Malignant Solitary Fibrous Tumor Research Articles

Rarest of the Rare – Malignant Solitary Fibrous Tumour of the Breast: Case Report and Literature Review

Rarest of the Rare – Malignant Solitary Fibrous Tumour of the Breast: Case Report and Literature Review

[Clinicopathological analysis of adult hepatic mesenchymal hamartoma].

Objective: To explore the clinicopathological and molecular genetic features of adult hepatic mesenchymal hamartoma (MHL). Methods: A total of five confirmed adult MHL cases diagnosed at the Pathology Department of the First Medical Center of the People's Liberation Army General Hospital between 2009 and 2022 were collected. Histomorphological observation and immunohistochemical staining were conducted. Gene detection was performed by next-generation sequencing. Results: Among the five cases, four were male and one was female, aged 46-67 years, with an average age of 56.2 years. The maximum diameter was 5.3-13.5cm, and the average diameter was 9.2cm. Tumors were generally cystic, solid, or mixed cystic-solid. Histopathologically, in four out of five cases of MHL, malignant transformation occurred, of which three cases were malignantly transformed into undifferentiated embryonal sarcoma and one case was malignantly transformed into a malignant solitary fibrous tumor. NAB2-STAT6 gene rearrangements were identified. Conclusion: Adult MHL is a rare kind of tumor with malignant potential, and it is difficult to diagnose with preoperative imaging examinations. A fine-needle biopsy is rarely used for diagnosis, but surgical resection of symptomatic or enlarged lesions is recommended to rule out the possibility of malignancy and further diagnosis. Genetic testing results revealed the complex genetic alterations in MHL, and it was found that adult MHL can malignantly transform into malignant solitary fibrous tumors. We believe that genome-wide analysis is necessary to determine the unique molecular characteristics of MHL and identify potential targets for therapeutic intervention.

Prognostic analysis of extrameningeal solitary fibrous tumor using the modified Demicco model: a clinicopathologic study of 111 Chinese cases.

Solitary fibrous tumor (SFT) represents a fibroblastic neoplasm exhibiting NAB2::STAT6 gene rearrangement, displaying diverse clinical manifestations, spanning from benign to malignant. To predict prognosis, the modified (four-variable) Demicco (mDemicco) model was introduced. This investigation aims to authenticate the mDemicco risk model's precision in Asian patients while investigating the clinicopathological and molecular factors linked to the prognosis of extrameningeal SFTs. Clinicopathological data from 111 extrameningeal SFT cases in East China, covering the period from 2010 to 2020, were thoroughly analyzed. The tumors were classified using the mDemicco model. Immunohistochemical evaluation of P16 and P53, molecular detection of TP53 and TERT promoter mutation, and fluorescence in situ hybridization for CDKN2A gene alterations were performed. Statistical methods were utilized to assess the associations between clinicopathological or molecular factors and prognosis. Histologically, only one parameter, the mitotic count, exhibited a statistical correlation with progression-free survival (PFS) and overall survival (OS). During the Kaplan-Meier analysis, the variation in PFS among the different risk groups exhibited a notable trend towards statistical significance. Nevertheless, 3 out of 74 patients classified as low-risk SFTs and 7 out of 21 patients classified as intermediate-risk exhibited disease progression. Among the 5 patients with TP53 mutations and/or mutant-type P53 immunophenotype, 3 experienced disease progression, including 2 intermediate-risk patients. Additionally, among the 4 patients with TERT promoter mutations who were followed up, 3 showed progression, including 2 intermediate-risk patients. Moreover, it was observed that hemizygous loss of CDKN2A was detected in more than 30% of one case, yet the patient exhibited a favorable survival outcome. The mDemicco risk model exhibits certain limitations when dealing with smaller tumor sizes, younger age groups, and occurrences of malignant and dedifferentiated SFTs. Furthermore, molecular factors, such as TP53 or TERT promoter mutations, may identify intermediate-risk SFTs with poorer prognoses.

