant therapy, it achieves better outcomes.


Assuntos
Neoplasias de Cabeça e Pescoço , Hemangiopericitoma , Neoplasias de Tecidos Moles , Pré-Escolar , Feminino , Seguimentos , Testa , Neoplasias de Cabeça e Pescoço/patologia , Neoplasias de Cabeça e Pescoço/cirurgia , Hemangiopericitoma/patologia , Hemangiopericitoma/cirurgia , Humanos , Recém-Nascido , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia , Fatores de Tempo , Carga Tumoral
4.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao ; 41(4): 512-516, 2019 Aug 30.
Artigo em Chinês | MEDLINE | ID: mdl-31484614

RESUMO

To study the clinicopathologic characteristics,immunohistochemical features,differential diagnosis,and prognosis of solitary fibrous tumours(SFT)/hemangiopericytomas(HPC)in the maters(meninx). Methods A series of 7 cases previously diagnosed as SFT/HPC at the Department of Pathology,Peking Union Medical College Hospital,during the period from 2008 to 2018 were analyzed for clinical data,histopathology,and immunohistochemical findings.The patients were followed up and the relevant literatures were reviewed. Results These seven patients included two males and 5 females aged 22 to 77 years(mean,49 years).Headache was the most common symptom.The magnetic resonance imaging of SFT/HPC showed irregularly contoured masses and dural tail sign was observed at the periphery of the lesion in 4 cases.The major axis of the tumor ranged from 1.8 cm to 10 cm(mean,4 cm).The tumors were located in the mater in 6 cases and in the spinal meninx in 1 case.The tumors were surgically removed in all cases.Under light microscope,the tumors were formed by long round,oval or spindle cells,with rich branching vascular pattern and varying quantity of collagenous fibers bands in both sparse areas and dense areas.According the WHO classification,2 cases were in WHO grade Ⅰ,2 cases in WHO grade Ⅱ,and 3 cases in WHO grade Ⅲ.Immunohistochemistry of the paraffin-embedded tissues in all cases showed positive immunoreativity for CD34 and vimentin in all seven cases,along with positive signal transducer and activator of transcription 6 in 4 cases,negative epithelial membrane antigen and S-100 in 7 cases,and negative progestational hormone and somatostatin receptor 2 in 6 cases.The Ki-67 index ranged from 1% to 15%.Five patients with follow-up data(including 1 current case)were alive,while 2 patients were lost to follow-up. Conclusions The SFT/HPC are rare in the maters(meninx)and is clinically difficult to be differentiated from other meningioma.The combination of CD34 and signal transducer and activator of transcription 6 helps to diagnose this disease.


Assuntos
Hemangiopericitoma/diagnóstico , Meninges/patologia , Tumores Fibrosos Solitários/diagnóstico , Adulto , Idoso , Antígenos CD34/metabolismo , Diagnóstico Diferencial , Feminino , Hemangiopericitoma/patologia , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Prognóstico , Fator de Transcrição STAT6/metabolismo , Tumores Fibrosos Solitários/patologia , Adulto Jovem
5.
Ann Ital Chir ; 82019 Jun 13.
Artigo em Inglês | MEDLINE | ID: mdl-31366738

RESUMO

BACKGROUND: Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm affecting soft tissues with a not well defined biological behavior. SFT occurs mostly in the pleura and the thorax, while extra-thoracic localization is uncommon and abdominal localization is very rare. Histologically, SFT is a well defined mass with splindle-cell proliferation in collagenous matrix with staghorn vascular network and CD34 reactive. CASE REPORT: A 64 years-old man with a history of recurrent gastric cancer previously treated with total gastrectomy, was admitted with contrast enhanced CT-scan diagnosis of a well demarcated oval mass of 4.8 cm with microcysts, vascularized in the arterial phase and with wash out in the tardive phase, located in the peritoneal side of right rectus abdominis muscle, suspected for metastatic gastric tumor. The patient underwent minilaparotomy and en-bloc excision of the lesion. Histologically the tumor was characterized by a hemangiopericitoma like growth pattern and the immunostaining was positive to CD34, CD99, BCL-2 and Vimentin. The definitive diagnosis was SFT with a proliferation index (Ki-67/MIB-1) <3%. In our case, chemotherapy was not indicated. At the 6-month follow-up, the patient is in good clinical conditions with no recurrence or metastasis. CONCLUSIONS: We reported a rare case of primitive SFT located in peritoneal side of the of right rectus abdominis muscle treated surgically, in a patient previously affected by gastric adenocarcinoma. In this case, SFT showed a benign behaviour during a short term follow-up. Dimensional pattern, histopathological features and curative surgery remain the most important indicators of clinical outcome. KEY WORDS: Abdominal wall, Hemangiopericitoma, SFT, Solitary fibrous tumorSpindle cell.


