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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 , Niño

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A solitary fibrous tumour (SFT)/haemangiopericytoma (HPC) accounts for approximately 1% of all vascular tumours, and 13-25% of them are present in the head and neck region. Here, a case of SFT in the left EAC has been reported with the longest duration follow-up among the previously reported cases.

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Solitary fibrous tumours (SFTs) of the urinary bladder are a rare mesenchymal neoplasm that occasionally has malignant potential. The tumour is characterised by haphazardly arranged spindle-shaped to ovoid cells, with a prominent, branching, thin-walled, dilated vasculature and NAB2-STAT6 gene rearrangement. While most SFTs are indolent in nature, difficulty arises predicting which SFTs are potentially malignant. There are now validated risk stratification tools to help identify which SFTs are likely to metastasize and help clinicians determine management. The mainstay of treatment for SFTs remains surgery, with emerging evidence in the combined use of surgery and radiotherapy.

RESUMEN

A 71-year-old male with a 4-month history of bulging, tearing, and redness in the right eye presented with vision loss, proptosis, conjunctival hyperemia, and chemosis. Magnetic resonance imaging showed a right intraconal solid mass with extraconal extension, hyper-intense in T2 sequences with heterogeneous contrast enhancement. Complete excision of the mass was performed through a lateral orbitotomy. Histological analysis revealed a neoplasm with high vessel density, solid growth of oval cells, a concentric proliferation of the wall of small vessels, and a weak and patchy positivity for smooth muscle actin. These findings were consistent with the diagnosis of myopericytoma. After surgery, visual acuity improved in the affected eye and after 18 months of follow-up there have been no signs of recurrence.

Asunto(s)

Exoftalmia , Myopericytoma , Neoplasias Orbitales , Anciano , Exoftalmia/diagnóstico , Humanos , Imagen por Resonancia Magnética , Masculino , Órbita/patología , Neoplasias Orbitales/diagnóstico por imagen , Neoplasias Orbitales/cirugía

RESUMEN

Haemangiopericytoma is a highly vascular tumour, which is a rare soft tissue lesion that may arise anywhere in the body, including the orbit. During its surgical resection, it is too friable for the surgeon to handle and it can bleed severely causing many problems to the surgeon. That is why many surgical approaches have been reported till now, aiming at total excision with minimal blood loss. In this case, total resection of an orbital haemangiopericytoma in a 61-year-old Caucasian woman, using an intraoperative 23G needle injection of 40% n-butyl-2-cyanoacrylate and 60% lipiodol, is presented. The lesion was directly injected under fluoroscopic visualization, after which it became firm enough to be surgically removed without significant bleeding.

Asunto(s)

Enbucrilato , Hemangiopericitoma , Aceite Etiodizado , Femenino , Hemangiopericitoma/cirugía , Humanos , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Órbita

RESUMEN

INTRODUCTION: Glomangiopericytoma is a rare neoplasm of low malignant potential. It is a rare type of haemangiopericytoma located in nasal cavity. This neoplasm has good prognosis and complete surgical excision is treatment of choice. This case report is representing one such neoplasm. This reporting is done in line with the SCARE criteria (Agha et al., 2018 [1]). CASE PRESENTATION: We presenting a case of 54 year old male patient from upper socioeconomic status who presented at our institution with history of surgery (endoscopic sinus surgery with Septoplasty) 15 days back. A Final histopathology report suggested glomangiopericytoma. Since primary surgery was not done as per oncologic principals, patient was advised for adjuvant radiation. At our institute patient was evaluated again. Revision surgery was done. Patient was discharged next day. DISCUSSION: Glomangiopericytoma is a rare neoplasm with incidence of less than 0.5% of all neoplasms of sinonasal cavity. Prognosis is very good after complete surgical excision. It often confuses clinicians with nasal polyps. Here also patient was operated initially considering as benign polyposis outside. Patient was re-operated again to ensure the complete clearance. CONCLUSION: This is the typical case of converting dual modality treatment to single modality with the help of knowledge, communication, transparent team work. This also a rare type of neoplasm and by reporting this rare case we are contributing to data pool of nasal tumors where lack of reporting is major obstacle in the formation of uniform treatment guidelines.