Pleural malignant fibrosolitary tumor: A rare entity

Solitary fibrous tumors of the pleura represent a rare histological entity, belonging to the groups of pleural tumors of sub-mesothelial origin , therefore some authors evoke the hypothesis of its proliferation from submesothelial tissue of the pleura .The clinical manifestations are varied and the certain diagnostic can only be made by anatomopathological examination which evaluates precise malignancy criteria. Total surgical removal is the only way to ensure a definitive cure. We report the case of 65-year-old woman with a malignant solitary fibrous tumor operated by thoracotomy with complete resection. Through this presentation, we would like to underline the rarity of this entity

A Rare Case of Malignant Solitary Fibrous Tumor on the Scalp

Solitary fibrous tumor (SFT) is an uncommon tumor that typically originates in the pleural cavity but may also be found in extra-pleura sites like the head and neck, spine, lungs, mediastinum, peritoneum, and pelvis. There are few reports of SFT of the scalp, and malignant SFT of the scalp appears to be so rare that only two cases of it were reported previously. A 47-year-old woman was admitted to our institution with a large, rapidly growing mass on the left parietal scalp. An analysis of her scalp condition and imaging findings of the mass revealed two different appearances between the superior and inferior halves; the scalp of the inferior half looked normal, but that of the superior half did not. Also, computed tomography findings of the mass confirmed different patterns between the superior and inferior halves. The mass with the abnormal superior half scalp was removed totally. The pathological diagnosis was malignant SFT, but the inferior mass was revealed to have a benign nature. The patient underwent prophylactic radiotherapy and experienced no local recurrence or distant metastasis at the final follow-up at 12 months. We present a third case of malignant SFT originating on the scalp and describe our clinical and surgical experience managing malignant SFT of the scalp.

SAT477 Consumptive Hypothyroidism In A Patient With Plasma Cell Neoplasm

Abstract Disclosure: G. Sidhu: None. I. Madahar: None. A. Haider: None. J. Giordano: None. Introduction: Thyroxine (T4) is converted into metabolically active form triiodothyronine (T3) by deiodinase enzyme 1 & 2 (D1 & D2). Deiodinase 3 converts T4 and T3 into metabolically inactive form reverse T3 (rT3)& di-iodothyronine (T2) . Consumptive hypothyroidism syndrome (CHS) is a very rare form of hypothyroidism seen in patients with neoplasms, which express high levels of D3 enzyme. Case: 68-year-old female withhistory of Multiple Myeloma (MM), POEMS, hyperlipidemia and hypothyroidism was admitted with acute kidney injury, cirrhosis of unclear etiology, large volume ascites and anasarca. She weighed 92 kg on admission. She was complaint with her 50 mcg of levothyroxine and on admission her TSH was 7.14 (N: 0.43-3.55 uIU/ml), Free T4 (FT4): <0.42 (N: 0.7-1.25 ng/dl), total T4 (TT4): 2.6 (N: 4.8-11.7 ug/dl), total T3 (TT3): <20 (N: 80-200 ng/dl). Oral LT4 was switched to IV form due to underlying concerns of malabsorption from anasarca. rT3 was noted to be 40 (N: 8-25 ng/dl). Despite escalated intravenous levothyroxine doses for seven days her FT4, FT3 continued to remain undetectable So her LT4 dose was increased to 75 mcg IV daily initially and eventually to 100 mcg IV daily and 125 mcg daily. On 10th day of hospitalization, after her IV LT4 dose was increased to 200 mcg daily, (almost 2.5 mcg/kg/day) her FT4 levels became detectable, 0.50, rT3: 68 and TT3: <20 and TT4: 3.1. Since her rT3 levels were noted to rise after increasing the dose of LT4, liothyronine (LT3) 5 mcg BID was added to her regimen. PET scan showed small sclerotic bone lesions suspicious for active malignancy. Chromogranin A was elevated 313 (N: <311 ng/ml). IR guided biopsy of scapular sclerotic lesion was done and specimen was sent University of Chicago to measure DI-3 activity in the specimen Pathology results were suggestive of plasma cell neoplasm but unfortunately deiodinase activity could not be quantified. Patient developed hepato-renal syndrome during hospitalization and her family decided to pursue hospice care. Discussion: Critical care illness thyroid disease is also associated with increased DI-3 activity and increases rT3, however T4 and TSH are also low in the setting of critical care illness, and most patient do not require supraphysiologic T4 replacements. The presence of D3 in normal tissues such as the brain & placenta has been hypothesized to protect tissues from the effects of excessive thyroid hormone. However, aberrant expression of the enzyme can lead to severe hypothyroidism from inactivation of both endogenously produced and exogenously administered thyroid hormone. Previously this rare syndrome was described in association with massive hemangiomas in children and in a single case of a hemangioendothelioma in an adult. However case reports in adults have been described in nonvascular tumors who required supraphysiologic doses of levothyroxine prior to the resection of a large malignant solitary fibrous tumor. Presentation Date: Saturday, June 17, 2023