Assuntos
Neoplasias Abdominais/cirurgia , Segunda Neoplasia Primária/cirurgia , Reto do Abdome/patologia , Tumores Fibrosos Solitários/cirurgia , Neoplasias Abdominais/química , Neoplasias Abdominais/diagnóstico , Neoplasias Abdominais/patologia , Adenocarcinoma/diagnóstico , Adenocarcinoma/secundário , Biomarcadores Tumorais , Diagnóstico Diferencial , Gastrectomia , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Segunda Neoplasia Primária/diagnóstico , Segunda Neoplasia Primária/patologia , Tumores Fibrosos Solitários/química , Tumores Fibrosos Solitários/diagnóstico , Tumores Fibrosos Solitários/patologia , Neoplasias Gástricas/cirurgia
6.
World Neurosurg ; 130: e383-e392, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31233926

RESUMO

OBJECTIVE: Solitary fibrous tumor/hemangiopericytoma (SFT/HPC) and meningioma exhibit similar radiographic features; however, they differ in their prognoses. Preoperative differentiation between them is important for determining the treatment and follow-up plan. The aim of this study was to determine the factors that can be used to differentiate SFT/HPC from meningioma and World Health Organization (WHO) grade I from grade II meningioma. METHODS: The analysis included 84 cases: 5 of SFT/HPC, 72 of WHO grade I meningioma, and 7 of WHO grade II meningioma. Clinical characteristics and conventional magnetic resonance imaging, perfusion magnetic resonance imaging, and magnetic resonance spectroscopy (MRS) LCModel parameters were evaluated via multivariate logistic regression analysis to identify the factors that distinguish SFT/HPC from meningioma. RESULTS: Patients with SFT/HPC were mostly men and were younger than those with meningioma. The percentage of T2-weighted images in meningioma was greater than that in SFT/HPC. There were significant differences between SFT/HPC and meningioma in levels of glutamate, phosphocholine, myo-inositol, or glycerophosphocholine + phosphocholine derived from long echo-time MRS, and myo-inositol derived from short echo-time MRS. Stepwise logistic regression analysis revealed that the age of <45 years and myo-inositol in short echo-time MRS of ≧6.347 were associated with a diagnosis of SFT/HPC with high sensitivity and specificity. However, no factors were found that differentiated WHO grade I meningioma from WHO grade II meningioma. CONCLUSIONS: Age and myo-inositol level calculated from MRS are useful factors for distinguishing SFT/HPC from meningioma preoperatively.


Assuntos
Hemangiopericitoma/diagnóstico , Neoplasias Meníngeas/diagnóstico , Meningioma/diagnóstico , Tumores Fibrosos Solitários/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Diagnóstico Diferencial , Feminino , Hemangiopericitoma/diagnóstico por imagem , Hemangiopericitoma/patologia , Humanos , Angiografia por Ressonância Magnética , Imagem por Ressonância Magnética , Espectroscopia de Ressonância Magnética , Masculino , Neoplasias Meníngeas/diagnóstico por imagem , Neoplasias Meníngeas/patologia , Meningioma/diagnóstico por imagem , Meningioma/patologia , Pessoa de Meia-Idade , Gradação de Tumores , Tumores Fibrosos Solitários/diagnóstico por imagem , Tumores Fibrosos Solitários/patologia , Adulto Jovem
7.
J Neuroradiol ; 46(5): 281-287, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31226327