RESUMEN

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.

Asunto(s)

Diagnóstico por Computador/métodos , Hemangiopericitoma/diagnóstico por imagen , Aprendizaje Automático , Imagen por Resonancia Magnética/métodos , Neoplasias Meníngeas/diagnóstico por imagen , Meningioma/diagnóstico por imagen , Neurorradiografía/métodos , Adulto , Femenino , Hemangiopericitoma/patología , Humanos , Masculino , Neoplasias Meníngeas/patología , Meningioma/patología , Persona de Mediana Edad , Cuidados Preoperatorios , Estudios Retrospectivos , Sensibilidad y Especificidad

RESUMEN

Haemangiopericytomas (HPCs) in the spinal canal are extremely rare and have only been infrequently reported. Severe and sustained spinal cord compression resulting from these intraspinal tumours may cause potentially irreversible neurological impairment. We described a rare case of primary intradural extramedullary HPC recovered from serious compression of thoracic spinal cord in a 65-year-old man suffered from a rapidly progressive disease with complete paralysis of the lower limbs. Magnetic resonance imaging (MRI) and spinal angiography exhibited an unusual hypervascular mass, which was confirmed as HPC by pathological examination, in the dorsal aspects of T7 to T8 spine. Total surgical resection was performed followed by adjuvant radiation. Moreover, this patient had been undergoing specific training of lower limbs from the onset of paralysis and continuous rehabilitation was conducted after discharge. Increased strength of his lower limb muscles was shown in the period of follow-up. Remarkably, four years after surgery, this patient was able to walk with sticks. No signs of local recurrence or metastasis occurred. Conclusively, surgical removal is still the preferred treatment for HPC, and adjuvant radiotherapy and/or chemotherapy may help control tumour recurrence or metastasis. Additionally, it is possible that rehabilitation training potentially promotes neurological function recovery to some extent.

Asunto(s)

Hemangiopericitoma/radioterapia , Hemangiopericitoma/cirugía , Neoplasias de la Médula Espinal/radioterapia , Neoplasias de la Médula Espinal/cirugía , Anciano , Humanos , Masculino , Procedimientos Neuroquirúrgicos , Radioterapia Adyuvante , Recuperación de la Función

RESUMEN

Vascular tumours of the liver represent an underrated chapter of medical and surgical hepatology. These tumours cover a wide spectrum ranging from the frequent and most benign hepatic haemangioma (HH), via the rare and intermediately aggressive hepatic epithelioid haemangioendothelioma (HEHE) to the rare and most malignant hepatic haemangiosarcoma (HHS). In contrast to the treatment algorithms for hepatocellular and cholangiocellular cancer, the diagnostic and therapeutic approaches to HEHE and HHS are not well developed. The related uncertainty is explained by their rare occurrence and their protean clinical, morphological (imaging) and histopathological presentation and behaviour. This article gives an update about these particular tumours based on the analysis of the recent literature and of the studies on vascular tumours published by the European Liver Intestine Transplantation Association (ELITA)-European Liver Transplant Registry (ELTR). It focuses also on the place of liver transplantation (LT) in the respective therapeutic algorithms. The differential diagnosis between these vascular and other tumour types may be very difficult. Correct diagnosis is of utmost importance and is based on a high index of clinical suspicion and on the integration of clinical, radiological, histological [including immunohistochemistry (IHC) and molecular biology findings]. Surgery, be it partial or total hepatectomy (LT), should be proposed whenever possible, because it is the therapeutic mainstay. In HEHE, LT provides excellent results, with long-term disease-free survivals (DFS) reaching 75%. Good results can be obtained even in case of (frequent) extrahepatic spread. Based on the extensive ELITA-ELTR study a HEHE-LT prognostic score has been proposed in order to estimate the risk of recurrence after LT. In contrast, results of surgery and LT are extremely poor for HHS, for the almost invariably rapid recurrence (within 6 months) and related death within 2 years. LT remains a contraindication for HHS. Due to the still important recurrence rate after surgical resection (25% in HEHE and almost 100% in HHS), there is an urgent need to develop pharmacological treatments targeting angiogenic and non-VEGF angiogenic pathways. To date, some prospective pilot studies and case reports have shown some short-term stabilisation of the disease in small groups of patients. In order to make progress, combination of surgery, anti-angiogenic and immunotherapy seems worthwhile. To complete the panel of vascular liver tumours, infantile haemangioendothelioma, haemangiopericytoma, nodular regenerative hyperplasia (NRH) and hepatic small vessel neoplasms (HSVN) are also discussed.

