RESUMEN
BACKGROUND: Although the imaging findings of intracranial solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) and meningioma are similar, their treatment and prognosis are quite different. Accurate preoperative identification of these two types of tumors is crucial for individualized treatment. OBJECTIVE: The aim of this study was to develop a radiomics model for the differentiation of intracranial SFT/HPC and meningioma based on multiparametric magnetic resonance imaging (mpMRI). MATERIAL AND METHODS: A total of 99 patients from July 2012 to July 2018 with histologically and immunohistochemically confirmed SFT/HPC (n = 40) or meningiomas (n = 59) were retrospectively analyzed. A total of 1118 features were extracted based on its image shape, intensity and texture features. The logistic regression (LR) and multi-layer artificial neural network (ANN) classifiers were used to classify SFT/HPC and meningioma. The predictive performance was calculated using receiver operating characteristic curves (ROC). RESULTS: We found significant difference in terms of sex between the SFT/HPC and meningioma group (χ2= 4.829, P < 0.05), but no significant difference was found in age (P > 0.05). The most significant radiomics features included five shape and four first-order level features. For the LR classifier, the prediction accuracy of SFT/HPC was 71.0% and meningioma was 78.7%. For the ANN classifier, the prediction accuracy of SFT/HPC was 83.9% and meningioma was 80.9%. Both of the two classifiers achieved a high accuracy rate, but ANN was better. CONCLUSIONS: Radiomics features, especially when combined with an ANN classifier, can provide satisfactory performance in distinguishing SFT/HPC and meningioma.
Asunto(s)
Hemangiopericitoma , Neoplasias Meníngeas , Meningioma , Imágenes de Resonancia Magnética Multiparamétrica , Tumores Fibrosos Solitarios , Humanos , Meningioma/diagnóstico por imagen , Meningioma/patología , Hemangiopericitoma/diagnóstico por imagen , Hemangiopericitoma/patología , Tumores Fibrosos Solitarios/diagnóstico por imagen , Tumores Fibrosos Solitarios/patología , Femenino , Masculino , Neoplasias Meníngeas/diagnóstico por imagen , Neoplasias Meníngeas/patología , Persona de Mediana Edad , Estudios Retrospectivos , Adulto , Imágenes de Resonancia Magnética Multiparamétrica/métodos , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/patología , Anciano , Diagnóstico Diferencial , Redes Neurales de la Computación , Imagen por Resonancia Magnética/métodos , RadiómicaRESUMEN
Sinonasal tumours of varying histology are not unusual in otolaryngology surgical practice. Juvenile angiofibroma (JAs) are vascular tumours usually occurring in adolescent male population; but rare in females. But similar clinical and radiological presentations are possible in females inducing strong suspicion of JA which needs to be ruled out by detailed evaluation. Here we present a case of a young female in her 20s who presented with a bleeding nasal mass which was finally diagnosed as sinonasal glomangiopericytoma which is a very rare sinonasal tumour. Tumours resembling JA do present in the female population but rarely turn out to be JA. A strong index of suspicion along with a handful of special blood investigations to rule out androgen insensitivity syndrome is mandatory.
Asunto(s)
Angiofibroma , Hemangiopericitoma , Humanos , Angiofibroma/diagnóstico , Angiofibroma/patología , Femenino , Diagnóstico Diferencial , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/patología , Hemangiopericitoma/diagnóstico por imagen , Neoplasias de los Senos Paranasales/diagnóstico , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/diagnóstico por imagen , AdultoRESUMEN
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.
