Case Of Solitary Fibrous Tumor Research Articles

A Rare Mesenchymal Neoplasm in an Uncommon Anatomical SiteSolitary Fibrous Tumour of Vulva

Previously, Solitary Fibrous Tumour (SFT) was thought to originate from the pleura, a relatively rare mesenchymal neoplasm with an indolent course. Vulva is the very uncommon site of extrathoracic SFT. This is the report of a very uncommon case of vulval SFT in a 30-year-old female with complaint of a swelling over vulval region. Fine Needle Aspiration Cytology (FNAC) of the growth was attempted twice and only blood was aspirated. The swelling was excised completely. On histological examination, the sections revealed a cellular tumour of ovoid-to-fusiform spindle cells with indistinct cell borders, with haphazard arrangement or patternless pattern along with proliferation of variable sized blood vessels. The diagnosis was finalised to be a benign stromal tumour (most probably SFT), which was confirmed on immunohistochemical examination using Cluster of Differentiation 34 (CD34), CD99 and Signal Transducer and Activator of Transcription 6 (STAT6) markers.

Liver metastases of a malignant solitary fibrous tumor: the important role of immunohistochemistry in this rare entity.

Solitary fibrous tumor (SFT) is a rare neoplasm of mesenchymal origin that normally appears in the pleura; however, it has been described in other extrapleural locations. This tumor is rarely malignant and only a few cases of metastatic SFT have been described.

Case Report: Molecular Characterization of Aggressive Malignant Retroperitoneal Solitary Fibrous Tumor: A Case Study

Solitary fibrous tumors (SFT) are mesenchymal neoplasms with a favorable prognosis usually originating from the visceral pleura. Rarely, they may occur at various extrapleural sites and show malignant behavior coupled with dedifferentiation. NAB2-STAT6 fusion gene and STAT6 nuclear expression are biomarkers for diagnosis of SFT in addition to CD34, Bcl-2, and CD99. Furthermore, several reports have shown specific NAB2-STAT6 fusion variants and loss of STAT6 protein expression are associated with malignancy. We report a rare case of retroperitoneal SFT which rapidly progressed to death within 35 days after admission. Autopsy found a primary tumor containing both benign and malignant histologies, with multiple metastatic sites similar to the malignant, dedifferentiated tumor. STAT6 was detected in the primary differentiated tumor but not in the primary dedifferentiated tumor or lung/liver metastases. However, the NAB2-STAT6 fusion gene (NAB2ex6/STAT6ex16 variant) was detected in the primary tumor and lung/liver metastases. Intriguingly, fusion gene expression at the transcriptional level was downregulated in the dedifferentiated tumors compared to the differentiated tumor. We further performed target DNA sequencing and found gene mutations in TP53, FLT3, and AR in the dedifferentiated tumors, with TP53 mutations especially found among them. We demonstrate that downregulation of NAB2-STAT6 fusion gene at the transcriptional level is associated with malignant SFT for the first time. Moreover, the present study supports the idea that TP53 mutations promote malignancy in SFTs.

A rare case of solitary fibrous tumor of the liver and highly differentiated rectal adenocarcinoma

Solitary fibrous tumors (SFT) are rare spindle cell mesenchymal neoplasms of presumably fibroblastic origin with undefined malignancy potential and rare metastasis. Their most frequent localization is in the pleura, where they have been first described. The incidence of solitary fibrous tumors localized in liver is extremely low. The clinical picture of SFT is nonspecific and is not due to the anatomical location of the tumor and it's size.In the majority of cases these tumors are benign. However, a number of them still may have signs of malignancy: an aggressive course with possible local recurrence and distant metastasis. The main method of treatment of hepatic solitary fibrous tumor (as well as SFT of other localization) with it's resectability is surgery. Radical removal of the tumor in the majority of cases leads to recovery. Subsequently, the operated patients should be subjected to strict dynamic observation with regularity as in malignant neoplasms (MN).The importance for chemotherapy and radiation therapy in the treatment of hepatic solitary fibrous tumor has not been determined in clinical guidelines to date.There are isolated reports of the use of chemotherapy or radiation therapy for the treatment of cases when tumor resection is not radical or there are signs of malignant neoplasm.In literature, there are numerous reports of a combination of a solitary fibrous tumor of any localization with malignant neoplasms of other organs in the same patients.In this report, we represent a clinical case of a 64-year-old woman, who had a solitary fibrous tumor of the liver and highly differentiated rectal adenocarcinoma. The patient underwent resection of the first segment of the left lobe of the liver. After a month and a half, transanal excision of the villous tumor of the rectum. The pathologic and immunohistochemical examination of the liver tumor revealed a malignant solitary fibrous tumor. After researching villous tumor of the rectum - a highly differentiated adenocarcinoma with a depth of invasion of the submucous layer of the intestinal wall up to 1/3 (T1sm1 according to Kikuchi). During dynamic observation for twenty-five months after the operation, the patient has no signs of tumor recurrence and metastasis.