Long-Term Surgical Outcome for Orbital Solitary Fibrous Tumors.

To report long-term outcomes after surgery for orbital solitary fibrous tumors. 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. 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. 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.

A diagnostic challenge; pelvic solitary fibrous tumor (SFT) mimicking Peri‐Anal gastrointestinal stromal tumor (GIST): A case report

Key Clinical MessageThe study reports a rare case of pelvic solitary fibrous tumor (SFT) that was initially considered as a peri‐anal gastrointestinal stromal tumor due to similar radiologic/pathologic features. SFT diagnosis can be challenging due to its rarity and wide range of diagnoses that must be ruled out precisely.AbstractSolitary fibrous tumors (SFTs) are rare tumors that can occur in any part of the body. Although usually benign, malignant SFTs have been reported, especially outside the lungs. Radiology can help with diagnosis, but immunohistochemistry is necessary to distinguish SFTs from other possible diagnoses such as gastrointestinal stromal tumors (GISTs). This study presents a rare case of pelvic SFT initially considered to be a peri‐anal GIST, highlighting the importance of accurate diagnosis given the rarity of SFTs and the need to rule out other potential diagnoses.

Malignant Solitary Fibrous Tumours of the Pleura Are Not All the Same: Analysis of Long-Term Outcomes and Evaluation of Risk Stratification Models in a Large Single-Centre Series.

Introduction: Malignant solitary fibrous tumours of the pleura (mSFTP) are extremely rare diseases (<5% of all pleural neoplasms) with unpredictable behaviour. Surgery remains the standard of care for these tumours; however, estimating patient prognosis and planning follow-up remain challenging. Several risk stratification models have been proposed, but a classification with diagnostic and prognostic potential has not been well standardised yet. The aim of this study was to analyse the clinicopathological data of mSFTP to investigate their prognostic features and to compare the performance of three risk stratification models proposed in the literature. Methods: Observational retrospective cohort study on all proven cases of mSFTP surgically resected with radical intent between 2000 and 2019 in a single centre. Demographic, surgical and pathological data were examined. All patients were risk-stratified by using three prediction models: modified Demicco, De Perrot and Tapias. Overall survival (OS) and disease-free survival (DFS) were analysed. Results: There were 21 men and 13 women (median age, 67 years, range, 23-83 years). Twenty-one patients (62%) were symptomatic. The median follow-up was 111 months (range, 6-258 months). The 5-year OS and DFS were 81.2% and 77.4%, respectively. Nine patients (26.5%) experimented recurrences. At univariate analysis, the presence of necrosis (p = 0.019), nuclear atypia (p = 0.006), dimension greater than 11.5 cm (median value of our cohort) (p = 0.037) and relapse/disease progression (p = 0.001) were independent prognostic factor of worse OS. The administration of adjuvant treatment was a protective independent factor for survival (p = 0.001). Radicality of resection (p = 0.005); tumour dimension (p = 0.013), presence of necrosis (p = 0.041) and nuclear atypia (p = 0.007) and pleural pattern (p = 0.011) were independent prognostic factors of worse DFS. Analysing the three risk stratification models, the Tapias score was revealed as the best index to predict both OS (p = 0.002) and DFS (p = 0.047) in patients with mSFTP. Conclusions: Using the risk stratification model proposed by Tapias, patients with the highest risk of recurrence could be identified at the time of surgery to establish a more frequent imaging surveillance and longer follow-up. The role of adjuvant treatment in mSFTP therapy has not been established yet, but further analysis on patients with a high risk of recurrence, stratified according to risk models, along with biomolecular panels may tailor future post-surgical therapies.