RESUMO

PURPOSE: To assess whether a machine-learning model based on texture analysis (TA) could yield a more accurate diagnosis in differentiating malignant haemangiopericytoma (HPC) from angiomatous meningioma (AM). MATERIALS AND METHODS: Sixty-seven pathologically confirmed cases, including 24 malignant HPCs and 43 AMs between May 2013 and September 2017 were retrospectively reviewed. In each case, 498 radiomic features, including 12 clinical features and 486 texture features from MRI sequences (T2-FLAIR, DWI and enhanced T1WI), were extracted. Three neuroradiologists independently made diagnoses by vision. Four Support Vector Machine (SVM) classifiers were built, one based on clinical features and three based on texture features from three MRI sequences after feature selection. The diagnostic abilities of these classifiers and three neuroradiologists were evaluated by receiver operating characteristic (ROC) analysis. RESULTS: Malignant HPCs were found to have larger sizes, slighter degrees of peritumoural oedema compared with AMs (P<0.05), and more serpentine-like vessels. The AUC of the enhanced T1WI-based classifier was 0.90, significantly higher than that of T2-FLAIR-based or DWI-based classifiers (0.77 and 0.73). The AUC of the SVM classifier based on clinical features was 0.66, slightly but not significantly lower than the performances of 3 neuroradiologists (AUC=0.69, 0.70 and 0.73). CONCLUSION: Machine-learning models based on clinical features alone could not provide a better diagnostic performance than that of radiologists. The SVM classifier built by texture features extracted from enhanced T1WI is a promising tool to differentiate malignant HPC from AM before surgery.


Assuntos
Diagnóstico por Computador/métodos , Hemangiopericitoma/diagnóstico por imagem , Aprendizado de Máquina , Imagem por Ressonância Magnética/métodos , Neoplasias Meníngeas/diagnóstico por imagem , Meningioma/diagnóstico por imagem , Neurorradiografia/métodos , Adulto , Feminino , Hemangiopericitoma/patologia , Humanos , Masculino , Neoplasias Meníngeas/patologia , Meningioma/patologia , Pessoa de Meia-Idade , Cuidados Pré-Operatórios , Estudos Retrospectivos , Sensibilidade e Especificidade
8.
J Neurooncol ; 143(3): 457-464, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31054100

RESUMO

BACKGROUND: Prior studies have highlighted infratentorial tumor location as a prognostic factor for solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) of the central nervous system (CNS), and spinal location is considered a positive prognostic factor for other tumors of the CNS. While SFT/HPC of the CNS is known to frequently arise from the spinal meninges, there are no case series that report outcomes for spinally located CNS tumors, and their prognosis in relation to intracranial and other CNS-located tumors is unknown. OBJECTIVE: To investigate outcomes for patients with SFT/HPC of the spinal meninges. METHODS: The Surveillance, Epidemiology, and End-Results Program was used to identify patients with SFT/HPC within the CNS from 1993-2015. We retrospectively analyzed the relationship between tumor location (spinal vs. Brain and other CNS) and survival. RESULTS: We identified 551 cases of CNS SFT/HPC, 64 (11.6%) of which were primary tumors of the spinal meninges. Spinal tumors were more likely than brain and other CNS tumors to be SFT vs. HPC (37.5 vs. 12%, p < 0.001), benign (42.2 vs. 20.3%, p < 0.001), and less than 5 cm (53.1 vs. 35.7%, p < 0.001). The 10-year survival rates for spinal and brain/other CNS tumors were 85 and 58%, respectively. Median survival time was significantly longer for spinal tumors (median survival not reached vs. 138 months, p = 0.03, HR = 0.41 [95% CI 0.18-0.94]). On multivariable analysis, spinal tumor location was associated with improved survival over tumors located in the brain and other CNS (HR = 0.36 [95% CI 0.15-0.89], p = 0.03). CONCLUSION: Spinal tumor location is associated with improved survival in patients with SFT/HPC of the CNS. Larger institutional studies are necessary to characterize the relationship between tumor location and other relevant factors such as presentation and amenability to gross-total resection and adjuvant radiotherapy. Future studies exploring optimal management of spinally located tumors are also needed.


Assuntos
Hemangiopericitoma/mortalidade , Tumores Fibrosos Solitários/mortalidade , Neoplasias da Coluna Vertebral/mortalidade , Neoplasias da Coluna Vertebral/patologia , Feminino , Seguimentos , Hemangiopericitoma/patologia , Hemangiopericitoma/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Programa de SEER , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/cirurgia , Neoplasias da Coluna Vertebral/cirurgia , Taxa de Sobrevida
9.
J Am Anim Hosp Assoc ; 55(4): 194-200, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31099606

