Incidence and time trends of sarcoma (2000-2013): results from the French network of cancer registries (FRANCIM).

BACKGROUND: The exhaustive collection of new sarcoma cases and their second histologic review offer a unique opportunity to study their incidence and time trends in France according to the major subtypes. METHODS: Data were collected from population-based cancer registries covering 22% of the French population. Crude and world age-standardized incidence rates (ASR) were estimated according to anatomic, histological and genetic groups, age and sex over the 2010-2013 period. RESULTS: Time trends in incidence were calculated by the annual percent change over the 2000-2013 period. During the most recent period (2010-2013), 3942 patients with sarcoma were included. The ASR of soft-tissue and bone sarcomas, and gastro-intestinal stromal tumors (GIST) were 2.1, 1.0 and 0.6, respectively. For the four most frequent histological subtypes (unclassified, leiomyosarcoma, GIST and liposarcoma), the ASR ranged from 0.4 to 0.7. ASRs were 1.9 for complex genomic and 1.3 for recurrent translocation sarcomas. The time-trend analysis showed a significant increase of sarcoma incidence rate between 2000 and 2005, which stabilized thereafter. Incidence rates increased for four histological subtypes (GIST, chondrosarcoma, myxofibrosarcoma, solitary fibrous tumors) and decreased for three (leiomyosarcomas, Kaposi sarcoma and fibrosarcoma). CONCLUSION: To our knowledge, this study is the first to investigate sarcoma incidence based on a systematic pathological review of these cancers and on the updated sarcoma classifications. Due to the paucity of literature on sarcomas, future studies using data from population-based cancer registries should consider a standardized inclusion criterion presented in our study to better describe and compare data between countries.

Surveillance for metastatic hemangiopericytoma-solitary fibrous tumors-systematic literature review on incidence, predictors and diagnosis of extra-cranial disease.

BACKGROUND: Intracranial hemangiopericytomas (HPC) and solitary fibrous tumors (SFTs) (HPC-SFT) are rare vascular tumors that resemble meningioma on imaging and predominantly affect young adults. HPC-SFT have a high rate of local recurrence with well-known propensity for extracranial metastases. This provides clinical dilemmas frequently encountered in oncology: (i) How should these patients be monitored long term? (ii) Which primary tumors are more likely to metastasize? OBJECTIVES: This systematic review aims to identify the incidence, common locations and time to presentation of extra-cranial metastases of HPC-SFT. We will assess the effect of primary tumor location, treatment, grade, patient age, gender and effect of local recurrence on rates of extra-cranial metastasis and discuss the ideal techniques by which patients with intracranial HPC-SFT should be monitored for extra-cranial metastases. METHODS: Using PRISMA guidelines the authors searched Pubmed. Search terms included hemangiopericytoma, HPC, solitary fibrous tumor/ tumour, SFT, HPC-SFT, extra-cranial metastases, metastases, recurrence, monitoring, follow-up. Studies were identified up to 1st February 2018. Reference lists of identified articles were reviewed to detect other relevant citations. Data were extracted using a standard data collection form and results organized into (i) general study/patient characteristics, (ii) location of extra-cranial metastases, (iii) methods by which metastases were detected and followed up and (iv) characteristics of primary tumors. RESULTS: Seventy-one studies were identified. Mean recorded follow up ranged from 4 to 312 months. Mean age at diagnosis was 42.0 years. The overall rate of extra-cranial metastasis was 28% (n = 251/904). The minimum time to extracranial metastases was 3 months and the maximum time was 372 months. In the 71 studies identified, where site of extra-cranial metastasis was specified, there were 347 metastases in 213 patients. The most common sites for metastases were bone (location not specified) (19.6%) followed by lung and pleura (18.4%), liver (17.6%), and vertebrae (14.1%). Extra-cranial metastatic disease is typically diagnosed following symptomatic presentation. There is little documentation of methods used to monitor patients with extra-cranial HPC-SFT and no clear surveillance paradigm observed. Higher primary tumor grade (WHO Grade III) was associated with a 1.88 (p = 0.016) increased risk of extra-cranial metastasis. Location and treatment of primary tumor, local recurrence, patient age and gender were not. CONCLUSION: Patients with intracranial HPC-SFT require periodic, long term monitoring for extra-cranial metastases. Metastases occur in any age group and can occur early and late. They vary in location and are typically diagnosed following symptomatic presentation. There is no suggested imaging modality for surveillance. Higher grade primary tumors have a greater risk of metastasis. Regular clinical review is essential with early imaging for symptoms of recurrence/metastasis with imaging modality dependent on clinical concern. Quality evidence for an imaging surveillance protocol in this heterogeneous group of patients is lacking. A multicenter study on appropriate surveillance may be of benefit.