Malignant Solitary Fibrous Tumour of the Visceral Pleura—Report of a Case—

Malignant Solitary Fibrous Tumour of the Visceral Pleura—Report of a Case—

Malignant Solitary Fibrous Tumor of the Nasal Cavity and Orbit: A Case Report.

Solitary fibrous tumors are well-recognized in the pleura and their occurrence in the nasal cavity is uncommon. They show a hemangiopericytoma-like vasculature, a "patternless pattern" of growth, and lesions characterized by both hypercellularity and hypocellularity. As a malignant form of SFT, mesenchymal neoplasm is extremely uncommon and poorly understood, although there are no established standards for making this diagnosis, it is nonetheless accepted. It can develop either de novo or within an already-existing benign SFT, and there are few published accounts of the cytopathologic characteristics of these tumors.In this case, a patient complained of tenderness on the right nasal root and diplopia for six months. Here, we report on a case of malignant solitary fibrous tumors in the nasal cavity, which extended into the orbit, and review the radiologic and pathologic features.

ODP267 Doege-Potter Syndrome in a Patient With Mediastinal Solitary Fibrous Tumor

Abstract N on-islet cell tumor hypoglycemia (NICTH) is a rare paraneoplastic syndromethat presents mostly with hypoinsulinemic hypoglycemia and is associated with benign and malignant neoplasms other than insulinoma. Doege-Potter Syndrome (DPS) is characterized when NICTH is secondary to a solitary fibrous tumor (SFT). The following is a case of DPS in which the patient presented with refractory hypoglycemia. An 81-year-old Caucasian male presented to emergency department with a 10-day history of worseningneuroglycopenic symptoms in fasting state. He experienced worsening night sweats, unsteadiness while walking, dizziness, and irritation, which resolved after breakfast each morning. Five years prior to this admission, he was diagnosed with a malignant mediastinal SFT (atypical spindle cell neoplasm). He was treated with radiation followed by mass resection (9.5×6.5×7.9 cm). Despite treatment with 3 lines of therapy of phase 1 clinical trial, his disease continued to progress with a right paraspinal mass and bilateral lung metastases being subsequently found. In ED, his initial blood glucose was 53 mg/dL. Despite receiving IV dextrose pushes and a continual dextrose infusion, he continued to be hypoglycemic. Upon admission, blood work revealed blood glucose of 41 mg/dL, low insulin level of 0.4 uIU/mL (2.6-24.9 uIU/mL) and low C-peptide level of 0.9 ng/mL (1.1-4.4ng/mL). Proinsulin (4.4pmol/L), Cortisol (17.2mcg/dL) and A1c (5.2%) were all within normal limits. Serum and urine ketones as well as a blood sulfonylurea panel were negative. He was started on hydrocortisone 50 mg 8 hourly for insulin-independent hypoglycemia. His IGF1 level was 46 ng/mL (55-166ng/mL), and IGF2 level was 564 ng/mL (333-967ng/mL) with a high IGF2: IGF1 ratio of 12. He was discharged home with same hydrocortisone regimen. On follow-up, hydrocortisone dose was reduced to 30-30-20 mg. He passed away 4 months later after being hospitalized for hypoxic respiratory failure secondary to a pleural effusion. Interestingly, he was maintained on the same regimen of hydrocortisone up until his death and did not have recurrence of severe hypoglycemic episodes. The main mechanism of NICTH is tumoral overexpression of the IGF2 gene and aberrant post-translational processing, resulting in partially processed high-molecular-weight IGF2 (big-IGF2). Big- IGF2 has a lower affinity for IGF-binding proteins, which favors its own availability to IGF1 and insulin related receptors resulting in profound hypoglycemia and occasionally acromegaloid features. Diagnosis of NICTH is typically made byan IGF2: IGF1 ratio of greater than 10 (normal 3: 1)due to unavailability of commercial assays for big-IGF2. Radical tumor resection is the definitive treatment of NICTH. In unresectable neoplasms, tumor debulking, or medical therapies such as glucocorticoids, recombinant GH, and glucagon are used to alleviate hypoglycemic symptoms. Exploring more about effectiveness of chemotherapy regimens in controlling NICTH will be helpful in inoperable tumors. Presentation: No date and time listed