Asunto(s)
Hemangiopericitoma , Neoplasias Meníngeas , Tumores Fibrosos Solitarios , Humanos , Francia/epidemiología , Hemangiopericitoma/epidemiología , Hemangiopericitoma/patología , Hemangiopericitoma/cirugía , Hemangiopericitoma/diagnóstico , Femenino , Masculino , Persona de Mediana Edad , Neoplasias Meníngeas/epidemiología , Neoplasias Meníngeas/patología , Neoplasias Meníngeas/cirugía , Neoplasias Meníngeas/diagnóstico , Tumores Fibrosos Solitarios/epidemiología , Tumores Fibrosos Solitarios/patología , Tumores Fibrosos Solitarios/cirugía , Tumores Fibrosos Solitarios/diagnóstico , Adulto , Anciano , Incidencia , Adulto Joven , Meningioma/epidemiología , Meningioma/patología , Meningioma/cirugía , Meningioma/diagnóstico , Adolescente , Anciano de 80 o más Años , NiñoRESUMEN
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.
Asunto(s)
Hemangiopericitoma , Tumores Fibrosos Solitarios , Humanos , Femenino , Persona de Mediana Edad , Nalgas/patología , Tumores Fibrosos Solitarios/patología , Tumores Fibrosos Solitarios/cirugía , Tumores Fibrosos Solitarios/terapia , Hemangiopericitoma/cirugía , Hemangiopericitoma/patología , Hemangiopericitoma/terapia , Resultado Fatal , Imagen por Resonancia Magnética , Neoplasias Pulmonares/patología , Neoplasias Pulmonares/secundario , Neoplasias Pulmonares/terapia , Neoplasias Pulmonares/cirugíaRESUMEN
OBJECTIVES: To present a case report of sinonasal glomangiopericytoma (GPC) in a female patient in her thirties and to highlight the importance of collecting pathology specimens even in routine sinus surgery cases. METHODS: A case report detailing the diagnosis of GPC in a female in her thirties, including her initial presentation, treatment, and follow-up, along with a brief review of the literature. RESULTS: Pathology of the collected specimen revealed sinonasal GPC along with chronic rhinosinusitis. Immunohistochemistry was positive for SMA, beta-catenin, and cyclin D1; and negative for STAT6, ERG, pankeratin, SOX10, and S100. CONCLUSION: This diagnosis expands the knowledge around the demographic profile of GPC patients. GPC should be included in the differential diagnosis of sinonasal masses, even in younger patients. The case highlights the importance of collecting the entire pathology specimen in all cases, even of ones that seem routine and benign.
Asunto(s)
Hemangiopericitoma , Humanos , Femenino , Hemangiopericitoma/patología , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/cirugía , Adulto , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/cirugía , Neoplasias de los Senos Paranasales/diagnóstico , InmunohistoquímicaRESUMEN
BACKGROUND: Myopericytoma is a rare spindle cell tumor of mesenchymal origin, typically benign, characterized by concentric proliferation of tumor cells around blood vessels within subcutaneous tissue. It primarily occurs in middle-aged adults and is often located in distal extremities, although cases have been reported in proximal extremities and head-neck regions. However, occurrences within the oral cavity are exceedingly rare. To date, literature reviews have identified only two cases in children under 10 years old and reported only five cases of myopericytoma occurring in the lip region. We provide a comprehensive review and analysis of all documented cases to better understand this condition. CASE PRESENTATION: A 7-year-old girl presented to oral and maxillofacial surgery with the discovery of a painless mass on the inner aspect of the upper lip. The diagnosis of myopericytoma was confirmed by histological examination (HE staining), alcian blue staining, and immunohistochemistry. CONCLUSIONS: Following surgical excision, there were no signs of recurrence at a 3-month follow-up. The pathological diagnosis of myopericytoma is quite challenging, and immunohistochemical testing is necessary.
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Hemangiopericitoma , Myopericytoma , Adulto , Persona de Mediana Edad , Femenino , Humanos , Niño , Myopericytoma/diagnóstico , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/cirugía , Hemangiopericitoma/patología , Labio , InmunohistoquímicaAsunto(s)
Hemangiopericitoma , Neoplasias Gástricas , Humanos , Neoplasias Gástricas/patología , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/diagnóstico por imagen , Hemangiopericitoma/patología , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/diagnóstico por imagen , Masculino , Femenino , Persona de Mediana Edad , Tomografía Computarizada por Rayos X , Anciano , Gastrectomía , Adenocarcinoma/patología , Adenocarcinoma/diagnósticoRESUMEN
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.