Solitary-fibrous tumor/hemangiopericytoma of the central nervous system: a population-based study.

The World Health Organization (WHO) classification of tumors of the central nervous system (CNS) was recently updated, restructuring solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) into one combined entity. This is the first population-based study to examine outcomes of SFT/HPC based on the new WHO guidelines. The Surveillance, Epidemiology, and End Results (SEER) database (1998-2013) was queried to examine age-adjusted incidence and prognostic factors associated with overall survival in 416 surgically resected cases. Age-adjusted incidence was calculated to be 3.77 per 10,000,000 and was rising. Median survival was 155 months, with 5- and 10-year survival rates of 78 and 61%, respectively. Younger age, Asian/Pacific Islander versus white race, benign histology, tumor location, gross-total resection (GTR), and GTR plus radiation (RT) versus subtotal resection were significantly associated with survival. In multivariable analysis, older age (HR = 1.038, p < 0.0001), infratentorial location (HR = 2.019, p = 0.038), GTR (HR = 0.313, p = 0.041), and GTR + RT (HR = 0.215, p = 0.008) were independent prognostic factors. In the HPC and borderline/malignant subgroups, GTR + RT was associated with significantly increased survival compared with GTR alone (HR = 0.537, p = 0.039 and HR = 0.525, p = 0.038). After eliminating patients that died within 3 months of diagnosis, GTR + RT was still associated with an incremental increase in survival (HR = 0.238, p = 0.031) over GTR alone (HR = 0.280, p = 0.054). GTR + RT may be optimal in the management CNS HPC and SFT/HPC tumors with borderline/malignant features. This study, in combination with existing literature, warrants further investigation of adjuvant radiation through a prospective clinical trial.

Intrathoracic solitary fibrous tumor - an international multicenter study on clinical outcome and novel circulating biomarkers.

Intrathoracic solitary fibrous tumor (SFT) is a rare disease. Radical resection is the standard of care. However, estimating prognosis and planning follow-up and treatment strategies remains challenging. Data were retrospectively collected by five international centers to explore outcome and biomarkers for predicting event-free-survival (EFS). 125 histological proven SFT patients (74 female; 59.2%; 104 benign; 83.2%) were analyzed. The one-, three-, five- and ten-year EFS after curative-intent surgery was 98%, 90%, 77% and 67%, respectively. Patients age (≥59 vs. <59 years hazard ratio (HR) 4.23, 95 confidence interval (CI) 1.56-11.47, p = 0.005), tumor-dignity (malignant vs. benign HR 6.98, CI 3.01-16.20, p <0.001), tumor-size (>10 cm vs. ≤10 cm HR 2.53, CI 1.10-5.83, p = 0.030), de Perrot staging (late vs. early HR 3.85, CI 1.65-8.98, p = 0.002) and resection margins (positive vs. negative HR 4.17, CI 1.15-15.17, p = 0,030) were associated with EFS. Furthermore, fibrinogen (elevated vs. normal HR 4.00, CI 1.49-10.72, p = 0.006) and the neutrophil-to-lymphocyte-ratio (NLR > 5 vs. < 5 HR 3.91, CI 1.40-10.89, p = 0.009) were prognostic after univariate analyses. After multivariate analyses tumor-dignity and fibrinogen remained as independent prognosticators. Besides validating the role of age, tumor-dignity, tumor-size, stage and resection margins, we identified for the first time inflammatory markers as prognosticators in SFT.