RESUMO

This retrospective study evaluated the postoperative outcome and clinical parameters associated with prognosis in 167 client-owned dogs with 167 hemangiopericytomas. Parameters that were reviewed for an association with long-term outcome included signalment, clinical history, results of staging tests, tumor and surgical variables, and administration of adjunctive therapy. History of previous surgery, type of surgery performed, status of surgical margins, tumor location, and whether adjunctive therapy was performed were associated with tumor recurrence. The distal forelimb was the most common location reported overall (46/167 [27.5%]). Dogs with tumors located at the tail/perineum had the fastest recurrence rate, with a median disease-free interval of ∼16 mo (505 days). Tumor grade alone was not associated with recurrence (P = .069), but when analyzing tumor grade and margin, low-grade tumors with dirty margins had a significantly shorter time to recurrence than low-grade tumors with either clean or narrow margins. Tumor location should be considered when assessing the treatment plan and follow-up recommendations for any hemangiopericytomas. Aggressive initial surgical treatment is recommended when possible to reduce the chance of local tumor recurrence.


Assuntos
Doenças do Cão/cirurgia , Hemangiopericitoma/veterinária , Neoplasias Esplênicas/veterinária , Animais , Terapia Combinada , Intervalo Livre de Doença , Doenças do Cão/patologia , Cães , Hemangiopericitoma/patologia , Hemangiopericitoma/cirurgia , Estudos Retrospectivos , Esplenectomia/veterinária , Neoplasias Esplênicas/patologia , Neoplasias Esplênicas/cirurgia , Resultado do Tratamento
10.
Radiographics ; 39(2): 468-490, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30844353

RESUMO

While intradural extramedullary spinal disease varies widely, identification of tumors in this location and their radiologic manifestations greatly facilitates narrowing of the diagnostic considerations. Meningioma and schwannoma are the two most common intradural extramedullary tumors, and both are associated with neurofibromatosis. Meningiomas are most common in the thoracic spine and show a strong female predilection and a clinical manifestation related to compression of the spinal cord or nerve roots. Schwannomas typically are associated with radicular pain and other sensory symptoms. Melanotic schwannoma frequently shows T1 hyperintensity at MRI related to the presence of paramagnetic free radicals in melanin. Neurofibroma, known for its T2 hyperintensity, frequently involves the cervical spine, where it may make surgical resection challenging. Less commonly, malignant peripheral nerve sheath tumor commonly mimics the imaging appearance of a schwannoma but has decidedly more aggressive biologic behavior. In the cauda equina, myxopapillary ependymoma and paraganglioma are believed to arise from the filum terminale and have characteristic imaging manifestations based on their underlying pathologic features. Recent identification of a common genetic marker has led to reclassification of what had previously been regarded as separate tumors and are now known as solitary fibrous tumor/hemangiopericytoma. In the proper clinical setting, the presence of nodular intradural enhancement strongly suggests the presence of leptomeningeal metastatic disease, even when results of cerebrospinal fluid analysis are negative. This article highlights the characteristic neuroimaging manifestations of these neoplasms, with emphasis on radiologic-pathologic correlation. See Illumination by Frazier .


Assuntos
Neuroimagem/métodos , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Meios de Contraste , Ependimoma/diagnóstico por imagem , Ependimoma/patologia , Feminino , Gadolínio , Hemangiopericitoma/diagnóstico por imagem , Hemangiopericitoma/patologia , Humanos , Imagem por Ressonância Magnética/métodos , Masculino , Neoplasias Meníngeas/diagnóstico por imagem , Neoplasias Meníngeas/patologia , Neoplasias Meníngeas/secundário , Meningioma/diagnóstico por imagem , Meningioma/patologia , Neurilemoma/diagnóstico por imagem , Neurilemoma/patologia , Neurofibroma/diagnóstico por imagem , Neurofibroma/patologia , Paraganglioma/diagnóstico por imagem , Paraganglioma/patologia , Neoplasias da Coluna Vertebral/patologia
11.
Neuropathology ; 39(2): 141-146, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30864263

RESUMO

This report describes the clinicopathological findings of a solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) of the central nervous system in a 59-year-old man with space-occupying lesions on both the left anterior basicranial and on the top tail of falx cerebri. The tumor showed small quantities of solid papillary areas and a prominent papillary structure, where atypical cells were compactly arranged along the fibrovascular core. The tumor cells of both components showed nuclear relocalization of the signal transducer and activator of transcription 6 protein, with very high specificity and sensitivity for the diagnosis of SFT/HPC. In the literature, only three cases of SFTs with a papillary pattern have been reported, but this case showed a complete papillary pattern. "Papillary" SFT/HPC is a rare morphological variant of SFTs/HPCs, and its differential diagnosis among intracranial tumors is an important factor which clinicians should bear in mind during diagnosis.