RESUMEN

AIMS: Solitary fibrous tumour (SFT) is a rare mesenchymal-derived neoplasm that can arise in any anatomical location in the body. SFT rarely metastasises, but aggressive behaviour is seen in a minority of cases, and relapses can occur several years after treatment. It would be a clinical advantage if high-risk patients could be identified before treatment. MATERIALS AND METHODS: We retrospectively analysed a population-based cohort of SFT to describe treatment, outcome, prognostic factors and to further validate a previously published risk assessment tool (D-score) based on age, tumour size and mitotic index. Seventy-two patients diagnosed with SFT in the Central, North and Southern Denmark regions between 1979 and 2013 were included in the study. RESULTS: For patients with localised disease at the time of diagnosis (n = 64) the 5 and 10 year overall survival was 86% (95% confidence interval 74-92) and 65% (95% confidence interval 50-78), respectively. Seventeen of 62 patients (27%) who were in remission after radical treatment developed recurrence with either local or distant disease. The 5 year recurrence-free survival was 83% (95% confidence interval 70-90) and the 10 year was 69% (95% confidence interval 53-81). The 5 year local recurrence-free survival was 96% (95% confidence interval 86-99) and the 10 year was 92% (95% confidence interval 76-96). The median time to both overall recurrence and local recurrence was 4.3 years. Metastatic or inoperable SFT had a poor prognosis with a median overall survival of 8.4 months (range 3.6-26.4) and a 5 year overall survival of 11% (95% confidence interval 2-30). A further validation of a risk assessment tool (D-score) confirmed that patients classified as high-risk had a significantly decreased overall survival, with a hazard ratio of 3.7 (95% confidence interval 1.1-12.3). CONCLUSIONS: This study showed that our management and outcome were comparable with other published studies describing SFT and confirmed the value of the D-score as a risk assessment tool. Because of late recurrences, long-term (e.g. 10 years) follow-up for moderate- and high-risk patients is recommended.

Asunto(s)

Modelos Estadísticos , Recurrencia Local de Neoplasia/patología , Medición de Riesgo/métodos , Tumores Fibrosos Solitarios/patología , Adolescente , Adulto , Anciano , Niño , Preescolar , Terapia Combinada , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Metástasis de la Neoplasia , Recurrencia Local de Neoplasia/terapia , Pronóstico , Estudios Retrospectivos , Tumores Fibrosos Solitarios/terapia , Tasa de Supervivencia , Adulto Joven

RESUMEN

INTRODUCTION: This study was performed to assess the relationship between tumour response and radiation dose in equivalent 2 Gy per fraction (EQD2). METHOD: A retrospective cohort analysis of 21 patients with a diagnosis of intracranial haemangiopericytoma between 2000 and 2013 was included in this study. A total of 39 lesions were analysed. The equivalent dose in 2 Gy per fraction was calculated by assigning an alpha-beta ratio of 12 Gy. A paired t-test compared dose (EQD2) and tumour response, and as the outcome was binary, a logistic regression analysis was performed. RESULTS: In total, there were 14 cases of progression and 25 cases of non-progression. It was estimated that for a one unit increase in EQD2, the odds of non-progression were increased by a factor of 1.13 (P = 0.026). After adjusting for PTV volume, the estimated effect of EQD2 (min) on tumour response was stronger, with an estimated odds ratio of 1.36 for an increase of one unit and an odds ratio of 21.6 for an increase of 10 units (P = 0.015). The dose range varied with varying PTV volumes. Based on the logistic model, the probability of having non-progression is larger than 50% for EQ2Dmin doses larger than 30-40 Gy, in particular for volumes larger than 3.67 cm3 . CONCLUSION: This study demonstrates that there is a relationship between dose (EQD2) and outcome. With increasing dose, the likelihood of regression is higher. When adjusted for PTV volume, the response appeared stronger. The dose varied significantly with changes in the size of the PTV.