Prediction of local and metastatic recurrence in solitary fibrous tumor: construction of a risk calculator in a multicenter cohort from the French Sarcoma Group (FSG) database.

BACKGROUND: Solitary fibrous tumors (SFT) are rare unusual ubiquitous soft tissue tumors that are presumed to be of fibroblastic differentiation. At present, the challenge is to establish accurate prognostic factors. PATIENTS AND METHODS: A total of 214 consecutive patients with SFT diagnosed in 24 participating cancer centers were entered into the European database (www.conticabase.org) to perform univariate and multivariate analysis for overall survival (OS), local recurrence incidence (LRI) and metastatic recurrence incidence (MRI) by taking competing risks into account. A prognostic model was constructed for LRI and MRI. Internal and external validations of the prognostic models were carried out. An individual risk calculator was carried out to quantify the risk of both local and metastatic recurrence. RESULTS: We restricted our analysis to 162 patients with local disease. Twenty patients (12.3%) were deceased at the time of analysis and the median OS was not reached. The LRI rates at 10 and 20 years were 19.2% and 38.6%, respectively. The MRI rates at 10 and 20 years were 31.4% and 49.8%, respectively. Multivariate analysis retained age and mitotic count tended to significance for predicting OS. The factors influencing LRI were viscera localization, radiotherapy and age. Mitotic count, tumor localization other than limb and age had independent values for MRI. Three prognostic groups for OS were defined based on the number of unfavorable prognostic factors and calculations were carried out to predict the risk of local and metastatic recurrence for individual patients. CONCLUSION: LRI and MRI rates increased between 10 and 20 years so relapses were delayed, suggesting that long-term monitoring is useful. This study also shows that different prognostic SFT sub-groups could benefit from different therapeutic strategies and that use of a survival calculator could become standard practice in SFTs to individualize treatment based on the clinical situation.

The demographic features, clinicopathologic characteristics, treatment outcome and disease-specific prognostic factors of solitary fibrous tumor: a population-based analysis.

BACKGROUND: Solitary fibrous tumor's (SFT) demographic features, clinicopathologic characteristics, treatment outcome and disease-specific prognostic factors were unexplored comprehensively. METHODS: SEER program was used to identify patients diagnosed with SFT from 1973 to 2012. Overall collected data were analyzed by using the SPSS 18.0. RESULTS: In total, 804 cases were found including 613 cases with SFT-specific mortality and 801 patients were analyzed for overall survival (OS). The 3-year disease specific survival (DSS), 5-year DSS and 10-year DSS were 73.3%, 65.7% and 53.3%. The 3-year OS, 5-year OS and 10-year OS were 71.9%, 63.3% and 47.3%. In the multivariate survival analysis, the age > 51 years (hazard ratio [HR] = 1.851 for DSS, P = 0.024 and HR = 1.652 for OS, P = 0.033; Reference [Ref] ≤ 51 years for DSS and ≤ 53 years for OS), SEER stage metastasized tumor (HR = 4.269 for DSS, P = 0.000 and HR = 2.905 for OS, P = 0.028, Ref - localized + regional tumor), pathologic grade III + IV (HR = 2.734 for DSS, P = 0.001 and HR = 2.585 for OS, P = 0.000, Ref - grade I + II) were adversely associated with DSS and OS. In addition, surgery was favorably associated with DSS (HR = 0.217, P = 0.045, Ref - surgery + radiotherapy). CONCLUSIONS: The surgery was an independent prognostic factor for DSS. The patient's age, SEER stage and pathologic grade were SFT-specific independent prognostic indicators for DSS and OS.

Clinical Characteristics and Outcomes for Solitary Fibrous Tumor (SFT): A Single Center Experience.