Assuntos
Neoplasias Encefálicas/patologia , Hemangiopericitoma/patologia , Tumores Fibrosos Solitários/patologia , Neoplasias Encefálicas/metabolismo , Hemangiopericitoma/metabolismo , Humanos , Masculino , Pessoa de Meia-Idade , Fator de Transcrição STAT6/metabolismo , Tumores Fibrosos Solitários/metabolismo
13.
Eur J Radiol ; 112: 186-191, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30777209

RESUMO

PURPOSE: To assess the role of histogram analysis of apparent diffusion coefficient (ADC) maps based on whole-tumor in differentiating intracranial solitary fibrous tumor/hemangiopericytoma (SFT/HPC) from angiomatous meningioma (AM). MATERIALS AND METHODS: Pathologically confirmed intracranial SFT/HPC (n = 15) and AM (n = 20) were retrospectively collected and their clinical and conventional MRI features were analyzed. Diffusion-weighted (DW) images (b = 0 and 1000 s/mm2) were processed with the mono-exponential model. Regions of interest covering the whole tumor were drawn on all slices of the ADC maps to obtain histogram parameters, including mean ADC (ADCmean), median ADC (ADCmedian), maximum ADC (ADCmax), minimum ADC (ADCmin), skewness and kurtosis, as well as the 5th, 10th, 25th, 75th, 90th and 95th percentile ADC (ADC5, ADC10, ADC25, ADC75, ADC90 and ADC95). Differences of histogram parameters between SFT/HPC and AM were compared using Mann-Whitney U test. Receiver operating characteristic (ROC) curve was used to determine the diagnostic performance. RESULTS: The ADCmin (P = 0.001) and ADC5 (P = 0.045) were significantly lower in SFT/HPCs than in AMs, while no significant difference was found in sex, age, conventional MRI features or any other histogram parameters between the two entities (P = 0.051-1.000). ADCmin showed the best diagnostic performance (area under curve [AUC], 0.86; sensitivity, 81.3%; specificity, 83.3%) in differentiating SFT/HPC from AM with optimal cutoff value being 569.00 × 10-6 mm2/s, followed by ADC5 (AUC, 0.72; sensitivity, 68.8%; specificity, 75%) with optimal cutoff value being 781.97 × 10-6 mm2/s. CONCLUSION: SFT/HPC and AM share similar conventional MR appearances. Whole-tumor histogram analysis of ADC maps may be a useful tool for differential diagnosis, with ADCmin and ADC5 being potential parameters.


Assuntos
Hemangiopericitoma/patologia , Neoplasias Infratentoriais/patologia , Neoplasias Meníngeas/patologia , Meningioma/patologia , Neoplasias da Base do Crânio/patologia , Tumores Fibrosos Solitários/patologia , Adulto , Idoso , Diagnóstico Diferencial , Imagem de Difusão por Ressonância Magnética/métodos , Métodos Epidemiológicos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
14.
Clin Nucl Med ; 44(4): e305-e307, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30762828

RESUMO

We report the case of a 50-year-old man, with previous history of grade 3 intracranial hemangiopericytoma with initial complete surgical resection, addressed for local recurrence. Surgical revision performed 18 months after initial surgery allowed only partial resection, leaving residual disease along the optic nerve. Complementary radiotherapy with proton was decided. F-FDG PET/CT and F-choline PET/CT were both performed for treatment planning. F-FDG PET showed no uptake of the residual tumor, whereas F-choline depicted highly metabolic residual disease uptake with excellent delineation of local recurrence. F-choline PET/CT appears as a useful PET tracer for hemagiopericytoma imaging.