RESUMEN

Solitary fibrous tumours/haemangiopericytomas (SFT/HPC) are fibroblastic mesenchymal neoplasms that harbour a genetic fusion of NAB2/STAT6. There are few reported cases in the central nervous system (CNS), of which spinal tumours are minority. We present a case of a 63-year-old woman with progressive paraparesis and a sensory level of T6. On the MRI we detected an intradural extramedullary lesion on T9-T10. We performed a laminectomy with an intraoperative ultrasound study in which we observed a heterogeneous lesion with an infiltrating pattern affecting the medulla at several points, and resection of the lesion. Immunohistological findings revealed positivity for vimentin, CD34 and STAT6; Ki-67 was 2%. A final diagnosis of SFT/HPC with SFT phenotype was made. SFT/HPC of the CNS is a rare neoplasm that should be taken into account in the differential diagnosis of these kinds of lesions, as clinical and MRI findings often lead to errors in diagnostic approach. The support of intraoperative ultrasound can assist in surgical decision-making.

Asunto(s)

Hemangiopericitoma/cirugía , Tumores Fibrosos Solitarios/cirugía , Neoplasias de la Médula Espinal/cirugía , Biomarcadores de Tumor/análisis , Descompresión Quirúrgica , Errores Diagnósticos , Femenino , Hemangiopericitoma/química , Hemangiopericitoma/complicaciones , Hemangiopericitoma/diagnóstico por imagen , Humanos , Laminectomía , Vértebras Lumbares/cirugía , Imagen por Resonancia Magnética , Neoplasias Meníngeas/diagnóstico , Meningioma/diagnóstico , Persona de Mediana Edad , Neuroimagen , Paraplejía/etiología , Tumores Fibrosos Solitarios/química , Tumores Fibrosos Solitarios/complicaciones , Tumores Fibrosos Solitarios/diagnóstico por imagen , Trastornos Somatosensoriales/etiología , Neoplasias de la Médula Espinal/química , Neoplasias de la Médula Espinal/complicaciones , Neoplasias de la Médula Espinal/diagnóstico por imagen , Fusión Vertebral , Estenosis Espinal/cirugía , Cirugía Asistida por Computador , Vértebras Torácicas , Ultrasonografía Intervencional

RESUMEN

BACKGROUND: To report on the outcome of patients diagnosed with central nervous system haemangiopericytoma (HPC) or solitary fibrous tumours (SFT) and identify factors that may influence recurrence and survival. MATERIAL AND METHODS: Between January 1977 and December 2016, a retrospective search identified 22 HPCs/SFTs. The patients underwent a total of 40 surgical resections and 63.6% received radiotherapy. Median follow-up was 7.8 years. RESULTS: Six patients (27.3%) were re-operated for tumour recurrence. At the end of the study, 15 patients (68.2%) had no residual tumour on the last imaging. Surgical recurrence-free survival at 5 years was 77.4%, [95% CI: 60.1-99.8]. None of the investigated variables was associated with recurrence. At the end of the study, 5 patients were deceased (22.7%) and only 10 patients (45.5%) had no residual tumour on the last imaging and were alive. Overall survival at 5 years was 95%, [95% CI: 85.9-100]. None of the investigated variables was associated with overall survival. Patients who received radiotherapy demonstrated neither a reduced risk of surgical recurrence (P=0.378) nor a longer overall survival (P=0.405). CONCLUSION: SFTs/HPCs are associated with a significant risk of recurrence that may reduce the survival. Even if we could not demonstrate their benefit in this limited series, we believe that tailored maximal tumour resection on initial surgery is beneficial and that adjuvant RT is useful for tumours displaying grade II or III, even in case of complete removal.