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Radioterapia de Iones Pesados , Hemangiopericitoma , Tumores Fibrosos Solitarios , Masculino , Humanos , Femenino , Protones , Recurrencia Local de Neoplasia/radioterapia , Estudios Retrospectivos , Hemangiopericitoma/radioterapia , Hemangiopericitoma/patología , Hemangiopericitoma/cirugía , Tumores Fibrosos Solitarios/radioterapia , Tumores Fibrosos Solitarios/patología , Radioterapia de Iones Pesados/efectos adversosRESUMEN
ABSTRACT: Sclerosing angiomatoid nodular transformation (SANT) is a reactive non-neoplastic, rare vascular lesion of the spleen. The histology shows multiple angiomatoid nodules surrounded by proliferative stroma. A 31-year-old lady presented with an abdominal mass for 6 months. Contrast-enhanced computed tomography (CECT) abdomen was suggestive of hemangiopericytoma/hemangioendothelioma. An open splenectomy was performed, and the resected specimen was sent for histopathology examination. The gross examination showed a bosselated mass present at the lower pole of the spleen measuring 8 × 8 cm with peripherally located coalescing red-brown nodules embedded in a dense fibrous stroma on the cut surface. On microscopy, multiple circumscribed angiomatoid nodules comprising irregular slit-like vascular channels lined by plump endothelial cells were seen embedded in dense sclerotic stroma. Because of the lack of specific diagnostic features, it is difficult to diagnose SANT clinically and radiologically. However, the typical histopathological findings are a clue in clinching the diagnosis.
Asunto(s)
Bazo , Esplenectomía , Tomografía Computarizada por Rayos X , Humanos , Adulto , Femenino , Bazo/patología , Bazo/diagnóstico por imagen , Neoplasias del Bazo/patología , Neoplasias del Bazo/cirugía , Neoplasias del Bazo/diagnóstico , Neoplasias del Bazo/diagnóstico por imagen , Histocitoquímica , Microscopía , Histiocitoma Fibroso Benigno/patología , Histiocitoma Fibroso Benigno/diagnóstico , Histiocitoma Fibroso Benigno/cirugía , Histiocitoma Fibroso Benigno/diagnóstico por imagen , Radiografía Abdominal , Hemangiopericitoma/patología , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/diagnóstico por imagen , Hemangiopericitoma/cirugíaRESUMEN
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.
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Hemangiopericitoma , Sarcoma , Síndrome de Trombocitopenia Febril Grave , Tumores Fibrosos Solitarios , Humanos , Adulto , Biomarcadores de Tumor , Tumores Fibrosos Solitarios/patología , Hemangiopericitoma/patologíaRESUMEN
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.
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Hemangiopericitoma , Tumores Fibrosos Solitarios , Humanos , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/patología , Hemangiopericitoma/cirugía , Tumores Fibrosos Solitarios/diagnóstico , Tumores Fibrosos Solitarios/patología , Tumores Fibrosos Solitarios/cirugía , Análisis de Supervivencia , Pronóstico , NomogramasRESUMEN
Objective: To investigate the clinicopathological features, immunophenotype, diagnosis and differential diagnosis of SRF-rearranged cellular perivascular myoid tumor. Methods: Two cases of SRF-rearranged cellular perivascular myoid tumor diagnosed in the Department of Pathology, Fudan University Shanghai Cancer Center from October 2021 to March 2022 were collected. Immunohistochemical staining, fluorescence in-situ hybridization (FISH) and next-generation sequencing (NGS) were performed, and the literature was reviewed. Results: Case 1, a 3-month-old boy presented with a painless tumor of the scalp, measuring about 2 cm in diameter. Case 2, a 3-year-old girl complained with a painless tumor of the knee, measuring approximately 1.5 cm in diameter. Microscopically, the tumor had a clear boundary and showed multinodular growth. The tumor was mainly composed of spindle cells arranged in long intersecting fascicles associated with thin, slit-like or branching ectatic vessels, focally forming hemangiopericytoma-like appearance. The tumor cells were