Assuntos
Neoplasias Encefálicas/diagnóstico por imagem , Colina/análogos & derivados , Hemangiopericitoma/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Neoplasias Encefálicas/patologia , Hemangiopericitoma/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasia Residual , Recidiva
15.
Pathol Res Pract ; 215(5): 983-987, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30739805

RESUMO

Sinonasal glomangiopericytoma (SNGP) is a neoplasm arising in the nasal cavity and paranasal sinuses that shows perivascular myoid differentiation. The diagnosis of SNGP may be diagnostically challenging due to a large number of potential mimics. In the present study, we sought to characterize the histological and molecular features of six cases of SNGP found in prior surgical pathology records over a 15-year period. The average age at diagnosis was 48.5 years (range: 31-78 years), and the male-to-female ratio was 1:1. Imaging studies in all six cases demonstrated avidly enhancing, lobulated soft tissue masses in the nasal cavity, extending into the sinuses and nasopharynx. Histologically, the tumors were unencapsulated and composed of a proliferation of closely packed, bland, and uniform spindle cells growing deep to an intact surface respiratory epithelium. The cells were separated by a distinctive vascular network ranging from capillaries to large vascular spaces. All cases demonstrated strong positivity for smooth muscle actin, cyclin D1, CD99, and ß-catenin (100%). Targeted sequencing revealed recurrent CTNNB1 missense mutations in all cases tested. Additionally, TLE1 was positive in all cases which has not been previously reported. No tested cases harbored SS18 translocations. We found that while no single marker resolves immunohistochemical overlap between SNGP and its histologic mimics, an extended immunohistochemical panel that includes ß-catenin, cyclin D1, STAT6, smooth muscle actin, pan-cytokeratin cocktails, S100, and SOX10 helps to support the diagnosis of SNGP in diagnostically challenging cases without the need for molecular studies.


Assuntos
Hemangiopericitoma/patologia , Cavidade Nasal/patologia , Neoplasias Nasais/patologia , Neoplasias dos Seios Paranasais/patologia , Adulto , Idoso , Biomarcadores Tumorais/análise , Feminino , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/genética , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Nasais/diagnóstico , Neoplasias Nasais/genética , Neoplasias dos Seios Paranasais/diagnóstico , Neoplasias dos Seios Paranasais/genética , Proteínas Repressoras/genética , beta Catenina/genética
16.
World Neurosurg ; 126: e48-e56, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30716501

RESUMO

BACKGROUND: Intracranial solitary fibrous tumors (ISFTs) are rare mesenchymal neoplasms originating in the meninges and characterized by very different biologic and clinical behaviors. Benign histotypes, such as hemangiopericytomas, are now considered a cellular phenotypic variant of this heterogeneous group of rare spindle-cell tumors. Owing to their rarity and resemblance to other, more common brain tumors, ISFTs are often poorly recognized and remain a diagnostic challenge. METHODS: We describe a surgical series of 29 patients treated for ISFTs confirmed histologically and through immunohistochemistry. We attempt to provide a focus on the natural history of these pathologies and the need for tailored management. RESULTS: This was a retrospective consecutive series of 29 patients with either solitary fibrous tumor (n = 14) or hemangiopericytoma (n = 15) over a 10-year period. Mean follow-up time was 37.71 months. Recurrence rate was 42.9% for solitary fibrous tumors versus 26.7% for hemangiopericytomas. STAT6 expression was 66.7% in hemangiopericytomas versus 42.9% in SFTs. CONCLUSIONS: Histopathology and immunohistochemical staining (characterized by positive expression of mainly STAT6 but also CD34, Bcl-2 protein, and vimentin) are key in diagnosis and management of ISFTs. Although ISFTs are still considered benign lesions with very rare aggressive evolution, their clinical behavior is largely unpredictable. This study highlights the importance of relying on immunohistochemistry for a thorough definition of the management strategy.


Assuntos
Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/patologia , Tumores Fibrosos Solitários/diagnóstico , Tumores Fibrosos Solitários/patologia , Adulto , Neoplasias Encefálicas/cirurgia , Feminino , Seguimentos , Hemangiopericitoma/patologia , Hemangiopericitoma/cirurgia , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Procedimentos Neurocirúrgicos , Estudos Retrospectivos , Fator de Transcrição STAT6/biossíntese , Fator de Transcrição STAT6/genética , Tumores Fibrosos Solitários/cirurgia , Análise de Sobrevida , Resultado do Tratamento
17.
Zhonghua Bing Li Xue Za Zhi ; 48(1): 31-36, 2019 Jan 08.
Artigo em Chinês | MEDLINE | ID: mdl-30641643