Solitary fibrous tumor (SFT) is a mesenchymal neoplasm of fibrous origin. The 2013 WHO classification of soft tissue tumors defines malignant forms as hypercellular, mitotically active (>4 mitosis/10 high-power fields), with cytological atypia, tumor necrosis, and/or infiltrative margins. With an IRB-approved protocol, we investigated patient records and clinicopathologic data from our Sarcoma Database to describe the clinical characteristics of both benign and malignant SFT. All pathology specimens were reviewed by two pathologists. Descriptive statistics and univariate/multivariate survival analysis were performed. Patient records and Social Security Death Index were used to evaluate vital status. Of 82 patients, 47 (57%) were women and 73 (89%) were Caucasian. Median age was 62 years (range, 20 to 89). Thirty-two (39%) patients succumbed to the disease. Primary tumor site was lung/pleura in 28 (34%), abdomen/pelvis in 23 (28%), extremity in 13 (16%), and head/neck in 9 (11%) patients. Pathology was described as benign in 42 (51%) and malignant in 40 (49%) patients. Compared to benign SFT, malignant histology is associated with larger tumor size, higher mitotic counts, metastatic disease at diagnosis, and greater use of chemotherapy and radiation therapy. Gender, age, and tumor site were not significantly different between benign and malignant subtypes. By univariate analysis, only benign vs. malignant variant and complete resection positively impacted overall survival (P = 0.02 and P<0.0001, respectively). In the multivariable analysis of overall survival, receiving chemotherapy or not receiving surgery were two variables significantly associated with higher failure rate in overall survival: patients with chemotherapy vs. no chemotherapy (P = 0.003, HR = 4.55, with 95% CI: 1.68-12.34) and patients without surgery vs. with surgery (P = 0.005, HR = 25.49, with 95% CI: 2.62-247.57). Clear survival differences exist between benign and malignant SFT. While surgery appears to be the best treatment option for benign and malignant SFT, better systemic therapies are needed to improve outcomes of patients with metastatic, malignant SFT.

Primary cardiac solitary fibrous tumors.

Primary cardiac solitary fibrous tumors were reviewed. They are classified as pericardial tumors. Their incidences are very rare. Only 16 cases were reported in the literature. Basically, surgical treatments are performed. Their prognoses are generally good, although malignant cases are also reported.

Solitary fibrous tumor of the liver: report of two cases and review of the literature.

A solitary fibrous tumor (SFT) of the liver is a rare neoplasm of mesenchymal origin. 59 cases have been reported in the literature. We report 2 patients who presented with a hepatic solitary fibrous tumor. The first case is a 65-year-old man who presented with an accidental finding of a large mass in the left liver. Biopsy revealed an SFT and left hepatectomy was performed. The diagnosis was confirmed by histopathology. The second case is an 87-year-old woman who presented with disturbances in her liver function tests. A Computed Tomography (CT) scan showed a large mass in the right liver. Surgery was contraindicated because of the patient's poor general condition. A biopsy was done and SFT was diagnosed histopathologically. SFT are usually benign but the risk of malignant transformation always exists, which mandates surgical resection as the optimal management of these tumors. However, because of the small sample size and the rarity of the entity, it is difficult to define the evolution, the risk factors and the malignant potential of these tumors.

[Solitary fibrous tumor of the orbit: Possibly recurrent in the long-run]. / Les tumeurs fibreuses solitaires de l'orbite : une entité pouvant récidiver à long terme.

Solitary fibrous tumor (SFT) is a rare etiology of progressive unilateral exophthalmia. The tumor is of mesenchymal origin and it is usually well defined. But recurrences can occur despite of complete surgical resection. Metastases have been observed. Tumors of the SFT spectrum are considered as benign or low-grade malignant. Histological features do not currently allow any prognosis. The most important prognostic factor is complete surgical resection. Craniofacial approaches provide a good view of the tumor extensions and orbital contents. Recurrent tumors must be surgically removed when possible. Complementary treatments have not proved effective. A very long-term follow-up is mandatory.

Thoracic neoplasms at the Jena reference center for soft tissue tumors.