ODP187 Doedge-Potter syndrome and papillary thyroid carcinoma: An unusual association

Abstract Hypoglycemia can occur in non-diabetic individuals. Doege-Potter is a rare paraneoplastic syndrome presented as a hypoinsulinemic hypoglycemia caused by ectopic secretion of insulin-like growth factor-2 (IGF-2) from a solitary fibrous tumor. There is recent evidence of the involvement of the IGF system in different malignancies as papillary thyroid carcinoma, being exceptional. A 69-year-old female was brought to Emergency after a routine analysis uncovered severe hypoglycemia. She mentioned a history of morning sickness, dizziness, and confusion for the last week that reverted after breakfast, and dry cough for three months. She had a 25-pack-year history of smoking, no history of alcohol use, no other antecedent of interest, no history ofdiabetes mellitus or weight loss surgery, nor chronic treatments. Physical examinations revealed diminished breath sounds in the left middle lung. A thorax radiography showed a submassive left pleural effusion and toracocentesis revealed a hemorrhagic exudate. Laboratory data showed normal GH, and cortisol levels. Despite marked hypoglycemia, insulin level was 0.4 μIU/mL (2,6-24,9), C-peptide 0.11ng/ml (1,1-4,4) and hydroxy butyrate &amp;lt;0.1 mg/dl. The serum IGF-2 level was 428μg/dL (350-1000), and the IGF-1 level was 55.4 μg/L(161-384). Anti-insulin antibodies and sulfonylureas concentration were negative. CT showed a solid mass concerning parietal pleura with areas of necrosis and calcifications, measuring 14.6×15.4×20cm. Low blood glucose with suppressed insulin, C-peptide and IGF-1 levels, elevated IGF-2/IGF-1 ratio (7,7, normal&amp;lt;3) and CT findings suggested the possibility of non-islet cell tumor hypoglycemia (NICTH). Corticoid treatment was initiated to manage the hypoglycemia. Biopsy of the mass showed a malignant solitary fibrous tumor, positive for CD34 and BCL2. After surgery, glucose and IGF-2 levels returned to normal, the patient didn't experience further episodes of hypoglycemia, and corticoid treatment was gradually suspended. A FDG-PET scan, part of extension study, found a PET-positive thyroid nodule. A thyroid ultrasound confirmed a solid, taller than wide thyroid nodule, measuring 6.7×6.4×9,5mm and fine-needle aspiration biopsy was suggestive of papillary thyroid carcinoma. Hemithyroidectomy was performed. The patient is with no evidence of disease. Doege-Potter syndrome is a rare cause of hypoglycemia, resulting from a tumor producing elevated levels of IGF-2. Diagnosis is suggested by low insulin, C-peptide and IGF-1 levels, normal or elevated IGF-2, and elevated IGF2/IGF1 ratio. High levels of ''big-IGF-2'' are associated with high risk papillary thyroid carcinoma, suggesting the relation between the IGF-system and tumor growth, nevertheless there are few cases reported describing this association. Surgical resection of the tumor is the treatment of choice. Presentation: No date and time listed

Malignant solitary fibrous tumor of the pleura: A narrative review of clinical characteristics, diagnosis and therapeutic options

Solitary fibrous tumor of the pleura (SFTP) is a rare mesenchymal neoplasm that originates from CD34+ dendritic stromal cells. While the majority of SFTPs are considered benign, an estimated 10%-20% are malignant. Malignant SFTP (MSFTP) is highly invasive and has propensities for local recurrence and distant metastasis. In recent years, an increasing number of MSFTPs have been reported. However, the diagnosis of MSFTP can be difficult owing to a lack of typical clinical manifestations and imaging features. To facilitate its diagnosis, the clinical, imaging, and histopathological characteristics of MSFTP are reviewed herein, followed by the clinical management such as surgery, radiotherapy, chemotherapy, targeted drugs and immunotherapy. Detection of NAB2-STAT6 fusion and expression of nuclear STAT6 can facilitate the diagnosis of SFTP. Furthermore, the recurrence risk stratification and prediction models of MSFTP as well as progress will be discussed. An improved understanding of these factors may contribute to more efficient diagnosis and management of MSFTP patients.