RESUMO

Objective: To investigate the clinical manifestations, imaging features, clinicopathologic features, and differential diagnosis of solitary fibrous tumors/anginoblastomas (SFT/HPCs) originating in the central nervous system. Methods: Sixty cases of SFT/HPCs originating in the central nervous system were collected at Nanjing Jinling Hospital, from January 1, 2008 to December 31, 2016. The clinical data, imaging data, histomorphologic changes and immunohistochemical finding were analyzed in the sixty cases. Results: The 60 cases included 26 males and 34 females, aged 14 to 85 (median 49) years. The main clinical manifestations were headache, dizziness with nausea and vomiting. Radiologically, the tumors were large, enhancing, solid and cystic masses attached to the dura. Histopathologically, the neoplasms were composed of spindle cells with oval nuclei, inconspicuous nucleoli and moderate amount of eosinophilic cytoplasm arranged in fascicles with areas of hyalinized stroma, myxoid changes and a staghorn vascular pattern. Immunohistochemically, tumor cells of all cases were positive for vimentin (100.0%, 60/60), STAT6 (98.3%, 59/60), CD34 (61.7%, 37/60), and the tumor cells were typically positive for CD99, bcl-2, EMA and SSTR2 as well.Negative for S-100 protein, SOX10, E-cadherin, GFAP. Ki-67 index ranged from 1% to 50%. Forty cases were followed up for 6 to 82 months with average of 40 months, 30 patients were alive and 10 patients died. Conclusions: Central nervous system SFT/HPCs can be aggressive and relapses may occur several years after diagnosis. STAT6 is highly sensitive and specific for the diagnosis. Complete tumor resection is optional treatment followed by radiotherapy and chemotherapy. There is a correlation between the prognosis and the location of the disease, the histological grade, Ki-67 index, and fusion gene variants.


Assuntos
Neoplasias do Sistema Nervoso Central , Hemangiopericitoma , Tumores Fibrosos Solitários , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais , Neoplasias do Sistema Nervoso Central/química , Neoplasias do Sistema Nervoso Central/complicações , Neoplasias do Sistema Nervoso Central/diagnóstico por imagem , Neoplasias do Sistema Nervoso Central/patologia , Diagnóstico Diferencial , Feminino , Hemangiopericitoma/química , Hemangiopericitoma/complicações , Hemangiopericitoma/diagnóstico por imagem , Hemangiopericitoma/patologia , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Prognóstico , Tumores Fibrosos Solitários/química , Tumores Fibrosos Solitários/complicações , Tumores Fibrosos Solitários/diagnóstico por imagem , Tumores Fibrosos Solitários/patologia , Adulto Jovem
18.
Technol Health Care ; 27(2): 137-147, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30664513

RESUMO

BACKGROUND: It is difficult to distinguish solitary of fibrous tumor/hemangiopericytoma (SFT/HPC) from atypical meningioma (AM) by conventional imaging.As far as we know,diffusion weighting imaging may identify them effectively. OBJECTIVE: The purpose of this study was to determine the role of apparent diffusion coefficient (ADC) values to distinguish and predict prognosis of solitary of fibrous tumor/hemangiopericytoma (SFT/HPC) (WHOII) and atypical meningioma (AM). METHODS: Preoperative diffusion-weighted imaging (DWI) of 30 cases with histopathologic and immunhistochemical testified SFT/HPC WHOII (n= 11) and AM (n= 19) were performed retrospectively. The ADC values of lesion, peritumoral edema, normal white matter and lesion NADC ratio (lesion ADC values/ADC values of normal white matter (NWN ADC)) were compared. The immunhistochemical markers (Ki-67, CD34, Vim, EMA, GFAP, S-100, PR, CD56) were compared. The correlation between the ADC values and Ki-67 index was evaluated. RESULTS: The mean lesion ADC values of SFT/HPC (1.15 ± 0.04 × 10-3 mm2/s) was significantly higher than that of AM (0.80 ± 0.04 × 10-3 mm2/s) (t= 23.824, p< 0.05). The mean NADC ratio was lower for AM (1.03 ± 0.06) compared with SFT/HPC (1.51 ± 0.05) (t= 23.105, p< 0.05). The mean edema ADC for SFT/HPC (1.47 ± 0.06 × 10-3 mm2/s) was lower compared with AM (1.68 ± 0.05 × 10-3 mm2/s) (t=-9.926, p< 0.05 ). There was no statistical difference between the two groups of NWM ADC (t=-1.475, p> 0.05) . The mean Ki-67 of SFT/HPC (7.18 ± 2.60%) was lower than the mean Ki-67 of AM (13.58 ± 4.50%) (t=-4.934, p< 0.05). The CD34 showed statistically differences between two groups (X2= 13.659, p< 0.05). The EMA also showed statistically differences between two groups (X2= 4.474, p< 0.05). Vim,GFAP, S-100, PR, CD56 showed no statistical difference in the two group (p> 0.05). The pearson analysis indicated that there was a negative correlation between lesion ADC and Ki-67 in SFT/HPC group (r=-0.770, p< 0.05) and AM group (r=-0.727, p< 0.05). There was also a negative correlation between lesion NADC ratio and Ki-67 in SFT/HPC group (r=-0.673, p< 0.05) and AM group (r=-0.707, p< 0.05). There was a positive correlation between edema ADC and Ki-67 in SFT/HPC group (r= 0.819, p< 0.05) and AM group (r= 0.942, p< 0.05). Furthermore,there was no correlation between NWM A DC and Ki-67 in SFT/HPC group (r=-0.403, p> 0.05) and AM group (r= 0.202, p> 0.05). CONCLUSIONS: The lesion ADC, lesion NADC ratio and edema ADC can distinguish the SFT/HPC WHO II from AM and be helpful to predict prognosis of the two tumors before operation. Further more, histopathologic and immunhistochemical can make a definite diagnosis of the two tumors.