A Case of Solitary Fibrous Tumor Presenting as Lower Neck Mass

Solitary fibrous tumor (SFT) is rare mesenchymal tumor usually arising from pleura. SFT can be found at all anatomic site in our body but incidence of SFT is much lower in head and neck region especially at lower neck area.<br/>We found a case of SFT that presented as a lower neck mass in a 41-year old woman. Ultrasonography showed a 3×1cm sized hypoechoic mass in the intermuscular fat plane of left lower neck, and computed tomography showed a well circumscribed, low-density mass with contrast enhancement. Fine needle aspiration showed no malignant cells with abundant red blood cells, but it was not possible to completely rule out malignant tumors or nodules clinically. Surgery was performed to make a definitive diagnosis and histopathology showed tightly packed, round to fusiform cells with staghorn shaped vessels at microscopic examination. The tumor cell were positive for CD34 but negative for CD31 and S-100 protein.

Determination of biological behavior of solitary fibrous tumors: correlation of expression of Ki-67, TPX2 and TERT mRNA subunit level and NAB2-STAT6 fusion compared to morphological aspects of SFTs.

We present a retrospective study of 65 cases of solitary fibrous tumors (SFTs) of several localizations including the most common site of origin in the pleura and lungs. SFTs are mesenchymal fibroblastic tumors with an unpredictable biological potential ranging from benign to malignant. We investigated morphologic characteristics, proliferation activity evaluated by immunohistochemical expression of Ki-67 antigen, and the existence of NAB2-STAT6 fusion gene together with Ki-67, TPX2, and TERT mRNA expression levels. The aim was to define relationships between proliferation activity and biological potential and progression of the disease. We measured Ki-67, TPX2, and TERT mRNA levels using quantitative real-time reverse transcription PCR (RQ-RT-PCR). We observed a significant association between increased Ki-67 and TERT mRNA levels and the SFTs with malignant potential. Also, we investigated the effect of TERT promoter mutation on telomerase activation and patient outcome in our SFT cohort. We verified that TERT promoter mutation was frequent (36.6%) and present in a majority of malignant SFTs and SFTs with uncertain biological behavior. TERT promoter mutation alone predicted the disease recurrence.

Malignant Solitary Fibrous Tumor of Ileum with Intussusception

Solitary fibrous tumor (SFT) is rare mesenchymal neoplasm of fibroblastic or myofibroblastic origin. Especially, extrapleural SFT is known to be extremely rare. Here, we report a case of malignant SFT of ileum, resulting in small bowel intussusception in a 74-year-old female patient.

A case of solitary fibrous tumor incidentally detected during COVID-19 infection and treated with video-assisted thoracoscopic surgery

Although solitary fibrous tumor is the most common benign pleural tumor, it is encountered incidentally and very rarely. A mass in the chest wall was detected in a computerized chest tomography taken due to COVID-19 infection in a 36-year-old female patient. The case with solitary fibrous tumor originating from the parietal pleura was treated with video-assisted thoracoscopic surgery. During the COVID-19 epidemic, compute-rized tomography of the thorax, which is frequently taken for lung involvement, can reveal pathologies related to thoracic structures as well as tumors originating from the pleura. Video-assisted thoracoscopic surgery re-commended for the excision of pedunculated and less than 5 cm lesions; We would like to emphasize that it can be safely applied in the treatment of solitary fibrous tumors that are sessile and do not exceed 5 cm.

CD34-negative solitary fibrous tumour of the broad ligament with doege-potter syndrome: a case report

Solitary fibrous tumours (SFT) are rare fibroblastic mesenchymal tumours that can occur in various anatomical sites but are extremely rare in the female genital tract. The broad ligament itself is a rare location for primary tumours and SFT in the broad ligament has only been described twice before in the literature. We report on a case of CD34-negative SFT in a 61-year-old female who presented with Doege-Potter syndrome. This case highlights the unusual presentation and location, atypical CD34 expression and utility of STAT6 immunohistochemistry for SFTs. 1.Chen S, Zheng Y, Chen L, et al. A broad ligament solitary fibrous tumor with Doege-Potter syndrome. Medicine 2018; 97: e12564.2.Zubor P, Kajo K, Szunyogh N, et al. A solitary fibrous tumor in the broad ligament of the uterus. Pathol Res Pract 2007; 203: 555–60.3.Ronchi A, Cozzolino I, Marino FZ, et al. Extrapleural solitary fibrous tumor: A distinct entity from pleural solitary fibrous tumor. An update on clinical, molecular and diagnostic features. Ann Diagn Pathol 2018; 34: 142–50.