EP07.01-017 Malignant Fibrous Solitary Tumour of the Pleura Is Not All the Same: Evaluation of Risk Stratification Models in a Large Single Centre Series

Malignant solitary fibrous tumours of the pleura (mSFTP) are extremely rare diseases (<5% of all pleural neoplasms) with unpredictable behaviour. Surgery remains the standard of care for these tumours, however, estimating prognosis and planning follow-up remains challenging. Several risk stratification models have been proposed, but a classification with diagnostic and prognostic potential has not been yet well standardised. The aim of this study is to analyse the clinicopathological data of mSFTP to investigate their prognostic features and to compare the performance of three risk stratification models proposed in the literature.

[Malignant solitary fibrous tumors: a clinicopathological and molecular genetic analysis].

Objective: To explore the clinicopathological features, immunophenotype and molecular genetic characteristics of malignant solitary fibrous tumor (MSFT). Methods: Seven cases of MSFT were collected from the First Affiliated Hospital of Zhengzhou University from July 2018 to December 2020. Immunohistochemistry, RNA-based NGS and DNA-based NGS were performed. Results Among the 7 patients, there were 5 males and 2 females with a median age of 53 years (37-69 years). Two tumors located at skull base, and one in the tentorium of cerebellum, parietal occipital region, occipital area, chest and buttock respectively. The maximum diameter of the tumor was 2.5-20.0 cm. Microscopically, typical hemangiopericomatoid structures were noted; the tumor was cellular, fusiform or oval, very pleomorphic, with necrosis and high mitotic figures (>4/10 HPF). In some cases, classical solitary fibrous tumor morphology and dedifferentiated region were observed. Immunohistochemically, the tumor was positive for CD34 (6/7), STAT6 (7/7), bcl-2 (7/7), but negative for S-100 (7/7); CKpan or EMA was positive to varying degrees; mutated p53 was noted (3/7); Ki-67 positive index was more than 10%. NAB2-STAT6 gene fusion was typically detected in all the 7 cases. In 4 cases, ZNF415-FGFR1, COPG1-MET, IPO11-LRRC70_ncRNA-PLAG1 and Clorf198-CD274 (PD-L1) gene fusions were also detected. NOTCH1 mutation was found in 7 cases and TP53 mutation in 4 cases. TERT promoter mutations were not detected in all the cases. Conclusions: MSFT is rare and needs to be differentiated from many other spindle cell tumors. Especially when tumors express epithelial markers, they are easily misdiagnosed as sarcomatoid carcinoma and synovial sarcoma, etc. Immunohistochemistry and molecular detection of NAB2-STAT6 gene fusion have important diagnostic values. NOTCH1 and TP53 mutations may be associated with the progression of MSFT. Some patients have FGFR1 gene fusion and MET gene fusion, which may be potential therapeutic targets.

Resection of giant malignant solitary fibrous pleural tumor after interventional embolization: a case report and literature review

BackgroundSolitary fibrous tumor of the pleura (SFTP) is a rare mesenchymal tumor that arises at various sites and typically originates from the pleura. Most patients with SFTPs are asymptomatic, unless the tumor is large. Approximately 20% of SFTP cases are malignant. There are few reports on imaging diagnoses and interventional treatments of SFTP. Here, we report a case of a giant SFTP that exhibited malignant behavior and underwent successful resection after embolization of the main supply artery of the tumor.Case presentationWe report a clinical case of a giant SFTP in a 66-year-old Chinese female patient complaining of chest tightness and cough for more than 2 months. Ten years ago, the patient had undergone a chest CT scan at a local hospital for cough. Computed tomography (CT) had revealed a mass in the right thoracic region, which was misdiagnosed as a pulmonary abscess by CT-guided biopsy. Therefore, the patient did not receive appropriate/complete treatment at that time. She was hospitalized again, because CT showed significant enlargement of the right thoracic mass, which caused her obvious symptoms of discomfort. The pathological results of CT-guided biopsy at our hospital confirmed SFTP. Considering the large size of the tumor and the rich blood supply, some of the main blood vessels were treated with embolization before surgical resection. A large tumor, about 23 cm × 16 cm × 15 cm in size, was then successfully removed by thoracic surgery. The diagnosis of malignant SFTP was confirmed by surgical pathology and immunohistochemistry.ConclusionImaging findings of SFTPs are not characteristic, especially when a tumor is large, the diagnosis is difficult, and the final diagnosis still depends on histological and immunohistochemical examinations. The two-stage surgical treatment described here, which involves first embolization of the main supplying artery of the large tumor and then complete surgical resection, is effective and safe for SFTPs. Whether needle biopsy or vascular embolization is performed, intervention plays a crucial role in the diagnosis and treatment of patients with SFTPs.