Assuntos
Hemangiopericitoma/patologia , Meningioma/patologia , Tumores Fibrosos Solitários/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Diagnóstico Diferencial , Imagem de Difusão por Ressonância Magnética , Feminino , Hemangiopericitoma/diagnóstico por imagem , Humanos , Masculino , Meningioma/diagnóstico por imagem , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Tumores Fibrosos Solitários/diagnóstico por imagem
19.
Brain Tumor Pathol ; 36(1): 20-26, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30603845

RESUMO

Solitary fibrous tumors/ hemangiopericytomas (SFT/HPC) are mesenchymal tumors that share a common genetic aberration and very rarely undergo dedifferentiation. We report a unique case of an intracranial anaplastic SFT/HPC with de-novo dedifferentiation, which pursued a rapidly fatal clinical course in a 41-year-old lady. The dedifferentiated component comprised a focal area of glandular formation with epithelial immunophenotype acquisition. The distinct biphasic pattern of the tumor imparted great diagnostic challenges to the pathologists. An increased awareness of SFT/HPCs with a diverse morphologic spectrum or even a biphasic histologic pattern is essential in working up such cases. We first attempted gamma knife radiosurgery in treating a recurrent dedifferentiated SFT/HPC; unfortunately it was to no avail. Although it is now known that SFT/HPC is characterized by NAB2-STAT6 gene fusion, the unavailability of targeted therapy against this molecular signature still results in a treatment dilemma.


Assuntos
Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/terapia , Hemangiopericitoma/patologia , Hemangiopericitoma/terapia , Adulto , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/genética , Desdiferenciação Celular , Evolução Fatal , Feminino , Fusão Gênica , Hemangiopericitoma/diagnóstico por imagem , Hemangiopericitoma/genética , Humanos , Imagem por Ressonância Magnética , Recidiva Local de Neoplasia , Radiocirurgia , Proteínas Repressoras/genética , Fator de Transcrição STAT6/genética , Tomografia Computadorizada por Raios X
20.
Clin Neuropathol ; 38(2): 74-79, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30499771

RESUMO

Solitary fibrous tumor/hemangiopericytoma seldom arises from the optic nerve sheath. Herein, we describe the clinical, radiological, and histological findings of a solitary fibrous tumor/hemangiopericytoma arising from the optic nerve sheath and occupying the optic canal. The diagnosis is challenging both clinically and histologically as it clinically simulates a commoner lesion, i.e., an optic nerve sheath meningioma, and histological features can be mistaken for a fibroblastic/transitional meningioma. Differentiating these lesions from meningioma is crucial both for therapeutic and prognostic reasons. Moreover, hemangiopericytoma with high-grade features arising within the brain is known to develop extracranial metastases, and hence warrants a close follow-up.
.


Assuntos
Hemangiopericitoma/patologia , Neoplasias Meníngeas/patologia , Meningioma/patologia , Tumores Fibrosos Solitários/patologia , Adulto , Diagnóstico Diferencial , Hemangiopericitoma/diagnóstico , Humanos , Neoplasias Meníngeas/diagnóstico , Meningioma/diagnóstico , Prognóstico , Tumores Fibrosos Solitários/diagnóstico
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