abundant, but there was no obvious atypia. Mitotic figures (3-4/10 HPF) were noted. H-caldesmon and SMA were positive in both cases. Case 1 showed diffuse and strong positivity for Desmin, and focally for CKpan. Ki-67 proliferation index was 20% and 30%, respectively. FISH displayed NCOA2 gene translocation in case 1 and the RELA gene translocation in case 2. NGS detected the SRF-NCOA2 gene fusion in case 1 and the SRF-RELA gene fusion in case 2. Both patients underwent local excisions. During the follow-up of 5-14 months, case 1 had no local recurrence, while case 2 developed local recurrence 1 year post operatively. Conclusions: SRF-rearranged cellular perivascular myoid tumor is a novel variant of perivascular cell tumor, which tends to occur in children and adolescents. The tumor forms a broad morphologic spectrum ranging from a pericytic pattern to a myoid pattern, and include hybrid tumors with a mixture of pericytic and myoid patterns. Due to its diffuse hypercellularity and increased mitotic figures and smooth muscle-like immunophenotype, the tumor is easy to be misdiagnosed as myogenic sarcomas. The tumor usually pursues a benign clinical course and rare cases may locally recur.
Asunto(s)
Hemangiopericitoma , Sarcoma , Neoplasias de los Tejidos Blandos , Preescolar , Femenino , Humanos , Lactante , Masculino , Biomarcadores de Tumor/análisis , Proteínas de Unión a Calmodulina , China , Hemangiopericitoma/patología , Sarcoma/patología , Neoplasias de los Tejidos Blandos/patologíaRESUMEN
OBJECTIVE: The authors aimed to explore the clinical outcomes and risk factors related to recurrence of and survival from solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) that were reclassified according to the 2021 WHO classification of central nervous system (CNS) tumors. METHODS: The authors retrospectively collected and analyzed the clinical and pathological data of SFTs and HPCs recorded from January 2007 to December 2021. Two neuropathologists reassessed pathological slides and regraded specimens on the basis of the 2021 WHO classification. The prognostic factors related to progression-free survival (PFS) and overall survival (OS) were statistically assessed with univariate and multivariate Cox regression analyses. RESULTS: A total of 146 patients (74 men and 72 women, mean ± SD [range] age 46.1 ± 14.3 [3-78] years) were reviewed, and 86, 35, and 25 patients were reclassified as having grade 1, 2, and 3 SFTs on the basis of the 2021 WHO classification, respectively. The median PFS and OS of the patients with WHO grade 1 SFT were 105 months and 199 months after initial diagnosis; for patients with WHO grade 2 SFT, 77 months and 145 months; and for patients with WHO grade 3 SFT, 44 months and 112 months, respectively. Of the entire cohort, 61 patients experienced local recurrence and 31 died, of whom 27 (87.1%) died of SFT and relevant complications. Ten patients had extracranial metastasis. In multivariate Cox regression analysis, subtotal resection (STR) (HR 4.648, 95% CI 2.601-8.304, p < 0.001), tumor located in the parasagittal or parafalx region (HR 2.105, 95% CI 1.099-4.033, p = 0.025), tumor in the vertebrae (HR 3.352, 95% CI 1.228-9.148, p = 0.018), WHO grade 2 SFT (HR 2.579, 95% CI 1.343-4.953, p = 0.004), and WHO grade 3 SFT (HR 5.814, 95% CI 2.887-11.712, p < 0.001) were significantly associated with shortened PFS, whereas STR (HR 3.217, 95% CI 1.435-7.210, p = 0.005) and WHO grade 3 SFT (HR 3.433, 95% CI 1.324-8.901, p = 0.011) were significantly associated with shortened OS. In univariate analyses, patients who received adjuvant radiotherapy (RT) after STR had longer PFS than patients who did not receive RT. CONCLUSIONS: The 2021 WHO classification of CNS tumors better predicted malignancy with different pathological grades, and in particular WHO grade 3 SFT had worse prognosis. Gross-total resection (GTR) can significantly prolong PFS and OS and should serve as the most important treatment method. Adjuvant RT was helpful for patients who underwent STR but not for patients who underwent GTR.