Solitary fibrous tumor of thoracic cavity, extra-thoracic sites and central nervous system: Clinicopathologic features and association with local recurrence and metastasis

Published risk stratification models of solitary fibrous tumor (SFT) have been associated with distant metastases outside the central nervous system (CNS), but have not been studied for tumors occurring in the CNS. In a retrospective review, we identified 72 cases of solitary fibrous tumor or hemangiopericytoma (HPC) diagnosed between January 2011 and December 2020 at our institution. The tumors involved the central nervous system (N = 17), thoracic cavity (N = 28), and extrathoracic sites (N = 27). The risk of local recurrence, distant metastasis, or death at 5 years was 57% (95% CI 23%, 76%) in the CNS, 24% (95% CI 2%, 41%) in the thoracic cavity, and 13% (95% CI 0%, 25%) in extrathoracic sites. By contrast, the risk of distant metastasis or death at 5 years was 13% (95% CI 0%, 29%) in CNS primaries, 5% (95% CI 0%, 14%) in thoracic primaries, and 14% (95% CI 0%, 27%) in extrathoracic primaries. Using the published 3- and 4-variable risk stratification models by Demicco et al., we retrospectively assessed our cases for risk of local recurrence, distant metastasis, and death. For tumors outside the CNS, we show that three- and four-variable risk stratification models were associated with recurrence-free survival in addition to the previously known association with distant metastasis (all P < 0.05). In contrast, inside the CNS, we show that neither risk model is a significantly associated with clinical behavior, and that WHO grade is likely the best available prognostic tool, though none of the differences were significant. The lack of significant differences can be likely explained by the younger median age (47 years vs 61 years) and smaller median tumor size (3.5 cm vs 5.6 cm), downgrading the risk stratification scores in CNS compared to non-CNS primaries. In conclusion, existing risk stratification models of SFT are not associated with clinical behavior for tumors arising inside the CNS, but are associated with local recurrence in addition to distant metastasis outside the CNS.

Clinical, Histological, and Molecular Features of Solitary Fibrous Tumor of Bone: A Single Institution Retrospective Review.

Simple SummarySolitary fibrous tumors arising from the bone are an extremely rare event and only few cases have been previously described in the literature. It is characterized by a prominent, branched vascularization, with a thin and dilated vascular texture defined as “staghorn” and by the presence of the NAB2-STAT6 gene rearrangement, present in about 90% of cases and considered a pathognomonic feature. In the present study, we described our series of 24 cases of primary solitary fibrous tumor of the bone to find any clinical and molecular prognostic factors and to compare them with those currently used for soft tissue solitary fibrous tumor and to evaluate the risk stratification system proposed by Demicco, in order to understand whether this system was able to correctly predict the risk of local and distant metastatic relapse even in the case of solitary fibrous tumor of the bone.Primary solitary fibrous tumor (SFT) of the bone is extremely rare, with only few cases reported in the literature. We retrieved all cases of primary SFT of the bone treated at our institution and we assessed the morphology and the immunohistochemical and molecular features to investigate the clinical outcome of primary SFT of the bone and any clinical relevance of clinical and histological criteria of aggressiveness currently adopted for the soft tissues counterpart. Morphologically, 15 cases evidenced high cellularity, cytologic atypia, and foci of necrosis and were associated with more than 4 mitotic figures/10 HPF. Immunohistochemical analysis showed an expression of CD34 and of STAT6 immunopositivity in 95% and in 100% of cases, respectively. The presence of NAB2-STAT6 chimeric transcripts was found in 10 out of 12 cases in which RT-PCR analysis was feasible, whereas TERT promoter mutations analysis was feasible in 16 cases and only a C-to-T substitution in a heterozygous state was found in one DNA sample for the C228T genetic variant. P53 variants were assessed in 12 cases: 11 (91.6%) cases showed a variation, while in one case, no alteration was found. Disease-specific survival was 64% at 5 years and 49% at 10 years. Statistical analysis showed no correlation between survival and all the clinicopathological and molecular parameters evaluated. In conclusion, at difference to SFT of soft tissues, aggressive behavior of primary SFT of the bone seems to be independent from mitotic count or any other clinicopathological and molecular features.

