Descriptive epidemiology of 399 histologically confirmed newly diagnosed meningeal solitary fibrous tumours and haemangiopericytomas in France: 2006-2015.

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.

A giant solitary fibrous tumor of the abdominal pelvic cavity: A case report and literature review.

RATIONALE: Solitary fibrous tumor (SFT) is a rare mesenchymal tumor, especially the giant one from the abdominal pelvic cavity. We report on a rare case of a giant SFT of the abdominal pelvic cavity to review the existing literature in detail to improve the diagnosis and treatment of SFT. PATIENT CONCERNS: The patient is a 52-year-old female who presented with 2 weeks of abdominal distension. Abdominal magnetic resonance imaging showed a giant mass (>20 cm) in the abdominal pelvic cavity, considered a mesenchymal tumor. She denies a history of tumor disease. DIAGNOSES: A whole abdomen bulge and a mass of about 18 cm × 10 cm on the right side and middle side were found in the physical examination after admission. Abdominal enhanced computed tomography revealed a giant cystic-solid mass located on the middle and right side of the abdominal pelvic cavity, measuring approximately 20.4 cm × 11.7 cm, with multiple cystic changes and necrosis and compression of adjacent organs and tissues, and marked inhomogeneous enhancement. INTERVENTIONS: The patient underwent an open abdominal pelvic cavity giant tumor operation to achieve a radical resection, and did not undergo chemotherapy or radiotherapy. OUTCOMES: The patient underwent open complete resection of a giant abdominal pelvic tumor with no complications and was diagnosed as SFT according to the pathology, immunohistochemistry showed that the tumor tested positive for CD34(+), STAT-6(+), and Ki-67 (10%). Abdominal computed tomography scans were performed 6 months after resection, and no signs of recurrence or metastasis were found. LESSONS: The clinical symptoms and imaging features of giant abdominal pelvic cavity SFT are not typical. Preoperative diagnosis is difficult and has the potential for malignancy. Based on the results of the current study, there is no standard treatment strategy around the world and the therapeutic effect of radiation therapy and chemotherapy is relatively limited. Thus, complete surgical resection and close clinical follow-up are advocated.

Robotic-assisted laparoscopic resection of a pelvic solitary fibrous tumour causing distal ureteric compression.

This case report describes a male in his late 40s with a 4 cm pelvic mass compressing the left distal ureter, resulting in left hydroureteronephrosis. Biopsy of the mass was suggestive of a solitary fibrous tumour. The patient underwent a robotic-assisted laparoscopic excision of the left pelvic mass. Intraoperatively, the mass was found to be densely adhered to the ureter, necessitating a left distal ureterectomy and ureteric reimplantation. Subsequent histopathological analysis revealed the mass was a solitary fibrous tumour with no evidence of malignancy.

Total management of hemangiopericytoma/solitary fibrous tumor of the buttock: A case report.

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.

Malignant solitary fibrous tumor of the kidney with IGF2 secretion and without hypoglycemia.

BACKGROUND: Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal tumor that mostly involves the pleura and infrequently involves extra-pleural sites. De novo SFT of the kidney is uncommon, and malignant SFT is extremely rare. CASE PRESENTATION: We report a case of a 51-year-old man with a large malignant SFT in the left kidney. Pathological examination confirmed the diagnosis of SFT based on typical morphology, nuclear STAT6 expression, and NAB2-STAT6 gene fusion. The malignant subtype was determined by a large tumor size (≥ 15 cm) and high mitotic counts (8/10 high-power fields). KRAS mutation was identified by DNA sequencing. Insulin-like growth factor 2 (IGF2) was diffusely and strongly expressed in tumor cells, however, hypoglycemia was not observed. Hyperglycemia and high adrenocorticotropic hormone (ACTH) concentration were observed one month after surgery. Hormone measurements revealed normal blood cortisol and aldosterone levels, and increased urinary free cortisol level. A pituitary microadenoma was identified using brain magnetic resonance imaging, which may be responsible for the promotion of hyperglycemia. CONCLUSIONS: We report a case of renal malignant SFT with a KRAS mutation, which was previously unreported in SFT and may be associated with its malignant behavior. Additionally, we emphasize that malignant SFT commonly causes severe hypoglycemia due to the production of IGF2. However, this effect may be masked by the presence of other lesions that promote hyperglycemia. Therefore, when encountering a malignant SFT with diffuse and strong IGF2 expression and without hypoglycemia, other lesions promoting hyperglycemia need to be ruled out.