Asunto(s)
Neoplasias del Sistema Nervioso Central , Hemangiopericitoma , Síndrome de Trombocitopenia Febril Grave , Tumores Fibrosos Solitarios , Masculino , Humanos , Femenino , Adulto , Persona de Mediana Edad , Estudios Retrospectivos , Hemangiopericitoma/cirugía , Hemangiopericitoma/patología , Tumores Fibrosos Solitarios/cirugía , Tumores Fibrosos Solitarios/patología , Pronóstico , Neoplasias del Sistema Nervioso Central/cirugía , Sistema Nervioso Central/patología , Factores de Riesgo , Organización Mundial de la Salud , Recurrencia Local de Neoplasia/epidemiologíaRESUMEN
Objective: To investigate the clinicopathological features, immunophenotype, diagnosis and differential diagnosis of SRF-rearranged cellular perivascular myoid tumor. Methods: Two cases of SRF-rearranged cellular perivascular myoid tumor diagnosed in the Department of Pathology, Fudan University Shanghai Cancer Center from October 2021 to March 2022 were collected. Immunohistochemical staining, fluorescence in-situ hybridization (FISH) and next-generation sequencing (NGS) were performed, and the literature was reviewed. Results: Case 1, a 3-month-old boy presented with a painless tumor of the scalp, measuring about 2 cm in diameter. Case 2, a 3-year-old girl complained with a painless tumor of the knee, measuring approximately 1.5 cm in diameter. Microscopically, the tumor had a clear boundary and showed multinodular growth. The tumor was mainly composed of spindle cells arranged in long intersecting fascicles associated with thin, slit-like or branching ectatic vessels, focally forming hemangiopericytoma-like appearance. The tumor cells were abundant, but there was no obvious atypia. Mitotic figures (3-4/10 HPF) were noted. H-caldesmon and SMA were positive in both cases. Case 1 showed diffuse and strong positivity for Desmin, and focally for CKpan. Ki-67 proliferation index was 20% and 30%, respectively. FISH displayed NCOA2 gene translocation in case 1 and the RELA gene translocation in case 2. NGS detected the SRF-NCOA2 gene fusion in case 1 and the SRF-RELA gene fusion in case 2. Both patients underwent local excisions. During the follow-up of 5-14 months, case 1 had no local recurrence, while case 2 developed local recurrence 1 year post operatively. Conclusions: SRF-rearranged cellular perivascular myoid tumor is a novel variant of perivascular cell tumor, which tends to occur in children and adolescents. The tumor forms a broad morphologic spectrum ranging from a pericytic pattern to a myoid pattern, and include hybrid tumors with a mixture of pericytic and myoid patterns. Due to its diffuse hypercellularity and increased mitotic figures and smooth muscle-like immunophenotype, the tumor is easy to be misdiagnosed as myogenic sarcomas. The tumor usually pursues a benign clinical course and rare cases may locally recur.
Asunto(s)
Preescolar , Femenino , Humanos , Lactante , Masculino , Biomarcadores de Tumor/análisis , Proteínas de Unión a Calmodulina , China , Hemangiopericitoma/patología , Sarcoma/patología , Neoplasias de los Tejidos Blandos/patologíaRESUMEN
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.