INTRODUCTION: The Jena Soft Tissue Tumor Reference Center is the major German pathology institute for consultation of malignant mesenchymal tumors. Here, we present the clinicopathological data on thoracic soft tissue tumors of a two-year period. METHODS: The tumors were analyzed according to their localization, type (soft tissue tumor, other tumor type, non-neoplastic lesion) and biological behavior. The frequency of the defined soft tissue tumor entities were considered after categorizing the cases according to the WHO Classification of Tumors. Gender and age were also assessed. RESULTS: In total, 1,071 cases of thoracic tumors were recorded within the 2 years. The majority were non-epithelial lesions (75.3%, n = 806/1,071), of which 68.1% (n = 549/806) were malignant or intermediate malignant. 107 non-epithelial lesions involved the lung and 37 the pleura. By far the most common lung and pleural tumors were undifferentiated sarcomas. In the lung, other frequent entities were solitary fibrous tumors, synovial sarcomas and leiomyosarcomas. We also recorded 13 non-epithelial tumors of the heart and 66 tumors of the breast with angiosarcomas being the most frequent subtype. There was a female predominance for vascular neoplasms, while men prevailed for adipocytic tumors. CONCLUSIONS: Our study provides information about the frequency, distribution, age and gender of patients with thoracic soft tissue tumors including several rare entities. Thus, it may help in the differential diagnosis of these neoplasms. In addition, we present a model highlighting the potential progression of lung carcinoma to undifferentiated sarcoma via the process of epithelial-mesenchymal transition.

The central nervous system solitary fibrous tumor: a review of clinical, imaging and pathologic findings among all reported cases from 1996 to 2010.

OBJECTIVE: Central nervous system (CNS) solitary fibrous tumor (SFT) is a rare lesion first identified as a unique entity in 1996. We describe two cases treated at the University of Florida followed by a review of all reported cases of CNS SFT between 1996 and 2010. METHODS: A review of the literature was performed to identify all reported cases of CNS SFT. RESULTS: 189 cases (including the two presented herein) were discovered, of which 46 were spinal and 143 were intracranial. Demographic, imaging, and pathologic findings are presented. Roughly 6% of reported lesions are malignant. Subtotal resection (STR) was associated with a 16-fold increased odds of recurrence (OR 15.9, 95% CI 5.5-46.1), although mean follow-up was shorter in those cases of GTR without recurrence. CONCLUSION: CNS SFT is a rare lesion. Six percent of lesions are malignant. GTR is superior to STR although the degree of superiority is not clear.

[Solitary fibrous tumor. Clinic and pathological study of 16 cases].

BACKGROUND: Solitary fibrous tumor (SFT) is a localized fibrous mesothelioma and was originally described as a benign pleural lesion. It is a mesenchymatous lesion that has been found in different structures and tissues of the human organism. It originates from a stromal fibroblastic cell positive to CD4. We undertook this study to determine the clinical and histopathological characteristics of SFT found from January 1, 2002--January 31, 2010 in a concentrated third-level general hospital in Mexico City. METHODS: We present 16 resected cases of different localizations: three pleural, three mediastinal, three lung, two oral cavity, one nasopharyngeal, one in the lateral aspect of the neck, one kidney, one paratesticular and another in the crural region. All were studied with hematoxylin and immunoperoxidase. RESULTS: There was similar affection in both genders. Mean age was 51 years (range: 43-81 years). Two pleural cases presented recurrence and one had hypoglycemia but none demonstrated metastases. No deaths were registered during a mean follow-up of 18 months. Histological pattern corresponded to a fusocellular growth that coincided with hemangiopericytoid zones in five cases and sarcomatoid in four. These cases were considered as mixed. All were positive to vimentin. CONCLUSIONS: Definitive diagnosis must be made by immunoperoxidase. Immediate treatment is surgical resection; relapses.

Concurrent solitary fibrous tumor and low-grade fibrillary astrocytoma of the cerebellum.

OBJECTIVE: We report the clinicopathologic features of a solitary fibrous tumor (SFT) having undergone malignant transformation and being intimately associated with a WHO Grade II astrocytoma. CLINICAL PRESENTATION: A 7-month old patient presented with delayed motor development and hydrocephalus. INTERVENTION: Histologic and immunocytologic methods were applied in the study of the tumors. Resection was initially employed and the SIOP protocol employing vincristine and carboplatin was applied upon tumor recurrence. CONCLUSION: The biologic basis for the association of SFT and astrocytoma is unknown. The complex lesion differs substantially from WHO Grade IV gliosarcoma and from gliofibroma, lesions in which the disparate elements are linked by metaplasia. Indeed, it may represent a collision tumor. Lastly, induction of the glioma by the solitary fibrous tumor, a mechanism invoked to explain the poorly understood "sarcoglioma," deserves consideration.