[Malignant Solitary Fibrous Tumor Compressing the Right Ventricle].

An 84-year-old woman was referred to our department with an abnormal mass detected on a chest computed tomography (CT) scan. The CT scan revealed a tumor between the sternum and the right ventricle, fed by the left internal thoracic artery. Multiple hepatic nodules were also observed. An ultrasound-guided biopsy was performed on the liver nodule, which was diagnosed as a solitary fibrous tumor. The tumor was compressing the heart, and the patient was at risk of sudden death, therefore, a decision was made to resect tumor. Preoperative embolization of the left internal thoracic artery was performed to prevent massive intraoperative bleeding. The tumor was resected via a median sternotomy approach. Intraoperatively, feeding vessels entering the tumor from the diaphragm were also identified. Total blood loss was 70 ml. The postoperative course was uneventful.

Solitary fibrous tumor within the mesorectum: literature review based on a case report of resection by transanal minimally invasive surgery (TAMIS).

PURPOSE: Solitary fibrous tumors (SFT) are a rare entity of in majority benign neoplasms. Nevertheless, up to 20% of cases show a malignant tendency with local infiltration or metastasis. Commonly arising in the thoracic cavity, only few cases of SFT of the mesorectal tissue have been reported in the literature. Complete surgical resection, classically by posterior approach, is the treatment of choice. The purpose of this review is to demonstrate the safety and suitability of transanal minimally invasive surgery (TAMIS) as a surgical approach for the resection of benign pararectal solid tumors. METHODS: We report the case of a 52-year-old man who was diagnosed incidentally with SFT of the distal mesorectum. Resection by TAMIS was performed. Based on this case, we describe the steps and potential benefits of this procedure and provide a comprehensive review of the literature. RESULTS: Histopathology confirms the completely resected SFT. After uneventful postoperative course and discharge on day four, follow-up was recommended by a multidisciplinary board by clinical examination and MRI, which showed a well-healed scar and no recurrence up to 3 years after resection. CONCLUSION: SFT of the mesorectum is a very rare entity. To our knowledge, this is the first report on a TAMIS resection for SFT, demonstrated as a safe approach for complete resection of benign pararectal solid tumors.

Orbital Solitary Fibrous Tumor in a Commercial Airline Pilot.

BACKGROUND: In the literature, central serous retinopathy (CSR) accompanying solitary fibrous tumors (SFT) in a pilot has not been reported. In airline pilots, mass effect-related symptoms such as diplopia, ptosis, etc., seen with orbital tumors may endanger flight safety.CASE REPORT: A 62-yr-old male commercial airline pilot presented with blurred vision in the right eye. He had been receiving treatment for 2 mo because of CSR. His visual acuity was 10/20 in the right eye and 20/20 in the left. During examination, ptosis and exophthalmos were noticed in the right eye. Ocular movements were free in all cardinal directions and there was downward displacement in the right eye. There was no diplopia. Magnetic resonance imaging revealed a 1.5- to 2-cm well-defined contrast-enhancing mass in the lateral extraconal orbit. His medical flight certificate was suspended for 3 mo due to decreased visual acuity and superior visual defect. Superior orbitotomy was performed without any complication. Ptosis and CSR had regressed 1 wk after surgery. All systemic and ophthalmological examinations met aviation medical certificate requirements. He returned to flight on the condition of being checked every 3 mo. At the 1-yr follow-up, there was no sign of recurrences of SFT or CSR.DISCUSSION: SFTs are slow-growing neoplasms that can manifest symptoms related to mass effect. In the current literature, there are no reported cases of the coexistence of orbital SFT and CSR or pilots able to resume flight duties only 1 wk after a successful orbitotomy and tumor resection surgery.Altinbas M, Ozpinar A, Akbaba M, Nacaroglu SA, Sargolzaeimoghaddam M, Sargolzaeimoghaddam M. Orbital solitary fibrous tumor in a commercial airline pilot. Aerosp Med Hum Perform. 2024; 95(6):333-336.

Rare presentation of solitary fibrous tumour in the floor of the mouth.