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Hemangiopericitoma , Programa de VERF , Tumores Fibrosos Solitarios , Humanos , Estudios Retrospectivos , Masculino , Femenino , Tumores Fibrosos Solitarios/terapia , Tumores Fibrosos Solitarios/mortalidad , Tumores Fibrosos Solitarios/patología , Tumores Fibrosos Solitarios/epidemiología , Persona de Mediana Edad , Hemangiopericitoma/terapia , Hemangiopericitoma/mortalidad , Hemangiopericitoma/patología , Hemangiopericitoma/epidemiología , Anciano , Pronóstico , Adulto , Neoplasias Encefálicas/terapia , Neoplasias Encefálicas/mortalidad , Neoplasias Encefálicas/patología , Neoplasias Encefálicas/epidemiología , Nomogramas , Clasificación del Tumor , Estimación de Kaplan-Meier , Adulto Joven , Anciano de 80 o más Años , Terapia CombinadaRESUMEN
Solitary fibrous tumors (SFTs) are mesenchymal neoplasms with variable clinical behavior depending on age, tumor site, and size, and pathologic factors such as mitoses and necrosis. Imaging features on computed tomography (CT) or magnetic resonance imaging (MRI) are not specific, and the diagnosis relies on histopathology with immunohistochemistry. SFTs arising from seminal vesicles is rare and reported in only eight earlier cases. We discuss the clinical, histopathologic and positron emission tomography (PET) imaging characteristics of a 54-year-old patient with SFT of the seminal vesicle. The patient was treated with robot-assisted seminal vesiculotomy and is doing well on follow-up at two years.
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Hemangiopericitoma , Síndrome de Trombocitopenia Febril Grave , Tumores Fibrosos Solitarios , Masculino , Humanos , Persona de Mediana Edad , Vesículas Seminales/diagnóstico por imagen , Vesículas Seminales/cirugía , Vesículas Seminales/patología , Síndrome de Trombocitopenia Febril Grave/patología , Tumores Fibrosos Solitarios/diagnóstico por imagen , Tumores Fibrosos Solitarios/cirugía , Hemangiopericitoma/patología , Tomografía Computarizada por Rayos XRESUMEN
Solitary fibrous tumours (SFTs) are rare mesenchymal neoplasms composed of spindle cells, most often occurring in the pleura. SFTs arising from the prostate are exceptionally rare, with only around 40 cases reported in literature to date. We report a man in his 60s who was referred to our clinic for elevated prostate-specific antigen and presented with mild obstructive lower urinary tract and defecatory symptoms. Prostate needle-core biopsy revealed neoplastic spindle cells that strongly expressed CD34. Cross-sectional imaging demonstrated a 12 cm locally advanced heterogeneous prostate mass with intravesical extension and mass effect on the anterior rectum. Radical cystoprostatectomy with orthotopic neobladder reconstruction was performed, and the diagnosis of primary prostatic SFT was made based on histological characteristics and immunophenotyping. We present diagnostic, clinical management and prognostic considerations in patients with primary prostatic SFT.
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Hemangiopericitoma , Síndrome de Trombocitopenia Febril Grave , Tumores Fibrosos Solitarios , Masculino , Humanos , Próstata/diagnóstico por imagen , Próstata/cirugía , Próstata/patología , Tumores Fibrosos Solitarios/diagnóstico por imagen , Tumores Fibrosos Solitarios/cirugía , Hemangiopericitoma/patología , Biopsia con Aguja GruesaRESUMEN
PURPOSE: To report long-term outcomes after surgery for orbital solitary fibrous tumors. METHODS: Retrospective review of patients with orbital solitary fibrous tumor, first seen between 1971 and 2022. Primary excisions were grouped as (A) surgically intact, (B) macroscopic, but with cell spillage, or (C) known incomplete excision. RESULTS: Fifty-nine patients (31 female; 53%) presented at a mean age of 43.0 years (19-82 years), with 5 patients (8.5%) having malignant solitary fibrous tumors. Average follow up was 11.4 years (median 7.8; range 1-43 years). There were 28 of 59 (47%) group A patients with 1 of 28 (3%) having recurrence, 20 of 59 (34%) group B having 6 of 20 (30%) recurrences, and 11 of 59 (19%) group C with 9 of 11 (82%) recurrence ( p < 0.001 for recurrence rate). At a mean of 8.9 (range 1-23.6) years after initial treatment, continued local tumor growth was evident in 16 (27%) patients, with higher-grade recurrence in 3 of 14 (21%) cases. No patient had systemic disease at presentation, but 2 of 59 patients (3%) developed metastases at 22 and 30 years after first treatment. The 10-year progression-free survival was 94% (group A), 60% (group B), and 36% (group C). Tumor disruption or incomplete excision (groups B + C) carries the highest risk of tumor recurrence (hazard ratio 15.0; 95% confidence interval, 1.98-114; p = 0.009), with no correlation to tumor size or histology. CONCLUSIONS: Orbital solitary fibrous tumors have a low recurrence rate with surgically intact excision; piecemeal excision, capsular disruption, or known incomplete resection have a high recurrence rate, which can occur decades later. Baseline postoperative scans is recommended, together with long-term clinical and interval imaging.