Giant solitary fibrous tumor of the pleura.

BACKGROUND: Solitary fibrous tumor is the second primary malignancy of the pleura and can reach up to 39 cm in diameter; however, to be referred to as 'giant' it must occupy at least 40% of the affected hemithorax. Although this tumor usually shows a benign behavior, malignancy criteria have been described. The aim of the study was to assess the initial evaluation, diagnostic procedures, surgical management, treatment outcome, and prognosis. METHODS: We performed a descriptive, observational, longitudinal, and retrospective study from 2002 to 2006 on patients who underwent surgery with a diagnosis of giant solitary fibrous tumor of the pleura. RESULTS: Six patients were included; 83.3% were females. Mean age was 48 years. All patients were symptomatic, mainly dyspnea, cough and chest pain; 66.7% were left-sided. Preoperative angiography and embolization were performed in 83.3% cases with successful surgical resection. The predominant blood supply was derived from the internal mammalian artery. Intraoperative complication rate was 17%. A vascular pedicle was found in 66.7%. The largest lesion was 40 cm in diameter and weighed 4500 g. Only one case showed high mitotic activity. Mean follow-up to date is 14 months. CONCLUSIONS: Symptomatology found was consistent with previous reports but in higher percentages. Accurate diagnosis is critical because surgical resection involves a potential cure; however, long-term follow-up is mandatory. Preoperative embolization is recommended due to tumor size.

Meningeal hemangiopericytomas and hemangiopericytoma/solitary fibrous tumors of extracranial soft tissues: a comparison.

The current World Health Organization (WHO) classification of central nervous system tumors lists meningeal hemangiopericytomas (HPC) and meningeal solitary fibrous tumors (SFT) as separate entities. On the contrary, SFT and HPC of soft tissues are regarded in the WHO soft tissue fascicle as features of the same entity. The clinical data, histology, and immunohistochemistry of 18 cases of meningeal HPC and 12 cases of peripheral soft tissue HPC-SFT were compared. Both intracranial and soft tissue lesions had significant similarities that included staghorn vasculature, necrotic areas, cytologic atypia, and positivities for CD99, collagen IV, and reticulin. Nevertheless, intracranial tumors were more cellular than HPC-SFT of soft tissues and had fewer collagen bands. Meningeal HPC in addition had more mitoses, higher Ki67 index, stained less intensely for CD34 and B-cell lymphoma 2 (BCL2) than HPC-SFT of soft tissues. Meningeal HPCs recurred in 13 out of 14 cases (92.9%). One of the patients died in the postoperative period for a recurrent lesion 5 years after the diagnosis, and another patient developed an extracranial metastasis 13 years after surgery. None of the six cases of HPC-SFT of soft tissues available for follow-up recurred. Both meningeal and soft tissue tumors appear to represent different features of the same entity. A more aggressive phenotype of the tumor together with incomplete surgical resection of intracranial lesions might explain the noticeable clinical difference between HPC of the meninges and HPC-SFT of soft tissues.

Orbital solitary fibrous tumor. Another rare case from Africa.

Solitary fibrous tumor (SFT) of the orbit, initially thought to be rare, is being increasingly documented in the medical literature. We here present the case of a 37-year-old Nigerian man who presented with left sided painless, vision-sparing proptosis. Cranial computed tomography scan showed a globular, left medial, orbital extraconal mass. The mass was excised, en bloc, via a medial orbitotomy procedure aided by left ethmoidectomy. Surgery was uncomplicated and histology of the excised tissue was reported as compatible with SFT. There was also a strong and diffuse immunostaining with CD 34. At 6-month follow-up, the left-sided proptosis has completely regressed. To the best of our knowledge, this is the second case of orbital SFT to be reported in an African.