Asunto(s)

Neoplasias del Sistema Nervioso Central/terapia , Hemangiopericitoma/terapia , Neoplasias Meníngeas/terapia , Tumores Fibrosos Solitarios/terapia , Adulto , Anciano , Neoplasias del Sistema Nervioso Central/mortalidad , Neoplasias del Sistema Nervioso Central/radioterapia , Neoplasias del Sistema Nervioso Central/cirugía , Femenino , Hemangiopericitoma/mortalidad , Hemangiopericitoma/radioterapia , Hemangiopericitoma/cirugía , Humanos , Masculino , Neoplasias Meníngeas/mortalidad , Neoplasias Meníngeas/radioterapia , Neoplasias Meníngeas/cirugía , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Pronóstico , Estudios Retrospectivos , Tumores Fibrosos Solitarios/mortalidad , Tumores Fibrosos Solitarios/radioterapia , Tumores Fibrosos Solitarios/cirugía , Análisis de Supervivencia , Resultado del Tratamiento , Adulto Joven

RESUMEN

This short review highlights significant changes and recent findings incorporated to varying extent in the WHO 2016 definition of a variety of tumours, including peripheral nerve sheath tumours, meningiomas, mesenchymal nonmeningothelial tumours, melanocytic tumours, lymphomas and histiocytic tumours, germ cell tumours and non-neuroendocrine pituitary tumours. Most notable classification changes include: adding 'hybrid nerve sheath tumours' to the spectrum of benign nerve sheath tumours; an updated definition of atypical meningioma (WHO grade II), including cases with brain invasion; recognizing dural solitary fibrous tumour (SFT) and haemangiopericytoma (HPC) as a single tumour entity characterized by NAB2 and STAT6 gene fusions for which the term SFT/HPC was chosen; recognizing that pituitary granular cell tumour, spindle cell oncocytoma, and pituicytoma all share nuclear expression of TTF-1, possibly representing a spectrum of a single nosological entity derived from posterior pituitary glial cells. The most significant diagnostic markers which have emerged include: inactivation of NF1, CDKN2A, and PRC2 components, SUZ12 and EED in MPNST, leading to neurofibromin and H3K27me3 expression loss; GNAQ and GNA11 mutations in CNS primary melanocytic tumours; BRAFV600E mutation in histiocytic tumours (Langerhans cell histiocytosis and Erdheim-Chester disease) and papillary craniopharyngioma, which provides both a diagnostic marker in the appropriate pathological setting and a therapeutic target. The WHO 2016 Classification has balanced cutting-edge knowledge on the molecular characteristics of the miscellaneous CNS tumours reviewed here with a practical approach for their daily diagnostic work-up. Much more progress can be expected in the classification of these neoplasms in the near future.

Asunto(s)

Neoplasias del Sistema Nervioso Central/clasificación , Neoplasias del Sistema Nervioso Central/diagnóstico , Neoplasias del Sistema Nervioso Central/patología , Humanos , Organización Mundial de la Salud

RESUMEN

Haemangiopericytoma of the head and neck (HN-HPC) is a rare neoplasm occurring in children. The rarity of the tumour poses a diagnostic and therapeutic challenge. Despite protocols that recommend the use of chemotherapy prior to surgery, no treatment standard has yet been proposed. This article reports the case of a 5-month-old infant with a rapidly growing, giant life-threatening HN-HPC that was treated successfully by two-stage surgery without adjuvant chemotherapy.

Asunto(s)

Arteria Carótida Externa/cirugía , Neoplasias de Cabeza y Cuello/cirugía , Hemangiopericitoma/cirugía , Arteria Carótida Externa/diagnóstico por imagen , Arteria Carótida Externa/patología , Femenino , Neoplasias de Cabeza y Cuello/patología , Hemangiopericitoma/diagnóstico por imagen , Hemangiopericitoma/patología , Humanos , Lactante , Imagen por Resonancia Magnética , Tomografía Computarizada por Rayos X

Asunto(s)

Hemangiopericitoma/diagnóstico , Neoplasias Renales/patología , Neoplasias Peritoneales/diagnóstico , Hemangiopericitoma/patología , Humanos , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Neoplasias Peritoneales/patología