A Case of Non-Islet Cell Tumor Hypoglycemia in Metastatic Solitary Fibrous Tumor

Abstract Introduction: Insulin like growth factor (IGF-2) mediated hypoglycemia secondary to solitary fibrous tumor (SFT), also known as Doege-Potter syndrome is a rare paraneoplastic syndrome. The tumor cells produce large amounts of high molecular weight IGF 2 precursor protein called “big IGF-2” which binds to insulin and IGF receptors in liver, muscle and other peripheral tissues. This causes reduced gluconeogenesis and increased uptake of glucose by the muscle and other tissues leading to hypoglycemia. Big IGF-2 also exerts central negative feedback of growth hormone causing reduction of IGF-I production. Most SFTs are benign and localized (approximately 78-88%). As a result, tumor excision alone would often lead to resolution of the hypoglycemia. We present a case of metastatic SFT with multiple metastasis managed with oral prednisone. Clinical Case: A 44-year-old man with metastatic SFT presented with bilateral humeral fractures. He has known metastatic disease to the brain, lung, liver, bony lytic lesions over a course of eleven years. It has progressed despite multiple chemotherapy and radiation therapies. Prior to admission, he had multiple syncopal episodes associated with fasting hypoglycemia. He reported capillary blood glucose values ranging between 30-50 mg/dl during these episodes which would improve after drinking juice or eating candy. There was no history of diabetes mellitus or use of oral hypoglycemic agents or insulin. On admission, he had a capillary blood glucose value of less than 20 mg/dl, which was confirmed by a serum glucose value of 18 mg/dl on basic metabolic panel. His renal, liver and thyroid function tests were normal. Significant labs include: serum glucose 17 mg/dl, C-peptide &amp;lt;0.10 ng/ml (n: 1-4 ng/ml), serum insulin &amp;lt;1.6 Uu/ml (n: &amp;lt;20 Uu/ml), beta-hydroxybutyrate &amp;lt;0.2 mmol/L (n: &amp;lt;0.3), cortisol 10.8 ug/dl(n: 5-15 ug/dl) glucagon 6 pg/ml(ref 8-57 pg/ml), insulin-like growth factor-1 (IGF 1) 20 ng/ml (n: 52-328), and IGF-2 level 380 ng/ml (267-616 ng/ml), improvement in blood glucose from 46 to 111 mg/dl after 1-gram glucagon administration. The IGF-2/IGF-1 ratio of 19 confirmed our clinical suspicion of non-islet cell tumor hypoglycemia (NICTH). He was started on prednisone 20 mg twice daily with marked improvement in hypoglycemia. Conclusion: NICTH is a rare cause of hypoglycemia and should be considered in the differential while evaluating hypoglycemia in malignancy. For diagnosing NICTH, assays for big IGF-II are not commercially available. However, the IGF-II:IGF-I ratio is considered to be a surrogate marker of big IGFII concentration. The normal ratio is 3 and ratio &amp;gt;10 is diagnostic of NICTH. In cases like ours where tumor resection is not possible, glucocorticoids are most effective in management of hypoglycemia by inhibiting big IGF2 production and stimulating gluconeogenesis.

A Malignant Solitary Fibrous Tumor of the Parotid Gland: A Case Report

A Solitary Fibrous Tumor (SFT) is a soft tissue tumor that appears exceedingly rare in the parotid gland. The literature review suggested that approximately 12%-22% of these cases behave aggressively. Besides, only 4 cases of histological malignant parotid gland SFT are reported in the English literature. We presented a 65-year-old cigarette-smoker man with a fix and tender mass over the left parotid and submandibular areas. On physical examination, the marginal branch of the facial nerve was paralyzed. The left total parotidectomy and neck dissection levels 1 and 2 were performed with preoperative embolization procedure and adjuvant radiotherapy for the patient. The results of the follow-up examination were unremarkable after 11 months. In this case report, we aimed to increase awareness on SFTs, although rare, among clinicians and pathologists. Accordingly, it should be considered in the differential diagnosis of soft-tissue tumors in the major salivary glands for better management.

Oral Solitary Fibrous Tumor: A Retrospective Clinico-Pathological Study and Long-Term Follow-Up.