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Hemangiopericitoma , Tumores Fibrosos Solitarios , Humanos , Femenino , Adulto , Recurrencia Local de Neoplasia/patología , Tumores Fibrosos Solitarios/cirugía , Tumores Fibrosos Solitarios/patología , Hemangiopericitoma/patología , Resultado del Tratamiento , Estudios RetrospectivosRESUMEN
Orbital primary solitary fibrous tumors (OPSFTs) are rare. To further characterize the clinical and pathological features of OPSFTs, 92 cases of OPSFT were analyzed to develop a risk prediction model. OPSFTs were equally distributed between males (n = 45) and females (n = 47) with a mean patient age of 40.8 years (median 39 years; range 5-70 years) at initial diagnosis. The mean tumor size was 2.79 cm (median 2.5 cm). Microscopically, the tumor cells were irregularly arranged in spindle, ovoid, or round shapes with varying amounts of collagen and branching blood vessels. Immunohistochemical staining showed positive STAT6 nuclear expression in all cases, loss of CD34 expression in seven cases, and a mean Ki-67 label index of 5.25% (range 1%-30%). All patients were initially surgically resected and had a median follow-up of 99 months: 33 patients recurred, 6 of whom presented with multiple recurrences and 1 with distant metastases. A predictive model for the risk of recurrence based on tumor size, mitosis, Ki-67 label index, and dominant constituent cell (DCC) was developed based on our results. In conclusion, OPSFTs are rare but can be reliably diagnosed based on characteristic morphological features and STAT6 immunohistochemistry. The rate of local recurrence of orbital tumors tends to be higher than the rate of distant metastases, which can be predicted by a risk stratification model specific to orbital tumors. Long-term clinical follow-up is recommended as advanced disease is common.
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Hemangiopericitoma , Neoplasias Orbitales , Tumores Fibrosos Solitarios , Masculino , Femenino , Humanos , Adulto , Neoplasias Orbitales/cirugía , Neoplasias Orbitales/patología , Antígeno Ki-67/metabolismo , Hemangiopericitoma/patología , Tumores Fibrosos Solitarios/cirugía , Tumores Fibrosos Solitarios/química , Inmunohistoquímica , Biomarcadores de Tumor , Factor de Transcripción STAT6RESUMEN
BACKGROUND: Head and neck solitary fibrous tumors (SFTs) are rare neoplasms, with few large-scale studies describing this entity. We evaluated the demographics and correlates of survival in a large series of SFT patients. METHODS: The 2004-2017 National Cancer Database was queried for head and neck SFT patients receiving definitive surgery. Cox proportional-hazards and Kaplan-Meier analyses assessed overall survival (OS). RESULTS: Of 135 patients, sinonasal (33.1%) and orbital (25.9%) SFTs were most common. Approximately 93% of SFTs were invasive and 64% were classified as hemangiopericytomas. The 5-year OS of skull base SFTs (84.5%) was lower than sinonasal (98.7%) and orbital (90.7%) SFTs (all p < 0.05). Government insurance exhibited higher mortality (HR 5.116; p < 0.001) and lower OS (p = 0.001). CONCLUSION: Head and neck SFTs presented with distinct prognoses based on anatomical origin. Overall survival was particularly worse in patients with skull base SFTs or government insurance. Prognostically, hemangiopericytomas were indistinct from other SFTs.