RESUMEN

To describe the outcome of patients diagnosed with central nervous system haemangiopericytoma (HPC) or solitary fibrous tumour (SFT) and identify factors that may influence recurrence and survival. Between January 2000 and September 2016, a retrospective search identified 55 HPCs/SFTs. The patients underwent a total of 101 surgical resections and 56.9% received radiation therapy. Median follow-up was 7.8 years. 28 patients (50.9%) were re-operated for tumour recurrence. At the end of the study, 21 patients (42%) had no residual tumour on the last scan. Surgical recurrence-free survival at 5 years was 75.2%, 95% CI [63.3-89.3] and, the median surgical recurrence-free survival was 7.4 years. In the adjusted analysis, venous sinus invasion (present vs. absent) (HR 3.39, 95% CI [1.16, 9.93], p = 0.026), completeness of resection (HR 0.38, 95% CI [0.15-0.97], p = 0.042) and tumour subtype (SFT vs. HPC) (HR 3.02, 95% CI[1.02, 8.91], p = 0.045) were established as independent prognostic factors. At the end of the study, 25 patients were deceased (45.5%). and only 15 patients (27.3%) had no residual tumour on the last scan and were alive. Overall survival at 5 years was 80.2, 95% CI [69.3-92.8] and the median overall survival was 13.1 years. None of the investigated variables was associated with overall survival. Patients who received radiation therapy demonstrated neither a reduced risk of surgical recurrence (p = 0.370) nor a longer overall survival (p = 1.000). SFTs/HPCs are associated with a significant risk of recurrence that may reduce the survival of the patients. Total tumour resection upon initial surgery is associated with a lower risk of relapse but not with a prolonged survival. We did not observe a significant improvement in any of the clinical outcomes after radiation therapy.

Asunto(s)

Hemangiopericitoma/diagnóstico , Hemangiopericitoma/terapia , Neoplasias Meníngeas/diagnóstico , Neoplasias Meníngeas/terapia , Tumores Fibrosos Solitarios/diagnóstico , Tumores Fibrosos Solitarios/terapia , Adulto , Femenino , Estudios de Seguimiento , Hemangiopericitoma/patología , Humanos , Masculino , Neoplasias Meníngeas/patología , Persona de Mediana Edad , Recurrencia Local de Neoplasia/diagnóstico , Recurrencia Local de Neoplasia/cirugía , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Tumores Fibrosos Solitarios/patología , Análisis de Supervivencia

RESUMEN

BACKGROUND: Solitary fibrous tumor is an uncommon soft tissue neoplasm with intermediate biological behavior, which rarely metastasizes. Malignant solitary fibrous tumor, although not clearly defined, is rarely described in the prostate. The present case is characterized by some peculiarities if compared with previously reported cases of prostatic malignant solitary fibrous tumor. Firstly, it does not show a homogeneous morphology: part of the neoplasm (about 50%) showed the features of a conventional solitary fibrous tumor, while the remaining part showed the features of a malignant solitary fibrous tumor. In addition, the case is the first malignant solitary fibrous tumor reaching a huge diameter of 20 cm and replacing all prostatic parenchyma. Interestingly, normal prostatic parenchyma was observed on left-lobe trans-rectal needle-core biopsies, but was totally absent in surgical specimen. Since radical prostatectomy was carried out about 4 months after the biopsies, such discordant data may suggest exceedingly rapid growth of the neoplasm. CASE PRESENTATION: We report a case of a 62-year-old male, presented at medical observation for urinary retention, constipation and an enlarged prostate gland. A trans-rectal prostatic biopsy showed a low-grade spindle cell neoplasm. Histopathological examination of the prostatectomy specimen showed patternless architecture with hypocellular and hypercellular areas and hemangiopericytoma-like vessels. In some fields the neoplasm was characterized by a high mitotic index and evident cellular atypia. Immunohistochemically, neoplastic cells were positive for CD34, bcl2, CD99, STAT6 and partially for PgR. The neoplasm was diagnosed as a malignant solitary fibrous tumor. CONCLUSIONS: The differential diagnosis of spindle cells tumors arising in the prostrate is broad and includes lesions of epithelial and mesenchymal origin, primary prostatic lesions such as stromal tumors of uncertain malignant potential and stromal sarcoma, as well as anatomically ubiquitous soft tissue neoplasms. Solitary fibrous tumors should be considered in cases of prostatic tumors with a spindled morphology, but malignancy in such tumors is extremely rare in the prostate. A review of literature showed only four additional cases. Because of the unpredictable biological behavior and the possibility of recurrence, a long-term clinical and instrumental follow-up is recommended.