Malignant solitary fibrous tumor of the pleura

BackgroundSolitary fibrous tumors of the pleura are rare diseases of the thoracic cavity. They frequently grow unnoticed until they exert compressive effects on adjacent organs. Treatment of solitary fibrous tumors of the pleura is surgical resection. Post-operative surveillance is recommended to identify early recurrent disease.Case presentationWe present a rare case of a 76-year-old female patient with no previous pulmonary history who presented with progressive dyspnea, fatigue, and involuntary weight loss. On chest X-ray and computed chest tomography scan, she was found to have a 16.7 cm × 12.8 cm × 10.1 cm bulky mass occupying the left hemithorax with associated compressive atelectasis of the lung. She underwent a computed tomography guided biopsy that revealed the mass to be a solitary fibrous tumor. The patient underwent left muscle sparing lateral thoracotomy with complete resection of the tumor. Post procedure, the left lung fully expanded. 18 months post-resection, she developed a 3.3 cm × 1.7 cm tumor along the left internal thoracic artery lymph node chain which was histologically identical to the resected tumor. The patient is currently being treated with bevacizumab and temozolomide.ConclusionSolitary fibrous tumors are very rare pleural tumors. Surgical resection is the treatment of choice followed by close post-operative surveillance.

Malignant progression of cerebellopontine angle solitary fibrous tumors following radiation: illustrative case.

BACKGROUNDIntracranial solitary fibrous tumors (ISFTs) are rare mesenchymal tumors originating in the meninges and constitute a heterogeneous group of clinical and biological behavior. Benign histotypes, such as hemangiopericytomas are now considered as a cellular phenotypic variant of this heterogenous group of rare spindle-cell tumors. IFSTs are poorly recognized and remain a diagnostic challenge due to rarity and resemblance to other brain tumors. Previously, IFSTs were thought to pursue a slow, indolent, and nonaggressive course, however, a growing body of literature based on longer follow-up demonstrates an unpredictable clinical course and an uncertain diagnosis.OBSERVATIONSA rare case report of malignant transformation of IFST following radiation therapy is reported. In this case a 60-year-old female who underwent gross total resection of the cerebellopontine angle tumor with histopathology consistent with solitary fibrous tumor followed by salvage stereotactic radiosurgery, presented with another recurrence after 2 years of surgery. The authors performed complete removal of the tumor with pathology now consistent with malignant solitary fibrous tumor. A recent follow-up magnetic resonance imaging did not show any recurrence or residual tumor, and the patient reports a generalized well-being.LESSONSThis report will help to understand the natural history and unusual clinical behavior of these intracranial tumors.

Buccal space malignant solitary fibrous tumour: a case report

Solitary fibrous tumour (SFT) occurs rarely in extrapleural sites such as head and neck region. Head and neck region is affected in 16% cases occurring usually in orbit and paranasal region and are benign variants. Though usually benign, Malignant SFT in buccal space is extremely rare and comes with challenges in diagnosis and management. We report a rare case of Malignant SFT in the buccal space in a 70-year-old- gentleman who presented with slow growing mass in left cheek. Patient was treated with surgical excision and adjuvant radiotherapy and is on regular follow up. Malignant SFTs are difficult to diagnose due to lack of specific histological pattern but certain IHC and genomic markers can help. Buccal space malignant SFT can be managed by wide local excision and adjuvant radiotherapy in cases of large tumour size and margin positive resection.