Background and Objectives: This was a retrospective single-center study to analyze and describe the clinical and histological features of all cases of oral solitary fibrous tumor (SFT). Study design: the study included all consecutive cases of oral SFT diagnosed between 2008–2018 at a single tertiary center. Materials and Methods: Clinical data was retrieved from medical charts. The diagnosis of oral SFT was based upon the morphologic features of the lesions, in routine hematoxylin and eosin (H&E) stained sections and confirmed by immunohistochemical analyses including CD34, CD99, Bcl2, and stains for STAT6. Results: Seven cases of oral SFT were found. Of these, three (42%) were in males and four (58%) in females. The age range was 24–63 years (mean 47 ± 13). Four (58%) lesions were located in the buccal mucosa, two (28%) in the labial mucosa and one (14%) on the floor of the mouth. The diameter ranged between 3–50 mm (mean 22 ± 14 mm). All patients were treated with local excision. Follow-up periods were between 2–74 months (mean 41 ± 27). No recurrences were reported. Conclusions: We present a series of oral SFT, which were all non-aggressive in presentation and did not recur after conservative surgery (local excision) over a relatively long follow-up period.

Solitary Fibrous Tumor in the Perianal Region: Report of Two Cases With an Atypical Location

A solitary fibrous tumor (SFT) is a mesenchymal neoplasm of spindle cells, initially described in the pleura. The World Health Organization (WHO) classifies the solitary fibrous tumor as a neoplasm with intermediate biological potential. Diagnostic images are essential for the diagnostic and therapeutic approach in this entity. The standard of treatment for this type of lesion is surgical resection with oncological margins larger than 1 cm. The solitary fibrous tumors located in the perianal, perineal, and pelvic regions are infrequent and represent a challenge in the clinical approach, mainly because the manifestations are nonspecific. Given the low incidence of this type of neoplasm, we present two cases of SFT in the perianal region managed in a high-complexity hospital.

Soliter fibrous tumor of diaphragm in a patient with larynx cancer: Case report

Introduction: Solitary fibrous tumor is a neoplasm of mesenchymal origin with benign and malignant forms. We aimed to present a case of solitary fibrous tumor which developed in a patient operated for laryngeal cancer and originated from diaphragm in the light of the literature. Case report: A 61-year-old male patient with tracheostomy with an undiagnosed lesion that appears to be almost 10 cm was referred to our clinic. Since it was a large volume mass, we chose to perform a thoracotomy over thorachoscopic approach. Discussion: Although solitary fibrous tumors most commonly occur in the pleura but may also originated from diaphragm, and our case is valuable that originates from diaphragmatic since there are less than 5 reported cases in literature for past two decades. Conclusion: Even in the case of recurrence, the main treatment remains as total surgical excision. Solitary fibrous tumors are usually detected because of compression symptoms. That is the main reason why we chose thoracotomy.

A Dedifferentiated Solitary Fibrous Tumor of Soft Tissue With Retiform and Papillary Features: Report of a Case and Review of the Literature

Abstract We report a case of a dedifferentiated solitary fibrous tumor (SFT) arising in the arm of a 74-year-old man, with the dedifferentiated component showing a unique retiform and papillary architecture. The patient presented with a tumor first noticed 12 years ago, which was diagnosed as a schwannoma. It remained clinically stable for about 10 years until he noticed growth of the mass 2 years ago. On comparative magnetic resonance imaging scans, while most of the tumor did not show major changes, a proximal lobule demonstrated increase in its size. After a new biopsy, which was interpreted as malignant, preoperative radiotherapy and surgical excision were performed. The surgical specimen had areas of typical SFT, with diffuse immunohistochemical expression of STAT6 and only focal CD34 and p16. The lobule that demonstrated growth showed a much more cellular, retiform/papillary proliferation, with plump atypical cells, necrosis, diffuse expression of STAT6 and p16, and complete lack of CD34 expression. With next-generation sequencing, the NAB2(3)-STAT6(18) fusion transcript was detected in both areas of the tumor. Only a few cases of SFT with papillary or retiform/papillary features were reported, most of them in the central nervous system, and none of them interpreted as a dedifferentiated SFT. To the best of our knowledge, these morphological features have not been reported in SFT of the soft tissue or in dedifferentiated SFT of any site. We think pathologists should be aware of this rare finding, which becomes particularly challenging in small biopsies or if classic areas of SFT are not found.

A Case of Solitary Fibrous Tumor in the Obturator Foramen

A Case of Solitary Fibrous Tumor in the Obturator Foramen

A case of solitary fibrous tumor arising in the meninge

A case of solitary fibrous tumor arising in the meninge

A Case of Solitary Fibrous Tumor of the Palate with Signs of Malignancy

A Case of Solitary Fibrous Tumor of the Palate with Signs of Malignancy