Asunto(s)

Recurrencia Local de Neoplasia/patología , Neoplasias de la Próstata/patología , Neoplasias de la Próstata/secundario , Tumores Fibrosos Solitarios/patología , Biopsia con Aguja/métodos , Diagnóstico Diferencial , Humanos , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia/diagnóstico , Próstata/patología , Neoplasias de la Próstata/diagnóstico , Tumores Fibrosos Solitarios/diagnóstico

RESUMEN

BACKGROUND: In the assessment of a pituitary mass, objective visual field testing represents a valuable means of evaluating mass effect, and thus in deciding whether surgical management is warranted. CASE PRESENTATION: In this vignette, we describe a 73 year-old lady who presented with a three-week history of frontal headache, and 'blurriness' in the left side of her vision, due to a WHO grade III anaplastic haemangiopericytoma compressing the optic chiasm. We report how timely investigations, including an iPad-based visual field test (Melbourne Rapid Field, (MRF)) conducted at the bedside aided swift and appropriate management of the patient. CONCLUSIONS: We envisage such a test having a role in assessing bed-bound patients in hospital where access to formal visual field testing is difficult, or indeed in rapid testing of visual fields at the bedside to screen for post-operative complications, such as haematoma.

Asunto(s)

Computadoras de Mano , Hemangiopericitoma/diagnóstico , Hemianopsia/diagnóstico , Quiasma Óptico/patología , Neoplasias Hipofisarias/diagnóstico , Pruebas en el Punto de Atención , Pruebas del Campo Visual/instrumentación , Anciano , Diagnóstico Diferencial , Femenino , Hemangiopericitoma/complicaciones , Hemianopsia/etiología , Humanos , Imagen por Resonancia Magnética , Neoplasias Hipofisarias/complicaciones , Agudeza Visual , Campos Visuales

RESUMEN

BACKGROUND: To report six cases of CD34+ fibroblastic mesenchymal tumours, which are uncommon neoplasms in the orbit. CASE PRESENTATION: Six patients presenting with proptosis and palpable mass who were later diagnosed with fibrous solitary tumours, fibrous histocytoma or haemangiopericytoma in the orbit were included. All patients received radiologic examinations and surgical excision for histopathology and immunohistochemistry examinations. Five patients had no recurrence after a minimum follow-up of 12 months. One patient (case 6) experienced recurrence twice, and had debulking surgeries each time. At present, the patient still has remnant tumour in the orbit, but no growth has been detected during the past two years. The tumour size will be closely monitored. CONCLUSIONS: Even though fibroblastic tumours are rarely found in the orbit, they can present as a palpable mass with proptosis. Complete surgical excision is important for long-term prognosis, and immunohistochemical study is helpful for confirming pathologic diagnosis.

Asunto(s)

Angiofibroma/diagnóstico , Antígenos CD34/inmunología , Hemangiopericitoma/diagnóstico , Histiocitoma Fibroso Benigno/diagnóstico , Órbita/patología , Neoplasias Orbitales/diagnóstico , Tumores Fibrosos Solitarios/diagnóstico , Adulto , Angiofibroma/complicaciones , Angiofibroma/inmunología , Exoftalmia/diagnóstico , Exoftalmia/etiología , Femenino , Hemangiopericitoma/complicaciones , Hemangiopericitoma/inmunología , Histiocitoma Fibroso Benigno/complicaciones , Histiocitoma Fibroso Benigno/inmunología , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Neoplasias Orbitales/complicaciones , Tumores Fibrosos Solitarios/complicaciones , Tumores Fibrosos Solitarios/inmunología , Adulto Joven