A male in his 20s, a tobacco chewer, presented to the outpatient department with a history of painless, slowly progressive swelling in the floor of the mouth. After a thorough history and clinical examination, MRI was done and the tumour was completely excised. Histopathological examination revealed the mass to be a solitary fibrous tumour, confirmed with immunohistochemical markers. On subsequent follow-ups, the patient was found to be asymptomatic with no clinical signs of recurrence.

Solitary fibrous tumor of the parapharyngeal space: report of 2 cases and a literature review.

The solitary fibrous tumor (SFT) is usually described as a lesion arising from the pleura. Rarely, it has been described in the parapharyngeal space (PS). This study aims to report two cases of SFT in the PS and to perform a literature review on this topic. Two patients undergoing surgical resection of a SFT in the PS, were reported. A literature review on SFT of the PS, was also performed. Two patients were analyzed. Both patients underwent surgical resection, followed by adjuvant radiotherapy, for SFT arising from the PS. The postoperative course was uneventful and both patients recovered well after the procedure. No recurrences were diagnosed during the followup. SFT of the PS is an infrequent entity. Surgical resection is the most used treatment, and adjuvant radiation should be considered in patients with recurrence risk factors or distant metastases.

Solitary fibrous tumor occurring in the colon as submucosal mesenchymal lesion: report of two cases and review of the literature.

Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm most often arising from the pleura and rarely in extra-pleural locations, including the gastrointestinal tract. We describe two cases of a SFT presenting as submucosal colonic lesion and review the literature on this lesion. One submucosal lesion was localized in the cecum and was 10 mm in size. The second lesion presented as a 17 mm submucosal rectal lesion. Both lesions presented as well-circumscribed submucosal lesions arranged in short fascicles, blending with abundant collagenous stroma. In both cases, the spindle cells were positive for CD34, STAT6 and CD99, and molecular studies showed NAB2:STAT6 fusion supporting the diagnosis of SFT. Both patients are alive and well 10 and 5 years post-excision, respectively. In conclusion, SFT can occur in the colon as a submucosal lesion and should be included in the differential diagnosis of colonic mesenchymal lesions.

Conditional survival analysis and dynamic survival prediction for intracranial solitary-fibrous tumor/hemangiopericytoma.

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.

Surgical Management of Craniospinal Axis Solitary Fibrous Tumors: A Single-Institution Case Series and Comprehensive Review of the Literature.

BACKGROUND AND OBJECTIVES: Meningeal solitary fibrous tumors (SFTs) comprise 0.4% of primary central nervous system neoplasms and carry metastatic potential. Disease course and optimal management are largely unknown, and there is currently no literature rigorously describing neurological outcomes in surgically managed SFTs. We present one of the largest craniospinal SFT series, analyze patient outcomes, and extensively review the associated literature. METHODS: All surgically managed SFTs at our institution between January 2005 and March 2023 were retrospectively reviewed. Patient demographics, tumor and radiographic features, treatment, and clinical outcomes were collected. Neurological function was quantified using Frankel grade and Neurologic Assessment in Neuro-Oncology scores. Descriptive statistics, multivariate analysis, log-rank test, and Kaplan-Meier survival analysis were performed. RESULTS: Twenty-one patients satisfied inclusion criteria. Tumor locations included 15 supratentorial, three infratentorial, and three spinal. All patients underwent surgical resection, and 16 (76.2%) underwent radiation. Six (28.6%) patients had tumor recurrence, and three (14.3%) developed metastasis. Younger age and higher postoperative Frankel grade were significantly associated with increased overall survival (OS) ( P = .011, P = .002, respectively). All patients symptomatically improved or stabilized after surgery, and Neurologic Assessment in Neuro-Oncology score ( P = .001) and functional status significantly improved postoperatively (Karnofsky Performance Status: 65.2 ± 25.2 vs 91.4 ± 13.5, P = .001). Sex, adjuvant radiation, and extent of resection were not significantly associated with OS. CONCLUSION: SFT of the central nervous system is a rare entity with a variable clinical course. Surgical resection was associated with improved postoperative functional and neurological status. Higher postoperative neurological function was significantly associated with OS. Further studies are warranted to validate a standardized treatment algorithm and investigate the efficacy of adjuvant radiation in SFT.